Vohmw 93 Number 4

Brief clinical and laboratory observations

point of view has been challenged.' Soy protein may induce changes in the mucosa of the small intestine, leading to mucosal atrophy very rapidly. Our patient showed mucosal alterations two hours after challenge which were similar to those described by A m e n t ' after four hours. The frequent complication of carbohydrate malabsorption found in diarrheal disease,' which may involve all carbohydrates as seen in our patient, is accompanied by large osmotic gradients in the intestine a n d / o r bile salt deconjugation. Our animal studies suggest that these conditions enhance intestinal macromolecular transport. ~"~ However, in h u m a n beings there may be other factors, such as prolonged protein-calorie m a l n u trition, which could contribute to this process. Regardless of the mechanism, increased absorption of antigen from the diet could sensitize the infant and be responsible for the allergic manifestations to dietary protein, as seen in our patient. Antige'n absorption may also account for the frequent observation of milk and egg antibodies in the serum of patients following diarrheal disease '~ and for the milk allergy found in association with intestinal disaccharidase deficiencies?' Selective exclusion of the offending agent or agents from the diet may permit oral feeding, and clinical improvement may be obtained, without having to resort to total parenteral nutrition in chronic diarrhea in children.

6 19

92. Green IlL, McCabe DR, and Merenstein GB: Protracted diarrhea and malnutrition in infancy: Change in intestinal morphology and disaccharidase activities during treatment with total intravenous nutrition or oral elemental diets, J PEDIATR87:695, 1975. 3. Sunshine P, Lebenthal E, and Marinkowich VA: Intractable diarrhea of infancy: Relationship to a severe gastrointestinal allergy, Clin Res 20:257, 1972. 4. Ament ME: Soy protein allergy-another cause of fiat intestinal mucosa, Gastroenterology 62:227 , 1972. 5. Cook CD: Probable gastrointestinal reaction to soybean, N Engl J Med 263:1076, 1960. 6. Mendoza J, Meyers J0 and Snyder R: Soybean sensitivity-a case report, Pediatrics 46:774, 1970. 7. Goldman AS, Anderson DW, and Sellers WA: Milk allergy. 1. Oral challenge with milk and isolated milk proteins in allergic children, Pediatrics 32:425, 1963. 8. Cooper M, Teichberg S, and Lifshitz F: Alterations in rat jejunal permeability to a macromolecular tracer during a hyperosmotic load, Lab Invest 38:447, 1978. 9. Fagundes-Neto U, Teichberg S, Bayne MA, Morton B, and Lifshitz F: Enhanced jejunal macromolecular absorption induced by bile salts, Fed Proc 37:699, 1978. 10. Gruskay FL, and Cooke RE: The gastrointestinal absorption of unaltered protein in normal infants and in infants recovering from diarrhea, Pediatrics 16:763, 1955. 11. Lubos MC, Gerrard JW, and Buchan DJ: Disaccharidase activities in milk sensitive and celiac patients, J PEDtX'rR 70:325, 1967.

REFERENCES I~ Lifshitz F: Carbohydrate problems in pediatric gastroenterology, Clin Gastroenterol 6:415, 1977.

Enteritis, abscess, and septicemia due to Yersinia enterocolitica in a child with thalassemia J. P. Butzler, M. Alexander, A. Segers, N. Cremer, and D. Blum, Brussels. Belgium

ttUMAN INFECTION with Yersinia enterocolitica has increased substantially in recent years, principally in Europe and Canada. The range of clinical patterns has extended similarly; apart from acute enterocolitis, the most c o m m o n are acute mesenteric hdenitis and terminal ileitis, erythema nodosum, and acute polyarthritis. 2 A few instances of septicemia in infants have been described. Attention has been drawn to the conditions underlying 9

From the Departments of Microbiology and Pediatrics, St Pierre Hospital-Free University of Brussels.

0022-3476/78/100619+03500~30/0 9 1978 The C. V. Mosby Co.

the infection, often hematologic disorders involving iron overload; for example, infections due to 1: enterocolitica are more frequent in adults with hemochromatosis? We report a 4-year-old child with thalassemia who developed an abscess and septicemia of intestinal origin due tO Y. enterocolitica. CASE R E P O R T Patient G.S., a girl of Sicilian origin, was first referred to a hospital at the age of 13 months because of anemia and failure to thrive. Thalassemia major ",,,'as suspected on the basis of the peripheral blood findings, and was confirmed by hemoglobin

6 20

Brief clinical and laboratory observations

The Journal of Pediatrics October 1978

Table. 1': enteroeolitica septicemia in c h i l d r e n - r e v i e w o f the literature

Patient

Reference I Sex

Age

Underl)'ing condition

I

Clinical features

Septic metastasis

I

2

F

2

Aplastic anemia

diarrhea, fever

2

I

F

1.5

Thalassemia

Acute gastroenteritis

3

l

F

1.6

Thalassemia

Fever, osteitis foot

Os

4 5

3 4

M M

9 9

Thalassemia Thalasscmia

Enteritis fever Abdominal pain, hepato-splenomegaly

Appendix Abscess foot

6 7

5 6

? F

2 3/12

Kwashiorkor -

Diarrhea, fever Diarrhea, peritonitis

8

8

M

12

Thalassemia

Syndrome of right iliac fossa

Terminal ileum

9

Present

F

4.9

Thalassemia

Fever, diarrhea

Appendix

electrophoresis (llbF 89%). Thereafter the child was transfused monthly with packed RBC to maintain the hemoglobin level above 8 gm/dl. After July, 1973, treatment included desferrioxamine injections, and vitamin C supplementation. The subsequent clinical course was uneventful except for moderate hepatosplenomegaly and a transient rise in transaminase values. At age 4 years and 9 months, she was admitted to the St. Pierre Hospital with fever, diarrhea, vomiting, and epigastric pain. Physical examination showed only mild pharyngitis, splenomega"ly, and a diffusely tender abdomen. Laboratory data included a hemoglobin level of 8.8 gm/dl a WBC count of 21,000 (polynuclear ceils 80%, lymphocytes 18%, monocytes I%, erythroblasts I%) and a platelet count of 240,000. Sedimentation rate was 48 ram/hour. Urine analysis was negative. A ltimbar puncture yielded normal CSF. After taking blood and stools for culture, the child was transfused and treatment with ampicillin 200 mg/kg/day (IV) was started. During the next two days there was no clinical improvement, but then blood and stool cultures were reported positive for Y. enterocolitica, serotype 0a. Because of the ineffectiveness of ampicillin against Y. enteroeolitica, which is a producer of beta laetamase, the treatment was changed to gentamicin (6 mg/kg/day). The sedimentation rate was then 65 mm, the WBC count 11,700 (80% polynuctear cells) and platelets 268,000/mm3; the Yersiniaantibody titer was I/3,200). Five days after gentamicin was started the child was afebrile and the stools were normal, but the abdomen was still tender and a 3 cm mass was felt in the right inferior quadrant; this suggested the possibility of an appeudicular abscess. On the tenth day of treatment, the child was n o longer complaining and stools were normal. 'The abdominal mass was less easy to feel. At this time, gentamicin was discontinued; two days later a barium enema showed a deformation of the cecum with an irregular pattern on its internal wall and adherence of the

Treatment Tetracycline + colistin Penicillin + colislin Chloramphenicol + streptomycin Tetracycline Cotrimoxazole + tetracycline Tetracycline Gentamicin + chloramphenicol Streptomycin after tetracycline Gentamicin

Outcome Death Recovery Recovery

Recovery Recovery

Recovery Death

Recovery

Recovery

terminal ileum. Although these changes were consistent with an appendicular abscess, no operation was performed because of the child's good clinical state. A radiologic examination ten days later showed a normal cecum and complete filling of the appendix. By this time the child was well. Sedimentation rate and WBC count were normal. Blood and stool cultures yielded no Yersinia but the antibody titer was still high (I/3,200). The child was discharged two weeks after cessation of treatment. During the following months she remained well while receiving regular transfusions, and the abdominal mass was no longer palpable. BACTERIOLOGY Two stool cultures and a blood culture yielded a gram-negative coccobacillus with the following characteristics: mobility negative ( - ) at 37~ but positive ( + ) at 22~ urease + , oxidase - , phenylalanine desaminase - , glucose + , sucrose + , lactose - , gas - , H2S --, rhamnose --, Citrate --. Antigenic typing using living organisms isolated from the stools and the blood showed that the organism was a Y. enterocolotica belonging to serotype 0a, chemotype 4, lysotype 8. This is the usual lysotype o f strains isolated from man in Western Europe. The minfmum inhibitory concentrations in /~g/ml o f antibiotics were: ampicillin 25, cephalothin 100, carbenicillin 200, chloramphenicol 0.39, kanamycin 1.56, gentamicin 0.19, colistin 0.39, and tetracycline 1.56. The bactericidal power o f the serum, assayed when the patient was receiving gentamicin, was 1/64 and 1/128, respectively, demonstrating the great effectiveness o f this antibiotic. Diagnostic serology carried out with unheated suspen-

Vohone 93 Nttmber 4

sions of Y. enterocolitica type 0, (from the patient) and type 0.~ (reference strain) showed the presence of specific antibodies to the homologous strain 0v There was a progressive decrease in the antibody titer to Y. enterocoliticia O~ in the patient's serum, from 1/3,200 to less than 1/50, over a period of 260 days from the date of the positive blood culture. DISCUSSION Infections by Y. enterocolitica tend to have some underlying pathology. In the adult it may be cirrhosis,, hemochromatosis, diabetes, a blood disorder, malnutrition, or treatment with immunodepressive drugs. A review of the literature on Y. enterocolitica septicemias occurring in infants (Table) confirms this fact. The septicemias are always of intestinal origin. In the young infant they occur following gastroenteritis, and in children over about 5 years ofage the arise as a complication of an appendicular syndrome. Six patients (including our own) had a hematologic disorder (thalassemia, aplastic anemia). The only patient in whom a predisposing factor did not exist was a very young breast-fed infant who had a progressive infection complicated by intestinal perforation and peritonitis: The role of iron i n resistance to infectior~s has recently been reviewed by Pearson and Robinson.' In vitro, an excess of iron favors bacterial multiplication, but one must be cautious about relating this fact to disorders 9 such as thalassemia. The above authors expressed doubt about the existence of an increased susceptibility to , infections in patients with thalassemia; most o f the infections occur in patients who have had splenectomy. It is nevertheless striking that only one of the patients 9described had had splenectomy at the time of their Yersinia septicemia. Factors other than a raised serum' iron concentration

Brief clinical attd laboratory observations

62I

may explain the greater frequency of infections in patients with thalassemia. In particular, hepatic cirrhosis allowing bacteria to pass by the reticuloendothelial system of the liver may be a contributory factor? Cirrhosis is only a late complication of thalassemia, however, and the young age of the patients studied makes this hypothesis rather improbable. Whatever m a y be the contributing factors, one must consider the possibility of an infection with Y. enterocolitica in every febrile intestinal syndrome occurring in patients with thalassemia, since early identification of the organism allows appropriate antibiotic therapy and may prevent septicemic complications. REFERENCES

I. Blum D, Viart P, and Dachy A: Septic/'mie .~ Yersinia cnterocolitica chez,deux enfants atteints de thalass~mie majeure, Arch Fr P~diatr 27:445, 1970. 2. Mollaret Htt, Omland T, Henrikson SD, Baero Pr, Rykner G, and Scavizzi M: Les septicrrnies humaines .5 "Yersinia enterocolitica". Apropos de dix-sept cas rrcents, la Pressc b,l~d 79:345, 1971. 3. Seigneurin R, Marchal MF, and Ons J: Septicemic Yersinia enterocolitica chez un enfant thalassrmique Mrd Mal lnf 2:317, 1972. 4. Hewstone AS, and Davidson GP: Yersinia cnterocolitica septicaemia with arthritis in a thalassemic child, Med J Aust 1:1035, 1972. 5. Rabson AR, Hallet AF, hnd Koornhof tlJ: Generalised Yersinia enterocolitica infection, J Infect Dis 131:447, 1975. 6. Kohl S, Jacobson JA, and Nanmid A: Yersinia enterocolitica infections in children, J PEDImR 89:77, 1976. 7. Pearson ttA, and Robinson JE: The role of iron in host resistance, Adv Pediatr 23:!, 1976. 8. Kiffer B, Rudloft J, Leli~vre-Gassin C, and Bury P: Affection :1 Yersinia enterocolitica. Une observation ~ forme pseudotumorale ct septicrmie chez un enfant atteint de maladie de Cooley, Ann M~d Nancy 15:847, 1976.

Ovarian fibromata in four successive generations Ra3~nonde A. Dumont-Herskowitz, M.D., Homa S. Safaii, M.D., and Boris Senior, M.D.,* Boston, Mass.

OVARIAN TUMORS rarely present in childhood. Teratomata make up the majority of childhood tumors, followed From the Pediatric Endocrine Metabolic Service and the Department of Pathology of Tufts-New England Medical Center. *Reprint address: New England Medical Center tlospital, 171 ltarrison Ave., Boston, MA 02111.

0022-3476/78/100621 +04500.40/0 9 1978 The C. V. Mosby Co.

in order of frequency by cystic and mucinous tumors. In only 5.8% of childhood examples does the tumor originate in the ovarian stroma. Most of the stromal tumors are of the granulosa-theca variety, a type of specialized stromal tumor; fibromata, a nonspecialized form, comprise only 1.5% of all childhood ovarian tumors.' Rare as ovarian tumors, particularly fibromata, are in