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Background/aim: Although the mucosectomy-commencing points on transanal endorectal pull-through (TAEPT) differ among reports, the optimal point is unclear. This study assessed the outcomes among different mucosectomy-commencing points., Methods: We conducted a nationwide survey from 2008 to 2012. The data of 1,087 Hirschsprung's disease patients were collected, and data on those who underwent TAEPT were extracted. The patients were divided according to the mucosectomy-commencing points into two groups: in Group A, mucosectomy was started ≥5 mm from the dentate line (DL), and in Group B, mucosectomy was started <5 mm from the DL. The extent of the aganglionic segment and postoperative complications in the month after TAEPT were compared., Results: The data of 327 patients were extracted (Group A, n=155; B, n=172). Aganglionosis extending to the sigmoid colon was the most frequent in both groups. Regarding postoperative complications, the patients of each group experienced enterocolitis (Group A: 8.4%; B: 7.6%) and incontinence (A: 3.9%; B: 2.9%). The incidence of rectal mucosal prolapse was significantly greater in Group B (4.1%); (A: 0%, p=0.02)., Conclusions: Although the outcomes of TAEPT were comparable in both groups, rectal mucosal prolapse was significantly frequent in patients in whom the commencing point was <5 mm from the DL., Type of Study: Retrospective study LEVEL OF EVIDENCE: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: Hirschsprung's disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz's band. The condition is reported be associated with a high mortality rate of 50-80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan., Methods: Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan., Results: A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel's procedure in one). Ziegler's myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes., Conclusion: HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.

Background: The Canadian Paediatric Triage and Acuity Scale (P-CTAS) is used and modified at hospitals as a triage tool for pediatric patients before they are seen in emergency rooms. Pediatric surgery patients account for very few of the many patients in emergency departments, but they should be triaged as emergency or urgent because they might be candidates for surgery. Problems with and improvements for triaging pediatric surgery patients using the P-CTAS were studied., Methods: This retrospective study evaluated all patients <16 years old who visited the emergency department of Kyorin University Hospital during an approximately 4 year period between 1 May 2005, and 11 February 2009. Pediatric surgery patients were divided into two groups to evaluate the efficiency of P-CTAS triage. Patients who needed emergency treatment were in group A, and the others were in group B., Results: Most group A patients were level I, II, or III (97%, 111/114). In contrast, 60% (71/119) of group B patients were level IV or greater. Some problems with and suggestions for the P-CTAS were identified. Many patients with trauma were under 1 year of age, and many with a foreign body were under 2 years of age. Age categories should be added for patients with trauma or foreign body aspiration. Patients with abdominal pain, and without anal bleeding or vomiting who are >2 years old are triaged as level IV and they accounted for 12% of patients with possible intussusception in this study. A category of 'possible intussusception' should be made for level II. Most patients with acute scrotum, whether operated on or not, were level III. 'Red or purple color of scrotal skin' and/or 'within 6 h from onset' could be added to level II for patients with acute scrotum., Conclusions: P-CTAS worked well for pediatric surgery patients, and it needs to be modified and improved for such patients based on these results., (© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.)

The lectin-binding patterns in Auerbach's plexus in the distal portions of the rat colon from 15- to 21-day-old foetuses, newborns, and adults were examined by light and electron microscopy using 16 different lectins (ConA, RCA-1, WGA, PNA, SBA, UEA-1, DBA, LCA, PHA-L, DSA, GS-1, VVA, MPA, BPA, MAA, and PSA). The binding of ConA was shown to increase after day 19 of gestation in parallel with differentiation of Auerbach's plexus, whereas the staining intensity for DSA and RCA-1 increased after day 17 of gestation in accordance with the appearance of the plexus. At the electron microscopical level, DSA binding sites were observed to be localized mainly in the plasma membrane, Golgi apparatus, and nuclear membrane of nerve cells. Positive sites were also observed in the axolemma and in the plasma membrane of nerve cell processes, Schwann cells, and the surrounding smooth muscle cells. PSA, PHA-L, LCA, and WGA showed constant staining during the development after day 15 of gestation. Other lectins, most of which are specific for O-glycosidic mucin-type sugar residues, were essentially negative throughout the developmental stages. Moreover, N-glycanase digestion significantly diminished the positive reactions. N-linked oligosaccharides may thus play important roles in the development and maturation of the Auerbach's plexus, and may be involved in the developmental defect of the plexus, e.g. as occurs in Hirschsprung's disease.

To clarify the pathogenesis of excessive proliferation of extrinsic nerve fibers in the aganglionic colon of patients with Hirschsprung's disease (HD), we immunohistochemically determined the role that exocytosis-related proteins play in the regulation of exocytosis using the antibody to HPC-1/syntaxin 1A, an exocytosis-related protein. Localization of exocytosis-related proteins (HPC-1/syntaxin 1A, N-ethylmalemide-sensitive fusion protein (NSF), soluble NSF attachment protein (SNAP), synaptotagmin, synaptobrevin, and synaptosome-associated protein 25 (SNAP-25)) was determined in surgical specimens obtained from normal proximal and aganglionic distal segments of the colon of 7 infant patients with HD. In the normal ganglionic colon, Auerbach's plexus, Meisner's plexus, nerve fibers in the muscle layer, and ganglion cells were immunopositive for all six kinds of antisera. In the aganglionic segments, numerous proliferated nerve fibers and hypertrophied nerve bundles were detected in the submucosal layer and myenteric layer by NSF, SNAP, synaptotagmin, synaptobrevin, and SNAP-25. However, HPC-1/syntaxin 1A was not recognized in the proliferated nerve fibers of the submucosal layer or the hypertrophied nerve bundles of the aganglionic segment. These findings show that immunoreactivity of HPC-1/syntaxin 1A was decreased in the affected bowel segments of patients with HD and may be related to the pathogenesis of extrinsic nerve-fiber proliferation in the aganglionic colon of HD.

A short-segment Hirschsprung disease (HSCR) patient associated with 21 trisomy showing point nucleotidic changes in both the receptor tyrosine kinase (RET) proto-oncogene and the endothelin-B receptor (EDNRB) gene is reported. A T to A heterozygous transition at the splicing donor site of the intron 10 in the RET proto-oncogene, and a G to A heterozygous substitution in non-coding region in the exon 1 of the EDNRB gene were observed. The familial analysis with these genes revealed that the origin of the former mutation was de novo and the latter one was maternal. No patient has been reported with two points mutations in different pathogenetically susceptible loci for HSCR. There is genetic evidence that the RET and EDNRB genes may interact in their susceptibility leading to HSCR.

A 5-year-old girl with congenital central hypoventilation syndrome associated with Hirschsprung's disease (Ondine-Hirschsprung syndrome) representing a missense mutation in exon 12 of the receptor tyrosine kinase (RET) proto-oncogene is reported. Using a direct sequencing technique, genomic DNA obtained from the patient's peripheral leukocytes was analyzed for its nucleotide sequences in all 20 exons of the RET proto-oncogene, seven regions of the 1st to the 7th exon of the endothelin-B receptor gene and endothelin 3 gene, including sequences corresponding to proteolytic cleavage sites. The analysis revealed that adenine at the 2116th base in the 12th exon in the RET proto-oncogene was substituted by guanine, supposedly resulting in a mutation of Thr 706 to Ala. No other mutational change was observed in the gene examined in this case. Mutation analysis has not been described previously on the gene in this disease complex. Mutation in this case might impair the maturation of the tyrosine kinase protein and subsequently cause neurocristopathy supposedly originating from the neural crest.

A large trichobezoar has been traditionally removed by open surgery, which has entailed an upper abdominal incision. With the advent of laparoscopic surgery, it became feasible to retrieve a foreign body from the stomach without the necessity of a large skin incision in the upper abdomen. A 7-year-old girl presenting with abdominal pain, nausea, and appetite loss was admitted and evaluated. Results of an upper gastrointestinal series showed a large mass in the stomach that extended into the duodenum. Endoscopical removal had been tried twice under general anesthesia and resulted only in the retrieval of the small portion of the trichobezoar in the duodenum; total removal seemed impossible with endoscopic techniques. Laparoscopic removal was then under-taken to avoid the surgical scar in the upper abdomen. The trichobezoar was successfully retrieved through a gastrotomy and removed via a small suprapubic incision. This approach may be the treatment of choice for future cases of trichobezoar when surgery is indicated.

The HPC-1/syntaxin-1A antigen was originally identified as a neuron-specific membrane protein in the central nervous system. The presence of HPC-1 antigen in the nervous system of the fetal rat gastrointestinal tract was immunohistochemically demonstrated using the antibody against HPC-1 to clarify the role of this protein in the development of the enteric nervous system. Rat gastrointestinal tract from 14-, 16-, 18-, and 20-day fetuses and adults were immunohistochemically examined for HPC-1 antigen by light microscopy. Acetylcholinesterase (AchE) activity was also examined as a comparison. HPC-1 activity was first detected on 18th day of gestation. AchE activity was first detected at the Auerbach's plexus of the esophagus on the 16th day of gestation. The presence of HPC-1 in the developing rat intestine revealed that the HPC-1 antigen may be a good indicator for expressing the maturation of enteric nervous system in the development of the enteric nervous system.

Prenatal diagnosis of surgically correctable fetal anomalies is increasing with the improvement of ultrasonographic techniques. In spite of theoretical advantages, the mortality rate in cases of CDH presenting in the early hours of life has remained high. We have experience of two consecutive cases of CDH, diagnosed prenatally. With maternal transport, planned delivery by Cesarean section, immediate resuscitation of the babies after birth, and prompt surgical intervention by pediatric surgeons, the babies were successfully managed even though they had severe hypoplasia of the left lung. Other reported cases of CDH, have died in the neonatal period mainly from associated lethal anomalies and contralateral lung hypoplasia and in spite of prenatal diagnosis and early surgical intervention. Most of these cases belong to group 3 in Ein's classification. Cases belonging to group 3 are virtually impossible to save with current medical techniques. The effects of prenatal diagnosis on the survival rate in CDH have been discouraging in those series which include these types of cases. The advantage of prenatal diagnosis and early surgical intervention are obvious in groups 1 and 2. Our cases show the importance of cooperation between obstetricians, neonatologists and pediatric surgeons in the prenatal and postnatal management of babies with CDH.

This paper introduces a new operation for biliary atresia that establishes successful biliary drainage by extending the portahepatic dissection, and decreases ascending cholangitis by incorporating a nonrefluxing intussuscepted draining conduit. An analysis of the postoperative results is presented. The usual dissection of the portahepatis is extended to include the area between and beneath the branches of the right portal vein to incorporate all potentially usable remnants of the intrahepatic ducts. Ascending cholangitis is decreased by interposing an intussuscepted ileocolic segment between the portahepatis and the abdominal wall. This extended dissection of the portahepatis was performed since 1978 in 15 infants with noncorrectable biliary atresia, and bile drainage was achieved in all. In ten infants an intussuscepted ileocolic conduit was constructed. Ascending cholangitis in these ten infants has been either completely absent or easily controlled by antibiotics. The draining bile was highly concentrated due to the water-absorbing capacity of the interposed colonic segment; therefore, fluid and electrolyte disturbances, which develop frequently in patients having jejunal conduits, have never been encountered.

Effects of myocardial hypertrophy caused by pressure overload on sarcolemmal Na+,K+-ATPase and positive inotropic action of strophanthidin were examined in cats. Partial ligation of the main pulmonary artery for four weeks resulted in right ventricular hypertrophy with no significant changes in left ventricular muscle. Hypertrophy was associated with a reduction in the number of active Na+,K+-ATPase units. Affinity of the remaining enzyme for [3H]ouabain was unchanged. No apparent right or left shift in dose-response curve for the positive inotropic effect of strophanthidin was observed and toxic concentrations of strophanthidin were unchanged; however, the degree of the positive inotropic effect produced by high concentrations of strophanthidin was significantly smaller in hypertrophied muscle. Moreover, decreases in developed tension rather than tachyarrhythmias was the predominant form of toxicity observed in hypertrophied muscle. These results indicate that myocardial hypertrophy reduces the number of active Na+,K+-ATPase units per milligram protein, decreases maximal positive inotropic effect of strophanthidin, and alters the prevailing form of strophanthidin toxicity.

In order to assess abnormalities in the adrenergic mechanism in the intestine of Hirschsprung's disease, catecholamine concentrations, alpha-adrenoceptors, and the effect of electrical field stimulation were examined in aganglionic segments of colon or rectum. The aganglionic segment had a higher concentration of norepinephrine, assayed with high performance liquid chromatography with an electrochemical detector, whereas concentrations of epinephrine or dopamine were similar in normal and pathological segments. In four patients with extensive aganglionosis, the norepinephrine concentration in aganglionic colon segments decreased progressively in descending, transverse, and ascending colon. The tissue content of alpha-adrenoceptors and their affinity assayed from the specific binding of [3H]dihydro-alpha-ergocryptine appeared similar in normal and aganglionic segments of the rectosigmoidal colon. Electrical field stimulation of normal rectosigmoidal colon segments caused relaxation at low frequencies and contraction at a very high frequency. Relaxation was not abolished by blocking concentrations of propranolol or phentolamine. In aganglionic segments, the predominant response to electrical field stimulation was contraction, which was inhibited by either atropine or tetrodotoxin. These results indicate that an alpha-adrenergic system and cholinergic innervation apparently exist in aganglionic colon segments and that dysfunction of the colon appears to result from lack of a nonadrenergic inhibitory system.

The role of an endogenous inhibitor of Na+,K+-ATPase in hypertension observed in one-kidney NaCl-loaded rats treated with deoxycorticosterone (DOC) was examined. Ouabain or digitoxin, an exogenous inhibitor of Na+,K+-ATPase, failed to cause hypertension in one-kidney NaCl-loaded rats without DOC treatment or one-kidney DOC-treated rats without NaCl loading. Moreover, neither ouabain nor digitoxin acted additively with a putative endogenous inhibitor of Na+,K+-ATPase to augment hypertension observed in one-kidney NaCl-loaded rats treated with DOC. The results do not support the hypothesis that an endogenous inhibitor of Na+,K+-ATPase plays an important role in the development or maintenance of hypertension in this animal model.