Scurvy Past and Present Part 10

The field of antis...o...b..tic foodstuffs is one which is fertile for future investigation. The antis...o...b..tic status of milk heated to various heights of temperature and subjected to various degrees of aging furnishes problems of great practical importance. A lack of growth has been noted when antis...o...b..tics are given which have been subjected to a high degree of heat (_e.g._, autoclaved orange juice). Whether this is due to the destruction of some other growth factor is a question which has been raised by several experimental studies and requires an answer.

It is quite possible that we shall find useful antis...o...b..tic foods which at present are unknown or unappreciated. The recent introduction of the swede, of the canned tomato and of germinated pulses suggests and even renders this probable. On the other hand, the recognition of the comparative poverty of the antis...o...b..tic factor in lime juice shows the importance of putting each foodstuff to the experimental test.

CHAPTER VII

SYMPTOMATOLOGY AND DIAGNOSIS

The ident.i.ty 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 pa.s.sive, 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 const.i.tutes 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 appet.i.te 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 a.s.sociated 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 b.l.o.o.d.y 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 s.p.a.ce 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 a.s.sociated 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 l.u.s.tre. 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 a.s.sume 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 antis...o...b..tics. 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 cla.s.sified as a hemorrhagic disease. Besides the bleeding into the gums, skin and bones, hemorrhage into the stomach may take place, giving rise to haematemesis, 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, a.s.sume a hemorrhagic character when occurring in a s...o...b..tic 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 s...o...b..tic 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 "s...o...b..tic 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 r.e.t.a.r.ded.

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 caec.u.m 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, s...o...b..tic 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 cla.s.sic 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 appet.i.te 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 papillae 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 alb.u.men 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 const.i.tute a rigid ent.i.ty, 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 11 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 antis...o...b..tic. Autolyzed yeast had been tried as a prophylactic antis...o...b..tic, but failed to bring about a gain. When, however, orange juice was subst.i.tuted for the yeast, a prompt growth-reaction resulted, a gain of 1 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, alb.u.min 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.

[Ill.u.s.tration: FIG. 15.--Chart of I. F., aged 7 months, showing a prompt effect on pulse, respiration and temperature of subst.i.tution 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 antis...o...b..tic 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 quant.i.ty was not large, considered in conjunction with the pallor and poor appet.i.te, 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 6 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 pota.s.sium 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 pa.s.ses 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 pa.s.s 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 antis...o...b..tic 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 ma.s.s, 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 ma.s.s 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 haematuria 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 sc.r.a.ped 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.