Indian Pediatr 2011;48: 641-642
Short-Term Corticosteroids for Celiac Crisis
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,
Correspondence to: Prof. Nedeljko Radlovic, Head,
Department of Gastroenterology and Nutrition, University Children’s
Hospital, 10 Tirsova, 11000 Belgrade, Serbia.
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.
eliac 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].
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 , 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 . 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).
Blood Laboratory Values on Admission
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.
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.
Competing interests: None stated.
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.
4. Gupta T, Mandot A, Desai D, Abraham P, Joshi A.
Celiac crisis with hypokalemic paralysis in a young lady. Indian J
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.
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.