Pediatric kidney transplantation: is it safe to perform during night-time or day-off?

PURPOSE: To investigate the impact of after-hours surgery on the outcomes of pediatric kidney transplantation (KT). METHODS: Medical records of pediatric KTs performed at a single institution between 2013 and 2021 were retrospectively reviewed. The population was split into three groups according to the incision time and calendar: ordinary day (8.00 AM - 6.30 PM), day-off, and night-time (6.30 PM - 8.00 AM). The following endpoints were compared: ischemia times, length of surgery, complications, delayed graft function (DGF), primary graft non-function (PGNF), and eGFR at three-month follow-up. RESULTS: Ninety-six non-living donor KTs were performed, median age 11 (IQR 4.3-14) years and median body weight 26 (IQR 13-50) kg. Forty-one (43%) were performed during night-time and 28 (29%) during day-off. Ischemia times were similar (p = 0.769, p = 0.536). Day-off KTs presented an extended length of surgery (p = 0.011). Thirty-two complications were reported in 31 KTs. No difference in the overall rate of complications, DGF, PNGF, and three-month eGFR was found (p = 0.669, p = 0.383, p = 0.949, p = 0.093). Post-operative bleedings were more common in days-off (p = 0.003). CONCLUSION: The number of pediatric KTs performed during after-hours was considerable. Even though similar outcomes were reported, more caution should be focused on the KTs performed in days-off to avoid severe complications.

Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP).

BACKGROUND: Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated. MATERIALS AND METHODS: Among over 1000 patients registered into the Tumori Rari in Età Pediatrica-rare tumors in pediatric age project-from 2000 to 2019, 50 were affected by PC/PGL. All clinical and therapeutic data were evaluated. RESULTS: Twenty-eight patients had PC and 22 had PGL. Age at diagnosis ranged between 5 and 17 years. Thirty-five patients had symptoms related to catecholamine hypersecretion; in 7 of 50 patients, diagnosis was incidental or done during assessment of a familial syndrome. In all cases, conventional imaging was effective to assess the presence of a tumor. In addition, 18 of 38 functional imaging studies were positive (61%). Forty-eight patients were eligible for surgery: a complete resection was more frequently achieved in PC than in PGL (26/28 vs 11/22). All relapses were treated with surgery alone, surgery plus medical treatment, or chemotherapy alone; one PC with metastasis at diagnosis received radiotherapy only. Forty-four patients were in the first, second, or third complete remission (10/50 recurred; 8/10 carried a germline mutation). Five of 50 patients were alive with disease. One patient died of disease. CONCLUSIONS: Surgery can be curative in most tumors but it may not be always effective in removing PGLs. Severe postsurgical sequelae may affect these patients. Genetic tests should always be considered in individuals affected, and genetic counseling should be offered to their families.

A survival analysis of cuffed tunneled silicon central venous catheters in children affected by short bowel syndrome: A lesson from the past.

BACKGROUND: Tunneled central venous catheters (CVC) are crucial in the management of children affected by short bowel syndrome (SBS). This work aims to investigate the outcomes of tunneled CVC and to identify factors influencing their survival. METHODS: All the children diagnosed with SBS and undergone a procedure of insertion of a tunneled CVC from 2010 to 2019 were included. Demographic data and surgical information about the procedures were collected. Regression models and Kaplan-Meier analysis were performed to estimate the survival. RESULTS: Eighteen patients, eight males (44%), with a median length of residual bowel measuring 72 cm (IQR 50-102 cm), were enrolled. Thirty-nine Broviac CVCs were inserted with a mean number of 2.2 CVCs per patient and 13365 line-days. The overall incidence of complications was 3.2/1000 line-days, and the incidence of central line associated bloodstream infections (CLABSI) was 1.1/1000 line-days. No episode of catheter thrombosis was reported. The median survival was 269 days (IQR 82-1814 days). The survival was negatively influenced by a younger age at insertion (R2 = 0.29; p < 0.001), 2.7 Fr diameter (median survival 76 days; p < 0.001) and the occurrence of complications (median survival 169 days; p = 0.002). The length of residual bowel was a mild risk factor for anticipated removal (OR 1.1; CI95 1.0-1.1; p = 0.05). CONCLUSION: CVC-related complications negatively influenced the survival of the line. An elder age at insertion together with a larger CVC diameter increased the survival of the line, while a shorter residual bowel was associated with an anticipated removal due to complications.

Metachronous Contralateral Cystadenoma After Ovariectomy for Juvenile Granulosa Cell Tumor in a Young Girl: An Uncommon Association.

BACKGROUND: A unique Case of metachronous contralateral cystadenoma diagnosed 2 years after a juvenile granulosa cell tumor (JGCT) of the ovary is reported. CASE: One year after a left ovariectomy for JGCT, a 7 year-old girl was found to have, during her standard ultrasound follow-up, a 18-mm cyst in the right ovary, which was initially considered to be a follicular cyst. Six months later, the mass appeared to be increased and characterized by multivacuolar features, and inhibin A was mildly elevated. A laparoscopic-assisted cystectomy was performed, sparing healthy ovarian tissue around. The pathology report showed a benign mucinous cystadenoma.

Muscle functional recovery is driven by extracellular vesicles combined with muscle extracellular matrix in a volumetric muscle loss murine model.

Biological scaffolds derived from decellularized tissues are being investigated as a promising approach to repair volumetric muscle losses (VML). Indeed, extracellular matrix (ECM) from decellularized tissues is highly biocompatible and mimics the original tissue. However, the development of fibrosis and the muscle stiffness still represents a major problem. Intercellular signals mediating tissue repair are conveyed via extracellular vesicles (EVs), biologically active nanoparticles secreted by the cells. This work aimed at using muscle ECM and human EVs derived from Wharton Jelly mesenchymal stromal cells (MSC EVs) to boost tissue regeneration in a VML murine model. Mice transplanted with muscle ECM and treated with PBS or MSC EVs were analyzed after 7 and 30 days. Flow cytometry, tissue analysis, qRT-PCR and physiology test were performed. We demonstrated that angiogenesis and myogenesis were enhanced while fibrosis was reduced after EV treatment. Moreover, the inflammation was directed toward tissue repair. M2-like, pro-regenerative macrophages were significantly increased in the MSC EVs treated group compared to control. Strikingly, the histological improvements were associated with enhanced functional recovery. These results suggest that human MSC EVs can be a naturally-derived boost able to ameliorate the efficacy of tissue-specific ECM in muscle regeneration up to the restored tissue function.

"Rendezvous" Procedure in Children with Cholecysto-Choledocholithiasis.

Introduction: Cholecysto-choledocolithiasis is a rare entity in children and its management is still challenging and controversial. The laparoendoscopic rendezvous (LERV) procedure, consisting of laparoscopic cholecystectomy and simultaneous endoscopic retrograde cholangiopancreatography for the management of symptomatic choledocholithiasis is well described in adult patients. However, in the literature, few reports about its application in the pediatric population have been recorded. Aim of the Study: The aim of the present study is to report our first successful cases of symptomatic cholecysto-choledocholithiasis LERV treatment. Methods: Two girls suffering of hemolytic disease presented to our third referral center with acute abdominal pain due to cholecysto-choledocholithiasis. Preoperative, perioperative, and postoperative data were retrospectively reviewed. Results: Surgery was performed without complications. The girls were dismissed once re-alimentation and re-canalization were achieved and had no other episodes of biliary cholic. Reported advantages of LERV include: a shorter in-hospital stay, a reduction in the number of procedures and anesthesia, and a reduced overall risk of complications. Conclusions: The promising result with our 2 cases suggest that, when performed in highly specialized centers, LERV is a safe procedure, which leads to considerable benefits, despite logistic and organizational difficulties.