Ready to Use Therapeutic Food (RUTF) in the Management of Severe Acute Malnutrition in India

Case Report

Indian Pediatr 2011;48: 641-642

Short-Term Corticosteroids for Celiac Crisis in Infants

Nedeljko P Radlovic, *Marija M Mladenovic, †Zorica M Stojsic, and Radivoj S Brdar

From the Department of Gastroenterology and Nutrition, University Children’s Hospital, Belgrade; *Pediatric Department, Health Center, Valjevo; and †Institute of Pathology, University Medical School, Belgrade, Serbia.

Correspondence to: Prof. Nedeljko Radlovic, Head, Department of Gastroenterology and Nutrition, University Children’s Hospital, 10 Tirsova, 11000 Belgrade, Serbia.
Email: [email protected]

Received: January 11, 2010;
Initial Review: January 29, 2010;
Accepted: May 3, 2010.

We report two infants with celiac crisis who continued to have persistent secretory diarrhea despite gluten and lactose free diet and supportive parenteral nutrition. The children were given corticosteroid therapy. After a five-day oral prednisone in the dose of 2 mg/kg/daily, both patients rapidly recovered.

Key words: Celiac crisis, Corticosteroids, Infants.

Celiac crisis (CC) is a rare and life-threatening complication of celiac disease (CD) [1-4]. Although possible at all ages, it is most often seen in children younger than two years of age [1-5]. Beside the usual measures, to achieve a successful recovery, glucocorticoid therapy is also occasionally necessary [1,2,5-7].

Case Report

We present two infants with severe celiac crisis who required a short-term administration of steroids. The diagnosis of celiac disease was based on the criteria of the European Society of Pediatric Gastroenterology, Hepatology and Nutrition [8], and that of celiac crisis on the acutization of chronic diarrhea followed by severe dehydration, metabolic acidosis, refusal of meals, abdominal distension, hypoproteinemic edema and a marked decrease of bodyweight [1]. Stool examination for bacterial infection, Rota-Adeno virus latex agglutination test, ova and parasites, as well as duodenal fluid for Giardia lamblia, were negative in both cases.

Case 1. An 8-month-old male infant presented with severe dehydration, abdominal distension, generalized edema and marked malnutrition caused by a persistent two-month diarrhea which intensified a week prior to admission. Glutin had been introduced in the diet at 3 months of age and diarrhea had started 3 months later. The child was lethargic, with cold peripheries. Rectal temperature was 35.9ºC, heart rate was 142/min, respiration 40/min and BP 50/30 mmHg. The child was malnourished with bodyweight 33% of the expected. There was perianal erythema and erosive changes. Stool pH was 5 and stool reducing substances was 1.5% (Clinitest 3+). Antibodies to tissue transglutaminase (ATTG) IgA was 68 U/mL.

Case 2. A 9-month-old female infant presented due to the worsening of a one and half-month progressive diarrhea followed by severe dehydration, abdominal distension, periorbital and pedal edema and malnutrition. This child had a rectal temperature of 36.80C, HR 144/min, RR 42/min and BP 55/30 mmHg. The child also had acute severe malnutrition. ATTG IgA was 88 U/mL. She also had gluten introduction in diet at 3 months and onset of diarrhea at 7.5 months.

Following correction of water, electrolyte and acid-base disturbances, both patients were started on supportive parenteral nutrition. After a week of treatment, the biopsy of small bowel mucosa was performed using the Watson’s modification of Crosby’s capsule for children with double port. In both children the mucosa, viewed under the dissecting microscope, was flat (Marsh score IIIc).

TABLE I

Blood Laboratory Values on Admission

Case	Sodium	Potassium	pH	Creatinin	Calcium	Magnesium	Phosphate	Albumin	Hb
	(mmol/L)	(mmol/L)		(µmol/L)	(mmol/L)	(mmol/L)	(mmol/L)	(g/L)	(g/L)
1	126	2.1	7.22	133	1.02	0.61	0.62	18	93
2	128	2.4	7.24	108	1.1	0.64	0.66	24	71

Although the condition of both infants stabilized under the applied therapy, due to persistent secretory diarrhea requiring a continual intravenous fluid substitution and also perpetual anorexia, in case 1 on the 10th and in case 2 on the 11th day after admission, we started oral prednisone therapy in the dose of 2 mg/kg/daily. After five days, both infants showed improvement in diarrhea and improved appetite. During the next week, prednisone therapy was gradually decreased and finally stopped. Under a strict gluten free diet, a two-week lactose free diet and four-month addition of iron, folic acid and multivitamin preparations, both patients achieved full recovery. After six-month treatment, serum transaminase activity was also normal. By provocation gluten tolerance test, in case 1 aged 5.8 years and in case 2 aged 5.6 years, we confirmed the diagnosis of celiac disease.

Discussion

Although celiac crisis usually resolves to rehydratation, correction of metabolic imbalance, gluten and lactose free diet, and supportive parenteral nutrition, the rapid recovery of the patient may sometimes fail [1,2]. In cases resistant to conventional therapy, several authors have reported positive experience with the use of intravenous corticosteroid therapy based on its anti-inflammatory effects, as well as positive influence on the bowel epithelium maturation [5-7].

We report two infants with CC resistant to conventional therapy. Both were breastfed for a short time and were early exposed to gluten. Also, in both, the disease was of long duration. In none of the cases, celiac crisis was precipitated with gastrointestinal or extraintestinal infection. Although being aware of side effects, the lack of response to gluten and lactose free diet and supportive parenteral nutrition prompted us to use prednisone. Additionally, the lack of gastrointestinal and extraintestinal infections made such therapeutic approach easier. Short-term, administration of corticosteroids was justified in both cases, with no side effects. However, this option should only be exercised in cases who are resistant to standard therapy.

Acknowledgment: Mrs. Tatjana Paunovic, English language editor of Serbian Archive of Medicine, on her help in the translation of this paper.

Contributors: NPR, MMM and RSB were involved in managing of the cases. ZMS, performed the pathological analysis of the small bowel mucosa samples. MMM did the literature search, acquired data and wrote the initial draft under the supervision of NPR. NPR was involved in critical revision of the manuscript and will serve as the guarantor.

Funding: None.

Competing interests: None stated.

References

1. Walker-Smith JA, Murch S. Coeliac Disease. In: Walker-Smith JA, Murch S, eds. Disease of Small Intestine in Childhood, 4th ed. Oxford: Issis Medical Media, 1999. p. 235-78.

2. Ciclitira PJ, King AL, Fraser JS. AGA technical review on celiac sprue. Gastroenterology. 2001;120:1526-40.

3. Walia A, Thapa BR. Celiac crisis. Indian Pediatr. 2005;42:1169.

4. Gupta T, Mandot A, Desai D, Abraham P, Joshi A. Celiac crisis with hypokalemic paralysis in a young lady. Indian J Gastroenterol. 2006;25:259-60.

5. Lloyd-Still JD, Grand RJ, Khaw KT, Schwachman H. The use of corticosteroids in celiac crisis. J Pediatr. 1972;81:1074-81.

6. Baranwal AK, Singhi SC, Thapa BR, Kakkar N. Celiac crisis. Indian J Pediatr. 2003;70:433-5.

7. Mones RL, Atienza KV, Youssef NN, Verga B, Mercer GO, Rosh JR. Celiac crisis in Modern Era. J Pediatr Gastroenterol Nutr. 2007;45:480-3.

8. Walker-Smith JA, Guandalini S, Schmitz J, Shmerling DH, Visakorpi JK. Revised criteria for diagnosis of coeliac disease. Report to working group of European Society of Paediatric Gastroenterology and Nutrition. Arch Dis Child. 1990;65:909-11.