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Radiological Case of the Month
Rivital Sela, MD;
Moshe Nussinovitch, MD;
Nitsa Ziv, MD;
Benjamin Volovitz, MD;
Jacob Amir, MD;
Beverly P. Wood, MD
From the Department of Paediatrics C and the Institute of Radiology,
Schneider Children's Medical Centre of Israel, Sackler School of Medicine,
Tel Aviv University, Petach Tikvah, Israel (Drs Sela, Nussinovitch, Ziv, Volovitz,
and Amir); and the University of Southern California, Keck School of Medicine,
Los Angeles (Dr Wood).
Arch Pediatr Adolesc Med. 2001;155:1273-1274.
A 3-YEAR-OLD BOY was referred for evaluation of unilateral testicular
swelling and abdominal and lower limb edema. His mother reported that he had
been coughing and vomiting for 3 weeks prior to admission. Swelling began
4 weeks before admission. Physical examination showed extensive peripheral
edema and ascites. There was no fever and his blood pressure was 106/56 mm
Hg.
Laboratory findings were as follows: white blood cell count, 26.3 x
109 (62.7% lymphocytes); hemoglobin, 14.4 g/dL; albumin, 1.7 g/dL;
potassium, 4.7 mmol/L; sodium, 132 mmol/L; glucose, 107 mg/dL (5.94 mmol/L);
normal urine protein and amylase content; positive urine cytomegalovirus antigen;
positive cytomegalovirus antibody, IgM and IgG; and stool 1-antitrypsin
level was 15.2 mg per gram of dry weight stool.
Abdominal ultrasound showed bilateral pleural effusions and a large
quantity of intraperitoneal fluid, an enlarged liver, and markedly thickened
gastric rugae (Figure 1).
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Figure 1.
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The boy was treated with antibiotics for his cough, intravenous albumin
and furosemide for edema, and a diet of protein-enriched food. The swelling
subsided and the abdominal diameter decreased during the next several weeks.
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Denouement and Discussion: Hypertrophic Gastropathy With Edema
Figure 1. Abdominal ultasound
shows marked thickening of gastric rugae.
A diagnosis of hypertrophic gastropathy with cytomegalovirus infection
was made. Hypertrophic gastropathy of childhood has been reported in children
with a mean age of 5 years. The initial symptoms include vomiting, diarrhea,
abdominal pain, and anorexia. On physical examination, peripheral edema is
usually present. Laboratory results show low serum albumin and protein-losing
enteropathy proven by analysis of chromium-labeled albumin or stool 1-antitrypsin.
There are 2 diagnostic criteria for hypertrophic gastropathy: (1) giant
gastric rugae by imaging, endoscopy, or laparotomy; and (2) characteristic
histologic findings of foveolar hyperplasia and cystic dilation of submucosal
glands.
The appearance of hypertrophic gastropathy has been analyzed by endoscopic
ultrasound and endoscopy in previous studies. In the study by Hizawa et al,1 every patient had giant gastric folds 13 to 20
mm in diameter resulting from thickening of the mucosal layer with or without
cystic components. By ultrasound study, the thickened mucosa was echogenic
and Helicobacter pylori was the causative agent in
most adult patients. Approximately 55 cases of hypertropic gastropathy in
children have been published. In contrast to the chronic course of Ménétrier
disease in adults, the pediatric cases are generally benign, self-limited,
and show complete resolution within a few weeks.2
The benign pediatric hypertrophic gastropathies have been associated with
infections, primarily cytomegalovirus3
and occasionally H pylori, herpes simplex, and mycoplasma.2, 4
Abdominal scintigraphy using technetium Tc 99m-labeled human serum albumin
delivered intravenously and direct measurement of protein in gastric juice
has proven that serum proteins are massively secreted in the stomach.5 Supportive treatment with a high-protein diet
and intravenous albumin transfusions is recommended. H2-receptor antagonist
use may improve symptoms.2, 6
AUTHOR INFORMATION
Reprints: Moshe Nussinovitch, MD, Department of Paediatrics C, Schneider
Children's Medical Centre of Israel, Petach Tikvah, Israel.
REFERENCES
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1. Hizawa K, Kawasaki M, Yao T, et al. Endoscopic ultrasound features of protein-losing gastropathy with hypertrophic
gastric folds. Endoscopy. 2000;32:394-397.
PUBMED
2. Kindermann A, Koletzko S. Protein-losing giant fold gastritis in childhooda case report
and differentiation from Menetrier disease of adulthood. Z Gastroenterol. 1998;36:165-171.
PUBMED
3. Eisenstat DD, Griffiths AM, Cutz E, Petric M, Drumm B. Acute cytomegalovirus infection in a child with Menetrier's disease. Gastroenterology. 1995;109:592-595.
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4. Ben Amitai D, Zahavi I, Dinari G, Garty BZ. Transient protein-losing hypertrophic gastropathy associated with Mycoplasma pneumoniae infection in childhood. J Pediatr Gastroenterol Nutr. 1992;14:237-239.
PUBMED
5. Yamada M, Sumazaki R, Adachi H, et al. Resolution of protein-losing hypertrophic gastropathy by eradication
of Helicobacter pylori. Eur J Pediatr. 1997;156:182-185.
PUBMED
6. Kaneko T, Akamatsu T, Gotoh A, et al. Remission of Ménétriér's disease after a prolonged
period with therapeutic eradication of Helicobacter pylori. Am J Gastroenterol. 1999;94:272-273.
PUBMED
SECTION EDITOR: BEVERLY P. WOOD, MD
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