Esophageal atresia with double tracheoesophageal fistula--a case report and review of the literature.

Esophageal atresia with double tracheoesophageal fistula (TEF) is a very rare anomaly, and the accurate preoperative diagnosis of proximal TEF is very difficult. This paper describes a baby girl who presented with esophageal atresia with double, proximal, and distal TEF. The distal TEF was diagnosed before operation, whereas the proximal TEF was found intraoperatively. Overlooking the presence of proximal TEF can lead to increased morbidity and mortality due to severe respiratory infection and the necessity of a second operation. Great care must therefore be taken to not overlook the presence of proximal TEF in patients with this anomaly.

Effects of omega-3 fatty acids in rat allogenic small intestinal transplantation.

The aim of this study was to estimate the effect of omega-3 fatty acids on the recipient and graft immune response after rat allogenic small intestinal transplantation. Seven-week-old Lewis rats were randomly assigned to one of three groups according to the diet received: an FO group (fish oil supplemented), an SB group (soy bean oil supplemented) or a control group (normal rat chow). The recipient Lewis rats were each given their respective group diet for 12 days, and then, on the 19th day of gestation, a 2 cm jejunum from the donor fetal Fischer rat was transplanted into the abdominal wall of the recipient rats using a non-vascular anastomotic technique. The recipient rats were killed on day 2 after transplantation, and the recipient plasma IL-2, IFN-gamma and IL-1 beta levels were determined. In addition, the histological findings of the graft were analyzed. The cytokine levels of the FO group were significantly lower than the other two groups. In order to determine the grade of rejection, the morphological findings were blindly graded on a scale of 0-4. The mean grade of the FO group was also significantly lower than the other two groups. Omega-3 fatty acids are therefore considered to have an immunosuppressive effect on rat allogenic small intestinal transplantation based on the recipient plasma IL-1 beta, TNF and IL-2 levels and the histological findings of the grafts.

Outcome of fetal cystic hygroma and experience of intrauterine treatment.

OBJECTIVE: To review our cases of fetal cystic hygroma and to examine the prognostic factors with the goal of establishing criteria for the intrauterine treatment for cystic hygroma. PATIENTS AND METHODS: Thirty-one cases of fetal cystic hygroma were managed by us from January 1988 to December 1997, and 21 cases were available for analysis. Three prognostic factors, namely chromosomal abnormality, structural anomaly and hydrops fetalis, were evaluated. We treated 2 cases of cystic hygroma associated with hydrops fetalis in utero using OK-432 injection under ultrasound guidance. RESULTS: The fetuses without any of the prognostic factors listed above showed a good prognosis throughout the fetal and neonatal periods. However, in this group, 2 infants with large tumors died of hemorrhage from the tumor at 6 months and 3 years of age, respectively. Cases with hydrops fetalis without chromosomal abnormalities or structural anomalies (5 cases) resulted in either intrauterine fetal death (IUFD, 2 cases) or early perinatal neonatal death (early PND, 3 cases). The cause of early PND was circulatory failure. Most of the hydrops cases with either a chromosomal abnormality or structural anomaly resulted in IUFD before 22 weeks of gestation. The size of the cyst decreased in 1 of 2 cases treated in utero. CONCLUSIONS: The fetal cases of cystic hygroma showing hydrops fetalis without chromosomal abnormalities or structural anomalies are considered to be possible candidates for intrauterine therapy. Those with very large cystic hygroma without any of the three prognostic factors are also thought to be candidates for fetal treatment. Based on our clinical experience, sclerotherapy using OK-432 is considered to be a treatment option in selected cases with fetal cystic hygroma.

The efficacy of predeposited autologous blood transfusions in general pediatric surgery.

Allogeneic blood transfusions are associated with a risk of infection, immunological reactions, immunosuppression, and the induction of antibodies in blood cells. We report our results of giving predeposited autologous blood transfusions (PABT) to children when it was anticipated that transfusions would be required for an elective operation. Autologous blood was collected for deposit from 16 patients ranging in age from 1 to 11 years old (mean 5.6 years old, mode 4 years old), and weighing from 9.7 to 42 kg (mean 20.8kg). They included 12 patients with pectus excavatum (funnel chest) and 4 patients with choledochal cyst (CBD). Blood was collected once from 2 patients and twice from the other 14 patients, then centrifuged and stored in a freezer at -80 degrees C. Between 7 and 14 ml/kg was collected at one time, the total mean volume of predeposited blood being 21.0 +/- 3.3 ml/kg for the children operated on for funnel chest, and 16.2 +/- 4.5 ml/ kg for those operated on for CBD. None of the patients required allogeneic transfusions and no complications occurred. PABT was found to be a safe and effective means for elective general pediatric surgical procedures for avoidance of allogeneic blood transfusion.

The validity of bioelectrical impedance phase angle for nutritional assessment in children.

BACKGROUND/PURPOSE: Bioelectrical impedance analysis (BIA) is a quick and noninvasive method for estimating body composition. Many prediction equations have been reported recently using bioelectrical impedance to calculate fat free mass (FFM) and fat mass (FM). These equations are based on the assumption that the composition and density of FFM are stable. In children, the composition and density of FFM vary according to age and clinical state, so the use of these equations is limited. However, phase angle is directly determined from resistance (Rz) and reactance (Xc) without equations and reflects body cell mass. The authors, therefore, investigated the validity of phase angle for nutritional assessment in children. METHODS: Bioelectrical impedance analysis and anthropometric measurements were performed in 81 patients, including 71 well-nourished and 10 malnourished children. RESULTS: Phase angle correlated with body weight (R = 0.818) and arm muscle circumference (r = 0.901) in well-nourished children. Malnourished patients showed lower phase angle than that of well-nourished children. CONCLUSION: Bioelectrical impedance phase angle is a useful parameter for nutritional assessment in children.

Transport of 1,5-anhydro-D-glucitol into insulinoma cells by a glucose-sensitive transport system.

The uptake of 1,5-anhydro-D-glucitol (1,5-AG) occurs by passive mechanisms in cells or tissues that have passive glucose transporters. It is known that serum 1,5-AG concentrations are reduced in patients with diabetes mellitus. To elucidate the metabolism of this substance and its physiological role in pancreatic beta-cells, we assayed 1,5-AG transport in the insulinoma-derived cell lines, RINr and MIN6. Both cell lines showed an insulin-insensitive, concentration-dependent uptake of 1,5-AG with a saturation time of approximately 120 min, and most of the 1,5-AG in the cytoplasm was in the free form. A biphasic saturation curve was obtained using a wide range of 1,5-AG concentrations, suggesting that accumulation was mediated by a high affinity and a low affinity transporter. The high affinity transporter had a K(m) of 10.4 in RINr cells and 13.0 mM in MIN6 cells, and the low affinity transporter had a K(m)100 times, being much higher than the physiological concentrations of 1,5-AG. These results indicate that the 1,5-AG carrier system in insulinoma cells is distinct from that in either the somatic cells or renal tubular cells. These findings also suggest that a unique 1,5-AG transport system is present in pancreatic beta-cells.

"Gut-feeling" or evidence-based approaches in the evaluation and treatment of human short-bowel syndrome.

Short bowel syndrome is the malabsorptive state that often follows extensive resection of the small intestine. Long-term survival without parenteral nutrition depends on the process of intestinal adaptation, through which the remaining small bowel gradually increases its absorptive capacity. The process of intestinal adaptation is almost exclusively luminal nutrient dependent. To date the clinical management of short bowel patients is mostly based on a 'trial and error' regimen because human data and randomised trials using trophic substances are lacking due to the small number of patients annually present in pediatric surgical centres. We evaluate here the currently available as well as some more recently developed methods of measuring intestinal absorption and adaptation in short bowel patients. New techniques such as measurements of (1) intestinal permeability and carbohydrate absorption using the sugar absorption test, (2) gastrointestinal hormone production of gastrin, cholecystokinin and peptide YY, (3) transmural potential difference of the gastrointestinal tract using electrophysiology and (4) mucosal enzyme expression of lactase and sucrase-isomaltase using immunohistochemistry were evaluated. These new techniques are promising in monitoring the process of adaptation of the remaining intestine and evaluating the effect of therapeutic interventions in patients with short bowel syndrome.

Fetal stabilization for antenatally diagnosed diaphragmatic hernia.

BACKGROUND/PURPOSE: Infants with congenital diaphragmatic hernia have pulmonary hypoplasia resulting in persistent pulmonary hypertension of neonates (PPHN), which is the main contributor to both high mortality and morbidity. The pulmonary artery bed in patients with congenital diaphragmatic hernia (CDH) is underdeveloped and is very sensitive to slight stimuli. It is, therefore, vital to avoid any factors that might increase pulmonary vascular resistance during the perinatal treatment of these patients. Recently, fetal anesthesia for perinatal stabilization in patients with CDH has been reported. However, the efficacy of this method remains controversial. The aim of this study is to analyze the benefits of fetal stabilization using fetal anesthesia in patients with CDH. METHODS: The authors have seen 9 cases of antenatally diagnosed CDH and attempted fetal stabilization. The indication for fetal stabilization was a lung thoracic ratio of less than 0.2, without any severe associated anomalies. The protocol for fetal stabilization was (1) monitoring the fetal respiratory movement and heart beat by ultrasonography, (2) the administration of morphine (20 to 30 mg) and diazepam (5 mg) to the mother, (3) the confirmation of any interruptions in fetal movement followed by a cesarean section, (4) pancuronimum (0.5 mg) was given through the umbilical vessels, (5) intubation before clamping of the umbilical cord, and (6) high-frequency oscillatory ventilation (HFO) without bagging. RESULTS: The lung-thratic ratio (LTR) was between 0.06 to 0.17 (average, 0.10+/-0.04). Operation was performed in 7 of 9 patients at between 2.5 and 27 hours after birth. The overall survival rate was 66.7% (6 of 9). All of the patients who underwent operation within 5 hours after birth survived. CONCLUSIONS: Perinatal stabilization using fetal anesthesia was found to be effective in preventing PPHN and shortening the period of preoperative stabilization. It also improved the survival rate of patients with severe CDH.

Alterations of the intramural nervous distributions in a chick intestinal atresia model.

The postoperative intestinal dysmotility seen in intestinal atresia (IA) is usually found in association with a dilatation of the proximal intestinal segment, but the etiology of this disorder is not yet fully understood. A chick IA model was made by cutting the postumbilical midgut on d 11 in ovo. The operated chicks were euthanized 2 d after hatching. The samples were divided into two groups according to the extent of the dilatation of proximal ileal segments. Cryostat sections were processed for immunohistochemistry by the use of antisera to protein gene product 9.5, vasoactive intestinal polypeptide, substance-P, and alpha-smooth muscle actin and were also stained by NADPH-diaphorase. Tn highly dilated proximal segments, a decreased number of protein gene product 9.5-positive fibers was found in both the circular muscle and submucous layers. The number of nerve fibers positive for vasoactive intestinal polypeptide, substance-P, and NADPH-diaphorase also decreased in the circular muscle layer, particularly in the deep muscular plexus. Hypertrophy and an alteration of the staining intensities in the circular muscle layer were also revealed by a-smooth muscle actin staining. The nerve distribution of the distal segments was indistinguishable from that of the age-matched controls and the sham-operated group. Abnormalities in the intramural nerves are only found in the proximal ileal segment of the IA models. The abnormal nerve distribution of the proximal segment might thus be implicated in the postoperative dysmotility of the intestine in IA.

[Recent advances in the nutritional management of pediatric surgical patients].

The prognosis of pediatric surgical patients is significantly improved by perioperative nutritional support. However, the morbidity and mortality rates from intestinal failure, including short gut syndrome and allied disorders of Hirschsprung's disease, remain high. Recent advance in nutrition support focus on the management of intestinal failure. A central venous catheter is commonly used for long-term total parenteral nutrition (TPN) in intestinal failure, although the incidence of catheter-related complications is still high. A nutrition support team approach might reduce such complications, with the exception of deep vein thrombosis, which is another serious problem in intestinal failure after long-term TPN on which more research is needed. A other serious complication in long-term TPN is liver dysfunction. It is postulated that bacterial translocation plays an important role in TPN-associated liver damage, although so far without evidence. Both cyclic TPN and enteral feeding are known to be effective in preventing liver damage. Since we are now familiar with methods of nutritional support little attention is paid to complications. Therefore nutritional management should be reevaluated from this standpoint.

Acute glucosuria after continuous glucocorticoid loading in the rat in vivo.

We investigated the effects of the continuous infusion of various steroids in rats on renal tubular reabsorption of glucose in vivo to elucidate the pathogenesis of steroid-induced glucosuria. Urinary glucose excretion increased 60 min after administration of dexamethasone (2.38 mM). By 120 min, urinary excretion of glucose was three times higher in the dexamethasone group than in the control group (24.1 +/- 4.6 versus 72.4 +/- 16.7 micromol); the plasma level of glucose did not increase. Dexamethasone had no effect on the resorption of 1,5-anhydro-D-glucitol, which is a glucose-resembling polyol that is actively absorbed by the renal tubules as glucose. Neither estradiol nor progesterone increased urinary excretion of glucose. These findings suggest that continuous administration of a high-dose glucocorticoid selectively influences the glucose reabsorption system in the kidney.

Antenatal diagnosis and surgical management of congenital cystic adenomatoid malformation of the lung.

We experienced 12 cases of congenital cystic adenomatoid malformation of the lung (CCAM) including 6 cases diagnosed antenatally. They were classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 3), group B presented with respiratory distress symptoms after birth (n = 6), and group C showed no respiratory symptoms (n = 3). All cases of group A were lost because of hydrops and respiratory failure due to pulmonary hypoplasia. Because a compression of the mass is thought to be a cause of hydrops, this group is considered to be a good indication for fetal treatment. All cases of group B showed progressive respiratory symptoms a few days after birth which were successfully treated surgically. In 2 of 3 cases of group C, the lesions decreased in size both antenatally and postnatally. We conclude that serial sonographic evaluations for fetal CCAM are important. If the fetus develops hydrops, fetal surgery is to be considered. If not, however, fetal surgery should not be done, because some lesions can shrink in size, or even disappear, while others can be treated successfully after birth by lobectomy or even segmentectomy.

Prevention of diabetes by thymic hormone in alloxan-treated rats.

We investigated the effects of facteur thymique sérique (FTS), a thymic peptide hormone, on alloxan- and streptozotocin-induced diabetes in rats. Pretreatment with intravenous injection of FTS significantly suppressed both alloxan- and streptozotocin-induced hyperglycemia. The effects of FTS were time dependent. FTS suppressed hyperglycemia in a dose range of 1-6600 micrograms/kg. Alloxan-induced hyperglycemia was completely prevented when FTS was injected in doses of 40-50 micrograms/kg 1 min before injection of alloxan. Histological examination of islet areas showed that alloxan-induced destruction of beta-cells was inhibited by FTS. FTS had no significant effects on lymphocyte subsets and immunity-related cells or on plasma superoxide dismutase activity and total glutathione level. The blood half-life time of exogenously injected FTS was short (2-3 min), indicating acute internalization of FTS into pancreatic beta-cells. Our results suggested that FTS acutely and directly blocks some initial effect of alloxan, preventing the destruction of beta-cells.

Transport and accumulation of 1,5-anhydro-D-glucitol in the human erythroleukemia cell line K-562.

The transport and intracellular accumulation of 1,5-anhydro-D-glucitol (AG) was studied in the human erythroleukemia cell line K-562 by gas chromatography-mass spectrometry in conjunction with liquid scintillation spectrometry. K-562 cells contained 106 +/- 6 nM/10(6) cells of free AG, primarily in the cytosol. Addition of physiologic amounts of AG to the extracellular medium resulted in rapid intracellular incorporation of AG, with a half-saturation time of 5 s. Intracellular accumulation was linear for 2 h and subsequently reached saturation. AG uptake was temperature and concentration dependent with an apparent Km of 127 mM. AG uptake and accumulation was not inhibited by fructose, fucose, galactose, mannose, glucose, or 3-O-methyl-D-glucose and was less affected by cytochalasin B or phloretin than that of 2-deoxyglucose. Phloridzin did not affect AG uptake but did inhibit 2-deoxyglucose uptake. Efflux of AG from K-562 cells depended on external AG concentration alone and was not affected by extracellular glucose concentration. Intracellular AG concentration decreased rapidly and reached zero within 10 min following removal of AG from the external medium. We therefore propose that both transport and countertransport of AG in K-562 cells are mediated by a specific carrier system.

Effects of surgical orthodontic treatment for malalignment due to the prolonged retention of deciduous canines in young dogs.

This study was conducted to clarify the effects of surgical orthodontic treatment for malalignment caused by the prolonged retention of deciduous canines in young dogs. Fifty-five dogs with malocclusion or pre-malocclusion were divided into two groups. Group 1 received surgical orthodontic treatment (32 dogs) using modification of Ross and Yamane's method of treating for malalignment due to prolonged retention of deciduous canine teeth; Group 2 received no treatment (23 dogs). Occurrence of malocclusion 1-3 weeks after treatment of the group receiving surgery was only 3.8%. However, malocclusion was seen in 73.4% of the non-treatment group approximately 2 years after the first examination. Surgical orthodontic treatment was very useful in treating malalignment due to prolonged retention of deciduous canines in young dogs.

New fluorescent probes E3810 and methoxy E3810 for determining distributions of the apical membrane and the acidic compartment of gastric acid secreting cells.

Substituted benzimidazoles such as omeprazole, E3810 and methoxy E3810 were inhibitors of gastric H+, K(+)-ATPase which is rich in the apical membrane of gastric parietal or oxyntic cells at the secreting state. The acid-activated compounds of omeprazole and methoxy E3810, which have methoxy group at the 5-position in the benzimidazole ring, are fluorescent (excitation wavelength = 370 nm; emission wavelength = 560 nm). The fluorescence disappeared when the activated compounds reacted with the ATPase or glutathione. Using this fluorescence property, the distribution of the intracellular acidic canalicular space in isolated single parietal cells was determined. On the other hand, irradiation with ultraviolet light (335 nm) of the acid-activated compound of E3810 which had been reacted with sulfhydryl group of the ATPase or glutathione resulted in a formation of a fluorescent compound (emission = 470 nm). Using this second fluorescence property, we determined the distribution of the apical membrane of the intracellular canaliculus of isolated single mammalian parietal cells and also the location of the apical membrane on the external surface of newt oxyntic cells.