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The Celiac Affection in Children (1) - Edinburgh Med. Journ., Vol. XXXV.-NO.IV., Oct. 1889, pp. 321-330
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Scott Adams

In 1994 I was diagnosed with celiac disease, which led me to create Celiac.com in 1995. I created this site for a single purpose: To help as many people as possible with celiac disease get diagnosed so they can begin to live happy, healthy gluten-free lives. Celiac.com was the first site on the Internet dedicated solely to celiac disease, and since then it has become an invaluable resource to people worldwide who seek information about celiac disease and the gluten-free diet.

In 1998 I created The Gluten-Free Mall, Your Special Diet Superstore! which was also another Internet first—it was the first gluten-free food site to offer a shopping cart-style interface, and the ability for people to order gluten-free products manufactured by many different companies at a single Web site.

I am also co-author of the book Cereal Killers, and founder and publisher of Journal of Gluten Sensitivity.

 
By Scott Adams
Published on 07/26/1996
 
The subject of this paper is one which has not been described, to my knowledge, in any of the text-books, either on general medicine or on the diseases of children. As it is one of great importance, and one which is readily overlooked, even by excellent physicians, I have thought that it would be of interest to publish a few of the cases which have come under my own observation of this somewhat uncommon disease. These cases are very similar, and it is therefore unnecessary to burden my paper with more than four, which will serve as examples of all.

By R.A. Gibbons, MD., M.R.C.P., Physician to The Grosvenor Hospital for Women and Children, Physician for the Diseases of Women and Children at the St. George's and St. James's Dispensary.

Footnote: (1) This title is adopted by Dr. Gee, who was the first to direct my attention to the disease, at the Hospital for Sick Children, Great Ormond Street, and I have therefore thought it best to retain it.

The subject of this paper is one which has not been described, to my knowledge, in any of the text-books, either on general medicine or on the diseases of children. As it is one of great importance, and one which is readily overlooked, even by excellent physicians, I have thought that it would be of interest to publish a few of the cases which have come under my own observation of this somewhat uncommon disease. These cases are very similar, and it is therefore unnecessary to burden my paper with more than four, which will serve as examples of all.

The name "coeliac" is derived from the Greek Koilia, which means belly. The "coeliac flux" is a well-known and o1d expression signifying diarrhea, in which the undigested food is passed from the bowel. The name of the affection under consideration does not necessarily imply diarrhea, and is taken for want of a better one; it means merely that the disease pertains to the abdomen. It has, however, the merit of not conveying anything actually erroneous. The disease is not distinctly a diarrhea although undoubtedly children suffering from this affection are liable to attacks of diarrhea. In the latter affection, if the larger bowel is affected, we get dysenteric symptoms; if the smaller bowel is the seat of the trouble, it is generally considered that we have three different conditions affecting it, namely, increased movement, increased secretions, or a diseased condition of the mucous membrane. The coeliac affection corresponds with neither of these conditions, as will presently be shown.

The following cases are epitomized from notes taken of some of the patients who have been under my care in private practice:

Case I -- J.F., a boy, aged 2 years, was first seen on the 26th of April 1883 (1)

He was lying in his nurse's lap, looking flushed, and complaining of pain over the abdomen. He had been languid and vomiting during the morning. I ascertained that the motions had been copious, frothy, and offensive. He had been latterly losing flesh. As to the previous history, I learned that the child had been brought up by hand, and there was nothing of importance to relate until last December, since which time he had been out of health, and seemed to be gradually getting worse. In that month they had sought advice, and he had caused his parents anxiety ever since, sometimes being a little better, at other times worse. On examination I found that the temperature was over 99, the pulse 108; but no definite disease could be made out. The examination of the chest yielded nothing. The abdomen was soft and doughy, but painful to the touch, and he cried out when any attempt was made to palpate. The spleen and liver were not enlarged, and I could detect no enlarged lymphatic glands. The flesh was soft and flabby. As he was having rather frequent actions of the bowels, a mixture of bismuth and compound tincture of camphor was ordered.

His diet consisted of cream, with two or three times as much water or scalded whey. For a drink at breakfast, at 11 o'clock in the morning, and at bedtime, whey made wit essence of rennet; if mixed with cream, it was to be scalded. Pounded raw meat prepared in the ordinary way, and rubbed through a sieve; of this one, two, or three tablespoonfuls to be taken for dinner, with a little sifted sugar or fruit jelly. A tablespoonful of mashed floury potato. Rusks, plain biscuits, or toast with fresh butter; Savory & Moore's food made with whey, and a teaspoonful of cream added. Half a pint of asses' milk in the morning, and the same quantity- in the evening. One or two teaspoonfuls of raw meat in the after part of the day. The following day I carefully examined some motion microscopically, and could detect no pus and nothing extraordinary.

Family History:-- The mother was a healthy woman. She had had no miscarriage.

She had no history of any important disease. There was no trace of tubercle on her side. Her mother suffered occasionally from gout. The child's father appeared a healthy man; he occasionally passed free uric acid, and had symptoms rather like those of renal calculus. There was no history of syphilis obtainable, and no reason to suspect it. There were two other children, a boy and girl, older than this patient. The girl had suffered from suppuration of the submaxillary glands.

The child improved considerably until 5th July, when he had an attack of watery- diarrhea, which prostrated him. The motions were extremely offensive. Dr. Gee saw him with me, and suggested, instead of the compound kino powder, which he was taking, that he should have minute doses of mercury and chalk every two hours; forty-eight hours later the diarrhea had stopped. Without going further into detail, it may be stated that this attack nearly proved fatal. He, however, gradually recovered, the first favorable sign being that the motion contained some solid material. There was no change in the diet, except that the meat was omitted and Brand's essence substituted.

The following month left London for the seaside. He occasionally had attacks of diarrhea there. Six months afterwards I again saw him, when he seemed in excellent spirits, looked fat, and, although flabby, ran about and played with the other children with apparent enjoyment.

This boy died of acute purpura haemorrhagica, the details of which illness I have already published in the Medical Times and Gazette for 1885.

Case II -- S.H., aged 2 years and 8 months. First seen on 10th May 1885

Her condition is as follows:-- She is very anaemic in appearance. She is not thin or wasted, but her flesh is flabby, and her legs and arms, though apparently well covered with flesh, are soft to the touch. Nothing is to be discovered on examination of the chest, the heart sounds being normal, and the lungs acting well. On palpation of the abdomen, it is found to be doughy, blown out with flatulence, and looking very large. The liver can be felt about one finger's breadth below the edge of the ribs, and the spleen is slightly enlarged. No enlarged lymphatic glands can be made out anywhere. She walks with difficulty, and has a great disinclination to go upstairs. A very little exertion makes her tired, and break out into a profuse perspiration, especially about the head. Her appetite is poor, and it is with great difficulty that she can be coaxed to eat. Her motions are light in color, frothy, and intensely offensive. Microscopically they contain nothing abnormal. Sometimes the actions vary from one to three in the twenty-four hours. The urine contains no albumen, sugar, or bile.

Previous History:-- With the exception of an acute attack of laryngitis, there is nothing to report about her former health. She was nursed as a baby by her mother. She first began to complain in July last, but as the weather was very hot, it was thought that she was suffering merely from the effects of the heat. At that time her motions were clay colored, and she was very pale and languid, with occasional attacks of diarrhea; but in the beginning not much attention was paid to these symptoms. Soon after, becoming worse, advice was sought concerning her; and in a letter to me from her medical attendant, it was stated that the saw her for what he considered were symptoms of mesenteric disease, but that she greatly improved under treatment. She soon afterwards relapsed, and owing to the extreme
debility and pallor, her case was considered hopeless.

Family History:-- Her mother is perfectly healthy, with no history of any importance. Her father is at present healthy; he suffered some years ago from abscess of the hip joint. From this he perfectly recovered, and is in excellent health. The other children, three sisters, are all healthy.


Treatment:-- She was placed upon a diet exactly similar to the other cases related, but in her case it seemed necessary, on account of exhaustion, to give as an addition brandy once or twice a day. Attacks of diarrhea were treated as they arose, but the medicine she principally took was iron in some form. Cod-liver oil could never be borne; it invariably caused looseness of the bowels. At one time she developed purpuric patches all over the lower part of the abdomen, which, however gradually disappeared; and she also had an attack of stomatitis, which lasted for about ten days. She was under observation for a considerable time during each year, and I constantly heard about her when she was not in London. The diet and general treatment were carefully attended to, and she was allowed to have chicken, eggs, tapioca, milk, and everything of a plain description. when I last heard, her condition was most satisfactory, and she had gained considerably in weight.

Case III -- E.H.W., aged 2 years and 1 month. Her mother came to consult me about her on the 10th of May, 1887, complaining that the child's digestion was out of order, and the first thing she noticed was that the motions were offensive and copious.

Previous History:-- The mother declared that she considered the child had not been thoroughly well ever since she was born. She was only suckled a fortnight, after which she had cows' milk and water, then donkeys' milk for a time, and then again cows' milk.

Family History:-- The patient is an only child. The mother is a healthy woman; has never suffered from any miscarriage; there is no history of tubercle. There is no history of syphilis obtainable, and no reason to suspect it.


The child has always been nervous and easily frightened. For several months past she has been dieted by the mother. Sometimes she would give her a little meat, and sometimes not. She often has attacks of sickness; the least thing causes her to vomit, and the food is very easily brought back in an undigested state. The motions are very large in quantity, pale in colour, and extremely offensive. On examination the child is found to be generally flabby, and does not look strong; she is pallid, but not thin, and there is no yellow tint of the skin. On examination of the chest, the right side is seen to be slightly flattened anteriorly, and the percussion note is of a higher pitch as compared with the left, probably from thickened pleura. The breath sounds are natural; the heart seems normal. The liver is one finger's breadth below the ribs. The spleen is not enlarged, and there are no enlarged lymphatic glands to be discovered. On examination of the blood, there is observed to be an excess of white cells. The red are less colored than is natural, and there are numerous lymphoid particles present. It is spanaemic, the number of cells being greatly diminished. The tongue is white and coated. She was ordered some perchloride of iron and cod-liver oil, with occasionally some decoction of aloes, and the diet was to consist of cream, whey, also asses' milk, raw meat, and potato.


A week later, I saw her on account of pain in the abdomen. Her legs were drawn up and she complained of great pain all over the abdomen. Her medicines were stopped, and I substituted for them a mixture containing bismuth and compound tincture of camphor, under which she improved. On the 11th of June she had an attack of sickness. She vomited frequently, and had three copious loose motions, the last being watery. This attack was probably because there had been some mistake about the milk, the child having had cows' milk instead of asses' milk during the morning.

When I last heard of her from the country, I was told that she seemed very much better and improved in every way. She had, however, to be extremely careful about food, and could not make any change with impugnity, -- in fact, the diet had had to be altered to an ordinary one with the greatest of care.

Case IV -- D.G., aged 2 years and 8 months. First seen on 9th September 1886. Her mother brought her to me, complaining of her not passing water for many hours, sometimes as many as 10 or 12 elapsing without any inclination on the part of the child to relieve herself.

Previous history:-- The child had been brought up by hand, and there is nothing of consequence to report concerning her, with the exception of a severe attack of laryngitis, from which she suffered a year previously. Family History.-- The mother is a delicate-looking woman, thin, and by no means strong. There is no distinct history of phthisis on either side, and there is no syphilitic history obtainable, and no reason to suspect it. There is one other child, older than the patient and perfectly healthy. She also had a severe attack of laryngitis when a baby, but nothing else of consequence.

On examination, she was seen to be a rather delicate-looking child, but apparently well developed, although her flesh was soft and flabby. No signs of rickets could be detected. The abdomen seemed large and doughy. The heart and lungs appeared to be normal. The spleen and liver were not enlarged, and no enlarged lymphatic glands could be felt. The urine proved to be distinctly albuminous and to contain crystals of uric acid. The mother, who had come with her from the country, had not brought the nurse, and could tell me nothing about the motions, or whether the bowels were regular or not. She was given a mixture containing some sulphate of soda and citte of potash. I soon afterwards received a letter telling me that the motions were copious, frothy, whitish, and intensely offensive, and that the bowels acted once or twice daily. On the 26th October I again saw her, and then heard from the mother that the child had been very poorly a fortnight previously, owing to what was considered due to a chill. She told me that the child was seized with what she termed "spasms of the abdomen," that she drew her legs up, and seemed in great pain. This attack of pain apparently lasted of several hours, and the mother states that she was not feverish. At that time the motions were too light in colour; they were very large and extremely offensive. She passed a slight amount of blood with the motion, but the mother is not quite sure whether this was merely from straining at the first, or whether it was mixed with the motion; probably the former would account for it, for I never again heard of the presence of blood. The nurse said the motions were so large in quantity that they quite astonished her, considering the amount of food eaten; that they were intensely offensive, and that there were never more than two in the twenty-four hours, although usually there was but one.

There being no doubt as to the diagnosis in this case, I ordered the same diet as in the previous cases, viz., cream and whey, pounded raw meat with sifted sugar and floury potato, and to have some compound decoction of aloes and cod-liver oil. On the 4th of November I saw her in what her mother described as her "stomach attacks," and I then found that she appeared to have considerable abdominal pain, with the legs drawn up. I therefore omitted the compound decoction of aloe sand the cod-liver oil, and gave a mixture containing some compound tincture of camphor. On a microscopical examination of the motions, it was found that they contained no pus. There were many long, rod-like virbios, and very numerous spores, like the penicilium glaucum.

It may be briefly stated that she was kept upon this diet for many months; that the attacks of pain from which she originally suffered gradually diminished in intensity until they entirely ceased, and that her general condition was greatly improved. The albumen disappeared form the urine; she became more muscular and walked without fatigue, and the motions gradually assumed a natural colour. This improvement, however, did not take place rapidly, but occurred during a period of about two and a half years. When I last heard of her, her condition was satisfactory, and although she could not be considered to be a perfectly robust child, there seemed to be nothing amiss with her. She ran about and played like other children, and seemed well. Her motions have always been natural for months past, an she has gradually returned to ordinary every day diet; but the nurse has been extremely careful in giving her only what has been ordered for her, and she has at all times been cautious not to give her anything which she feared would din the least disagree with her. The child has, therefore, had every possible care regarding her treatment.

The coeliac disease, amongst hospital patients, is popularly called "consumption of the bowels," meaning by this one the several affections to which they apply this term. This disease is characterized by the passage of large, loose, white or whitish, frothy, intensely foetid motions; by pallor of the skin; wasting; loss of muscular strength, and by the fact that it is most frequently met with in children between 1 and 5 years of age. The youngest child I have seen attacked by it was between 10 and 11 months old. I have seen it in children with perfectly healthy parents, and, indeed, with sisters and brothers in every way healthy. In only one family can I trace any history of struma on the side of the father. No history of tubercle can be traced in the family of any of the patients whose cases are related, except one, and in that, consumption occurred in a paternal uncle of the patient, and in all there is no history of syphilis obtainable, and no reason to suspect it. In most cases there is a history to be obtained of gout, either on the side of the father or mother, or in distant relations. The examination of the faeces themselves throws no light upon the matter. In those I have submitted to careful microscopical examination I have never been able to discover anything abnormal, and I have failed to detect any tubercle bacilli although carefully searched for.

Pathology:-- Under this head there is really nothing to be detailed. Examination of the bowel after death has led to the discovery of nothing. There is no sign of wasting of the mucous membrane, of engorgement, or of ulceration, and this statement refers to the whole of the intestinal tract. I am not aware that any wasting of Lieberkuhn's follicles has been detected. The liver and the spleen are not remarkable for anything abnormal. The spleen, however, is occasionally enlarged. The blood usually has signs of anaemia and except for the excess of small lymphoid particles, might pass of ordinary anaemic blood. As, however, these small particles are not peculiar to this affection, they really need hardly to be noticed, for I have myself found them, on many occasions, especially in children suffering empyema. Therefore, taking all the facts into consideration, it is quite clear that up to the present moment nothing has occurred to throw any light upon the pathology of this extraordinary affection.

Diagnosis:-- About this there can be no difficulty whatever, for the signs and symptoms are so clear that they cannot be mistaken. It is, however, of the greatest importance to examine the motions for one's self, for I am certain that if this had been done in several of the cases which have come under my notice, I should not have been the first to discover the child was suffering from anything beyond a little general debility. About this point one cannot be too strong, for, as there are usually no signs of ordinary diarrhea, and possibly but one action a day, the nurse, if not alarmed by the foetor, is apt to think nothing of the bowel, but to be more struck with the pallor and the generally increasing weakness of the child. The signs are so clearly indicative of this one affection, that there is no other with which it could be confounded.

Prognosis:-- Unfortunately this is usually grave, and the majority of children suffering from this disease die. I have, however, with the exception of one, in the cases that have come under my observation, had the good fortune to witness their improvement; and I think it was partly due to the careful nursing that they had in their own homes, and to the fact that they have been the children of people who have been well off, and who have been able to give them everything that has been ordered, regardless of expense. I feel certain that this has a great deal to do with the well-being of the children, for, in one case, the second on the list, I am sure that without the most constant care and watching on the part of the mother and nurse, she would have died. As to the time at which death will take place, it is impossible to say, for, however bad these little patients are, they frequently have extraordinary rallies, and seem better for a time; but, without any warning, relapses take place, and after apparently progressing most favorably, they suddenly become exhausted, and rapidly fade away, usually from attacks of watery diarrhea.

Symptoms and Progress:-- The onset is usually gradual, and there is nothing definite to call attention to the disease. The nurse or the mother generally states that the child has been a little out of health for some weeks, or possibly months, and yet there has been nothing special to cause alarm, with the result that no treatment has been adopted. The child becomes pale and anaemic, the blood evidently being affected, and sometimes there is a puffiness about the face. Possibly amongst the first indications, the motions have been marked by the nurse, and this has generally been owing to the colour being different, but above all, on account of their intensely foetid odor. This mal-odor is sometimes overpowering, and those who have not encountered can hardly realize it. The motions themselves, as already pointed out, are very large, soft, whitish in colour, and frothy. They resemble very much ordinary oatmeal porridge. The actions may be two or three in a day, but usually there is only one large one, and that may take place at a time to which the child is accustomed. Now and then there are attacks of so called diarrhea, that is to say, the child may have frequent evacuations of the bowels, that they may be very loose, and that they may be occasionally watery if the attack is severe, but these attacks of diarrhea are not an essential part of the disease, except in the cases of some children who have these constant attacks of diarrhea throughout the whole of the illness. The appetite is poor, usually capricious, sometimes large. The tongue is frequently coated with a whitish fur, at other times perfectly clean. In some cases there are attacks of crampy abdominal pain, possibly due to ordinary colic. These attacks were well marked in case 4. The abdomen is generally soft and doughy, although occasionally distended and hard. The liver is generally unaffected: now and then the spleen is found to be enlarged: no enlarged lymphatic glands can be detected. As a rule, the urine has nothing remarkable about it; occasionally it contains a trace of albumen. The temper of the child seems variable, most frequently he is extremely irritable, fretful, capricious, or peevish. Nothing seems to please him, and altogether he is quite unlike himself. Sometimes he is heavy and languid, complaining of headache, and wishing to lie down a good deal. The special senses seem to be normal. In all the cases that I have examined the various tendon reflexes were unaffected.

The most remarkable sign about these children is that of loss of muscular power, with apparently no great loss of flesh; that is to say, to look at, the children do not seem to have wasted much, but on feeling the legs or arms, it is at once evident how soft and flabby and out of condition they are. A curious point is that they have a great objection to going upstairs, and in one case, on of the first symptoms which struck the nurse as being extraordinary was that the child did not care to mount the staircase, and that she had invariably to be carried up. The progress of this affection is as a rule slow. The children seem to gradually become more feeble, to waste away, and to lose power. Occasionally an attack of watery diarrhea, in those children who have been accustomed to suffer from diarrhea, may carry off the patient. In the case of colicky attacks I have seen a child in imminent danger from collapse. In non-fatal cases recovery is extremely gradual. An important point to note in this connection, is the tendency to relapses, each relapse taking a considerable time to make up for lost ground. When improvement begins to take place, it is usually shown in the healthier condition of the evacuations. The colour becomes better, the motions are more formed, and above all, less copious and less offensive. The child gains in strength, but is a long time gaining in colour. The muscular strength is the last to return, and mounting the stairs, or jumping, are amongst the very last things that can be done. The appetite generally improves but slowly, and is always capricious, or nearly so. Another important point concerning these patients is that the least thing apparently upsets them, and the smallest error in diet will bring on copious actions of the bowels, cause vomiting, or make the child quite ill again.