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This report describes the case of an ileal perforation secondary to clamping of the exomphalos minor in 3-day-old girl. Eighteen accidental clamping or cutting cases were found in the published work. It is thought that this defect resulted from either the clamping, ligation or cutting of the bowel in a small unrecognized omphalocele. These complications are extremely rare, but should be held in mind when performing an umbilical ligation.

A previous study documented the effects of maternal corticosteroid treatment on structural growth and functional development in fetal lungs associated with pathogenic conditions such as oligohydramnios using immunohistochemical and morphometric analyses. The purpose of the present study was to examine the effect of maternal dexamethasone treatment the expression of lamellar body in type II pneumocytes of the fetal rabbit lungs with hypoplasia induced by oligohydramnios using electron microscopy. Using an amniotic shunting rabbit model, pregnant rabbits were injected intravenously with either 0.1 ml of saline or 0.25 mg/kg/day of dexamethasone in 0.1 ml of saline 48 and 24 h before the delivery of fetuses, at day 30 of gestation. The number of lamellar bodies per type II pneumocyte was counted in each group using electron micrographs. The number of lamellar bodies per type II pneumocyte in the lungs of the shunted group that received saline was consistently and significantly less than that of the other three groups (5.49 +/- 2.07 vs. 7.34 +/- 2.27: shunted group that received dexamethasone, 7.58 +/- 2.08: non-shunted group that received saline, 7.79 +/- 1.90: non-shunted group that received dexamethasone; P < 0.01). These results suggest that maternal dexamethasone treatment accelerates the maturation of the surfactant system, especially the expression of lamellar bodies in type II pneumocytes, even in hypoplastic lungs induced by oligohydramnios.

10年間に当科で異物誤飲, 誤嚥の診断または疑いで受診した118症例について検討を行った. このうち異物誤飲, 誤嚥と診断されたのは小児群 (15歳以下) 47例, 成人例 (16歳以上) 18例であった. 異物なしと診断された症例は小児群51例, 成人群2例であった. 消化管異物, 気道異物ともに乳幼児と高齢者に多い傾向を認めた. 消化管異物では小児群はボタン型電池, 硬貨などX線非透過性異物が多く, 成人群は義歯とPTP (press through pack) が多かった. 診断は比較的容易で, 合併症もみられなかった. 気道異物では小児群はピーナッツを含む豆類が多く, そのほとんどがX線過性異物であったが, 成人群では歯科用金属が多く, ほとんどがX線非透過性異物であった. 小児のピーナッツ症例は診断までに時間がかかり, 摘出後も重篤な合併症をきたしていた. 異物なしと診断された症例の中に, X線透過性異物が診断できなかった症例が含まれている可能性が考えられた.

We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks' gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks' gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.

An unusual case of biliary atresia with jejunal atresia is herein described. Only 12 cases demonstrating biliary atresia associated with a jejunal atresia have been previously reported in Japan. The pathogenesis of biliary atresia is thought to be secondary to the influence of jejunal atresia.

We report a case of coexistent congenital cytomegalovirus (CMV) infection with pseudo-Hirschsprung's disease in a female infant born at 39 weeks' gestation. The serologic data, urinary viral culture, and gene expression by in situ hybridization all suggested a CMV infection, and the pathologic findings of resected bowel specimens showed aganglionosis. Thus, congenital CMV infection may be etiologically associated with Hirschsprung's disease in some children.

Morphometric analyses of the immunohistochemical expression of surfactant apoprotein A (SP-A) were carried out on the bronchioles of human congenital diaphragmatic hernia (CDH) neonates and then compared with those in a gestational and postnatal age-matched control group. There was no difference in SP-A expression between lung specimens of the control group and unaffected lungs in the CDH group. However, compared with both these lungs, the ipsilateral lungs of the CDH group showed a significant increase in SP-A expression, namely, the number of SP-A-positive cells per bronchiole, the number of SP-A-positive cells per unit perimeter of bronchiole, and the number of SP-A-positive cells per unit bronchiolar surface area. These results suggest that in lungs of CDH cases, especially ipsilateral lungs, there is a possible delay in the functional maturation or development of SP-A synthesis by the bronchiole, and this retardation may play a role in the postnatal respiratory insufficiency observed in CDH patients.

子宮腟留血腫を呈した処女膜閉鎖症(imperforate hymen: 以後IHと略記)の1例を経験したので報告する.患者は腹痛を主訴とする12歳,女児.卵巣嚢腫疑いで受診.外陰部視診で膨隆し,閉鎖した処女膜が認められた.腹部超音波検査, CTおよびMRIでは子宮・膣腔が拡張していたが,他の泌尿生殖器の異常は認められなかった.処女膜十字切開術を施行したところ約600mlの貯留血の排泄をみた.術後は癒着による再閉鎖などもみられず経過良好であった. 思春期発症のIH本邦報告例35例を集計した. IHは稀な疾患であり,腹部腫瘤として思春期の女性で初潮のない場合は,鑑別診断の一つとして念頭に置き外陰部の診察を必ず行うことが大切である.

Morphometrical analyses of the immunohistochemical expression of bombesin, which is one of the peptides produced by pulmonary neuroendocrine (PNE) cells, were carried out on the bronchioles of human congenital diaphragmatic hernia (CDH) neonates, and the findings were then compared with those in a gestational and postnatal age-matched control group. As a result, no difference was found in the number of bombesin-positive cells between the lungs of the control group and the unaffected side lungs in the CDH group except for the ratio of the bombesin-positive cells per unit of the bronchiolar surface area (P < 0.05). However, compared with the lungs in the control group, the affected side of the lungs in the CDH group showed a significant increase in the expression of bombesin, namely, the ratio of the bombesin-positive cells per bronchiole (P < 0.05), the ratio of the bombesin-positive cells per unit perimeter of the bronchioles (P < 0.05), and the ratio of the bombesin-positive cells per unit of the bronchiolar surface area (P < 0.01). These results thus suggest that hyperplasia of the PNE-cell system in the lungs of the CDH cases, especially on the affected side, exists in human fetuses. We also further speculate that PNE cells may thus play a role in the problems associated with CDH during intrauterine life in human beings.

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and persistent pulmonary hypertension. Recent experimental studies suggest that surfactant deficiency may also contribute to CDH pathophysiology. In this report, the authors immunohistochemically and morphometrically examined whether or not the hypoplastic lungs of CDH are associated with the immaturity of the surfactant system, especially alveolar type II cell function. Nine autopsy cases with CDH were immunohistochemically examined for the expression of surfactant apoprotein, using anti-IgG against human surfactant apoprotein A (SP-A), and the findings were compared with those in a gestational and postnatal age-matched control group. The lung/body weight ratio in the CDH was less than that in the controls (0.010 +/- 0.005 versus 0.021 +/- 0.013, P < .01). The radial alveolar count (RAC) of the CDH cases were also significantly less than that of the control cases (2.10 +/- 0.52 versus 3.48 +/- 0.39, P < .01). In the CDH cases, the RAC of the lung on the affected side were also significantly less than that of the lung on the unaffected side (1.71 +/- 0.34 versus 2.50 +/- 0.26, P < .01). In the immunohistochemical distribution of SP-A, compared with the control cases, the number of SP-A-positive cells on the alveolar septa of the CDH cases decreased in number, and this immunohistochemical reaction was weak even in positive type II cells. In addition, the immunoreaction observed in the alveolar type II cells of the unaffected side lungs in the four CDH cases was stronger than that of the unaffected side lungs. These results suggest that in the lungs of the CDH cases, especially on the affected side, there is a possible delay in both the structural growth and functional maturation or development of SP-A synthesis by alveolar type II cells, and this retardation of the functional maturation in alveolar type II cells is also considered to play a role in postnatal respiratory insufficiency in CDH patients.

Cholelithiasis is an extremely unusual finding in infancy. The following article describes the case of a 2-month-old male with a VACTER association who presented with persistent and progressive obstructive jaundice and acholic stool due to cholelithiasis. Thirty cases under the age of 1, including our case, have previously been reported in the Japanese literature. Twenty-eight cases had predisposing factors. The calculi were present only in the gallbladder in 18 cases, in the common bile duct or cystic duct or both in 7 cases, and in the gallbladder and common bile duct in 3 cases. Ten cases of stones were radiopaque, which thus made the plain abdominal roentgenogram findings very valuable. Nine cases underwent operation including cholecystectomy in 3 cases, choledocholithotomy in 3 cases, and cholecystolithotomy in 1 case, while the procedure was unknown in 2 cases. Recently, the number of reported cases of cholelithiasis in infants has gradually increased and today these cases are most often diagnosed by ultrasonography, because the examination is easy to perform and not invasive.

An unusual case of imperforate anus with sigmoid colonic atresia and ileal stenosis is described. Only six cases of imperforate anus associated with colonic atresia have previously been reported in Japan. The possibility of other alimentary obstructions should thus be considered when anorectal anomalies are observed.

Aim: The present study was carried out using electron microscopy to evaluate the expression of lamellar bodies in type II pneumocytes of fetal rabbit lungs with hypoplasia induced by oligohydramnios. Methods: Using an amniotic shunting rabbit model, the lungs obtained from 32 fetuses, including 16 experimental fetuses (shunting group) and 16 control fetuses (control group), were used for electron microscopic studies. At least 250 type II pneumocytes per fetus were photographed. The number of lamellar bodies per type II pneumocyte was counted. Results: The number of lamellar bodies per type II pneumocyte of the shunting group was consistently and significantly less than that of the control group (5.49 ± 2.07 vs 7.58 ± 2.08; P

MethodsWe performed a urinary tract obstruction (UTO) surgical procedure at 93-107 days' gestation in lambs to investigate the relationship between pulmonary hypoplasia and the appearance of pulmonary endocrine cells by quantitative analysis of respiratory tract cells using light microscopic immunohistochemistry.ResultsUTO produced a significant reduction in lung weight, lung/body weight ratio, air capacity, air capacity/body weight ratio (p < 0.01) and radial alveolar count (p < 0.05), which indicated the presence of lung hypoplasia. These foetuses also showed a significant increase in the number of neuron-specific enolase (NSE)- positive pulmonary endocrine cells, expressed as the number of NSE-positive cells per bronchus (p < 0.01) or bronchiole (p < 0.05), the number of NSE-positive cells per unit perimeter of bronchus or bronchiole (p < 0.01), and the number of NSE-positive cells per unit bronchial or bronchiolar surface area (p < 0.01).ConclusionThese results suggest that UTO significantly retards and modifies the structural growth and functional development of pulmonary endocrine cells in NSE expression. We speculate that pulmonary endocrine cells and their mediators may play a role in the problems associated with UTO during intrauterine life.

A 2-month-old female infant with a peritoneal loose body presenting as an abdominal mass and urinary tract infection is described. Peritoneal loose bodies are usually found in the pelvis of adult patients. A peritoneal loose body should be considered in the differential diagnosis of a mobile lesion found in the abdomen.

A case of transverse colon volvulus in a child with mental retardation and epilepsy is described. Previously reported cases in Japanese children are reviewed. A 540 degrees, counterclockwise volvulus of the transverse colon caused ischemia requiring resection. Possible factors related to pathogenesis are discussed, and diagnostic and therapeutic measures are outlined. Volvulus of the transverse colon is extremely rare in children.

A case of congenital intraspinal neuroblastoma with paraplegia in a newborn is described, and a review of previously reported cases is presented. Calcification within the spinal canal and abdominal mass was noted. At age 75 days, the patient underwent a resection of almost the entire retroperitonial tumor. A magnetic resonance scan showed hepatic metastasis, and chemotherapy was changed to a more intensive regimen. The liver metastasis disappeared; however, neurological signs persisted. A literature review of 38 cases showed 26 patients had a laminectomy and 12 patients did not. Treatment with or without laminectomy was associated with a poor prognosis for neurological recovery, suggesting cord compression must occur antenatally, and irreversible damage occurs before birth. Congenital intraspinal neuroblastoma has a relatively good prognosis (survival rate, 91.9%), although complete neurological recovery is highly unusual. Based on these findings, the authors conclude that a laminectomy is not indicated for those patients.