Scorbutus (Scurvy)

SCORBUTUS 233 velop it completely in a cotton jacket and then surround it with hotwater bags or bottles. The general management should be much the same as described in the chapter on Malnutrition. They require no drugs, but a great deal of careful nursing. CHAPTER VI. DISEASES DUE TO FAULTY NUTRITION. The diseases due to faulty nutrition are numerous. There are two, however, which have been so clearly shown to originate in this way that they may be put in a class by themselves. These are scorbutus and rickets. The prevailing opinion of the medical profession is that both of these are essentially " food-diseases." The purpose of considering them in connection with the disturbances of nutrition is to emphasise this relationship. SCORBUTUS {Scurvy). Scorbutus is a constitutional disease due to some prolonged error in diet. It is characterised by spongy, bleeding gums, swellings and ecchymoses about the joints, especially the knee and ankle, ha3morrhages from the nose, and occasionally from other mucous membranes, extreme hyperesthesia, and often pseudo-paralysis of the lower extremities. Added to these local symptoms there is in advanced cases a general cachexia with marked ansemia. While scorbutus and rickets are very frequently associated, they can not be considered as different forms of the same disease. Cases of scorbutus were, however, described in older writings under the title of Acute Rickets. Scurvy was well recognised and graphically described by Glisson as long ago as the middle of the seventeenth century. For our modern knowledge of the pathology of this disease we are indebted to the observations of Barlow and Cheadle. On the continent of Europe scurvy is most frequently known as Barlow's disease. For the statistical matter here presented I am indebted to the report of the American Paediatric Society's Collective Investigation of Infantile Scurvy in 1898, embracing 379 cases, reported by 138 observers. Of these, 31 cases were from my own practice. Etiology. — Age is an important factor; more than four-fifths of the cases occur between the sixth and the fifteenth months, and half of them between the seventh and the tenth months. Scurvy has been seen in infants under a month old. The great majority of the cases reported have been observed in private practice, often in the best surroundings. Previous disease is not a factor of much importance. Most of the chil- 234 NUTRITION. dren attacked have been in good health up to the development of scurvy. In about one-fourth of the number some previous derangement of the digestive tract has existed. The only etiological factor yet known to bear any constant relation to the production of scurvy is diet. The important facts regarding the previous diet brought out by the Society's investigation are as follows : ' Breast-milk in 12 cases; alone in 10. Previous food ^ Raw cow's milk Pasteurised milk Condensed milk Sterilised milk ^ Proprietary infant-foods 5 " " " 4. 20 " " " 16. 60 " " " 32. 107 " " " 68. 214 " This table shows that while scurvy may occasionally develop with almost any variety of food, three stand out prominently — viz., proprietary infant-foods, condensed milk, and sterilised milk. In all of these it would appear that something needed for normal healthy nutrition is wanting. Scurvy is not likely to follow unless an improper diet is continued for a long period, usually several months. In some instances where it developed in nursing infants, the nurse's milk has been examined and found totally inadequate to the needs of nutrition, many of the children having exhibited serious disturbances of nutrition before any signs of scurvy appeared. In several of the cases reported as occurring with a diet of raw or pasteurised milk it seems certain that the milk formula used was at fault, the most common error in those I have seen being low protein. Several cases have come under my personal observation where children had been kept for four or five months upon percentageswhich should have been continued only a few weeks. However, I have seen at least three cases of scurvy which developed while taking pasteurised milk where the percentages employed could hardly have been the explanation, and the heating (167° F. for thirty minutes) seemed to be the cause. However, I believe scurvy to be an exceedingly rare result of the pasteurisation of milk, 80 rare, indeed, as not to be weighed against its immense advantages. With the lower temperature now generally employed (155° F.) it need not be feared. The number of cases occurring while upon a diet of sterilised milk (usually heated to 212°' F. for one hour) is so large that we are driven to the conclusion that the heating alone was the cause, especially since prompt recovery has frequently followed when no other change was made than to discontinue the heating. These facts show that sterilised milk should not be continued as the sole diet for long periods — i. e., for several months — and that its possible danger should be kept in mind. No one fact in the etiology of scurvy is better established than its development after the prolonged use of condensed milk or the propri- SCORBUTUS. 235 etary infant-foods. Scurvy occurs not only when the foods are used with condensed or with sterilised milk, but also, though less frequently, with fresh milk. The inference is that these preparations cause scurvy not only by what they lack, but possibly by something wliieb they contain. Some have ascribed the results to the ferments present. This view has some support in the occurrence of scurvy after the prolonged use of peptonised milk, an infrequent but a well-established fact. In this respect, as with reference to sterilised milk, my personal experience, including now nearly one hundred cases of scurvy, coincides with the findings of the Society's report. While it may be regarded as established that the cause of scurvy is dietetic, no single dietetic error can be held responsible for the disease. None of the theories yet advanced in explanation of how diet causes scurvy is wholly satisfactory. Lesions. — The most marked effects of scurvy are seen in the bones, blood-vessels, and the blood. The number of recorded autopsies is not large, only six being included in the Society's report. I have myself had the opportunity of making examinations in three cases. The findings are remarkably uniform, but represent, of course, the extreme results of the disease. The most striking lesion is subperiosteal haemorrhage, which is practically constant and may occur almost anywhere in the body, but affects chiefly the bones of tlie lower extremities; it is often very extensive, and may reach from the knee to the great trochanter, or from the ankle nearly to tlie knee. Extravasations may also be found between the muscles, and blood may infiltrate the cellular tissue in the neighbourhood of the joints. Besides these lesions resulting from haemorrhagic periostitis the bone itself may be affected. Separation of the epiphysis from the shaft of some of the long bones, generally at the lower end of the femur or lower end of the tibia, is found in most of the fatal cases. Notwithstanding the serious lesions near the large joints, the joints themselves are usually normal. The minute bone changes are somewhat similar to those of rickets. But there are also differences of importance. The disposition to haemorrhage, which is altogether the most characteristic feature of scurvy, is entirely wanting in rickets. The visceral lesions are inconstant. Those most frequently found are small haemorrhages beneath the pleura, pericardium, and peritonaeum, sometimes into the various organs, also broncho-pneumonia, and nephritis. There may be small extravasations found upon the surface of any of the mucous membranes. The alterations in the blood-vessels are undoubtedly an important factor in bringing about the disposition to haemorrhage, but as yet they have been very imperfectly studied. The changes in the blood, in the gums, and the lesions of the skin will be considered with the symptoms. 236 . • NUTRITION. Symptoms. — In many cases a period of indisposition, fretfulness, pallor, and failing nutrition precedes the local symptoms, but usually tenderness of the legs is the first symptom noticed. In the beginning this is occasional and so slight as to cause the infant to cry only upon being handled. Later it becomes almost constant and is very acute. At first this soreness is not very definitely localised, but is generally more marked about the knees and ankles. Some swelling may be noticed, often just above the ankle-joints. Coincident with these may be seen the changes in the mouth. The gums are of a deep purplish colour, swollen, particularly about the upper central incisors, and may quite cover the teeth. They bleed from the slightest irritation, and sometimes spontaneously. The child now becomes fretful and cross, sleeps badly, loses colour, weight, and appetite. He may become quite cachectic in appearance. All these symptoms come on very gradually, often with periods of a few days in which apparent improvement is seen. Sometimes they may continue for several weeks without making any perceptible impression upon the child's previously good condition. Fig. 33. — Scuuvy Showing Characteristic Swellings and Posture. Patient 8i months old, fed exclusively upon malted milk after the age of 3 months. Epiphyseal separation at the upper extremity of both humeri, lower extremity of both femora and lower extremity of left tibia. Prompt and complete recovery. If the disease is recognised, and proper treatment instituted, rapid improvement follows, with complete and permanent recovery. If not recognised, and the faulty diet is continued, the disease advances to the more severe form. The tenderness of the legs becomes exquisite, so that any movement or even the slightest touch causes the child to scream with pain or apprehension. The posture is very characteristic. There is semiflexion of thighs and legs and outward rotation at the hip. (See Fig. 33.) In this position the child often lies motionless and voluntary movements of the extremities can not be excited. Paralysis is often suspected. The disability is chiefly owing to the extreme pain which motion provokes, but may depend upon epiphyseal separation. Small ecehymoses are frequently seen about any of the large joints, resembling SCORBUTUS. 237 the ordinary " black-and-blue " spots, and these often confirm the opinion previously formed triat the chikl has met with some accident. The swelling near the joints, particularly the knee, may be so great that the limb is nearly twice the size of its fellow. The mouth symptoms are usually striking. In addition to spongy, swollen, bleeding gums, dark purplish bags may be seen over teeth not yet through. There may be bleeding from the roof of the mouth or from the pharynx. The pain is sometimes so severe as seriously to interfere with taking food ; there is moderate though rarely extreme salivation. Blood may be vomited or passed with the fasces or the urine. In the severe cases the stools are rarely normal, more or less catarrhal colitis usually being present. The general condition is one of grave anaemia, accompanied by a marked cachexia and progressive wasting. The child cries almost constantly, sleeps little, and is truly a pitiable object. Slight fever is often present. Unless recognised and the cause removed, the condition grows steadily worse, the symptoms continuing until death occurs either by a slow asthenia, or suddenly from heart failure, or from some intercurrent disease, sucli as broncho-pneumonia or acute gastro-enteritis. The duration of the illness in the fatal cases is from two to four months. The onset is gradual in the great majority of the cases, the earliest symptoms noticed in the order of frequency being pain and tenderness of the legs, soreness and sponginess of the gums, disability, anaemia, cutaneous haemorrhages, and very rarely haematuria. Pain and tenderness are very prominent, being noted in 95 per cent of the Society's cases ; in the majority they were present only on motion or handling. The location of the pain and tenderness in 184 cases was as follows: Lower extremities alone, 133; upper extremities alone, 2; lower and upper, 42; lower and trunk, 7. In all but two cases, therefore, the lower extremities were affected, the lower part of the thigh and the leg just above the ankle being the usual seat. Disability, or pseudo-paralysis, is a very common symptom, and in all severe cases a constant one. It exists in varying degrees from a slight disinclination to use the limb to complete helplessness. In many cases it is more marked than the pain, and has led to a diagnosis of poliomyelitis. Swellings are associated with pain and tenderness in most of the severe cases. They are most marked near the joints, but may extend for some distance along the shafts of the bones. In nearly all cases the location is the lower part of the thigh or the lower part of the leg, and usually of both sides. Swellings are occasionally seen near the wrists, elbows, shoulders, and hip-joints; in rare cases, over the ribs, scapula, or ilium. Eedness is not generally present, but the parts may have a dark purplish colour. It is to the haemorrhages that both the swellings and the discoloration are chiefly due. 238 NUTRITION. Protrusion of the eyeball is present in about ten per cent of the cases; an extreme exophthalmus is sometimes seen, and is due to orbital haemorrhage. The gums are affected in nearly all cases, the exceptions being those recognised and treated early. HaBmorrhage occurs in about one-half the cases, and frequently there is ulceration not unlike that of a mercurial stomatitis. It is rather curious that, though the lower teeth are cut first, the upper gum is almost always most affected, and in the milder cases usually alone involved. Of 45 cases in which no teeth had been cut, the gums were affected in 24 and iiornial in "31. This is sufficient to disprove the old opinion that the gums are affected only when teeth have appeared. The severe inflammation and ulceration sometimes seen seem to be the result of secondary infection. Haemorrhages beneath the skin are present in about half the cases. They are rarely extensive, usually multiple, and their location is no doubt often determined by a sliglit traumatism. Haemorrhages from the mucous membranes are not quite so frequent. There may be bleeding from the gums, nose, bowels, kidneys, and rarely from the stomach. Haemorrhages in most cases are frequently repeated, but seldom profuse. Epiphyseal separation is seen only in very severe cases. It is most frequently either of the lower epiphysis of the femur or the tibia, or the upper epiphysis of the humerus, and is often bilateral. The actual separation may be caused by some slight injury, the condition of the bone predisposing to this occurrence. In three cases of my own with separation which recovered, rapid union occurred under anti-scorbutic treatment. Anaemia is slight in the early stage, but increases as the disease progresses. Blood examinations may show great reduction of the haemoglobin, sometimes to thirty-five or forty per cent; also in nearly all cases a proportionate reduction of the red cells. The changes are those of an ordinary secondary anaemia. The urine contains albumin in one-fourth of the cases ; in nearly half of those containing albumin casts also are found. In rare cases haematuria has been an early symptom. Blood cells usually in moderate numbers are found in practically all but the mildest cases, and are of some diagnostic importance. Evidences of general malnutrition are present in all advanced cases, varying, of course, greatly in degree. In a few infants under my own observation the weight, colour, and general appearance of health have continued in spite of very decided local symptoms. In most of them the impaired nutrition is shown by loss of appetite, occasional attacks of vomiting, and still more frequently by derangements of the bowels, which vary from slight indigestion to a serious catarrhal condition of SCORBUTUS. 239 both small and large intestine. It is with tlie latter that the discharge of blood is usually seen. Associatioii with Rickets. — In the Society's investigation great pains were taken to obtain definite and accurate data regarding this. Of the cases, 340 in number, in which this point was noted, symptoms of rickets were present in 1-52, or 45 per cent; these symptoms were recorded as slight in 72; marked in 64; and not specified in 16. In the remainder of the cases, 55 per cent, it is definitely stated that symptoms of rickets were absent. It is also stated that in 50 of t\^e patients which were rachitic, the rickets antedated the development of the scurvy. From these facts it would seem to ])e pretty well established that though rickets and scurvy have points of resemljlance, such as the age when they are seen, bony changes, dependence on defective nutrition, etc., they can not be regarded as different forms of the same disease. The two most striking characteristics of scurvy — viz., tendency to haemorrhages and prompt curability by fresh food and fruit juices — have no counterpart in rickets. However, their co-existence in the same patient is of common occurrence. Diagnosis. — The disease w^ith which infantile scurv}' is most frequently confounded is rheumatism. In fully four-fifths of the cases which have come to my own notice this has been the previous diagnosis. The extreme rarity of rheumatisjn under one year should always make one cautious; pain and tenderness of the legs only, should, in an infant, invariably suggest scurvy rather than rheumatism. The extreme disability has often led to a diagnosis of poliomyelitis, l)ut here again the acute tenderness should set one right. Many cases of scurvy come into the hands of the orthopaedic surgeon with a diagnosis of joint or spinal disease. Where the swelling was mainly of one limb I have twice known a diagnosis of malignant disease to be made, from the cachexia, the shape of the swelling, the discoloration, and the pain. I have known two cases to be operated upon by eminent surgeons, once with a diagnosis of sarcoma and once of ostitis of both tibiae. Not until the subperiosteal haemorrhages and epiphyseal separation were discovered was the nature of the trouble suspected. The diagnosis of scurvy seldom presents any difficulties to one who has once seen a case. No one need err if the essential features of the disease are kept in mind : the extreme soreness of the legs, spongy, swollen gums, swelling near the large joints, a tendency to haemorrhages, and usually a history of the prolonged use of some proprietary infant food, or sterilised or condensed milk. The epiphysitis of hereditary syphilis has many symptoms in common with scurvy, but it usually occurs at an earlier age (before the fifth month) and other evidences of syphilis are usually present. If any doubt exists, this will be removed by the prompt improvement and generally rapid cure following an anti-scorbutic diet. 240 NUTRITION. Prognosis. — This is invariably good if the disease is recognised early. No patients with symptoms so serious improve with such marvellous rapidity as do the great majority of those witii scurvy, under proper management. The figures of the Society's report on this point are interesting. The average duration of the disease before treatment was begun in over three hundred cases was somewhat over three weeks. In eighty per cent striking improvement was noticed during the first week of treatment, and in forty per cent within three days. Over two-thirds of these cases were well within three weeks, and nearly one-third within one week, after the beginning of treatment. It is only when the disease is of long standing, wlien the malnutrition is severe, or when serious complications, usually involving the digestive tract, are present that the symptoms persist and the issue becomes doubtful. It is difficult to tell what the exact mortality of scurvy is. Any case allowed to go on may result fatally. The younger the infant the more likely is this to occur. I have seen four deaths in nearly one hundred cases. In one of my patients death resulted from haemorrhage which followed an incision into an epiphyseal swelling at the lower end of the femur, made before I saw the patient, and which persisted despite all treatment. Barlow's early article included thirtyone cases with seven deaths. It is rare that scurvy leaves any permanent effects. Recovery is not only rapid but complete. Relapses are extremely rare and have been observed only in one or two cases, where chronic indigestion existed of so extreme a character that proper feeding was impossible. The after-effects are usually the result of prolonged malnutrition, of which the attack of scurvy w^as only one manifestation. Treatment. — This is remarkably simple — viz., to discontinue all proprietary foods, condensed milk or sterilised milk, and to substitute a diet of fresh cow's milk, modified to suit the child's digestion. With this treatment alone improvement will soon begin and gradually complete recovery takes place. However, when fresh fruit juice is added improvement is much more rapid. It should always be combined with the change in diet. Orange juice is to be preferred, but the juice of any fresh ripe fruit will answer the purpose. Oranges should be sweet and fresh. From two to four ounces a day are required, best in divided doses, given about one hour before the milk-feeding. It may be given plain, or diluted with water. In some cases, when not well tolerated by the stomach, it is better given at night, when no food is taken. Potato also has marked anti-scorbutic properties, and may be given in the form of a puree to infants as young as eight or ten months. The only really difficult cases to manage are those in which the general condition approaches one of marasmus, or when scurvy is accompanied by marked gastric or intestinal disturbance. When an intestinal catarrh is present, with the bowels moving five or six times a day, one may hesi- RICKETS. 241 tate to give the fruit juice for fear of increasing these symptoms. In a number of instances I have seen intestinal symptoms, which had resisted ordinary measures, immediately improved by the fruit juice, thus establishing their intimate connection with the scorbutic condition. Other things of value are fresh beef juice, and for older children all fresh vegetables, especially potato. The anaemia and malnutrition call for iron, cod-liver oil, and other tonics, which should be given after active symptoms of the disease have disappeared. Infants with scurvy should be handled as little as possible, and should be particularly protected againt exposure in their extremely susceptible condition. The affected limbs should be immobilised by splints during the period of marked symptoms, always if epiphyseal separation has taken place, and in many other severe cases. RICKETS (Rachitis). Eickets is" a chronic disease of nutrition. While the only important anatomical changes are found in the bones, it is not to be regarded as a bone disease; but as a very complex pathological process, the result of disturbed metabolism, which affects chiefly the bones, but also muscles, ligaments, mucous membranes, and nearly all the organs of the body, particularly the nervous system. It occurs especially between the ages of six and eighteen months. It is not very common in the country, but is exceedingly frequent in most large cities. While not a fatal disease per se, rickets adds very greatly to the danger from all acute diseases in infancy, and even to some degree also to those of later life. Under proper conditions of diet and hygiene it tends to spontaneous recovery. Etiology. — Certain facts in the causation of rickets are well known. It is closely related to improper feeding and bad hygienic surroundings. It is not common in nursing children unless lactation is unduly prolonged,^ as, for example, where nursing is continued for fifteen to eighteen months without other food. Artificially fed children are much more prone to the disease, especially those who are badly fed. The diet in these cases is most frequently deficient in fat, and often at the same time in protein, while it is apt to contain an excess of carbohydrates. It is somewhat difficult to separate the effects which these different factors produce. It appears, however, that the most important factor is the deficiency in fat. Rickets is exceedingly common in children reared upon the proprietary foods, nearly all of which are very low in fat and contain an excess of carbohydrates. It is also common in children who are reared upon sweetened condensed milk, and for precisely ' An exception to this statement must be made in the case of Italian and Negro children. In this class as observed in New York it is not uncommon to see marked rickets in those getting nothing but the breast. 17