Treatment of Hepatitis C in a Case of Pediatric B-Cell Acute Leukemia.

The prevalence of hepatitis C virus (HCV) infection in Pediatric patients with lymphoproliferative diseases has most commonly been reported with B cell Non-Hodgkin lymphoma. Case studies have reported the requirement of dose reduction or suspension of chemotherapy in 80% of Pediatric ALL cases who are anti-HCV positive owing to hepatotoxicity. The standard of care anti HCV therapy in children aged 3-17 years had been peginterferon and ribavirin for 48 weeks. FDA approved pan-genotypic, anti- HCV regimen, sofosbuvir/velpatasvir [SOF/VEL], for the Pediatric population >6yrs of age or >17 kg body weight in March 2020. We herein report a case of an HCV infected Pediatric B cell ALL patient who was treated with SOF/VEL concomitantly with an intensive chemotherapy regimen. Child tolerated the full dose chemotherapy along with antivirals for 12 weeks and was in morphological remission with sustained virological response.

SARS-CoV-2 infection in a pediatric acute leukemia patient on chemotherapy and concurrent sofosbuvir/velpatasvir for HCV.

There are new targets identified by experimental and animal research for treatment of SARS-COV-2 (Severe acute respiratory syndrome-Corona Virus-2) infection. Out of many clinical trials registered, there are ongoing human studies highlighting Sofosbuvir's possible role in the treatment of Covid-19 (Coronavirus Disease 2019). Here we present a case of acute leukemia on directly acting antiviral therapy (DAAs) for HCV infection mitigating SARS-COV-2 infection in a patient undergoing chemotherapy. The child was undergoing chemotherapy, along with directly acting antiviral for acute hepatitis C infection. He initially had features of hypoxia and radiological evidence of covid-19. He had an uneventful course and tested negative ten days after onset of illness. With ongoing trials on Sofosbuvir in covid 19 treatment, our finding, albeit coincidental, points to the possible role even in immune-compromised children.

A hungry Histiocyte, altered immunity and myriad of problems: Diagnostic challenges for Pediatric HLH.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is an immune deregulation disorder with varied clinical presentation which clinically overlaps with widespread tropical infections. METHODS: We conducted a retrospective chart review of children diagnosed with HLH at our center from February-2017 to October-2020. RESULTS: Out of the nine diagnosed patients, genetic predisposition was present in three children; two had identified infectious triggers. The mean age of presentation was 30 months with male predominance. The most common clinical findings were fever, organomegaly, and pancytopenia. The median value of fibrinogen was-156 mg/dL, ferritin-12 957 ng/mL and for triglycerides-349 mg/dL, respectively. In children with identified genetic predisposition, serum ferritin levels were usually more than 10 000 ng/mL. The majority of our patients had evidence of hemophagocytosis on bone marrow examination. In our experience, although nonspecific, very high ferritin and serum triglycerides with low fibrinogen in a patient with bi-cytopenia, pancytopenia was the most suggestive evidence of HLH. Genetic evaluation in our series identified three children, one with primary HLH genetic mutation and two with underlying immune deficiency syndrome. The presence of HLH in the accelerated phase of Chediak-Higashi and AD Hyper IgE syndrome with HLH is extremely rare. Leishmaniasis (in nonendemic area) and Ebstein-Barr virus (EBV) was identified as an infectious trigger in two cases. Most of our cases received treatment as per HLH 2004 protocol. Three children died during the initial diagnosis and treatment. HLH with subcutaneous panniculitis-like T-cell lymphoma recovered well. CONCLUSION: HLH remains a life-threatening disorder associated with a variety of underlying illnesses as highlighted by our case series.

Ellis-van Creveld syndrome: report of two cases.

BACKGROUND: Ellis-van Creveld syndrome (EVC syndrome, MIM 225500) or chondroectodermal dysplasia is a rare, autosomal recessive disorder. This syndrome is characterized by a tetrad of chondrodystrophy, post axial polydactyly, and hidrotic ectodermal dysplasia, mostly involving teeth and nails and a high frequency of congenital cardiac anomalies, most frequently a common atrium. The genetic basis of this disorder has been identified as mutations in the Evc and Evc2 genes. We present a report of two affected siblings with features consistent with those of the syndrome. METHODS: A 2-month-old child with features of lower respiratory tract infection was admitted to the pediatric emergency department. Detailed examination revealed skeletal anomalies such as limb shortening and polydactyly in both hands. On cardiac evaluation, ventricular septal defect was found. There were no neonatal teeth. A diagnosis of EVC syndrome was made based on the findings. RESULTS: Screening of family members revealed that the elder sibling had features consistent with those of EVC syndrome. He was 4 years old, yet undiagnosed with short bones, polydactyly, partial anodontia and ventricular septal defect. The third child and the parents were unaffected. The treatment of this disorder is primarily supportive particularly for associated cardio-respiratory problems. The parents were extensively counseled for regular follow-up. CONCLUSIONS: The diagnosis of this syndrome is based on clinical grounds supported by radiological evaluation. Prenatal diagnosis is possible by ultrasonography and genetic testing. Genetic counseling is required to make the parents aware of the risk of recurrences.

Fetotoxicity of warfarin anticoagulation.

INTRODUCTION: Fetal warfarin syndrome (warfarin embryopathy) is a consequence of maternal ingestion of warfarin during pregnancy. Warfarin fetotoxicity comprises wide range of manifestations including dysmorphology in neonate with characteristic classical features of nasal hypoplasia and stippling of epiphyses. MATERIALS AND METHODS: Here we present a case of a neonate whose mother was on unsupervised warfarin prophylaxis throughout pregnancy. A brief review of literature with suitable options for anticoagulation during pregnancy is discussed. CONCLUSION: The final consensus over LMWH and warfarin in the first trimester is yet to be finalised. The treatment of warfarin embryopathy is symptomatic. Long term sequels in survivors are still not known.