Julius Xavier Scott

Intermittent clobazam therapy in febrile seizures.

Objective: To evaluate the efficacy of intermittent clobazam therapy in preventing the recurrence of febrile seizures and to assess its safety.Methods: The study was a prospective, randomized, double-blind placebo-controlled trial conducted in the Department of Child Health, Christian Medical College Hospital, Vellore between July 2001 and September 2002. Neurologically normal children between 6 months and 3 years of age with a history of febrile seizures and no evidence of acute CNS infection or EEG abnormality were included into the study. 19 children in a clobazam group and 20 in the placebo group were randomly allocated. Temperature reduction measures with paractamol and tepid sponging were advised to all children. In addition the dispensed medication was to be administered at the onset of fever and continued for 48 hours irrespective of the duration of fever. The children were then monitored for seizures and adverse effects of clobazam. The children were followed up for a mean period of 9.9 months. The analysis was done on the number of febrile episodes in both the groups.Results: There were a total of 110 episodes of fever during the study period. Mean number of febrile episodes in the clobazam group was 3.1 and in placebo group 2.56. Six (12.5%) of the 48 episodes in placebo group and one (1.7%) of 60 episodes in clobazam group had seizure recurrence. This was statistically significant (p=0.01). Drowsiness and weakness were present equally in both clobazam and placebo group whereas ataxia was present only in the clobazam group, the difference being statistically significant (p=0.04).Conclusion: Intermittent clobazam therapy is an effective measure in the prevention of recurrence of febrile seizures. The ataxia due to clobazam was much lower than that reported with diazepam.

Propranolol Therapy for Infantile Hemangioma.

ContextThere has been widespread interest surrounding the use of beta-blockers (i.e. propranolol, timolol, nadolol, acebutolol) in the treatment of infantile hemangiomas (IH).ObjectiveTo review literature evaluating treatment of IH with propranolol.Evidence AcquisitionWe conducted a literature search on PubMed and investigated for case reports, case series, and controlled trials by using search terms including “hemangioma” and “propranolol.”ResultsData suggest that beta-blockers are efficacious in cutaneous, orbital, subglottic, and hepatic hemangiomas and assist in the resolution of ulcerated hemangiomas. Improvement has also been documented in children with PHACE syndrome. Propranolol produces favorable results in children who do not respond to steroids and with no long-term adverse effects. Propranolol should be administered with caution due to rare but serious side effects including hypoglycemia, wheezing, hypotension, and bradycardia. Additionally, recurrence of lesions following the cessation of treatment has been documented.ConclusionsAlthough large-scale randomized controlled trials must be conducted in order to further evaluate the safety and the possible role of propranolol in the treatment of IH, the reviewed literature suggests that propranolol carries promise as a potential replacement for corticosteroids as first-line therapy or as a part of a multimodal approach.

Hemophagocytic syndrome associated with Plasmodium falciparum infection

Hemophagocytic syndrome (HPS) has been associated with infections, hematological malignancies and autoimmune conditions. Malaria is rarely reported to cause HPS. We report a case of an 11-month-old infant with fever, hepatosplenomegaly, pancytopenia, high serum ferritin, hypertriglyceridemia, and bone marrow hemophagocytosis, consistent with hemophagocytic syndrome. Gametocytes of plasmodium falciparum were identified on bone marrow aspiration. Rapid recovery was observed after treatment with antimalarials.

Single dose rasburicase in the management of tumor lysis syndrome in childhood acute lymphoblastic leukemia: A case series

Tumor lysis syndrome (TLS) occurs in malignancies with high proliferative potential and tumor burden, such as lymphomas and leukemias. TLS syndrome is an oncologic emergency, requiring prompt intervention. The metabolic derangements cause acute kidney failure and may lead to cardiac arrhythmias, seizures, and death. With the advent of rasburicase, a recombinant urate oxidase, there has been a decline in the TLS-mediated renal failure and the need for dialysis. The recommended regimen and doses pose a heavy financial burden for patients in developing countries like India. With data and studies proving a similar efficacy for the reduced dose and lesser number of rasburicase, we report here a case series of seven children with acute leukemias, whose TLS was managed by a single dose of rasburicase. A retrospective analysis of case records of seven children with acute lymphoblastic leukemia and TLS, admitted to our Pediatric Oncology Unit of our Hospital between the period 2011 and 2013, was done. All our patients responded to a single dose, indicating that in appropriately monitored patients, single dose followed by as-needed dosing can be cost-saving.

Posterior reversible encephalopathy syndrome in a child with autoimmune lymphoproliferative syndrome: Case report and review of literature

Posterior reversible encephalopathy syndrome (PRES) is characterized by headache, nausea, vomiting, seizures and visual disturbances. PRES has been usually associated with hypertension, chronic renal disease, malignancy and chemotherapeutic agents. We report the association of PRES with Autoimmune lymphoproliferative syndrome, which to our best knowledge has not been reported before.

Multidisciplinary management of hepatoblastoma in children: Experience from a developing country.

Advances in chemotherapy, liver resection techniques, and pediatric liver transplantation have vastly improved survival in children with hepatoblastoma (HB). These are best managed by a multidisciplinary team (MDT) in a setting where all treatment options are available. Until recently, this was difficult to achieve in India.

Impact of cancer support groups on childhood cancer treatment and abandonment in a private pediatric oncology centre.

Aims: To analyze the impact of two cancer support groups in the treatment and abandonment of childhood cancer. Materials and Methods: This is a retrospective review of children with cancer funded and non-funded who were treated at Kanchi Kamakoti CHILDS Trust Hospital from 2010 to 2013. A total of 100 patients were funded, 57 by Ray of Light Foundation and 43 by Pediatric Lymphoma Project and 70 non-funded. Results: The total current survival of 80%, including those who have completed treatment and those currently undergoing treatment, is comparable in both the groups. Abandonment of treatment after initiating therapy was not seen in the financially supported group whereas abandonment of treatment after initiation was seen in one child in the non-funded group. Conclusions: Besides intensive treatment with good supportive care, financial support also has an important impact on compliance and abandonment in all socioeconomic strata of society. Financial support from private cancer support groups also has its impact beyond the patient and family, in reducing the burden on government institutions by non-governmental funding in private sector. Improvement in the delivery of pediatric oncology care in developing countries could be done by financial support from the private sector.

Extensive nail changes in a toddler with multisystemic Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a multisystem disorder involving various organs. Nail changes in LCH are extremely rare. We present this case report of extensive nail changes in an 18‐month‐old child with multisystem LCH.

Acute renal failure as an initial manifestation of acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Acute renal failure is a well-recognized complication of ALL after initiation of chemotherapy. Renal failure as the primary manifestation of ALL is rare. Here, we report three children who presented with acute renal failure and hyperuricemia and were subsequently diagnosed to have ALL.

Neonate with haemophagocytic lymphohistiocytosis secondary to dengue infection: a case report.

We report the first case of haemophagocytic lymphohistiocytosis (HLH) in a neonate secondary to primary Dengue virus infection. This neonate presented in the third week of life with fever, shock and hepatosplenomegaly and was diagnosed to have Dengue infection by serology and HLH was confirmed on bone marrow.