Current status of intractable pediatric gastroesophageal reflux disease in Japan: a nationwide survey.

Purpose: A nationwide survey was conducted to investigate the current status of pediatric gastroesophageal reflux disease (GERD) in Japan, with special reference to the characteristics of intractable GERD.

Methods: Data were collected using a questionnaire from facilities specializing in the treatment of pediatric GERD in Japan. Intractable GERD was defined as follows: I, no symptomatic improvement after 8 weeks of optimal medical treatment (OMT) plus fundoplication; II, no symptomatic improvement after 8 weeks of OMT and with no indications for fundoplication; and III, no symptomatic improvement after surgery without OMT.

Importance of plasma ghrelin levels with special reference to nutritional metabolism and energy expenditure in pediatric patients with severe motor and intellectual disabilities.

Background & Aims: Nutritional metabolism is complex in pediatric patients with severe motor and intellectual disability (SMID), and therefore, appropriate estimation of the energy requirements is difficult. Focusing on ghrelin's role in energy metabolism regulation, we investigated plasma ghrelin levels in pediatric SMID patients and analyzed its nutritional significance as a regulatory marker of energy reserve.

Methods: Fasting plasma total, acyl, and des-acyl ghrelin levels in 40 patients with SMID, including cerebral palsy (CP) (n = 20) and muscular disease (MD) (n = 8), and healthy controls (n = 13) were investigated.

The outcomes of transanal endorectal pull-through for Hirschsprung's disease according to the mucosectomy-commencing points: A study based on the results of a nationwide survey in Japan.

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.

Nationwide survey of outcome in patients with extensive aganglionosis in Japan.

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.

Characteristics of gastroesophageal reflux in pediatric patients with neurological impairment.

Purpose: To clarify the characteristics of gastroesophageal reflux (GER) in pediatric patients with neurological impairment (NI).

Methods: Twenty-six patients with NI, 1-17-year-old, fed via a nasogastric tube were categorized based on the absence (group I) or presence (group II) of abnormal GER, defined with multichannel intraluminal impedance-pH monitoring as %time with an esophageal pH <4.0 being ≥5.

Efficacy of the traditional Japanese medicine goreisan for the resolution of spermatic cord hydrocele in children.

Purpose: Goreisan, a traditional Japanese medicine, has previously been used for hydrostatic modulation. This retrospective study investigated the efficacy of goreisan for spermatic cord hydrocele resolution in children.

Methods: Seventy-two boys treated for spermatic cord hydrocele between 2012 and 2015 were included; Goreisan was administered to 16 [group G, median age 3 (1-8) years], and 56 were followed without medication [group C, median age 1 (0-8) years].

Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction.

Purpose: The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction.

Materials And Methods: We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development.

Results: The patients consisted of three boys and one girl, who ranged in age from 77 to 109months.

Cardiac fetal ultrasonographic parameters for predicting outcomes of isolated left-sided congenital diaphragmatic hernia.

Background: The purpose of this study was to investigate fetal cardiac ultrasonographic parameters in relation to the outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH).

Methods: Subjects comprised 33 fetuses with fetal CDH and 99 control fetuses without cardiac and lung anomalies. Three parameters, cardiothoracic area ratio (CTAR), the ratio of the diameter of the main pulmonary artery to that of the ascending aorta (MPA/AAo), and the ratio of the diameter of the tricuspid valve to that of the mitral valve (TV/MV) were compared between fetal CDH and control subjects and between survival (SG) and non-survival (NSG) groups.

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan.

Background: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan.

Methods: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan.

Causal relationship between delayed gastric emptying and gastroesophageal reflux in patients with neurological impairment.

Purpose: To investigate the causal relationship between delayed gastric emptying (DGE) and gastroesophageal reflux (GER) in patients with neurological impairment (NI).

Methods: Subjects included 30 NI patients (age, 1-34 years; median, 6 years). Combined multichannel intraluminal impedance-pH (MII-pH) monitoring and (13)C-breath test evaluated acid/non-acid reflux episodes (RE) and gastric emptying rates, respectively.

Mesenteric hernia causing bowel obstruction in very low-birthweight infants.

Internal hernia through a mesenteric defect, called mesenteric hernia, is an uncommon cause of acute intestinal obstruction in newborns. Strangulated mesenteric hernia results in intestinal necrosis or perforation and progressive deterioration with fatal outcome, especially when it occurs in low-birthweight infants. We report two very low-birthweight (VLBW) infants, who presented with acute intestinal obstruction related to mesenteric defect.

Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: a report of a nationwide survey in Japan.

Background/purpose: A nationwide survey was conducted to identify the clinical presentation of pediatric chronic intestinal pseudo-obstruction (CIPO) in Japan.

Methods: Data were collected via a questionnaire, ensuring patient anonymity, from facilities that treat pediatric gastrointestinal diseases in Japan.

Results: Ninety-two responses were collected from forty-seven facilities.

Does laparoscopy-aided gastrostomy placement improve or worsen gastroesophageal reflux in patients with neurological impairment?

Background/purpose: The purpose of this study was to retrospectively investigate whether laparoscopy-aided gastrostomy placement (LGP) improved or worsened gastroesophageal reflux (GER) in neurological impairment (NI) patients.

Methods: Subjects included 26 NI patients nourished via nasogastric tubes (age, 1-17years; median, 6years). They were divided into groups based on the percentage of time with an esophageal pH <4.

Selenium deficiency in children and adolescents nourished by parenteral nutrition and/or selenium-deficient enteral formula.

The authors analyzed serum selenium levels of 95 children and adolescents with intestinal dysfunction and/or neurological disabilities [age range: 7 months-20 years; mean±standard deviation (SD): 8.0±5.3 years] who received parenteral nutrition (PN) and/or enteral nutrition (EN) with either reduced or no selenium doses for more than 3 months.

Esophago-gastric motility and nutritional management in a child with ATR-X syndrome.

X-linked alpha thalassemia mental retardation (ATR-X) syndrome is an X-linked recessive disorder that often involves gastrointestinal symptoms. Aspiration pneumonia related to gastroesophageal reflux has been reported as the major cause of death, but gastrointestinal function has not been well investigated. The present report describes a child with ATR-X syndrome who suffered from periodical episodes of refractory vomiting.

Physiological analysis of the effects of rikkunshito on acid and non-acid gastroesophageal reflux using pH-multichannel intraluminal impedance monitoring.

Purpose: To clarify the effects of rikkunshito on acid reflux, non-acid reflux, and esophageal clearance in patients with gastroesophageal reflux disease (GERD).

Methods: We enrolled seven patients with vomiting and/or stridor (median 6 years; 1 month-17 years), with a percent total time of esophageal pH <4.0 (reflux index) over 4.

The clinical significance of intrahepatic cystic lesions in postoperative patients with biliary atresia.

Purpose: To clarify the significance of intrahepatic cystic lesions (ICLs) after Kasai procedure for biliary atresia (BA), we reviewed the BA patients with special reference to the shape of ICLs.

Methods: For the last two decades, 75 cases have been followed up at our institution. The patients were divided into two groups: ICLs (+) with ICL and ICLs (-) without ICL.

Importance of evaluating for cow's milk allergy in pediatric surgical patients with functional bowel symptoms.

Background: Cow's milk allergy (CMA) can cause functional bowel symptoms. This can lead to confusion and difficulty in managing pediatric surgical patients who also have CMA. We examined CMA's effect on the management of pediatric surgical patients in our institute.