Sirolimus-induced Hypertriglyceridemia Leads to Acute Pancreatitis and Diabetic Ketoacidosis Post Stem Cell Transplant for Sickle Cell Disease.

Sirolimus (mammalian target of rapamycin inhibitor) is a potent immunosuppressive agent, used in patients receiving hematopoietic stem cell transplant (HSCT) for Graft vs Host disease prophylaxis. Compared to calcineurin inhibitors, sirolimus has no neurotoxicity or nephrotoxicity, but sirolimus causes dose-dependent thrombocytopenia, leukopenia, delayed wound healing, hyperlipidemia, and hypertriglyceridemia. Here we report a case of acute pancreatitis and diabetic ketoacidosis in a patient with sickle cell disease post haploidentical family donor HSCT which was managed conservatively without plasmapheresis. Based on our review of the literature, this is the first reported case of developing acute pancreatitis as an adverse effect of sirolimus-induced hypertriglyceridemia leading to diabetic ketoacidosis in a recipient of HSCT.

Efficacy of therapeutic plasma exchange in pediatric cases of acute liver failure as an extracorporeal liver support system.

BACKGROUND: Acute liver failure in the pediatric population is often accompanied by deranged metabolism, severe encephalopathy and coagulopathy. A liver transplant is the most viable option for the management of such patients. Therapeutic plasma exchange (TPE) is helpful in improving the liver biochemistry profile, thereby, increasing their likelihood of undergoing a liver transplant METHOD: The study was conducted over a period of 3 years (January 2018 to December 2021). Indications mainly consisted of ALF with hepatic encephalopathy, worsening liver parameters in spite of medical management, and candidacy for undergoing a liver transplant. Plasma exchange was performed daily or alternatively until the patient recovered, succumbed, or was stable enough to undergo a transplant. Biochemical parameters serum bilirubin, ALT, AST serum ammonia serum urea, serum creatinine were recorded before and after TPE sessions. RESULTS: The study group comprised 14 patients of which a total of 28 TPE was performed. There were a total of 5 cases of cryptogenic ALF, 4 of Wilson disease, 2 cases each of infection-related ALF and autoimmune hepatitis, and a single case of drug-induced hepatitis. A total of 5 out of 14 patients underwent a liver transplant and amongst the 9 who did not undergo a transplant, 4 patients expired due to septic shock syndrome; the remaining 5 were discharged in a stable condition following TPE sessions. The disease-free survival was 78.9% and the transplant-free survival was 35.71%. CONCLUSION: TPE plays a crucial role in improving the biochemistry profile of the liver in children with liver failure.

Refractory Status Asthmaticus: A Case for Unconventional Therapies.

Status Asthmaticus is a common reason for Emergency Room visits in children. Most of the asthma flares are successfully managed by use of ß agonist and steroids. If these therapies fail to halt the progression of asthma, a number of medical therapies may be used to treat it. However, the data supporting the use of these therapies are conflicting. We present successful use of Extracorporeal Membrane Oxygenation and isoflurane in a child with Refractory Status Asthmaticus.

Feasibility of initiating early enteral nutrition after congenital heart surgery in neonates and infants.

OBJECTIVE: To assess the feasibility of initiating enteral nutrition support with first 24 h of congenital heart repairs in neonates and Infants and its impact on outcomes following surgery. DESIGN: It is a prospective randomized control single blind study. SETTING: It is a single centre prospective study carried out in a tertiary care centre at Pediatric cardiac intensive care unit. PATIENTS: All patients with the cyanotic congenital heart disease with increase pulmonary blood flow, weighing less than 5 kg and undergoing congenital heart repair during the study period were included in the study. Patients with single ventricle status, those undergoing palliative procedures (PA band), open chest, requiring ECMO before leaving operating room, having any other contraindication for starting enteral feeding or those who refuse for consent were excluded from study. INTERVENTIONS: The patients were randomized into two groups. Group 1 received trophic feeds (10-20 ml/kg/day) starting 4-6 h after surgery while feeds children in group 2 were kept NPO and received feeds after 48 h after surgery. MEASUREMENTS AND MAIN RESULTS: 15 children enrolled in both the groups. Both pre-operative and intraoperative variables were comparable in both the groups. There was no complication (vomiting, diarrhea, NEC, bowel necrosis) noted in the children who received feeds after surgery. Mean duration of mechanical ventilation in the feeds group was 58.2 ± 4.71 h, which was less then significantly less than those in the NPO group (P value 0.05). Similarly, duration of ICU stay was only 179.04 ± 41.28 h in feeds group as compared to 228.72 ± 85.44 h in the NPO group. CONCLUSIONS: Neonates and Infants tolerate feeds immediately following congenital heart repairs. Moreover, feeds appear to decrease duration of mechanical ventilation and duration of ICU stay.