CHAPTER VII_Scurvy, Past and Present

SYMPTOMATOLOGY AND DIAGNOSIS

The identity of scurvy in the infant, in the young child and in the

adult is thoroughly established and requires no further substantiation.

There are, however, sufficient differences between the symptoms of adult

scurvy and those of Barlow's disease to render it advisable to consider

them separately. These distinctions are due largely to the fact that the

former disorder affects mature tissues, whereas the latter is engrafted

upon tissues which are in the process of rapid growth and development.

The symptomatology is influenced also by the striking differences in

environment--the passive, shielded existence of the infant, contrasted

with the active and exposed life of the adult. Although we shall,

therefore, treat adult and infantile scurvy separately, it should be

borne in mind that, from an etiologic and pathologic viewpoint, such a

division is artificial and is resorted to merely for purposes of

clarity.

=Adult Scurvy.=--The earliest sign of scurvy is usually a change in the

complexion of the individual. His color becomes sallow or muddy, an

aspect difficult to describe, but one which is characteristic, and

constitutes an important danger signal to the eye of the experienced

physician. About the same time the patient loses his accustomed vigor,

seemingly becomes indolent and complains of tiring quickly, and of

breathlessness. He may experience fleeting pains in the joints and

limbs, especially in the legs, symptoms which are frequently attributed

to rheumatism. At this early stage the appetite may still be normal,

there is usually no loss in weight, but merely a general malaise which

is significant, although in no way distinctive. Very soon the gums

become sore, bleed readily, and are found to be congested, spongy, and

somewhat hemorrhagic at their edges. Absolute reliance must not,

however, be placed on this sign for early diagnosis, as at times it does

not appear until later. Careful examination at this stage will disclose

petechial spots on the body, more especially on the legs, at the site of

the hair follicles, or even larger ecchymoses, depending upon the

hemorrhagic tendency of the individual, his exposure to bruising, the

adequacy of his diet, and secondary infection. Less frequently bleeding

from the nose occurs early, or the eyelid suddenly becomes swollen and

purple, or the urine shows the presence of blood.

These signs progress steadily with a varying degree of rapidity. The

complexion becomes more dingy and somewhat brownish, the weakness

increases so that the slightest exertion causes breathlessness and

palpitation, and the gums become spongy and even fungous. If there is

infection of the gums and the teeth are carious, the breath is extremely

foul--a sign long associated with scurvy. Later the teeth become loose

and may fall out, and the alveolar process undergoes necrosis. The

surface hemorrhages increase in severity, large effusions appearing on

the trunk, on the extremities, and less often beneath the mucous

membrane of the mouth. A bloody diarrhoea may take the place of the

constipation which is generally noted earlier in the disease. There are

at this time hemorrhages into the muscles and deeper tissues, especially

into the calves of the legs, giving rise to hard, brawny, tender

swellings which have been termed "scurvy sclerosis." This is sometimes

the earliest sign noted by the patient and may puzzle the physician who

has not met with it before. The swelling may be found in the popliteal

space or at the site of the tendo Achilles, and result in lameness and

contracture of the neighboring joint. Frequently there is slight edema

of the ankles associated with a glossiness of the extensor surfaces of

the legs. This infiltration differs from ordinary edema in being firm

and not pitting on pressure. The skin is dry and rough, the follicles

being unusually elevated;[46] the hair likewise is dry and loses its

lustre. Not infrequently subperiosteal hemorrhages occur, giving rise to

exquisitely tender swellings, especially of the tibia or of the femur,

or of the ramus of the lower jaw, as has been noted in connection with

guinea-pig scurvy. If there are wounds or ulcers they assume a

hemorrhagic aspect, the edges becoming bluish or livid and showing no

tendency to heal; even scars which have existed for many years change in

color and show an altered state of nutrition, and ulcers long healed

break out afresh.

[46] As the result of an experience with thousands of cases of scurvy in

the Serbian army, Wiltshire has recently laid great emphasis on this

follicular hyperkeratosis. He states that the earliest recognizable sign

of scurvy is an enlargement of the hair follicles of the inner and

anterior aspects of the thigh and upper leg, which show numerous conical

elevations about the size of a pin's head. A hair, broken or unbroken,

frequently pierces the follicle.

Nowadays, the disease usually does not reach this stage, and rarely

progresses further. If, however, the patient remains untreated, he

becomes progressively weaker and more lethargic; there is frequent

palpitation, shortness of breath, and increasing loss of weight. The

pains in the limbs render him helpless and an object of pity. Marked

edema may be added to the picture as the result of starvation, so that

the legs become swollen, and even the face becomes bloated. Hemorrhages

into the skin as large as the palm of the hand appear on different parts

of the body. The gums swell to such an extent that they overlap and may

even hide the teeth and protrude from the mouth as foul fungoid growth.

Death comes about in various ways. Frequently sudden and fatal syncope

occurs, due to heart weakness or to the pouring out of fluid into the

pleural or the pericardial cavities. Another frequent cause of death is

secondary infection, resulting in pneumonia, which finally ends the

suffering of the patient. The fatal outcome is thus described in the

narrative of Lord Anson's voyage:

"Many of our people, though confined to their hammocks, ate and

drank heartily, were cheerful, and talked with much seeming vigor,

and in a loud, strong tone of voice; and yet, on their being the

least moved, though it was only from one part of the ship to

another, and that in their hammocks, they have immediately expired;

and others, who have confided in their seeming strength, and have

resolved to get out of their hammocks, have died before they could

well reach the deck. And it was no uncommon thing for those who

could do some kind of duty, and walk the deck, to drop down dead in

an instant, on any endeavor to act with their utmost vigor; many of

our people having perished in this manner during the course of this

voyage."

The disease may develop and progress in various ways. It may remain

latent for a long period and be cured by some accidental change of

diet, or, as more frequently occurs, it runs a moderately acute course,

and is promptly cured by means of antiscorbutics. In the days when

scurvy was common and widespread it sometimes became chronic, developing

into the "inveterate scurvy" of the older authors, which was notably

resistant to treatment. Harvey, in his treatise published in 1685,

states that "a mild scurvy may continue or be protracted to ten, twenty,

or thirty years."

In addition to the general picture of the disease which we have

presented, mention should be made of other less common symptoms. As is

well known, one of the characteristic signs of scurvy is _hemorrhage_.

Indeed, in many of the systematic treatises of medicine it is classified

as a hemorrhagic disease. Besides the bleeding into the gums, skin and

bones, hemorrhage into the stomach may take place, giving rise to

hæmatemesis, or there may be hemorrhage into the eye, under the

conjunctiva or into the anterior chamber, leading to the destruction of

the eyeball. A very unusual form is meningeal bleeding, giving rise to

symptoms of apoplexy. It may be stated in general that hemorrhage

dominates the picture of scurvy. Eruptions which in normal individuals

are simply macular or papular, assume a hemorrhagic character when

occurring in a scorbutic individual. This phenomenon was noted in the

recent war in connection with the eruption of typhus fever, and has been

observed by military and naval surgeons in numerous expeditions.

Scurvy reduces the nutritional state of probably all the cells and

tissues of the body. If the resistance is still further lowered by

exposure, nutritional disturbances will result more readily than where

the tissues are normal and well nourished. For this reason we believe

that scurvy may predispose to _frostbite_. Reports of congelations

occurring in the trenches in the course of the World War tend to confirm

our opinion that scurvy was a predisposing factor in many of these

cases. This has been true in other wars. For example, Munson writes that

"during the Crimean War the temperature was never very low and a report

of the times suggests that the large number of congelations observed

among the soldiers might well be regarded as gangrene owing to a

scorbutic tendency exaggerated by the cold."

In connection with the involvement of the gums, another typical symptom

of scurvy, it should be remembered that this sign may appear late and

therefore fail to be of value for early diagnosis, and that it occurs

also in purpura and thus may lead to error. This is especially the case

if there is pyorrhoea. As is well known, hemorrhages of the gums

appear only where teeth are present, and are absent in the edentulous

gums of old people as well as in babies who have no teeth. Immerman is

probably correct in believing that an injury is always necessary to

produce a hemorrhagic lesion in scurvy, and that this explains the early

involvement of the gums and also their non-implication in the absence of

teeth.

It is a common belief that separation of the epiphyses occurs only in

infants and young children, and not in the scurvy of adults. This,

however, is not correct, as in severe adult scurvy there is frequently a

separation of the epiphyses of the long bones of the lower extremities

or of the ribs, the latter resulting in a sinking of the sternum.

The pulse is sometimes slow and feeble, having been recorded as low as

40 beats per minute, but more frequently is rapid, in the neighborhood

of 140. It is, however, almost invariably unduly excited by emotion or

by mild physical activity. Frequently there is a low type of fever,

which has been termed "scorbutic fever," but which probably should be

regarded as a complication of the disease rather than as an intrinsic

symptom.

There is little tendency to the formation of pus. Although the lymphatic

glands are frequently enlarged and effusions into the tissues and into

cavities of the body are by no means uncommon, they show little tendency

to become purulent. In the severe cases described by the older authors,

the breaking down of the glands in the inguinal region--buboes--is

frequently noted. The urine is apt to be scanty, becoming much more

profuse following treatment. Perspiration is also retarded.

A peculiar symptom reported in connection with numerous epidemics of

scurvy, both on sea and on land, is _nyctalopia or night-blindness_. The

patients can see fairly well during the day, but have very little vision

as soon as darkness develops. This phenomenon has puzzled many

observers, as nothing abnormal has been found on examination of the

eyes. Recently O'Shea, who met with many cases of this nature among

soldiers, has reported that in an ophthalmic examination of 22 cases the

only abnormality was pallor of the optic disc in 3 cases. This weakness

of sight is due to the general nutritional weakness and has been

reported in connection with other exhausting and nutritional

diseases--for example, hunger edema. More rarely there is day-blindness.

As a complication, dysentery may be mentioned. This has been described

by Schreiber and others in scurvy epidemics occurring in the course of

the World War. Jaundice may appear, and might be expected to occur more

often in view of the marked congestion of the upper duodenum found so

frequently at necropsy.

Pericarditis, hydrothorax, pleurisy with effusion, pneumonia, are common

complications of severe forms of scurvy. Lind reports that the dominant

complication varies in different epidemics; that on one cruise many

cases of diarrhoea would occur and on another many pulmonary

infections.

O'Shea reports the exceptional case of a man who was operated upon for

acute appendicitis. A large hemorrhage in the wall of the cæcum was

found, as well as some other hemorrhages in the peritoneal cavity. This

report is interesting, not so much from a diagnostic standpoint as

because "contrary to what might have been expected, scorbutic cases when

operated upon showed no particular tendency to hemorrhage."

=Infantile Scurvy.=--The stereotyped picture of infantile scurvy and the

one which this term commonly suggests, is that of the acute form of the

disease. In _acute infantile scurvy_ we have to do generally with a

poorly-nourished, pale infant with a peculiarly alert and worried

expression. As we approach its bed it whimpers or cries out in terror.

Frequently its posture is characteristic, as it lies quietly on its back

with one thigh everted and flexed on the abdomen. Examination shows that

one or even both thighs are swollen and exquisitely tender, or that

there is merely tenderness, the baby shrieking at the slightest pressure

upon the lower end of the femur. If teeth are present, the adjacent gums

are red, swollen and bleed readily. This is the syndrome which the

medical student is taught to carry away to guide him in his everyday

practice. It is the acute, florid type, and presents a striking picture,

but must not be regarded as the common form of the disorder. If we are

to diagnose infantile scurvy early and not overlook its more subtle

manifestations, the classic textbook description must be augmented by

portrayals of types of the disorder which are less crude and more

difficult to recognize--of "subacute" and of "latent" scurvy.

The commoner form, which we have termed "_subacute infantile scurvy_,"

comprises a large number of symptoms which are inconclusive

individually, and frequently escape correct interpretation. The affected

baby is usually in the second half of the first year of life, and does

not gain in weight or gains but slightly for weeks. It may be fairly

well nourished, but is pale or sallow, with perhaps slight edema of the

upper eyelids. The mother or nurse complains that the child is irritable

and peevish, and that the appetite is poor or capricious. The gums show

a lividity or slight peridental hemorrhage, which on subsequent

examination may be no longer visible, and may have consisted merely of a

rim of crimson edging the borders of the upper gum, perhaps behind an

upper incisor, as Still pointed out. On closer examination it may be

observed that the papillæ of the tip of the tongue are markedly

congested, and that a petechial spot is to be seen on its frenum, on the

palpebral conjunctiva, or here and there on the surface of the body,

more especially where there are erosions, eczema or other skin lesions.

Attention may be called to tenderness of the lower thighs, which in some

instances is definite, in others so ill-defined and fleeting that it is

impossible to convince oneself of its significance or even reality.

There may be slight edema over the crests of the tibia, of a kind which

does not pit on pressure. The knee-jerks are almost always markedly

exaggerated. The urine is diminished in volume but is generally normal

or contains a trace of albumen and red and white blood-cells. The pulse

is frequently rapid, and becomes markedly rapid and irregular on the

slightest excitement. The respirations are also rapid (Fig. 15).

These symptoms do not constitute a rigid entity, but are subject to

manifold variations. The syndrome may be rendered less typical and clear

by the fact that the infant has gained steadily rather than lost in

weight, as is sometimes the case if the food has been insufficient

during the first few months of life. Roentgenograms of the bones may

show the "white line" at the epiphyses first described by Fraenkel (Fig.

20) or a thickening of the periosteum. However, too great reliance

should not be placed on these signs in making an early diagnosis of

this disorder, as neither is invariably present.

An instance of subacute scurvy, which in many respects is typical, is

the following:

I. F., girl, was seen when 3 months old, weighing somewhat over 8

pounds. She was given Schloss milk, 4 ounces, and then 5 ounces

every three hours, and did well, weighing 11¼ pounds two months

later. As she failed to gain for some weeks, although getting 6

ounces of food, it was thought that this might be due to the fact

that she was getting pasteurized milk and had never received an

antiscorbutic. Autolyzed yeast had been tried as a prophylactic

antiscorbutic, but failed to bring about a gain. When, however,

orange juice was substituted for the yeast, a prompt growth-reaction

resulted, a gain of 1¼ pounds in four weeks. Accompanying this

lack of gain in weight there were many of the other symptoms

enumerated above; irritability, pallor, slight tenderness of the

lower ends of the femora, albumin and a few red and white cells in

the urine. The pulse- or heart-beat was frequently over 150, and the

respiration 60 (Fig. 15). The diagnosis of subacute scurvy was

substantiated by the prompt subsidence of all symptoms when orange

juice was administered.

[Illustration: FIG. 15.--Chart of I. F., aged 7 months, showing a prompt

effect on pulse, respiration and temperature of substitution of orange

juice (30 c.c.) for autolyzed yeast (30 c.c.) and a further response

when the former was replaced by potato (15 g.).]

Infantile scurvy may be dormant for a long time. The diagnosis of

_latent scurvy_ is based mainly on the reaction to specific therapy, on

the marked improvement when orange juice, tomato, potato or other

antiscorbutic food is given. The symptoms themselves are suggestive, and

do not enable an absolute diagnosis to be made. In our experience with

many cases of this kind the usual course has been as follows: The infant

has been generally from 6 to 9 months of age, and fed for a considerable

period on pasteurized milk, which may or may not have been prepared with

cereal decoction. Nor has it been material whether gruels also had been

given. When about 6 months of age the baby ceased to thrive, to gain

satisfactorily, to look healthy, and to feed as it should. The most

careful investigation or physical examination has failed to solve the

difficulty. On the other hand, the history of a diet of heated milk,

especially if the quantity was not large, considered in conjunction with

the pallor and poor appetite, the increased knee-jerks, and perhaps a

rapid pulse and respiration (the cardiorespiratory syndrome), has

awakened suspicion. Orange juice or canned tomato, prescribed in such

cases with a view to diagnosis as well as to treatment, frequently

brings about a magic result. The following case, the weight chart of

which is reproduced (Fig. 14), is fairly typical of this abnormal

nutritional state:

H. S., boy, born December 15, 1915, was artificially fed until

January 4, 1916, when he weighed 6½ pounds. He was given 28

ounces of Schloss milk a day. (This was prepared from pasteurized

milk which was not heated a second time. It contains per litre

(quart) 140 c.c. of whole milk, 140 c.c. of 20 per cent. cream, 50

g. of dextrimaltose, 5 g. of plasmon, 0.2 g. of potassium chlorate,

and 700 c.c. of water.) By March 1 he weighed 9 pounds, and gained

three-quarters of a pound more in the course of this month. During

April he gained only 4 ounces. As will be seen from the chart,

there was almost a cessation of gain from April 10 to May 3,

although yeast was added to the diet. May 2, orange juice was given.

The weight advanced at once, the color and the general appearance

improved, and an eczematous condition of the face rapidly healed. It

will be noted from the chart that the gain occurred, although the

food intake remained the same.

Epicrisis: A baby 4 months old with latent scurvy, which existed

since he was at least 3 months of age.

_This condition of latent scurvy is probably the commonest type of the

disorder, especially in the larger cities where almost the entire milk

supply for infants is pasteurized._ It usually passes unrecognized. Most

infants fortunately are given orange juice by the time they are 6 months

of age, and may receive a small amount of vegetable or potato before

they are much older, so that they are protected from serious harm in

this way. But there is no doubt a considerable number, especially those

peculiarly susceptible, who quite unbeknown to anyone pass through the

state of latent scurvy.

If this large group of cases were included in the incidence of infantile

scurvy, we should not look upon it as a disorder which occurs rarely

during the first six months of life.

When scurvy goes unrecognized or untreated for a long time, or the

antiscorbutic content of the food is exceptionally small, or the patient

unusually susceptible, the disorder may progress and resemble the

advanced cases described in connection with the adult type of this

disease. Happily such instances are rare. One of the most typical and

vivid descriptions of _an extreme case_ of infantile scurvy is that

reported by Vincent:

The infant lay in its bed extremely apathetic and barely conscious.

Its face was ashy gray in color, the respirations were extremely

frequent, the pulse-rate was 144 per minute, and the temperature

103.2°. When touched it moaned feebly, and made no attempt at

movement. The mouth was kept open, the lower jaw hanging away from

the face. There was a complete absence of muscular tone, so that the

infant appeared to be quite incapable of voluntary movement.

The mouth presented a horrible appearance. No sign of the teeth

could be discovered, though it was stated that several had appeared.

All that could be seen was a purple mass, which was so extensive

that on superficial inspection it was difficult to distinguish

between the upper and lower jaws, despite their wide separation.

Scattered over this purple mass were areas of necrosing tissue, the

odor of which was extremely unpleasant.

Petechial hemorrhages were distributed over the back and limbs, and

a large patch of extravasated blood was found in the region of the

left hip.

Tenderness was present in all the limbs, as manifested by moaning

and by the facial expression. There was a general enlargement over

both humeri throughout their length; the ulna and radius did not

appear to be thus affected, but the index-finger of the right hand

was enlarged, especially at the junction of the metacarpal bone with

the first phalanx, the enlargement being at each side of the joint.

In the legs the signs were extreme. At both knee-joints the skin was

tightly stretched over the swollen epiphyses; the tenderness also

was greater than at any other part.

Bleeding from the gums and nose had occurred; no history of

hæmaturia could be obtained. The motions were semisolid, green, and

offensive. During the last twenty-four hours the infant had refused

food.

The baby was given large amounts of lemon juice and subcutaneous

injections of salt solution and the necrosing surfaces of the gums

were scraped and swabbed with boracic solution. By the third day the

pulse was 100, the temperature 99.8°, the odor from the mouth

scarcely noticeable, and the general condition distinctly improved.

It continued to improve and to gain in weight and when seen at the

end of the sixth week of treatment it was doing well and was quite

happy.

It will be well to consider in detail the signs and symptoms which may

develop in the course of scurvy.

=Hemorrhage.=--_Hemorrhage of the gums_ is one of the characteristic

signs of scurvy. For a reason not clearly understood it involves first

and foremost the tissues about the upper incisors. If, however, we fix

our attention too narrowly to this region we may be led into error; in

several instances we have first encountered hemorrhages about the molar

or the canine teeth, which had been overlooked because the anterior part

of the gums had been found normal. Where teeth are absent or not in the

course of eruption hemorrhages do not appear. At the onset the gums may

be merely deep red or bluish red, especially if they overlie upper

incisors which are close to the surface. Hemorrhage is particularly apt

to occur where the edges of the teeth have just broken through the

mucous membrane. In this connection the question arises as to whether

every hemorrhage of the gums in infants is to be considered a sign of

scurvy. This is a matter of some diagnostic importance. We have seen

hemorrhages of the gums at the site of erupting molar teeth where, as

prolonged observation proved, not even latent scurvy existed. This sign

should not, therefore, be regarded as pathognomonic. In two infants

entirely free from scurvy we have noted slight hemorrhage of the gums

overlying incisor teeth. It should be well understood that such an

occurrence is most exceptional; it is to be attributed probably to

bacterial invasion or to a constitutional hemorrhagic condition. One of

these cases was the following:

The infant was 8½ months old. It had been nursed by the mother up

to this time and was well nourished, but when first seen had some

fever, probably due to a grippe infection. About ten days later

distinct linear hemorrhages of the gums were noted over the two

upper incisor teeth. No treatment was instituted for this condition,

and it healed within a week. There was no subsequent sign of similar

hemorrhage or of other scorbutic manifestation in the months that

the baby was under observation.

The localization of the hemorrhage in the gums is due largely to trauma,

occasioned by the sharp contact of the jaws or of the nursing-bottle.

Local infection plays almost no rôle in infants, although in the adult

where there is caries of the teeth it frequently incites hemorrhage.

Dental caries and gingival infection may lead to local hemorrhage, even

where the nutritional conditions are normal.

Subperiosteal hemorrhage is a sign distinctive of infantile scurvy,

although it must be borne in mind that it may take place in the scurvy

of adults. It involves most frequently the lower end of the femur and

the tibia, but occurs in connection with the humerus, the mandible, the

scapula and other bones.[47] The hemorrhage usually manifests itself as

a swelling which appears suddenly at the lower end of the femur or

femora. It is brought about by trauma, at times in the course of

diapering, or by manipulation in testing for local tenderness. The

swelling is very tender, and varies in size from an enlargement which is

difficult to appreciate, to one which renders the leg fully twice its

normal circumference (Fig. 18). It may involve merely a small part of

the long bone or extend up or down the shaft for a long distance. As

might be supposed from the nature of this lesion, the enlargement

persists for weeks, frequently long after the gums and the general

symptoms have disappeared. During this period it becomes harder and less

tender, and may develop the consistency of bone; it is in this stage

that such swellings have been diagnosed as new growths, and that

incision or even amputation of the leg has been resorted to. In

subacute cases the swelling--which must be regarded as hemorrhagic

rather than scorbutic--may be absorbed gradually in spite of the fact

that no antiscorbutic food has been given. This has led to the mistaken

conclusion that the scurvy has been cured without dietetic treatment.

[47] There seems to be some misconception as to the pathogenesis of the

subperiosteal hemorrhage in scurvy. In most reports this lesion is

described as if it resulted from a hemorrhage burrowing its way beneath

the periosteum and raising it from the subjacent bone. In point of fact,

such an event is impossible, as will be fully realized when one

experiences the great difficulty in separating periosteum from normal

bone. The scorbutic process involves the periosteum so that it is no

longer normal but becomes insecurely attached to the shaft of the bone,

and is readily stripped off by hemorrhage.

Subperiosteal hemorrhage may be clearly seen by means of the fluoroscope

or in X-ray photographs (Figs. 16 and 17). The shaft of the bone appears

surmounted by an elongated blood-clot, which is more or less distinct

according to its age and density. It may become calcified, as clearly

seen in figures. More often the periosteum undergoes calcification or

ossification, especially near the site of the separation of the

epiphysis. This gives rise to a bizarre radiographic picture which may

be difficult to interpret--the opaque strip or streamer being almost

unrecognizable as periosteum (Fig. 17).

[Illustration: FIG. 16.--Infant 11 months old. Separation of lower

epiphysis of femur. Fraying of end of femur and head of tibia.

Subperiosteal hemorrhage surrounding lower part of shaft of femur, with

calcification of periosteum and of clot.]

[Illustration: FIG. 17.--Infant 11½ months old. Separation of lower

epiphysis of femur with marked subperiosteal hemorrhage. Typical

periosteal "tags" or "streamers." The connection of these "streamers"

with the periosteal layer is evident.]

Hess and Unger observed that in several instances where subperiosteal

hemorrhage had been diagnosed, X-ray examination disclosed that the

swelling of the thigh was due mainly to infiltration of the muscles and

subcutaneous tissue. It is surprising how an infiltration of serum gives

rise to a swelling which resembles in appearance and consistency the

classical subperiosteal tumor.

The skin, mucous membranes and subcutaneous tissues are frequently the

sites of hemorrhage. There is a difference of opinion as to how

frequently petechial hemorrhages occur in scurvy, particularly as to

whether they are encountered early in this disorder. Great variation in

this regard may be noted in individuals and in groups of cases occurring

at different times. In the cases reported in 1914 by Hess and Fish,

petechial hemorrhages were frequently an early sign, to such an extent

that they led to a study of the blood and blood-vessels in this

disorder. The hemorrhages in this "scurvy epidemic" were the result of a

complication of scurvy with an infectious disease. It is not necessary,

however, for infection to exist to bring about a rupture of the small

vessels. The idiosyncrasy of the individual has to be considered as well

as the fact that infants have a tendency to develop minute skin

hemorrhages, especially such as have an exudative diathesis. In the

course of scurvy, petechiæ may be found not only in the skin, but in

the mucosa of the mouth, especially overlying the hard palate, and also

in the palpebral conjunctiva, identical with the minute petechiæ so

significant of general sepsis. In addition to these minute hemorrhages

larger ones are not infrequently found in various parts of the body,

especially in the neighborhood of the joints. They appear as

discolorations of various intensities and shades, and are often

interpreted as being merely the result of bruises. These have been

encountered most often about the knee-joint, on the forehead, or in the

concha of the external ear, where they may best be seen by means of

transmitted light.

A form of hemorrhage which must be especially mentioned, although it is

very infrequent, is that taking place into the orbit, leading to a

_proptosis of the eyeball_, usually the left (Still). This sign should

be borne in mind, as it occurs occasionally before other symptoms have

rendered the diagnosis clear, and may lead to a diagnosis of tumor.

As mentioned above, hemorrhages into the muscles or between the muscle

planes are very common in adults, leading to hard swellings, the typical

"scurvy sclerosis." Such effusions occur much less frequently in

infants, due probably to their lack of activity. In addition to these

hemorrhages there are serous effusions of the muscles similar to those

which are found in the pleural and pericardial cavities. These effusions

are very striking at necropsy, when one incises the muscles--for

example, the muscles of the thigh. During life they are frequently

mistaken for subperiosteal hemorrhages.

Less frequently there are _hemorrhages into the internal organs_. These,

however, play a comparatively insignificant rôle in the symptomatology

of this disease. At postmortem examination we find numerous hemorrhages

of the pleura, pericardium and peritoneum, which rarely produce

symptoms during life. Still records a case with marked abdominal pain

and swelling, which he believed to have been due to hemorrhage into the

wall of the intestine. As previously mentioned, O'Shea met with a case

of hemorrhage into the cæcum which was mistakenly operated upon for

appendicitis. Hæmothorax and hæemopericardium occur, especially

associated with local inflammatory processes of tuberculous nature. The

clinical aspect of hemorrhage of the gastro-intestinal and the

genito-urinary tracts will be considered elsewhere.

In the scurvy of adults as well as that of infants, _the nails and the

hair_ are altered by the nutritional condition. Mention has been made of

the hyperkeratosis recently emphasized by Wiltshire as an early sign,

occurring especially on the thighs and legs. The skin is frequently dry,

the so-called "goose skin" that is seen in some poor nutritional states.

The nails are thin, brittle and lined; at times small hemorrhages will

be noted beneath them. The hair also becomes thin and dry, and there is

a tendency for petechial hemorrhages to develop at the roots.

In a paper on the therapeutic value of yeast and of wheat embryo the

author called attention to the fact that _eczema_ may occur in

connection with infantile scurvy, and be cured by means of orange juice.

We have met with eight cases of eczema in infantile scurvy, which, in

almost every instance, have yielded promptly to an antiscorbutic, thus

proving their scorbutic nature. A case of this kind is the following:

M. L., seven months old, was getting "Molkenadaptierte" milk, and in

addition autolyzed yeast. On May 25th it developed nasal diphtheria,

but soon afterward did well. On June 9th it was gaining, but its

pulse was 160 and respirations 80. A few days later it developed

marked eczema about the neck and to a less extent on the back and

buttocks. The "capillary resistance test" was negative.

Cardiographic tracings showed merely a simple tachycardia. A few

days later petechial spots appeared at the site of the eczema. On

June 17th orange juice was given. The appetite improved, the

cardiorespiratory syndrome disappeared, and the child began to gain.

The eczema also cleared up rapidly without any local treatment.

We wish to draw particular attention to this skin condition, as it is

generally not mentioned, or has been regarded merely as a chance

occurrence. The report of the American Pediatric Society includes two

cases of eczema as a complicating condition. This symptom is of special

interest in view of the fact that a similar skin lesion constitutes one

of the typical signs of pellagra. In a case of infantile scurvy we have

seen an eruption at the nape of the neck which was symmetrical and

greatly resembled that of pellagra. Andrews refers to the occurrence of

eczema in his description of infantile beriberi.

In a paper published a few years ago attention was drawn by Hess and

Fish to the fact that infantile scurvy frequently is associated with the

exudative diathesis of Czerny, a pathological condition which

predisposes to the development of exudations of the skin and the mucous

membranes. Infants suffering from this condition--intertrigo, eczema,

recurrent bronchitis--seem to be particularly susceptible to scurvy and

to develop it more quickly than others.

As is well known, _edema_ constitutes a not infrequent symptom of adult

scurvy. It has not, however, been accorded any place in the

symptomatology of infantile scurvy. We do not refer to the edema in

connection with subperiosteal hemorrhage or separation of the epiphyses

of the long bones, but a mild and peculiar form which is seen early in

the disease. It involves most regularly the upper eyelids, and the

legs--especially the skin covering the lower part of the tibiæ. In the

latter site it differs from edema as usually encountered, in that it

does not pit on pressure; it is firm, tense, causing some glossiness of

the overlying skin, which is rendered difficult to wrinkle or to pinch

between the fingers. Not infrequently the skin is slightly reddened, a

sign of interest, in view of a similar, although much more intense,

hyperæmia seen in pellegra.

In addition to this very mild edema there may be marked swelling,

resulting in what might be called, following the terminology of

beriberi, "wet scurvy." The legs, body and even the face may be swollen.

This has been frequently described in adult scurvy, and occasionally in

infantile scurvy. The first case of infantile scurvy described in

America, that of Northrup, had marked edema of the scrotum. Edema is

frequently met with in "ship beriberi," a disorder considered by some

writers to be a combination of beriberi and scurvy.

The symptom leading to the diagnosis of scurvy most often is

_tenderness_ or swelling of one of the extremities, as the antecedent

clinical signs, comprising latent scurvy, are generally overlooked.

These manifestations involve usually the distal end of the thigh or

thighs. The tenderness is elicited most readily by pressure just above

the knee, which causes the baby to wince, and to quickly flex the thigh,

a reaction termed by Heubner "the jumping-jack phenomenon." As a result

of pain and tenderness, the leg lies often immobile in a state of

pseudo-paralysis (Fig. 18). There may be tenderness elsewhere than in

the long bones. Kerley refers to two cases showing tenderness of the

spine, and we have seen a similar case. Not infrequently there is

tenderness of the chest wall, the earliest symptom noted by nurse or

mother being unaccountable crying whenever the baby is lifted by the

thorax. This is largely due to the sensitiveness of the ends of the

cartilage and bone which are pressed together at their junction.

[Illustration: FIG. 18.--Infant with marked scurvy. Characteristic

posture and swelling of right thigh.]

An early sign of infantile scurvy is _beading of the ribs_--the

development of a "rosary" similar to that characteristic of rickets

(Fig. 19). This has recently been described by Hess and Unger in an

article devoted to this subject. That this rosary is truly scorbutic and

not rhachitic is proved by the fact that it recedes rapidly when

antiscorbutic foodstuff is given, and that it remains uninfluenced by

treatment with cod liver oil. A similar scorbutic rosary occurs in

guinea-pig scurvy, but has been termed "pseudo-rhachitic." It is

important that this sign should be recognized, as it is probable that

much of the confusion regarding the relationship and frequent

association of these two diseases is due to considering the beading

rhachitic. The interpretation of infantile scurvy as "acute rickets,"

the view held previous to the writings of Barlow, was based largely on

the development of the rosary. To-day the error is made of regarding

early scurvy as chronic rickets; the rickets supposed to be occasioned

by a diet of condensed milk is probably more often scurvy. This beading

differs generally from the round knobby "rosary" usually encountered. It

is more angular, the junction taking on a step-like form, as if the

abutting ends of the cartilage and the bone were of unequal size, and

not well fitted to each other. In the accompanying radiograph (Fig. 19)

it will be noted that the "beads" present an irregular appearance.

[Illustration: FIG. 19.--Same infant as in figure 17. Scorbutic beading

of the ribs (rosary). This developed on a diet which included cod liver

oil, and decreased when an antiscorbutic was given. Note peculiar ragged

appearance of "beads."]

In Figs. 6, 16 and 17 will be seen illustrations of a _separation of the

epiphyses_ of the head of the humerus, and of partial and of complete

separation of the lower ends of the femora. This is a frequent lesion of

fully developed scurvy in infants, children, and even in young adults.

It is most frequent at the lower end of the femur, the upper end of the

tibia, the head of the humerus, and the costochondral junctions. It is

to these epiphyseal separations that the term fracture or infraction

usually refers. Union is remarkably perfect even where no splint has

been employed, and nature has effected the cure (Fig. 7). Occasionally

there is some deformity, as when coxa vara develops. The callus is often

remarkably large; an old callus sometimes undergoes destruction in the

course of scurvy.

We have referred to use of röntgenograms in connection with separation

of the epiphyses, subperiosteal hemorrhage, cardiac enlargement and

beading of the ribs. In addition to its application in these

connections, the X-ray may be of service to show a peculiar alteration

of the ends of the long bones--_the white line of Fraenkel_. This is

portrayed in Fig. 20. It is best seen at the lower ends of the radius

and femur, and appears as a white, transverse, somewhat irregular band.

Its diagnostic value has been greatly exaggerated, as it is frequently

not present when the disease is advanced (observe radiographs

illustrating separation of the epiphyses). This sign should therefore

not be relied on for establishing the diagnosis. Furthermore, changes

may be seen in connection with rickets (cases receiving antiscorbutic

diet) which are very difficult to differentiate from the "white line."

It cannot be employed as a criterion of the progress of the case, as it

may persist for months after all other signs and symptoms have

disappeared.

[Illustration: FIG. 20.--Radiograph. Infant 14 months of age, showing

"_white line_" at wrist some months after cure of scurvy.]

The joints may be involved in scurvy. In most instances, however, where

swelling of the joints is diagnosed, the lesion is periarticular. An

effusion of serum or of blood does occur occasionally into the joints

and has been found at operation, at necropsy, and by puncture. If these

effusions are allowed to go undisturbed, to be absorbed as a result of

antiscorbutic treatment, they rarely suppurate. Czerny and Keller

report the articular fluid as invariably sterile.

The cardiovascular system has been given but scant attention in

connection with scurvy. Adults complain not infrequently of palpitation

and pain over the pericardium, or rather of a tightness or oppression in

the chest. Little information is given regarding the size of the heart.

Darling described enlargement of the heart, especially a right-sided

hypertrophy, which he thought was pathognomonic of the Rand type of

scurvy. The pulse is described in some cases as slow, and in others as

rapid. In descriptions of infantile scurvy the entire subject is

generally passed over without mention--for example, in the excellent

report of the American Pediatric Society nothing whatsoever is stated

regarding the heart's action or the pulse. Barlow wrote: "There is

nothing to note regarding the heart and lungs."

In a paper written a few years ago, it was pointed out by the author

that there is frequently enlargement of the heart, and more especially

of the right heart. This can be elicited at the bedside and has been

substantiated in numerous cases by means of the Röntgen-ray, which

demonstrates not only enlargement of the heart, but also a marked

broadening at its base, at the site of the large vessels (Fig. 21).

These phenomena resemble closely the description of Reinhard in cases of

beriberi.

[Illustration: FIG. 21.--Radiograph. Scorbutic infant 14 months of age,

showing cardiac enlargement and broadening of shadow at base of heart.]

Necropsy protocols usually are incomplete and unsatisfactory in their

descriptions of the heart. The excellent monograph of Schoedel and

Nauwerk, however, which reports five careful necropsies, contains the

following data regarding three:

1. Pericardial fluid somewhat increased, both ventricles moderately

dilated, the right somewhat hypertrophic.

2. The heart showed a hypertrophy of the right and left ventricles, as

well as dilatation of the right ventricle.

3. The right ventricle dilated and slightly hypertrophied, the muscles

pale and tough.

In addition to this enlargement of the heart, or perhaps associated with

it, there is a combination of signs which has been termed "_the

cardiorespiratory syndrome_" (Hess). It will be noted in the above

description of a case of subacute scurvy, that the pulse- or heart-beat

was frequently over 150, and the respiration 60. These phenomena were

noted in several instances before their significance and intimate

relationship to scurvy were realized. The heart-beat not infrequently is

found to be 200 per minute, and to be characterized by marked

lability--increasing to an astonishing degree as the result of slight

exertion or excitement. A mild febrile disturbance causing a rise of

temperature to little more than 100° F. will send the pulse-rate up 30

beats. It must not be thought that this refers to severe cases; the

babies we have in mind are similar to the one cited as an instance of

subacute scurvy. Apparently they are not ill, but show merely some

tenderness of the thighs, pallor, and the other minor signs described.

The cardiographic tracings showed a simple tachycardia with an

exceptionally tall T-wave in some tracings, such as is commonly seen in

exophthalmic goitre (Fig. 22).

[Illustration: FIG. 22.--Electrocardiogram in case showing

cardiorespiratory syndrome. Tachycardia with exceptionally tall T-wave.]

The rapidity of respirations is perhaps a more delicate indicator of

this disturbance than the pulse and has been found to be markedly

affected when the latter was merely slightly increased in rate. For

example, in one instance the respirations were 64, 60 and 64 on three

successive days, while the pulse was 124, 141 and 136; in other words,

there was a 2:1 instead of the normal 4:1 pulse-respiration ratio. The

accompanying chart (Fig. 15) illustrates the phenomenon in all its

details better than a verbal description. There is one point in

connection with it, to which especial attention should be called. This

is a reaction evident at a glance at the chart--the sharp drop in the

pulse and in the respiratory rate when orange juice was given. It is the

essence of the phenomenon; a therapeutic response which proves that the

rapidity is scorbutic in nature.

The main involvement of the _respiratory system_ in scurvy is the

polypnoea just described in connection with the cardiorespiratory

syndrome. There is no aphonia, a sign so typical of adult and of

infantile beriberi, although at times the voice is abnormal and whining.

The lungs frequently show some dullness posteriorly, which may be due to

engorgement or to the pressure of the enlarged heart. Pneumonia is a

frequent complication and edema a terminal event. Hydrothorax associated

with hydropericardium is of frequent occurrence, and was noted in the

early description of this disease in adults and in the first account of

Barlow. These effusions rarely progress to what may be termed the

clinical degree and under antiscorbutic treatment are rapidly absorbed.

It is commonly thought that scurvy does not involve _the nervous

system_; that this is a feature which distinguishes it sharply from

beriberi, another "deficiency disease." This view is incorrect, for the

nervous system is probably affected in many cases of scurvy. The

rapidity and lability of the pulse, combined with the rapid

respirations, would seem to be due to a disturbance of the vagus

mechanism. It is true that in beriberi the vagus is involved to a still

greater extent, especially its recurrent laryngeal branch which brings

about the characteristic aphonia. In scurvy the knee-reflexes are

generally increased. Very rarely they are absent in infantile scurvy, as

described in adults. It is impossible to judge whether the pain and

tenderness in infants are due in part to a sensitiveness of the nerve

trunks as well as of the periosteum. Careful studies in adult scurvy

should furnish an answer to this question. No methodical examination

for areas of anæsthesia or paræsthesia, signs which occur so frequently

in connection with beriberi, has been carried out in scurvy. In certain

epidemics, however, pains in the limbs have been prominent symptoms.

The optic discs are generally pale in both infants and in adults, with

occasional signs of neuredema. Nyctalopia, so frequently encountered,

must be regarded as a circulatory symptom rather than as one of nervous

origin.

In a recent paper the author described a focal degeneration of the

lumbar cord in a case of infantile scurvy, the lesion involving mainly

the anterior horn cells (Figs. 3 and 4). In view of this report it would

be well to watch for corresponding clinical signs of involvement of the

spinal cord. Herpes has been described in connection with both adult and

infantile scurvy. In one of the early cases in the American literature

Fruitnight reported a case with herpes in a girl five years of age. In

considering the rôle of the nervous system, mention should be made of

cases where sweating constituted an important symptom. Finkelstein lays

particular stress on this symptom in infantile scurvy. We have not met

with it frequently; possibly it is due in part to complicating rickets.

As would be expected, the nervous system is at times the site of

hemorrhage. Such lesions cannot, however, be considered essentially

nervous. For instance, hemorrhage into the meninges may occur, as in the

case of Sammis, where there was "a general clonic convulsion" before

death, and a blood-clot 2½ inches long by ½ inch wide was found at

necropsy between the dura and arachnoid. Fife reported a similar case.

Finkelstein also has drawn attention to the occurrence of meningeal

hemorrhage, and Hess and Fish reported obtaining bloody cerebrospinal

fluid from a case with meningeal symptoms. Recently Aschoff and Koch

have depicted hemorrhages in the sheath of the sciatic nerve, which

undoubtedly must have given rise to symptoms during life.

In view of many of these symptoms, especially those involving the vagus,

scurvy must be looked upon as a disorder which may seriously affect the

nervous system. Furthermore, when we note the marked reaction brought

about by the antiscorbutic vitamine--for example, the sharp fall in the

rate of respirations and of pulse, as shown in Fig. 15, after giving

orange juice, we must conclude that the antiscorbutic vitamine

functions, at least indirectly as an antineuritic vitamine--that it must

possess this character to allay the various nervous signs of this

disorder.

The urinary system is frequently involved in the course of scurvy. Among

38 cases Still reports that 89 per cent. gave evidence of urinary

changes and that 60 per cent. showed hæmaturia. Finkelstein found

urinary signs in at least a third of his cases. Our figures, the result

of a study of subacute and mildly acute cases, correspond more nearly

with those of Finkelstein.

The occurrence of pronounced renal hemorrhage as a first symptom of

scurvy is emphasized in many descriptions of this disease, and has

impressed itself in the minds of physicians. It is true that this occurs

sometimes at the onset, as does hemorrhage into or about the joints, or

hemorrhage behind the eyeball. It is well to bear these possibilities in

mind, but they must be regarded as very exceptional early signs of this

disorder. We have encountered frank hæmaturia but once in the early

stage of infantile scurvy. The blood emanates generally from the

kidneys, although the submucous hemorrhages of the bladder as well as in

the urethra, described both in man and in guinea-pigs, indicate that the

blood in the urine may have its origin lower down in the tract. This

bleeding should be regarded not as a sign of nephritis, but rather as a

hemorrhagic manifestation. It is less frequent in adults than in

infants. O'Shea reports some degree of hemorrhage in 15 per cent. of his

cases (adults).

A true nephritis, however, may occur in connection with scurvy. There

may be albumen and many casts, or a urine loaded with casts and

cylindroids. These peculiar casts may appear suddenly, as in the

alimentary intoxication of infants, and disappear just as rapidly when

antiscorbutic treatment is given. The urine may contain a large number

of pus cells as in pyelitis. This condition may be accompanied by

irregular fever, but in two instances we have encountered it where the

temperature was normal. It is to be regarded, probably, merely as one of

the manifestations of secondary infection so commonly associated with

scurvy. Some pus cells may continue to be present in the urine for a

period of months. This is likewise true of the red cells. We have under

observation at present an infant which had subacute scurvy almost three

years ago and still has red blood-cells in the urine.

Oliguria is a common symptom of both adult and infantile scurvy. Lind

mentioned this symptom, and in this connection remarks on the beneficent

effect of antiscorbutic treatment. Charpentier called attention to the

fact that in a case of scurvy the urine decreased from 1250 g. to 800 g.

The report of the American Pediatric Society mentions scanty urine in 9

cases and suppression of urine in one. This sign, however, was not

emphasized until recently, when Gerstenberger, and Hess and Unger drew

attention to its frequent occurrence in infants. It has some diagnostic

significance and should be borne in mind where a decreased excretion of

urine is reported. A counterpart of this symptom is the sudden

outpouring of urine frequently noted after antiscorbutic treatment has

been instituted. This polyuria accounts for the loss of weight or lack

of gain which sometimes accompanies unmistakable general improvement,

and which is difficult otherwise to understand (Fig. 23). It is

interesting to learn that oliguria occurs commonly in both adult and

infantile beriberi.

[Illustration: FIG. 23.--Joseph G., aged 9 months. Chart showing

stationary weight (due to oliguria followed by diuresis) in spite of

marked variation of fluid intake. A=Schloss milk; B=cod liver oil; C=egg

yolk; D=1 ounce of orange juice; E=potato (orange juice stopped).]

One of the earliest, as well as one of the most constant symptoms of

scurvy, is a _lack of appetite_. It is a typical sign of latent scurvy,

although occasionally we have met with cases where the appetite remained

unimpaired until the hemorrhagic stage was reached. In adults there is

sometimes bulimia and a marked capriciousness of the appetite. Anorexia

is a true scorbutic symptom, disappearing with remarkable rapidity when

antiscorbutic food is given, and not capable of alleviation by tincture

of gentian or other vegetable bitters. Whether it depends upon a lack of

secretions in the gastro-intestinal tract is not known, as there has

been no thorough study of this aspect of the disorder. The hydrochloric

acid generally is deficient in cases of scurvy. Recently McCarrison has

laid emphasis on the importance of the impairment of the digestion and

assimilative function in scurvy. This subject gains added interest in

view of the recent reports of Uhlmann as well as of Voegtlin, showing

that water-soluble vitamine acts as a stimulant for the various

secretions of the gastro-intestinal tract.

As a result of McCollum's statement that scurvy is due mainly to

_constipation_, marked attention has been directed recently to the

action of the bowels in this disorder. This question has been discussed

in the chapter on etiology, and, therefore, will be referred to in this

place merely from the clinical viewpoint. In our experience the activity

of the bowel varies greatly in cases of latent or subacute scurvy. In a

great many instances it has been normal; more often there has been

slight constipation, and exceptionally there has been irregular

diarrhoea. In other words, no causative relationship or parallelism

could be observed between the emptying of the intestinal tract and the

development of scurvy. This in general has been the experience of

others. In the report of the American Pediatric Society the bowels are

stated as having been regular in 74 cases, irregular in 15, constipated

in 126, and diarrhoeal in 65. It may be added that we were unable to

cure scurvy by means of liquid petrolatum or phenolphthalein, either in

infants or in guinea-pigs, and likewise unable to protect guinea-pigs

from scurvy by means of various laxatives. On the other hand, opium

given in the form of the camphorated tincture did not lead to an

intensification of the symptoms, although, in one case, the bowels did

not move for over three days.

As complications involving the gastro-intestinal tract may be mentioned

the vomiting of blood, which is stated in the above report as occurring

in 2 of the 361 cases, as well as bleeding from the bowel, which was

noted in 37 cases, in 12 of which there was bloody diarrhoea. However,

these are late symptoms, and correspond to the mycotic ulcers which are

so frequently found, especially in the large intestine, in cases of

scurvy. Mention may again be made of the fact that hemorrhages may occur

under the peritoneum and give rise to symptoms simulating appendicitis

or general peritonitis.

Jaundice has been described in connection with certain epidemics of

scurvy. To our knowledge it has not been reported in infants.

The presence of worms has been frequently reported in the bowel or in

the stool of patients suffering from beriberi. There have been no

similar investigations in relation to scurvy. It would be interesting to

inquire into this question, as it is quite possible that a lack of

antiscorbutic foodstuff may favor the presence of parasites in the

intestinal canal.

Before closing this consideration of the involvement of the alimentary

tract, we would call attention to the relation of stomatitis to scurvy.

Among adults this is a common complication. In infants it is uncommon,

due to the absence of carious teeth and secondary infection; we have

encountered it in but two instances. Stomatitis is of importance in this

connection, as it frequently develops on the basis of malnutrition,

scurvy being one of the disorders which may constitute the substratum.

Such may be the case where stomatitis occurs in epidemic form--for

example, among large bodies of troops. It may be remarked that

stomatitis at times was a very common disease among the soldiers in the

recent war.

TABLE 4

THE PLATELETS AND OTHER BLOOD-CELLS IN SCURVY

======================================================================

-----+------+-----------+--------+-----------+---------+--------------

M. H.| 5/ 3 | 280,000 | ...... | ......... | .. |

| 5/ 4 | 248,000 | 10,000 | ......... | .. |

| 5/ 5 | ....... | ...... | ......... | .. |Boiled orange

| 5/ 8 | ....... | ...... | 4,300,000 | 35 |

| 5/16 | ....... | 15,900 | ......... | .. |

| 7/ 2 | ....... | 6,800 | 5,456,000 | 40 |Well but pale.

A. L.| 5/ 3 | 300,000 | ...... | ......... | .. |

| 5/ 5 | ....... | 21,000 | ......... | .. |

| 5/ 9 | ....... | ...... | 5,480,000 | .. |

| 5/11 | 382,000 | ...... | ......... | .. |

| 5/13 | ....... | ...... | ......... | 65 |

| 5/16 | ....... | 11,500 | ......... | .. |

H. C.| 5/ 8 | 320,000 | 20,000 | ......... | .. |

| 5/ 9 | ....... | ...... | 5,340,000 | .. |

| 5/13 | ....... | ...... | ......... | 70 |

| 5/16 | 362,000 | ...... | ......... | .. |

B. B.| 5/ 4 | 496,000 | 21,000 | ......... | .. |A severe case.

| 5/13 | 585,000 | 14,000 | 3,200,000 | 70 |

| 5/18 | ....... | 17,600 | ......... | .. |

| 7/13 | ....... | 40,000 |=7,672,000=| 82 |Has gained well

| 7/15 | ....... | ...... |=7,640,000=| 88 |

H. Y.| 5/15 | 560,000 | ...... | ......... | .. |

| 5/16 | 424,000 | ...... | ......... | .. |

| 7/ 9 | ....... | ...... | 5,750,000 | 45 |

-----------------------------------------------------------------------

Scurvy is associated with an alteration of both _the blood and the

blood-vessels_. The characteristic pallor, which is one of the most

common as well as earliest symptoms, is due in a large measure to the

anemia. This anemia is of the secondary type, but has definite

peculiarities, and does not resemble that encountered in the course of

tuberculosis, rickets or marasmus. The hemoglobin is greatly diminished,

far out of proportion to the decrease in the number of the red cells.

Not infrequently we will find a hemoglobin index of 0.5. Table 4, above

taken from the article on this subject by Hess and Fish (1914), brings

out the details of the blood-picture. It shows that there may be a

polycythemica, which may persist after the other signs of the disorder

have disappeared. Brandt has recently made similar observations,

reporting in one instance over ten million red cells two months after

treatment. In soldiers suffering from scurvy Wassermann has encountered

cases where, during convalescence, the red-cell count has risen to over

six or seven millions and the hemoglobin to 110 or 120 per cent. Under

the microscope the red cells show poikilocytosis, anisocytosis and a

lack of hemoglobin; they are slightly enlarged, with the occasional

occurrence of exceptionally large cells resembling the "dropsical cells"

described in connection with chlorosis. Sometimes a few nucleated red

cells and myeloblasts are seen; megaloblasts are also reported.[48] The

blood-picture bears a remarkable similarity to that of chlorosis, a

point of interest, in view of the fact that both scurvy and chlorosis

have been attributed to a disordered function of the endocrine glands.

The "dropsical cells" suggest a disturbance of the salt balance in the

plasma. In some cases we have found a decreased fragility of the red

cells, which also has been described in chlorosis.

[48] Senator regarded the marrow in scurvy as being aplastic. Nobécourt,

Tixier and Maillet report postmortem examinations where the marrow

showed an increased number of myelocytes and nucleated red cells of

various kinds. They consider the typical blood change an intense myeloid

reaction of the blood.

The total number of leucocytes is slightly increased. In our cases the

mononuclear cells have averaged 66 per cent., which is somewhat high

even for infants. This has been the experience of Labor, who, however,

also describes an eosinophilia during convalescence, a phenomenon which

we have not encountered. Some describe a marked increase in the

polynuclear cells, which, probably, is to be regarded as the reaction to

secondary infections. There is indeed a marked difference of opinion in

regard to the morphology of the blood in scurvy in adults as well as in

infants. Some found a large number of one type of cell--for example,

nucleated red cells, myelocytes, eosinophiles--whereas others have

failed to observe an increase of these cells. The divergent reports

probably should be attributed to the fact that the investigators are

describing scurvy of various grades of severity, of different stages of

development, or complicated by intercurrent disease.

Nobécourt, Tixier, and Maillet have questioned whether there is always

complete recovery from this anemia, which is severe from the standpoint

of hemoglobin and iron. The older authors reported instances where men

have been weakly and ailing for the remainder of their lives after an

attack of scurvy. In some infants pallor and anemia may persist for

months after apparent cure; however, this is the exception rather than

the rule.

In view of the fact that scurvy frequently is classed as a hemorrhagic

disease, and that hemorrhages play such an important rôle in its

symptomatology, a consideration of the factors concerned in the

_coagulability of the blood_ is of interest. In an investigation (Hess

and Fish) it was found that the oxalated plasma (of blood taken directly

from a vein) showed a slightly delayed coagulation time--eight to

fourteen minutes. The "bleeding time" carried out according to the

simple method of Duke was slightly increased. Holt reports a case where

a child bled to death following incision into an epiphyseal swelling at

the lower end of the femur. The number of blood platelets is increased,

running parallel, as is usually the case, with the number of red cells

(Table 4). This increase in the blood-platelets, recently confirmed by

Tobler and by Brandt, is a very exceptional phenomenon, and was not

anticipated in connection with a disorder characterized by hemorrhage.

The antithrombin content of the plasma is normal.

The investigation was directed to a study of the integrity of the

blood-vessels in order to account for the hemorrhages. To this end the

"capillary resistance test" was devised.[49] In the majority of cases

this was found to be "positive" (the blood-vessels showing an increased

permeability) and to become negative when antiscorbutics were given and

the symptoms disappeared. This shows that the cellular structure of the

vessels is altered in the course of scurvy, and indicates probably that

this is an important cause of the hemorrhages. The edema of the face and

ankles, the outflow of serum into the body cavities and into the muscles

(Barlow) must be regarded as other evidences of the inadequacy of the

vessel walls. The tendency of children with exudative diathesis to

develop scurvy is perhaps still another manifestation of vascular

weakness. This point of view has been strengthened recently by the

pathological studies of Aschoff and Koch, who regard scurvy as a

nutritional disorder in which there is a lack of some colloidal

substance needed for the normal structure of the vessels.

[49] A blood-pressure band, or tourniquet, is placed about the arm, and

the pressure increased until the forearm becomes cyanosed and the radial

pulse is almost obliterated. The pressure is then maintained at this

level for 3 minutes. The principle of this test consists in subjecting

the capillaries and venules to increased intra-vascular pressure to

observe whether this strain results in the escape of blood. In infants

the pressure was usually raised to 90 mm.; in some cases it had to be

raised higher in order to entirely obstruct the return flow of the

blood.

The test is considered to be "positive" when the forearm shows many

petechial spots. In normal infants petechiæ were almost always absent,

or there were few to be seen. This is not a specific test for scurvy,

but demonstrates a weakness of the vessel walls, whatsoever may be the

cause. It is found to be positive in the majority of cases of scurvy.

When one makes a subcutaneous puncture in infants suffering from scurvy,

a small hemorrhage very often develops at the site of the puncture

wound. This is not the case when one makes a hypodermic puncture in a

normal person or in a hemophiliac, although it does occur in cases of

purpura. This "stick test" is not a constant sign of scurvy, but, like

the capillary resistance test, was found in many cases and disappeared

with the subsidence of the disorder. It shows that the cells of the skin

and subcutaneous tissues are affected, and possibly that their

thromboplastic power is diminished.

=Nutrition and Growth.=--The general nutrition suffers in scurvy as the

disease progresses. It is a mistake, however, to picture the scorbutic

individual, either adult or infant, as in a state of malnutrition. Not

infrequently he appears well nourished, an appearance which is

heightened by the slight edema of the face. Infants generally for a

period of weeks or months preceding the onset maintain a stationary

weight. This may be the only sign of the scorbutic condition. For

example:

An infant seen in 1915 gained about one-half a pound during the

months of February, March, April and May. At this time it was

somewhat over 9 months of age and had never received raw milk or

other antiscorbutic food. In June it was given orange-peel juice,

and gained 2 pounds within a month. There were no other scorbutic

signs or symptoms, and no loss of appetite during the months of

February and March, although the baby was suffering from a

progressive scurvy.

The growth impulse of the body throughout an attack of scurvy remains

unimpaired, being merely in an inactive or quiescent state. Fig. 14

shows this very well, demonstrating that when an antiscorbutic food is

added to the dietary the gain may be abnormally great--there may be

supergrowth. Generally such marked increases are due to an increase in

the consumption of food, following the stimulation of the appetite.

However, decided gain in weight may follow the giving of orange juice or

other antiscorbutic despite the fact that the intake of food is

maintained at the same level.

[Illustration: FIG. 24.--Development of scurvy in spite of normal gain

in weight in a baby who had been underfed since birth.]

Although it may be stated as a principle that the development of scurvy

is accompanied by a failure to gain in weight, there are exceptions to

this rule. Under certain conditions the weight may follow a perfectly

normal course during the entire period. Fig. 24 illustrates this

clinical paradox:

A baby was seen in January, when it was 7½ months of age. Toward

the end of February, in spite of constant and normal gain in weight,

he manifested unmistakable signs of scurvy--peridental hemorrhage

over the upper incisor teeth, which were erupting, and tenderness of

the lower ends of the femora. The scorbutic nature of these signs

were substantiated by their prompt subsidence on the administration

of orange juice. We explain the phenomenon as follows: This baby had

been starved in a two-fold sense throughout the first months of its

life--it had received a diet lacking in caloric value as well as

deficient in antiscorbutic vitamine. Its growth impulse had been

held in abeyance for months by both of these factors. When

sufficient calories were supplied in the dietary, growth was no

longer repressed, and a steady gain resulted in spite of the

continued inadequacy of the antiscorbutic factor.

It has been shown that during the period of infancy undernourishment

must be extreme to occasion stunting of growth in length. In animals

Aron demonstrated that lack of nutrition led to a decrease of the fat

and of the muscle of the body, but that the skeleton nevertheless

continued to grow, and the ash content of the body to increase. In

marasmus, or infantile atrophy, the baby usually grows in length,

although its weight remains stationary or decreases. In scurvy we have

shown that there is frequently a definite retardation of growth in

length, an observation which has been recently confirmed by Epstein in

babies which developed this disorder in the foundling asylum of Prague

during the war. This fact shows how profoundly the metabolism must be

disturbed by this disorder. Figure 25 portrays this retardation in

growth and the sharp reaction when orange juice was added to the

dietary. It also demonstrates that the growth impulse remains unimpaired

and capable of quick response when the essential food factor is

furnished.

[Illustration: FIG. 25.--Showing retardation of growth in length during

the period when no orange juice was given and supergrowth when it was

given once more, O. J.=orange juice. O. P. J.= orange-peel juice. Lower

curve represents the normal.]

=Fever.=--Fever frequently accompanies scurvy. It is generally of a low

grade, ranging between 100° and 101°, as may be seen in Fig. 15. There

is a difference of opinion as to whether the rise of temperature should

be considered as truly scorbutic in nature, as "scorbutic fever," or

regarded merely as a condition grafted upon the nutritional disturbance.

A phenomenon which might seem to argue for its essential scorbutic

character is the sharp subsidence on giving antiscorbutic food. On the

other hand, this may quite as well be interpreted as due to a secondary

reaction, checking the absorption of toxins or bacteria. High

temperatures--for example, fever of 103° or over--are attributable to a

complicating infection and should lead to careful examination for the

source of the trouble; pyelitis should particularly be borne in mind. In

a recent case fever of uncertain origin disappeared following the

transfusion of blood.

We have already considered numerous _complications_ of scurvy, and shall

therefore not go over this ground again. Many of them are due to

hemorrhages or to serous effusions in various parts of the body. Another

large group in adults as well as in infants are the result of infection.

The respiratory tract is particularly susceptible, pneumonia

constituting the most common cause of death. In infants we meet with

frequent attacks of "grippe," widespread occurrence of _nasal

diphtheria_, furunculosis and torpid ulcers of the skin, pyelitis,

otitis, adenitis, etc. We have encountered nasal diphtheria--with

typical bloody mucous discharge--so frequently in connection with

scurvy, that where this local infection occurs among a group of infants

they should be carefully examined for latent or mild scurvy. Aschoff and

Koch recently have laid emphasis on the frequency with which diphtheria

complicated scurvy among adults (soldiers). Dysentery is another

complication resulting from an invasion of bacteria. Local infections

occur more often in adults than in infants--cervical adenitis following

gingival pyorrhoea, "bubo" of the groin following infection of the

lower extremity, abscess of the calf of the leg following hemorrhage

into this region.

Scurvy sometimes occurs in _epidemic form_, especially in the army, but

also, as in Russia, among the civilian population. This results when a

large group of individuals have been maintained on a limited and

inadequate ration, and especially where this nutritional condition is

complicated by intercurrent infection. It should not be interpreted as

evidence of the bacterial origin of scurvy. A few years ago the author

reported an epidemic of scurvy in connection with an outbreak of grippe

in an infant asylum. Twelve infants in one ward were affected. The signs

were atypical--an undue degree of hemorrhage occurring at atypical sites

(Table 5). It will be noted from the table that the ages of the infants,

the distribution of the hemorrhages, the development of signs (in some

instances) in spite of antiscorbutic treatment, the sharply defined

epidemic character, distinguish these cases from the scurvy commonly

seen. This is an instance where latent scurvy was prematurely changed to

acute scurvy by an intercurrent ward infection; an epidemic of grippe

precipitated a pseudo-epidemic of scurvy. It is important, especially

for army surgeons, to bear in mind that where latent scurvy exists a

bacterial invasion will lend the disorder a hemorrhagic character. This

has been noted during the recent war in connection with typhus fever on

the Eastern front, and was remarked upon during the Crimean War and our

War of the Rebellion. Some years ago Wherry made a similar observation

in the course of experiments with the plague bacillus--guinea-pigs fed

on a cereal diet developed far more hemorrhages subsequent to infection

than those which received cabbage in addition.

TABLE 5

DATA OF EPIDEMIC OF SCURVY

====================================================================

|Mos| _oz._| rhages | | |

-----+---+------+----------+-------+--------------+-----------------

1. | 6½| 9 6|Humerus, |Apr. 19|Breast milk |Grippe since end

2. | 5| 12 14|External |May 4|Pasteurized |Twitchings and

L. S.| | | ear, | | milk formula;| convulsions;

3. | 10| 12 8|Femur |Apr. 19|Pasteurized |Grippe end of

4. | 5| 7 |Both ears;|Apr. 29|Breast milk |Grippe through-

5. | 10| 15 13|Ear and |Apr. 27|Pasteurized |Two teeth;

6. | 4| 8 4|Tibia |May 8|Pasteurized |Grippe end of

7. | 2| 6 4|Abdomen |Mar. 7|Breast milk |Grippe; probable

--------------------------------------------------------------------

DIAGNOSIS

A correct and early _diagnosis_ of scurvy is the more important in view

of the fact that we possess a specific remedy, and that the disorder is

not self-limited. Recognition generally presents little difficulties for

those who have seen cases, but is a stumbling block where the

symptomatology has been gleaned merely from the textbooks. It has been

our experience that medical students who were conversant with scurvy

from a theoretical standpoint failed to diagnose a case presented to

them in the clinic. Where diagnosis is uncertain, the most important aid

is an exact knowledge of the previous diet, and observation of the

reaction of the patient to antiscorbutic treatment. These diagnostic

points should be constantly remembered in relation to the discussion

which follows, and will not be reiterated in the differentiation of

scurvy from the various other diseases.

The scurvy of adults and of infants are very similar. The main

difference is the subjective symptoms in the adult--pains in various

parts of the body--and the fact that the gums are frequently the site of

infection and ulceration, as well as of hemorrhage. It might be thought

that when scurvy occurs in epidemic form it would be readily

recognized, but experience shows that for months it may permeate the

ranks of troops or the inmates of almshouses, and pass as rheumatism.

This is the cardinal diagnostic error in adult as well as in infantile

scurvy--time and again, and in spite of urgent and repeated warnings,

patients continue to be treated for _rheumatism_. Holt writes: "In fully

four-fifths of the cases which have come to my own notice this

(rheumatism) has been the previous diagnosis." Such has been our

experience. The diagnosis should not be difficult. In sporadic cases,

the individual has limited his diet usually on account of indigestion,

or diarrhoea, or following some dietetic whim or medical advice given

months previously. Where scurvy occurs _en masse_ it may follow an

inability to obtain fresh food--as during war, on shipboard, in the

Tropics or in the Arctic regions--or be the result of a misplaced

reliance on some article of food--for example, dehydrated vegetables.

More careful investigation will disclose that the "rheumatic" pains and

tenderness are not in the joints but in the muscles and tendons. The

calf muscles are frequently painful and excessively tender and somewhat

swollen or infiltrated; the hamstring tendons or the tendo Achilles may

be sore and slightly swollen and the site of slight subcutaneous

hemorrhages. In some cases there is bone tenderness, pain on percussion

of the shins or of the sternum. These signs may be accompanied by, or

even precede, hemorrhages in the gums. The diagnosis of rheumatism in

infants indicates a lack of knowledge of pediatrics, as this disease is

hardly ever encountered in babies under a year and a half of age.

It is not always easy to differentiate scurvy from _purpura_. In either

disease the gums may be hemorrhagic and swollen, there may be scattered

subcutaneous hemorrhages and pains in the limbs. Occasionally, as in a

case seen a few years ago, we may be forced to resort to the dietetic

test. A close inquiry into the previous diet, however, a history of

previous attacks of purpura, the number, and especially the large size,

of the subcutaneous hemorrhages, and above all, the diminished number of

blood-platelets, should make diagnosis possible. In scurvy the platelets

are almost always over 300,000 per cubic millimetre, whereas in purpura

they are reduced to less than 200,000.

In the army it may be difficult to distinguish scurvy from _beriberi_,

especially if they occur side by side as in the recent English campaign

in Mesopotamia. There may be a combination of the two diseases, a

picture similar to ship-beriberi, regarded by Nocht as a hybrid of these

diseases. The diagnosis is rendered more difficult, as at times scurvy

is associated with signs of neuritis. We shall have to depend on the

involvement of the gums and the hemorrhages in scurvy, and on the

hyperæsthesia, paræsthesia, and anæsthesia in beriberi; marked edema

points to the latter disease.

We have thus far had in mind frank and outspoken cases of scurvy. When

we come to consider latent or early cases, the diagnosis is more

difficult and may have to be merely tentative. All that need be added,

in view of the clinical picture sketched above, is that this condition

should not be forgotten in treating adults who have malaise and

indefinite "rheumatic" pains and, more particularly, in relation to

infants who fail to gain, whose appetite is capricious, whose

disposition has become fretful and who have developed the sallow

scorbutic complexion. This warning is particularly opportune at present

in the United States, where pasteurized milk is fed so extensively to

infants, and an antiscorbutic food is not always given.

In addition to the symptoms just enumerated, tenderness of the bones,

especially of the distal ends of the femora, should be sought for, the

urine should be examined carefully for red blood-cells, and perhaps the

ends of the long bones radiographed for "the white line" of Fraenkel.

The experience of Comby with infantile scurvy is illuminating. Among the

fifty-five cases which he has seen, the diagnosis was erroneous in

forty-five, and among thirteen cases recently met with, the physician

failed to recognize the disorder in all but two. The infants had been

given sodium salicylate, had been treated with electricity and massage

for the supposed acute poliomyelitis, or given mercury for syphilis, or

incisions or trephining had been carried out for acute osteomyelitis.

Some had been put into plaster casts for coxalgia or for Potts' disease.

This experience requires little comment. It should be added, however,

that in the course of an epidemic of poliomyelitis, such mistakes are

apt to happen, and, to our knowledge, did occur in the recent epidemic.

In regard to "_the pseudo-paralysis" of congenital syphilis_ diagnosed

as scurvy, it should be remembered that this lesion occurs almost always

before the fifth month of life. A history of previous papular eruption,

the bilateral enlargement of the epitrochlear glands, and the Wassermann

test should suffice to establish the correct diagnosis.

Besides the clinical conditions enumerated above, we may add the

following, which have been confused with scurvy: Neuritis, hemorrhagic

nephritis, calculus of the urinary tract, renal tumor, orbital tumor,

appendicitis, peritonitis, pleurisy and pneumonia. Holt writes as

follows: "I have known two cases to be operated upon by eminent

surgeons, once with a diagnosis of sarcoma and once of ostitis of both

tibiæ. Not until the subperiosteal hemorrhages and epiphyseal

separations were discovered was the nature of the trouble suspected."

Recently we saw a case of fracture of the distal end of the femur

diagnosed as infantile scurvy; the baby had caught its thigh between the

bars of the crib and snapped the bone in trying to extricate itself.

Finkelstein adds acute endocarditis, hemorrhagic septicæmia with

multiple bone swellings, and leukæmia as having been confused with

scurvy.

In an interesting account of scurvy in the Russian army during the

recent war, Hoerschelmann states that tired soldiers at times feign

scurvy. They produce a "pseudo-scurvy" by means of scratching the gums

with their nails or rubbing them with tobacco, and at the same time

bring about hemorrhages of the skin by means of trauma.

Scurvy in the breast-fed infant has been fully considered under

etiology. We wish merely to state again that one cannot be too cautious

in venturing this diagnosis in a nursing baby. The great majority of

reported cases are not scurvy, but bacterial infections, syphilis, or

various forms of intoxication. In establishing a diagnosis the same

principles hold as in the case of bottle-fed infants.

The most important consideration in the diagnosis of scurvy is to keep

in mind the heterogeneous character of its symptoms, and the manifold

diseases with which it may be confused. Surgeons should be alert to this

danger when about to perform operations for osteomyelitis or bone

tumor. The mistakes occur because cases are infrequently seen and

because the signs, being dependent largely upon hemorrhage, occur in

such varied locations of the body. Where diagnosis cannot be made from

the signs or symptoms, the most important aid is a thorough acquaintance

with the previous diet of the individual and observation of his reaction

to antiscorbutic treatment.