Parenteral nutrition (PN) in children with short bowel syndrome is crucial and lifesaving. Taking care of such patients requires interprofessional practice and multiple team resource management. Home PN (HPN) usage allows patients and families to live regular lives outside hospitals. We share our experiences for the last two decades and identify the risk factors for complications and mortality. A retrospective study of HPN patients was conducted between January 2000 and February 2022. Medical records of age, body weight, diagnosis, length of residual intestines, HPN period, central line attempts, complications, weaning, and survival were collected and analyzed. The patients were classified as HPN free, HPN dependent, and mortality groups. A total of 25 patients received HPN at our outpatient clinic, and one was excluded for the adult age of disease onset. There were 13 patients (54.1%) who were successfully weaned from HPN until the record-enroled date. The overall mortality rate was 20.8% (five patients). All mortality cases had prolonged cholestasis, Child Class B or C, and a positive Pediatric End-Stage Liver Disease (PELD) score. For HPN dependence, extended resection and multiple central line placement were two significant independent factors. Cholestasis, Child Class B or C, and positive PELD score were the most important risk factors for mortality. The central line-related complication rate was not different in all patient groups. The overall central line infection rate was 1.58 per 1000 catheter days. Caution should be addressed to prevent cholestasis and intestinal failure-associated liver disease during the HPN period, to prevent mortality. By understanding the risks of HPN dependence and mortality, preventive procedures could be addressed earlier.

This study aims to evaluate the surgical management and outcomes of pediatric paravertebral tumors, particularly focusing on dumbbell tumors, within a single medical center. Employing a retrospective analysis at Taichung Veterans General Hospital from January 2016 to February 2022, we included pediatric patients diagnosed with paravertebral tumors confirmed via MRI and histopathology, who underwent surgical treatment. We aimed to assess the extent of tumor resection as the primary outcome, with postoperative complications, recurrence rates, and neurological function as secondary outcomes, utilizing descriptive statistics. In the given period, four pediatric patients, two males and two females aged 53-204 months, were treated at our institute for paravertebral tumors using CT and MRI for diagnosis. The tumors were located in the posterior mediastinum (3 patients) and retroperitoneum (1 patient), with varied MRI findings. Diagnoses included Ganglioneuroma, Schwannoma, Plexiform neurofibroma, and Spindle cell sarcoma. Treatments involved minimally invasive surgeries, with three cases using intraoperative neurological monitoring. Despite a mean operative time of 376 min and a 13-day average hospital stay, one patient experienced recurrence requiring chemotherapy, another had distant metastasis, and one died at a two-year median follow-up. Our study concludes that CT and MRI are crucial for diagnosing paravertebral tumors and planning surgery, and a multidisciplinary approach is essential for optimal functional preservation in pediatric patients. These findings emphasize the necessity for ongoing research to refine treatment strategies and improve patient outcomes in this complex clinical area.

This study aims to elucidate the relationship between intestinal obstruction and carnitine deficiency in neonates and infants. We retrospectively reviewed medical records of 330 neonates and infants, younger than six months, who underwent surgery for intestinal obstruction at our institute from January 2009 to April 2022. The analysis focused on clinical symptoms, related signs, complications, and etiology of the intestinal obstruction. Tandem mass spectrometry (MS/MS) or urine organic acid analysis was conducted for 47 patients, revealing carnitine deficiency in 16 patients. The incidence of carnitine deficiency was 34.0% in the suspicious group and 4.8% overall, significantly higher than in the general population in Taiwan. Notably, patients with carnitine deficiency experienced prolonged ileus, with a mean fasting duration of 41.7 days (range 7.8–65.5 days), compared to 10.8 days (range 8.2–13.4 days) in patients without carnitine deficiency. Carnitine replacement therapy was administered to twelve patients at dosages ranging from 32 to 90 mg/kg/day. One patient exhibited a drug allergy with skin rashes. Our findings suggest that carnitine deficiency should be considered in cases of neonatal and infant intestinal obstruction. Replacement therapy is straightforward and can be prognostically beneficial for some patients. Therefore, we recommend generalizing MS/MS and urine organic acid analysis, particularly for patients with prolonged ileus.