Surgical closure of the larynx for intractable aspiration pneumonia: cannula-free care and minimizing the risk of developing trachea-innominate artery fistula.

There is a risk of developing a fatal trachea-innominate artery fistula following laryngotracheal separation for the prevention of intractable aspiration pneumonia. We developed a novel technique of surgical closure of the larynx to avoid this complication and provide long-term cannula-free care.

Pre-operative Serum Albumin Predicts Native Liver Survival in Biliary Atresia.

BACKGROUND: To predict native liver survival (NLS) after Kasai portoenterostomy (KP) for biliary atresia (BA) using pre-operative clinical data. METHODS: Pre-operative data were collected from 29 patients with BA who underwent KP at our department between 1989 and 2017 and were analysed including serum albumin, bilirubin, prothrombin time-international normalised ratio, body height, body weight, age at KP, paediatric end-stage liver disease score calculated using the pre-operative data and the period of NLS. RESULTS: The 10-year NLS rate of all patients was 51%. A multivariate analysis revealed that among all factors, the pre-KP serum albumin level was the only independent predictor of NLS (P = 0.04, hazard ratio = 0.269, 95% confidence interval = 0.077-0.934). The area under the receiver operating characteristic curve for NLS, determined using pre-KP serum albumin was 0.760 and 3.75 mg/dl was selected as the cut-off value. There was a significant difference in NLS between patients with high (≥3.8 mg/dl) and low (≤3.7 mg/dl) pre-KP serum albumin (90.0% vs. 31.5%, P < 0.01). CONCLUSIONS: Decreased pre-KP serum albumin may reflect not only functional impairment of the liver, but also the inflammatory process, which is hypothesized to occur during its advancement. The pre-KP serum albumin level may be a good prognostic factor for NLS in post-KP BA patients.

A single institution experience of Eppikajutsuto for the treatment of lymphatic malformations in children.

BACKGROUND: Eppikajutsuto (TJ-28) is an herbal medicine recently reported to be effective in treating lymphatic malformations (LMs). We report our experience concerning the clinical efficacy of TJ-28 for LMs. METHODS: Medical records of 10 LM cases treated with TJ-28 between 2016 and 2018 were reviewed. TJ-28 was given at 0.3 g/kg/day and then increased to 0.5 if no improvement was noted after the first three months of treatment. Their clinical data were collected, and LM volume indices (depth×width×height) were measured with the first (LMVI-F) and latest (LMVI-L) imaging studies. The response rates were calculated as 1-LMVI-L / LMVI-F (%). RESULTS: The median age at the diagnosis and treatment period was 1.5 years and 17.5 months, respectively. LMs were located in the neck (six), mesenterium or retroperitoneum (three), and inguinal region (one). The median response rate was 83%, including 100% in three cases and the apparent improvement of obstructive airway symptoms in one case. One case underwent surgery for insufficient improvement, and another that showed no effect is being considered for surgery. Most of the satisfactory outcomes were demonstrated in the first six months of treatment. CONCLUSION: TJ-28 seems to be effective in treating LMs in children, especially early in treatment. LEVEL OF EVIDENCE: IV.

Combined Fundal and Segmental Adenomyomatosis of the Gallbladder in a Child: A Rare Case Report.

Adenomyomatosis of the gallbladder (AMG) is characterized by mucosal hyperplasia leading to invagination through the thickened muscle layer, which is relatively common in adults, but is rare in childhood. We report a 12-year-old boy with adenomyomatosis of the gallbladder combined segmental and fundal type. This combined type is rare in adults and is first reported here in childhood. Although initial imaging with computed tomography (CT) suggested the presence of a circular solid mass-like lesion because of its rare morphology, repeated ultrasonography (US) was useful for leading to a correct diagnosis.

Mizoribine as sole immunosuppressive agent in islet xenotransplantation models: a candidate immunosuppressant causing no adverse effects on islets.

Mizoribine (MZ) inhibits the differentiation and proliferation of helper T and B cells after antigen recognition by suppressing the purine biosynthesis pathway and nucleic acid synthesis. MZ has been used in kidney transplantation, but distinct data are unavailable for islet transplantation. The present study investigated the efficacy of MZ for islet xenotransplantation. Immunosuppressive effects of MZ were determined by mixed lymphocyte reaction (MLR) assay in vitro. Toxicities for Wistar rat islets were determined by adenosine triphosphate (ATP) contents of islets during 3-day culture and stimulation index in response to glucose after culture. Immunosuppressive effects in vivo were tested in a Wistar-to-B6 islet xenotransplantation model. MZ was administered continuously for 28 days subcutaneously or intramuscularly. MZ inhibited MLR response by approximately 50% at 0.1 µg/ml. ATP contents decreased with MZ >100 µg/ml, while stimulation index was maintained. Continuous infusion of MZ at 10 mg/kg maintained blood concentrations at 0.13-0.19 µg/ml, while intramuscular injection of MZ at 100 mg/kg/day (peak 520 µg/ml at 1 h postinjection) resulted in below measurable levels (<0.03 µg/ml) within 24 h. Graft survival was significantly prolonged following continuous infusion of 10 mg/kg/day compared to controls (31.0 ± 9.5 vs. 13.2 ± 5.2 days; p = 0.002). Furthermore, animals with intramuscular injection at doses of 3.2, 10, or 100 mg/kg/day showed significantly longer graft survival (20.0 ± 7.5, 22.0 ± 7.31, and 24.5 ± 8.1 days, respectively; p < 0.05 each). Histological examination showed significant suppression of lymphocyte infiltration by MZ administration. MZ showed immunosuppressive effects in an experimental islet xenotransplantation model without adverse effects on endocrine function of islet grafts.

Preservation of pancreatic islets in cold UW solution before transplantation.

Culture of islets prior to transplantation needs to be revisited for maintaining functional islet capacity. This study was conducted to compare cold UW (University of Wisconsin) preservation with conventional culture based on insulin secretory capacity in vitro and in vivo. Islets isolated from Wistar rats were either cultured for 24 h at 37°C in RPMI1640 medium or DMEM containing various concentrations of glucose or preserved for the same period in UW solution or in DMEM solution at 4°C. The islet yield in UW group, but not in other groups, was maintained as comparable with that of fresh islets. Insulin secretory capacity in response to glucose was maintained only in the islets of UW group, but not in other groups. SCID mice given 300 IEQ islets of UW group showed gradual restoration of normoglycemia as found in the mice given freshly isolated islets. Meanwhile, those mice given cultured islets for 24 h at 37°C in RPMI1640 medium showed rapid decrease of blood glucose levels on day 1 followed by relatively elevated levels on day 2, suggesting unstable insulin secretory capacity of islets.   Morphological staining with anti-HMGB1 (high mobility group B1) antibody revealed central damage of islets in all culture groups regardless of glucose concentration and in islets of cold DMEM group, whereas those in the UW group were quite intact. These results suggest that cold preservation in UW solution is simple and beneficial in protecting islets morphologically and functionally before transplantation.

Congenital esophageal stenosis because of tracheobronchial remnant and treated by circular myectomy: a case report.

Congenital esophageal stenosis (CES) is a rare anomaly, and appropriate management is not well established. We performed myectomy of the esophageal wall in a child with critical esophageal stenosis caused by tracheobronchial remnant (TBR). An 18-month-old boy was admitted to our hospital having frequent vomiting and failure to thrive. Esophagography and esophagoscopy showed abrupt stenosis at the lower esophageal wall. Balloon dilatation was performed but was ineffective. Surgery was performed under a diagnosis of CES because of TBR. Cartilage was palpable in the stenotic esophageal wall, and extirpation of the muscular layer of the stenotic portion was performed, leaving the mucosal layer intact. The muscular layer was closed loosely using interrupted 5-0 absorbable sutures to match the oral and anal sides together. Postoperatively, the esophageal passage was improved to the point that the patient was able to take solid foods without vomiting. This successful outcome suggests that circular myectomy of the TBR is worth recommending as a surgical procedure for short segment and stenosis of patients with CES because of TBR.