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Ménétrier's disease in children: Report of a patient and review of sixteen other cases
GASTROENTEROLOCY
CASE
1981;80:1042-7
REPORTS
M&Wrier’s Disease in Children: Report of a Patient and Review of Sixteen Other Cases J. P. CHOURAQUI, C. C. ROY, P. BROCHU, C. L. MORIN, and A. M. WEBER Service de Gastro-Ent&ologie Sainte-Justine et l’Universit6
et DBpartements de Pathologie de Mont&al, Montreal Qubbec,
M&&trier’s disease is one of the rarer causes of protein-losing enteropathies seen in childhood. At onset of symptoms, the present case and 35 others reported so far were younger than 10 yr of age. Edema was usually more conspicuous than gastrointestinal symptoms. In contrast to the usual chronicity of the disease in adults, the illness is self-limited, transient, and benign. A number of gastrointestinal disorders are associated with protein-losing enteropathy in the pediatric age group (12). MBn&rier’s disease, characterized by giant hypertrophy of the gastric rugae, is one of the rarer entities. Since the initial description by Men6trier (16)in 1888,only 15 cases have been reported in the pediatric age group (3-15)as opposed to more than 200 in adults (17,18). In this paper we present an additional patient and review other pediatric cases.
Case Report M. H., a boy of 8 yr, 8 mo, was admitted to HBpital Sainte-Justine in Montreal for evaluation of anasarca. The child was in good health until 10 days before admission when he developed epigastric pain. This pain typically occurred after meals, was of short duration, and disappeared spontaneously. Associated symptoms included anorexia, nausea, and vomiting. The patient denied fever and diarrhea, and there was no melena or hematemesis. Ten days before admission, his mother noted swelling of his eyelids, followed by puffiness of his face and, subsequently, by peripheral edema. The family history was unremarkable. Received August 5, 1980. Accepted December 1, 1980. Address requests for reprints to: CC. Roy, M.D., HGpital Sainte-Justine, 3175 Ste-Catherine Road, MontrBal, Quhbec. The authors thank Dr. Gordon F. Vawter, Children’s Hospital Medical Center, Boston, for his help in reviewing the slides and for helpful discussions. 0 1981 by the American Gastroenterological Association OOlS-5085/81/051042-06$02.50
H. GREGOIRE, et de Radiologie,
HBpital
Canada
Physical examination revealed a well-developed boy (50th percentile for height and weight) with normal vital signs. He had generalized pitting edema associated with a small left pleural effusion, and a discrete amount of ascitic fluid. Otherwise, the physical examination was within normal limits. Within 2 wk after admission, and in the absence of any treatment, the patient felt better. He became free of abdominal pain, the pleural effusion disappeared, and the peripheral edema markedly decreased as the patient lost 4 kg. Follow-up examinations 2 and 4 mo after admission revealed a boy in perfect health. Blood counts, protein electrophoretic patterns, and immunoglobulins obtained during the 4 mo of follow-up appear in Table 1.Other studies, including those for cholesterol, triglycerides, carotene, SGOT, SGPT, alkaline phosphatase, coagulation screening, D-xylose absorption, sweat test, BUN, and urine analysis, were normal. An upper gastrointestinal examination showed marked hypertrophy as well as irregularity of the mucosal folds mainly in the upper part of the stomach: the antrum was spared (Figure 1). A repeat barium meal after 4 mo of follow-up showed disappearance of the above findings. Gastroscopic examination confirmed the presence of large rugae that were prominent along the greater curvature and the anterior aspect of the fundus. Biopsies were performed. Gastrin determinations made on four different occasions gave normal results. Gastric acid studies were carried out both in the fasting state and after betazole (Histalog) stimulation (1 mg/kg body wt). The basal acid output (BAO) was 3.9 mEq/h while the peak acid output (PAO) was 5.8 mEq/ h. Four months later, repeat testing revealed a BAO of 2.1 mEq/h and a satisfactory response to betazole with a PA0 of 11.4 mEq/h. The protein concentration of fasting gastric juice was 5.9 mg/ml (N c 3.5) (19). One month after admission, radiochromated (“‘Cr) albumin tests showed that 1.5% (N < 0.5%) of the administered dose was excreted in a s-day stool collection; in a repeat test 3 mo later, the result was 0.6%. Antinuclear antibodies as well as antibodies against DNA, mitochondria, smooth muscle, and parietal cells were determined on four occasions and were always absent. Because of a previous report suggesting the possibility of a cytomegalovirus (CMV) infection, antibodies against CMV were measured on two occasions and were
CASE
1981;80:1042-7
REPORTS
M&Wrier’s Disease in Children: Report of a Patient and Review of Sixteen Other Cases J. P. CHOURAQUI, C. C. ROY, P. BROCHU, C. L. MORIN, and A. M. WEBER Service de Gastro-Ent&ologie Sainte-Justine et l’Universit6
et DBpartements de Pathologie de Mont&al, Montreal Qubbec,
M&&trier’s disease is one of the rarer causes of protein-losing enteropathies seen in childhood. At onset of symptoms, the present case and 35 others reported so far were younger than 10 yr of age. Edema was usually more conspicuous than gastrointestinal symptoms. In contrast to the usual chronicity of the disease in adults, the illness is self-limited, transient, and benign. A number of gastrointestinal disorders are associated with protein-losing enteropathy in the pediatric age group (12). MBn&rier’s disease, characterized by giant hypertrophy of the gastric rugae, is one of the rarer entities. Since the initial description by Men6trier (16)in 1888,only 15 cases have been reported in the pediatric age group (3-15)as opposed to more than 200 in adults (17,18). In this paper we present an additional patient and review other pediatric cases.
Case Report M. H., a boy of 8 yr, 8 mo, was admitted to HBpital Sainte-Justine in Montreal for evaluation of anasarca. The child was in good health until 10 days before admission when he developed epigastric pain. This pain typically occurred after meals, was of short duration, and disappeared spontaneously. Associated symptoms included anorexia, nausea, and vomiting. The patient denied fever and diarrhea, and there was no melena or hematemesis. Ten days before admission, his mother noted swelling of his eyelids, followed by puffiness of his face and, subsequently, by peripheral edema. The family history was unremarkable. Received August 5, 1980. Accepted December 1, 1980. Address requests for reprints to: CC. Roy, M.D., HGpital Sainte-Justine, 3175 Ste-Catherine Road, MontrBal, Quhbec. The authors thank Dr. Gordon F. Vawter, Children’s Hospital Medical Center, Boston, for his help in reviewing the slides and for helpful discussions. 0 1981 by the American Gastroenterological Association OOlS-5085/81/051042-06$02.50
H. GREGOIRE, et de Radiologie,
HBpital
Canada
Physical examination revealed a well-developed boy (50th percentile for height and weight) with normal vital signs. He had generalized pitting edema associated with a small left pleural effusion, and a discrete amount of ascitic fluid. Otherwise, the physical examination was within normal limits. Within 2 wk after admission, and in the absence of any treatment, the patient felt better. He became free of abdominal pain, the pleural effusion disappeared, and the peripheral edema markedly decreased as the patient lost 4 kg. Follow-up examinations 2 and 4 mo after admission revealed a boy in perfect health. Blood counts, protein electrophoretic patterns, and immunoglobulins obtained during the 4 mo of follow-up appear in Table 1.Other studies, including those for cholesterol, triglycerides, carotene, SGOT, SGPT, alkaline phosphatase, coagulation screening, D-xylose absorption, sweat test, BUN, and urine analysis, were normal. An upper gastrointestinal examination showed marked hypertrophy as well as irregularity of the mucosal folds mainly in the upper part of the stomach: the antrum was spared (Figure 1). A repeat barium meal after 4 mo of follow-up showed disappearance of the above findings. Gastroscopic examination confirmed the presence of large rugae that were prominent along the greater curvature and the anterior aspect of the fundus. Biopsies were performed. Gastrin determinations made on four different occasions gave normal results. Gastric acid studies were carried out both in the fasting state and after betazole (Histalog) stimulation (1 mg/kg body wt). The basal acid output (BAO) was 3.9 mEq/h while the peak acid output (PAO) was 5.8 mEq/ h. Four months later, repeat testing revealed a BAO of 2.1 mEq/h and a satisfactory response to betazole with a PA0 of 11.4 mEq/h. The protein concentration of fasting gastric juice was 5.9 mg/ml (N c 3.5) (19). One month after admission, radiochromated (“‘Cr) albumin tests showed that 1.5% (N < 0.5%) of the administered dose was excreted in a s-day stool collection; in a repeat test 3 mo later, the result was 0.6%. Antinuclear antibodies as well as antibodies against DNA, mitochondria, smooth muscle, and parietal cells were determined on four occasions and were always absent. Because of a previous report suggesting the possibility of a cytomegalovirus (CMV) infection, antibodies against CMV were measured on two occasions and were