Propranolol Monotherapy in Multifocal/Diffuse Infantile Hepatic Hemangiomas in Indian Children: A Case Series.

Introduction: Infantile hepatic hemangioma (IHH) is the most common benign liver tumor in children, and multifocal and diffuse tumors often become life-threatening, necessitating therapy. Propranolol is now considered the first choice of therapy with ample data in Caucasian children. We present a series of nine Indian children with multifocal (n = 5) and diffuse (n = 4) IHH treated with propranolol monotherapy. Methods: This was a retrospective clinical data-based single-center study. Propranolol was used at a median dose of 3.2 mg/kg/day (range 3-3.3 mg/kg/day) for a median duration of 12 months (range 6-32 months). Results: The presentations of IHH (either in isolation or combination) were hypothyroidism in six patients (diagnosed by elevated serum TSH levels), heart failure in three (diagnosed based on clinical and echocardiographic features), and imaging evidence of macrovascular shunting in two patients. A good response to propranolol monotherapy (with a median dose of 3.2 mg/kg/day for a median duration of 12 months) was observed in eight patients, with a poor response in one. One patient experienced recurrence but responded adequately to propranolol retreatment. Conclusions: Our data reiterate the excellent response (88.9% responded) and safety profile with propranolol monotherapy in complicated IHH and strengthen the data in Asian (Indian) children. It includes the maximum proportion of complicated IHH treated with propranolol in East and South Asia, and the largest series from India.

Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting.

Background Hepatoblastoma (HB) is a rare neoplasm of the liver, accounting for about 1% of all pediatric cancers. The aim of the present study is to report our experience with HBs over a period of five years from a tertiary center in Eastern India. Methodology This is a retrospective observational study. The data of all patients who were diagnosed with HB between August 2015 and December 2020 was reviewed. Results Twenty-three patients who were diagnosed and treated for HB at our center were included in the study. Sixteen (69.5%) of them were male. The median age of presentation was 14 (range, 3-58) months. An abdominal lump (n=23, 100%) and abdominal pain (n=11, 47.8%) were the most common presenting symptoms. The median level of serum alpha-fetoprotein at the time of initial evaluation was 8000 (878-1,280,000) ng/dL. The mean size of the largest focus in its largest dimension was 12.03±3.77 cm. The epithelial variant (n=22, 95.7%) was the most common histological subtype. One (4.3%), 10 (43.4%), 11 (47.8%), and one (4.3%) patient were found to have pre-treatment extent of tumor (PRETEXT) stages 1, 2, 3, and 4, respectively. Fifteen (65.2%) children were classified as standard risk and seven (34.7%) children as high risk. All the patients received neoadjuvant chemotherapy (NACT). The most commonly performed surgery was right hepatectomy (n=12, 52.1%). There were three (13%) cases of perioperative mortality. Four postoperative complications developed in three (13%) patients. Four (17.3%) patients developed chemotherapy-related complications. The median duration of follow-up was 31 (range, 0-58) months. Three (13%) patients developed relapses of the disease. Overall, five-year survival in our series was 73.9%. Conclusion This study shows that the overall outcomes of HB in a resource-limited setting such as ours are good with the adoption of multi-modality treatment. Managing chemotherapy-induced complications and making liver transplantation more feasible will improve the results further.

Immune Response to Double Dose Hepatitis-B Vaccine using Four Dose Schedule in HIV Infected Children.

OBJECTIVE: To assess the immune response to hepatitis B vaccination in HIV infected children using 4 double dose schedule administered at 0-1-2-6 mo. METHODS: In a prospective observational study, 40 HIV infected children were vaccinated with hepatitis B virus (HBV) vaccine (20 mcg, 1 ml IM at anterolateral aspect of thigh of recombinant DNA vaccine) at 0-1-2-6 mo. Anti-HBsAb titre were assessed 4-8 wk after the last dose. Clinical severity was assessed according to WHO staging. Immune status of the patients was assessed using CD 4+ counts before the vaccination. RESULTS: Of the total 40 patients, 33 (28 boys, 5 girls) completed the study. Six patients were in pre-antiretroviral therapy (ART) care while 27 were receiving ART for a mean duration of 2.06 y. Ten patients belonged to WHO clinical stage 1, and 17, 2 and 4 patients to WHO clinical stage 2, 3 and 4 respectively. Median CD4+ cell count was 738/mm(3) and 28 patients had mild or no immunosuppression. Out of the total 33 patients who completed followup, only 2 patients (6 %) did not seroconvert (Anti HBsAb titre at end of study <10 IU/L), the rest (94 %) achieved different levels of Anti HBsAb titre at end of study. Twenty two (66 %) patients had anti HBsAb titre more than 1000 IU/L, 8 (24 %) had titre between 100 and 1000 IU/L and one (3 %) patient had level of 10-99.99 IU/L. CONCLUSIONS: In HIV-infected children who have no or mild immunosuppression, four dose, double dose schedule of HBV vaccine achieves very high seroconversion rates.