Collagenous sprue in a 3‐month‐old infant

Collagenous sprue (CS) is a severe malabsorption disorder, the etiology of which has not been well defined. Herein, we report the case of a 3‐month‐old infant with CS who responded to steroid and immunomodulator treatment and presented a thick subepithelial collagen band. A 3‐month‐old Japanese girl presented with severe watery diarrhea that lasted for 2 weeks. She was admitted to the referring hospital, but symptomatic improvement was not achieved with fasting and rehydration. Gastroduodenal endoscopy showed an edematous duodenal mucosal surface. Duodenal biopsy indicated severe villous atrophy with infiltration of mostly CD8‐positive T cells; and deposition of subepithelial collagen was confirmed. The subepithelial collagen deposits, however, had disappeared after treatment. Historically, child‐onset CS is extremely rare and this case is likely to be the youngest case of infantile CS. The present case suggests that CS should be considered as a differential diagnosis for intractable diarrhea, even in infants.     

Gastric metaplasia: a frequently overlooked feature of duodenal biopsy specimens in untreated celiac disease

BACKGROUND: Duodenal gastric metaplasia is rarely reported in untreated celiac disease, although it is seen in 60% to 100% of duodenal biopsy specimens in nonceliac patients with histologic duodenitis. The low incidence could represent underreporting, a decreased incidence in pediatric patients generally, or the more distal sampling site that is customary for most biopsy specimens that are obtained to diagnose celiac disease. It could also be a unique feature of the inflammatory reaction that characterizes this disease. The purpose of this study was to examine the incidence of gastric metaplasia in duodenal specimens from children with untreated celiac disease with special reference to patient age and biopsy site. METHOD: Formalin-fixed paraffin-embedded specimens of duodenal mucosa were selected from the pathology department's archival material. Sections were either stained histochemically or by immunochemical methods, according to an antigen-retrieval protocol. Forty-four duodenal specimens from untreated patients with celiac disease (n = 22) and control subjects of similar age with normal histology (n = 22) were examined. Ten of each were obtained during upper endoscopy from the proximal duodenum (proximal site) and 12 of each by Crosby capsule near the ligament of Treitz (distal site). RESULTS: All specimens from patients with celiac disease exhibited marked villous atrophy. None had been noted to have gastric metaplasia during routine examination of sections stained by hematoxylin and eosin. Fifteen (68%) of 22 of the celiac specimens and 2 of 22 (9%) control specimens contained gastric metaplasia, identified as patches of gastric-type cells containing MUC5AC (gastric mucin), pS2 (gastric trefoil factor) and neutral (periodic acid-Schiff-positive) mucin. Five of the seven celiac specimens that had no metaplasia showed increased numbers of goblet cells expressing gastric markers. The incidence of gastric metaplasia was not different for endoscopic (70%) or capsule (67%) specimens. Sixty-eight percent (7/11) of patients aged less than 3 years had gastric metaplasia. CONCLUSION: The presence of gastric metaplasia has been previously underreported in celiac disease specimens. Detection would be improved by the routine use of period acid-Schiff/ alcian blue staining. The incidence of gastric metaplasia in celiac disease is not significantly influenced by biopsy site or age at time of the biopsy.     

小儿胃十二指肠炎内镜与病理检查对照分析

为探讨内镜检查小儿胃十二指肠炎的准确性，将２４２例有上消化道症状的患儿内镜下诊断和粘膜组织病理学检查做对照比较。结果表明，慢性浅表性胃炎、浅表性十二指肠炎的内镜下诊断符合率分别为７６．１％、８１．４％，慢性萎缩性胃炎为２５．０％。正常胃十二指肠粘膜和胃十二指肠炎粘膜改变的诊断在内镜、病理检查中有一定的差异。当胃粘膜镜下观察显示明显萎缩性改变特征时，慢性萎缩性胃炎诊断符合率较高；而显示红白相间等改变时，诊断符合率较低。提示将内镜、病理检查对比研究、了解胃十二指肠病之间的关系，可提高内镜诊断符合率并对临床治疗有重要的指导意义。     

Disorders of humoral regulation of the digestive organ functions in children with malabsorption syndromes]

The authors provide the data obtained during examination of 36 children with celiac disease and 18 children with lactase deficiency. The children's age ranged from 8 months to 15 years. All the children underwent spot biopsy of the gastric and duodenal mucosa followed by immunomorphological PAP-staining of the biopsy specimens and count of the number of gastrin- and somatostatin-producing cells. Gastrin in the blood serum was measured by radioimmunoassay. The children with celiac disease manifested an increase of the number of somatostatin-producing cells in the duodenum and decrease of their number in the pyloric part of the stomach, seen in the acute phase of the disease. The number of gastrin-producing cells remained unchanged. The level of gastrin declined in the acute phase and increased during a remission. The alterations described were found to be related to the atrophic processes in the small intestinal mucosa. In lactase deficiency, no significant alterations were established in the number of pyloric and duodenal endocrine cells or in blood gastrin level.     

Gastroduodenal pathology in pyelonephritis in children

The authors describe lesions of the upper alimentary tract in 91 children aged 4 to 15 years suffering from pyelonephritis. Intragastric pH-metry, endoscopy and histological examination of biopsy specimens from the gastric and duodenal mucosa were carried out, as a result of which gastroduodenal pathology was diagnosed in 3/4 of the patients. In 69.4% of the children, biopsy specimens of the mucous membrane of the antrum of the stomach showed Campylobacter pylori. Such combined pathology requires, in addition to the treatment of pyelonephritis correction of the diet, administration of agents that may contribute to the decrease of upper alimentary tract inflammation. If Campylobacter pylori are detected, the treatment eliminating those bacteria is to be administered.     

Intestinal perforation in a two-year-old child with eosinophilic gastroenteritis

A two-year-old boy underwent a laparatomy for an intestinal perforation due to eosinophilic gastroenteritis. He had marked peripheral blood eosinophilia and a small duodenal biopsy showed heavy eosinophilic infiltration in the mucosa. After 1 1/2 year on a restricted diet, a control duodenal biopsy showed only slight eosinophilia. Perforation of the small intestine is a rare but serious complication in eosinophilic gastroenteritis.     

Intestinal Perforation in a Two-Year-Old Child with Eosinophilic Gastroenteritis

ABSTRACT. A two-year-old boy underwent a laparatomy for an intestinal perforation due to eosinophilic gastroenteritis. He had marked peripheral blood eosinophilia and a small duodenal biopsy showed heavy eosinophilic infiltration in the mucosa. After 1 1/2 year on a restricted diet, a control duodenal biopsy showed only slight eosinophilia. Perforation of the small intestine is a rare but serious complication in eosinophilic gastroenteritis.     

Duodenal hematoma after upper endoscopy and biopsy in a 4-year-old girl

We describe a 4-year-old girl who developed a duodenal hematoma after upper endoscopy and biopsy. Although rare, duodenal hematoma formation can occur after upper endoscopy and biopsy in otherwise healthy children. A young child presenting with a duodenal hematoma in the absence of a clear mechanism of either previous gastrointestinal procedures or injury to the abdomen should have an extensive evaluation to exclude child abuse. A thorough evaluation for other medical causes, such as a coagulopathy, should be performed simultaneously.     

Tangier disease in a Turkish family

In this report we describe the upper gastrointestinal tractus involvement in a rare genetic disease of lipid metabolism. A 12‐year‐old boy presented with sore throat and fever. On physical examination, orange–yellow tonsils and adenoid tissue were noted. Mild hepatosplenomegaly was present. Lipid profile was compatible with Tangier disease (TD). Endoscopy of the upper gastrointestinal tract showed white‐yellowish fatty deposits on the gastric mucosa. Microscopically, biopsy specimens contained numerous histiocytes with a foamy cytoplasm packed in the lamina propria of the gastric mucosa and at the crypt basement of the duodenum. His sister, 8 years old, was also diagnosed with TD based on abnormal lipid profile and orange–yellow tonsils. TD is a rare familial disorder of lipid metabolism, characterized by deposition of cholesteryl esters, probably involving the entirety of the gastrointestinal tract from the mouth to the anus.     

Jejunal varices diagnosed by capsule endoscopy in patients with post‐liver transplant portal hypertension

Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%‐10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7‐16 years) with a median Hgb of 2.8 g/dL (range 1.8‐6.8 g/dL). Upper endoscopy was negative for significant esophageal varices, gastric varices, and bleeding portal gastropathy in all three children. All three patients had significant jejunal varices noted on CE in mid‐jejunum. Jejunal varices were described as large prominent bluish vessels underneath visualized mucosa, one with evidence of recent bleeding. The results led to venoplasty of the portal vein in two patients and a decompressive shunt in one patient with resolution of GI bleed and anemia. CE is useful to diagnose intestinal varices in children with portal hypertension and GI bleeding following liver transplant.     

Hp感染儿童胃十二指肠粘膜IL-8及IL-8 mRNA的研究

目的　检测Hp感染儿童胃、十二指肠粘膜中IL 8及IL 8mRNA的变化 ,从蛋白和分子水平探讨Hp对细胞因子IL 8的影响 ,以及IL 8在Hp相关性胃十二指肠疾病中的作用。方法　胃镜下取胃窦及十二指肠粘膜活检标本 ,用ELISA法和RT PCR半定量法测定胃及十二指肠粘膜中IL 8的含量和IL 8mRNA的表达量。结果　Hp阳性者胃粘膜IL 8为 2 4 66～ 177 77pg/mg ,IL 8mRNA为2 3 7～ 4 99;Hp阴性者胃粘膜IL 8为 2 94～ 12 98pg/mg,IL 8mRNA为 0 0 5～ 0 44 ;差异有显著意义(t分别为 12 3 4和 2 9 2 9,P <0 0 1)。Hp阳性者十二指肠粘膜IL 8为 12 98～ 177 77pg/mg ,IL 8mRNA为 1 2 2～ 1 80 ;Hp阴性者十二指肠粘膜IL 8为 2 0 4～ 10 43pg/mg ,IL 8mRNA为 0 0 1～ 0 2 3 ;差异有显著意义 (t分别为 7 18和 3 7 2 0 ,P <0 0 1)。活动性胃炎胃粘膜IL 8为 12 98～ 177 77pg/mg,IL 8mRNA为 0 5 1～ 4 99;非活动性胃炎胃粘膜IL 8为 2 0 4～ 10 43pg/mg,IL 8mRNA为 0 0 1～ 0 44 ;差异有显著意义 (t分别为 10 66和 18 62 ,P <0 0 1)。活动性胃炎十二指肠粘膜IL 8为 5 2 8～ 47 76pg/mg ,IL 8mRNA为 0 2 3～ 1 87;非活动性胃炎十二指肠粘膜IL 8为 3 19～ 8 14pg/mg,IL 8mRNA为0 0 1～ 0 2 0 ;差异有显著意义 (     

Campylobacter pyloridis-associated primary gastritis in children

Campylobacter pyloridis are spiral-shaped Gram-negative bacteria that have recently been associated with gastritis and peptic ulcer diseases in adults. The organisms have been identified in biopsy specimens of the gastric antrum by staining sections with silver stain. To determine the frequency of colonization of the antral mucosa with C pyloridis in association with gastritis in childhood, we retrospectively reviewed 53 cases in patients (mean age 11.9 years) in whom upper endoscopy with antral biopsy was performed for the evaluation of symptoms referable to the upper gastrointestinal tract. A silver impregnation method was used to detect the presence of C pyloridis. Nineteen of 53 antral biopsy specimens showed evidence of mucosal inflammation. Ten of these 19 patients had no apparent predisposing etiology for gastritis, and six of these ten had C pyloridis identified on antral biopsies. Nine of the 19 patients had secondary causes of gastric inflammation (two gastroduodenal Crohn disease, two eosinophilic gastroenteritis, four drug related, and one dys-gamma-globulinemia and lymphonodular hyperplasia). In contrast, none of these nine patients had C pyloridis demonstrated on antral biopsies. Silver stain for C pyloridis was negative in all 34 of the 53 children who had normal antral histologic findings. C pyloridis was not demonstrated by silver stain on any of the duodenal sections, although eight of the 19 children with gastritis and nine of the 34 children with normal antral histologic findings had histologic evidence of duodenal inflammation. We conclude that C pyloridis is associated with primary antral gastritis but not with secondary gastritis in the pediatric population.     

Intraluminal duodenal diverticulum in a child: incidental onset possibly associated with the ingestion of a foreign body

Funnel-type intraluminal duodenal diverticulum (windsock web) is a rare congenital malformation. A 4-year-old boy with vomiting and abdominal pain for several weeks was referred to the hospital. A plain abdominal X-ray on admission disclosed a double bubble sign. Abdominal echography and CT disclosed a foreign body lodged in the alimentary tract. After the foreign body was removed with a fibrescope, endoscopy showed a stenotic descending portion where the foreign body was located. An upper gastro-intestinal contrast study demonstrated a post-bulbar duodenal stenosis with a barium-filled pear-shaped sac in the descending portion of the duodenum. Surgical exploration was done under the diagnosis of windsock web of the duodenum. A simple excision of the web at its base was carried out. A hole 7 mm in diameter was found at the edge of the web. The microscopic appearance of the resected specimen was characterized by the duodenal mucosa with an extensive chronic inflammation lining both sides of the diverticulum and the lack of muscular layer of mucosa. Conclusion If an ingested material is not excreted in the stool, possible clogging in the intestinal tract should always be considered and a further intensive examination is warranted. Received: 2 September 1997 / Accepted in revised form: 1 July 1998     

原发性小肠淋巴管扩张症2例并文献复习

目的 探讨小肠淋巴管扩张症的临床表现，诊断、治疗及预后等。方法 2例患儿除一般的血清学、尿液、超声心动图、腹部B超和腹部CT等检查外，均接受胃镜和（或）小肠镜检查并取黏膜行病理活检。结果 例1，5岁发病，以反复水肿起病，小肠镜检查发现空肠、回肠弥漫性白色粟米样改变，病理提示肠黏膜固有膜内及黏膜肌层内可见到较多扩张淋巴管，淋巴管内皮细胞增生不明显。例2，5月龄发病，以水肿、腹泻起病，胃镜检查发现十二指肠黏膜弥漫性白色粟米样改变，正常绒毛结构消失，病理提示个别绒毛黏膜内可见轻度扩张淋巴管。2例患儿均除外引起小肠淋巴管扩张的继发性因素，结合起病年龄小，考虑原发性小肠淋巴管扩张症，其中例1诊断为小肠弥漫性淋巴管扩张。这2例患儿通过中链三酰甘油乳剂治疗后，随访症状明显缓解。结论 小肠淋巴管扩张症在儿童中可能并不少见。对于反复低蛋白血症、反复水肿或慢性腹泻的患儿要警惕原发性小肠淋巴管扩张症，应尽早行内镜及病理活检，并通过特殊的饮食控制疾病。     

Gastroduodenal endoscopic-histologic correlation in pediatric patients

The histopathologic abnormalities in endoscopic biopsies from the stomach and duodenal bulb were correlated with the visual findings of upper gastrointestinal endoscopy in 94 children (mean age, 8.4 years; 51 boys and 43 girls) with a variety of complaints. Histology was graded by observers blinded to the endoscopic findings, and both endoscopy and histology were graded using scales reflecting increasing severity with increasing grade. In all three locations studied (gastric body, antrum, and duodenal bulb), endoscopic grade was significantly higher than the histologic grade. Correlation was especially poor with mild endoscopic findings such as erythema and granularity/nodularity, which had little predictive value for histologic inflammation. Few patients had severe disease with eight of 94 having ulcer by endoscopy and seven of 94 having greater than grade 2 histologic disease in any location. We conclude that endoscopy without biopsy should not be used to diagnose gastroduodenal inflammation in pediatric patients.     

Intraluminal duodenal diverticulum in a neonate

Intraluminal duodenal diverticulum (IDD) is a very rare entity in the neonate. We present an unusual case in which the diverticulum was located in the first portion of the duodenum. Although the infant had symptoms similar to hypertrophic pyloric stenosis, an IDD was suspected preoperatively from upper gastrointestinal radiography. Total excision of the diverticulum was performed and the patient has been well. The microscopic appearance of the resected specimen revealed normal duodenal mucosa lining the inside and outside of the sac.     

十二指肠霜斑样溃疡的组织学观察

目的　探讨儿童十二指肠霜斑样溃疡的性质及临床病理特征。方法　观察 117例十二指肠霜斑样溃疡的内镜表现 ,对其胃窦部及十二指肠球部标本均行HE染色 ,观察病理改变 ,Giemsa染色诊断HP感染 ,十二指肠黏膜兼作AB(pH值 2 5 ) /PAS染色 ,观察十二指肠胃上皮化生 ,并进行治疗随访。结果　 117例霜斑样溃疡均表现为在充血、水肿的黏膜面有点状或片状的白色霜斑苔覆盖 ,检出率占同期 5 10 6例内镜检查数的 2 2 9% ,同期 2 38例十二指肠溃疡的 4 9 2 %。病理形态学以淋巴细胞、浆细胞及中性粒细胞浸润为主 ,常伴有浅表糜烂。病理诊断为慢性活动性十二指肠炎 6 1例 ,慢性十二指肠炎 5 0例 ,嗜酸性十二指肠炎 6例。 6 1例慢性活动性十二指肠炎中伴有浅表糜烂 4 5例。十二指肠霜斑样溃疡中窦部HP检出率 5 8 1% (6 8/ 117) ,球部HP检出率 11 1% (13/ 117) ;球部胃上皮化生检出率 31 1% (37/ 117) ;均明显高于同期球部正常及慢性十二指肠炎标本 (P均 <0 0 0 1)。 2 1例经抑酸或加抗菌治疗后 4周内镜复查 ,病变愈合 ,无瘢痕形成。结论　儿童十二指肠霜斑样溃疡是十二指肠炎症或糜烂的一种特殊表现 ,不是真正溃疡 ,其发生与窦部或球部HP感染、胃酸分泌增加有关 ,治疗以抑酸抗菌为主 ,预后良好     

Acute interstitial nephritis in T‐cell leukemia in a pediatric patient

Acute lymphoid leukemia is the most frequently occurring malignancy in childhood, but acute tubulointerstitial nephritis with associated acute renal failure as the leading manifestation of leukemia is extremely rare. Only a few pediatric cases have been described in the literature. We present a surprising case in which physical examination and initial investigation were not typical for leukemia. Ultrasound showed only modest kidney enlargement while laboratory results indicated acute renal failure. Renal biopsy indicated tubulointerstitial nephritis, and subsequent steroid treatment led to sudden clinical improvement. One month later, however, the patient returned with typical clinical features of leukemia. Re‐evaluation of the original kidney biopsy block indicated T‐cell acute lymphoid leukemia. The present case highlights the importance of renal biopsy.     

Life‐threatening acute gastric dilatation with aorta compression in a 3‐year‐old child

We report a fatal case of acute gastric dilatation in a 3‐year old boy who presented with severe abdominal pain and massive gastric distension in the emergency room. On physical examination the patient was in shock due to acute abdomen and lower limb ischemia. Initial laboratory findings showed multi‐organ failure with acute renal failure and pancreatitis. Abdominal computed tomography (CT) showed marked dilatation of the stomach resulting in compression of the abdominal aorta. The left kidney, spleen and pancreas were not visible on CT due to the necrotic changes. The patient was quickly stabilized by initial volume resuscitation, but suddenly deteriorated immediately after gastric decompression via nasogastric tube, and died from multi‐organ failure 3 h after initial presentation.