Expression and identification of a new splice variant of neuroglycan C, a transmembrane chondroitin sulfate proteoglycan, in the human brain

Neuroglycan C (NGC) is a transmembrane chondroitin sulfate proteoglycan with an EGF module. We studied the expression of NGC in the human brain, mainly in the hippocampus, and confirmed some observations by conducting experiments using rat brain. In humans, NGC mRNA was expressed exclusively in the brain, especially in the immature brain. The telencephalon, including the hippocampus and neocortex, showed strong mRNA expression. NGC was immunolocalized to neuropils in the hippocampus and neocortex of the adult rat. RT-PCR experiments showed that four splice variants (NGC-I, -II, -III, and -IV) were expressed in the adult human hippocampus. By Western blotting, the expression as proteins of all splice variants except NGC-II was confirmed in the adult rat hippocampus. NGC-IV, which was first found in the present study, had the shortest cytoplasmic domain among the four variants. NGC-IV mRNA was expressed by neurons, but not by astrocytes, in culture prepared from the fetal rat hippocampus, suggesting that NGC-IV plays a role specific to neurons. In addition, the human NGC gene, which is registered as CSPG5, comprised six exons and was approximately 19 kb in size. In exon 2, a single nucleotide polymorphism resulting in Val188Gly in the NGC ectodomain was observed.     

Type-A long-gap esophageal atresia treated by thoracoscopic esophagoesophagostomy after sequential extrathoracic esophageal elongation (Kimura’s technique)

Introduction

We report four cases of long-gap esophageal atresia (LGEA) treated with thoracoscopic esophagoesophagostomy (TEE) after sequential extrathoracic esophageal elongation (SEEE: Kimura’s technique).

Methods

All initially had gastrostomy, then SEEE. The proximal end of the esophagus was introduced into the apex of the thorax under direct vision during TEE.

Results

Mean birth weight was 1.9 kg. Mean gestational age was 35.0 weeks (range 30–39); mean age at initial esophagostomy was 43.2 days (range 15–110); SEEE was performed for a mean of 3 times (range 2–4) at mean intervals of 5.3 months (range 2–10), with the upper esophageal segment lengthened by a mean of 2.1 cm each time (range 1.1–3.5). Mean age and weight at TEE were 22.3 months and 9.8 kg, respectively. Gap was initially 4.5 vertebrae (range 4–5). Mean operating time was 9.6 h. TEE was successful in 3; 1 required thoracotomy, then re-anastomosis after 11 months of anastomosis leakage; 2 cases with post-TEE anastomosis leakage were treated conservatively. Postoperatively, all cases required fundoplication and esophageal dilatation (2, 3, 5, 8 times, respectively). At mean follow-up of 3.6 years, 2 eat normally and 2 eat minced food.

Conclusions

TEE after SEEE appears to be a feasible option for treating LGEA.     

Effective bladder preservation strategy with low-dose radiation therapy and concurrent intraarterial chemotherapy for muscle-invasive bladder cancer

Purpose The aim of this study was to evaluate retrospectively the toxicity and response, bladder preservation, and survival of patients with muscle-invasive bladder cancer treated with multimodality therapy consisting of low-dose radiation therapy (RT) and concurrent intraarterial chemotherapy (IACT). Methods and materials Between November 1999 and July 2005, a total of 27 consecutive, previously untreated patients with muscle-invasive bladder cancer underwent transurethral bladder tumor resection followed by concurrent low-dose RT and IACT. Patients who achieved a complete response (CR) were followed up closely without further therapy, and patients who did not achieve a CR underwent further treatment. Results Complete response was achieved in 22 of 27 patients (81%). Of these 22 patients, 7 developed recurrences, and 3 died of their disease. In five patients who did not achieve CR, one died from bone metastases. The 3-year overall survival rate was 81%, with a median follow-up time of 27 months; and 22 of 27 patients (81%) with a preserved bladder were tumor-free at the last follow-up. Three patients (11%) developed grade 3 acute hematological toxicity. Conclusion Multimodality therapy consisting of low-dose RT and concurrent IACT for muscle-invasive bladder cancer can achieve survival rates similar to those in patients treated with radical cystectomy, with successful bladder preservation and minimal adverse effects.     

Effects of rikkunshito on the clinical symptoms and esophageal acid exposure in children with symptomatic gastroesophageal reflux

Rikkunshito (TJ-43), a herbal medicine consisting of eight herbs, is used to treat chronic dyspepsia. Studies have shown that TJ-43 improves human gastric emptying. This study investigated the effects of TJ-43 on the clinical symptoms and esophageal acid exposure in children with symptomatic gastroesophageal reflux (GER). Eight children, aged from 2 months to 15 years (median age 4 years), were studied. Six of them had neurological impairment. TJ-43 (0.3 g/kg/day) was given orally or via nasogastric tubes in three divided doses before meals for 7 days. Their symptoms were frequent emesis in four, nausea in two, and hematemesis and stridor in one each. Twenty-four-hour esophageal pH monitoring was conducted using multichannel pH electrodes located at the distal esophagus (P1) and 10 cm proximal to P1 (P2). The clinical symptoms and esophageal pH were compared before and after TJ-43 therapy for 1 week. The frequency of emesis decreased in three patients. Other symptoms, including nausea, hematemesis, and stridor, were relieved in the remaining patients. Measured at the distal pH electrode, the percentage time of esophageal pH < 4.0 and the mean duration of reflux decreased significantly (P < 0.05). However, the number of acid reflux per hour did not change significantly, and no pH parameters measured at the proximal electrode differed significantly. The short-term administration of TJ-43 relieved symptoms and reduced the distal esophageal acid exposure through improved esophageal acid clearance.     

Risk factors for pneumothorax associated with isolated congenital diaphragmatic hernia: results of a Japanese multicenter study

This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax. A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study. Among the 495 neonates with isolated CDH, 52 (10.5%) developed pneumothorax. Eighteen (34.6%) patients developed pneumothorax before surgery, while 34 (65.4%) developed pneumothorax after surgery. The log-rank test showed that the cumulative survival rate was significantly lower in patients with pneumothorax than in those without pneumothorax. Univariate analysis revealed significant differences between patients with pneumothorax and those without pneumothorax with regard to the best oxygenation index within 24 h after birth, mean airway pressure (MAP) higher than 16 cmH2O, diaphragmatic defect size, and need for patch closure. Multiple logistic regression analysis indicated that only the MAP was associated with an increased risk of pneumothorax. The cumulative survival rate was significantly lower in isolated CDH patients with pneumothorax than in those without pneumothorax. A higher MAP was a risk factor for pneumothorax in CDH patients.     

Potential role of the PD-L1 expression and tumor-infiltrating lymphocytes on neuroblastoma

Pediatric Surgery International - The programmed death 1 (PD-1)/programmed death ligand 1 (PD-L1) pathway has garnered much attention for its roles in clinical oncology. The aim of this study was...     

Surface Ultrastructure of Rheumatoid Articular Cartilage

Six rheumatoid articular cartilage specimens, which appeared grossly normal and were shown to be free of pannus when examined under the light microscope, were examined electron microscopically. For comparison, normal-appearing cartilage specimens from 2 patients with meniscus injury and 2 with degenerative joint disease (DJD) were also examined. In all cases the normal-appearing joint surface of rheumatoid cartilage was abnormal. Amorphous-appearing material was present to a depth varying between 6 and 25 m̈m. Some of this material had the appearance of fibrin deposited at the cartilage surface, but much appeared to represent breakdown products of the cartilage matrix, i.e. degraded collagen and proteoglycan. DJD cartilage did not show similar changes. The findings suggest that the surface of rheumatoid articular cartilage, even when grossly normal in appearance, is degraded by enzymes either present in the synovial fluid or released by polymorphonuclear cells in close contact with the cartilage surface.     

A case of extensive pharyngeal vascular malformation successfully treated with Kampo medicine

Objective

To present the efficacy of Japanese-traditional medicine (Kampo) for a case with vascular malformation.

Appropriate timing of surgery for neonates with congenital diaphragmatic hernia: early or delayed repair?

Purpose

This study was aimed to evaluate the influence of timing of surgery on patient outcomes, and to clarify appropriate timing of surgery in neonates with congenital diaphragmatic hernia (CDH).

Methods

A total of 477 neonates with isolated CDH were included. Patients were classified into two groups by timing of surgery: early repair (ER) (≤48 h) and delayed repair (DR) (>48 h). The primary outcome was 90-day survival, with treatment duration (ventilation, oxygen, and hospitalization) being a secondary outcome. To adjust for disease severity, patients were stratified into three severities by Apgar score 1 min (“mild” 8–10, “moderate” 4–7, and “severe” 0–3), and outcomes were compared between ER and DR within each severity.

Results

Although 90-day survival was significantly different among the three severities (“mild” 97%, “moderate” 89%, and “severe” 76%, p = 0.002), there were no differences in 90-day survival between DR and ER within each severity. In “mild”, there were no differences in treatment duration between ER and DR. In “moderate”, treatment duration was shorter in ER than DR (ventilation 11 vs. 16 days, oxygen 15 vs. 20 days, and hospitalization 34 vs. 48 days). In “severe”, treatment duration was shorter in ER than DR, while the best OI was higher in DR than ER.

Conclusions

Timing of CDH repair seems to have no influence on 90-day survival regardless of disease severity. Patients with moderate severity may benefit from the early repair by reducing treatment duration.

     

Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia

Purpose

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients.

Methods

BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests. Lung perfusion scintigraphy and cardiac ultrasound were performed to diagnose HPS. Cardiac catheterization was conducted to diagnose PoPH.

Results

The study population consisted of 88 BA patients. The median follow-up duration was 11.6 years (range 0.8–26.0 years). Six patients (6.8%) developed HPS and three patients (3.4%) developed PoPH. The median age of onset of HPS was significantly younger than that of PoPH (HPS: 4 years, PoPH: 15 years, P < 0.019). Two patients (66%) with PoPH died, while all patients with HPS survivied.

Conclusion

The onset of HPS was significantly earlier than that of PoPH. The mortality rate was high in patients with PoPH. Teenagers with BA should receive routine cardiac echocardiograms to detect PH in its early stages.