Dinesh Yadav

Clinical spectrum and treatment outcome of West Syndrome in children from Northern India

PURPOSE This study was intended to document the clinical profile and treatment outcome of West syndrome in children attending a tertiary care center in Northern India. METHOD Data were collected by a retrospective chart review of children diagnosed with West syndrome between January 2008 and January 2012. Information was recorded pertaining to the age at onset and presentation, etiology, and associated co-morbidities; results of electroencephalography (EEG) and neuroimaging; treatment given; and final outcome. The following drugs were used for treatment: pyridoxine, prednisolone, vigabatrin, sodium valproate, nitrazepam, topiramate, and levetiracetam. The response was categorized as spasm cessation, partial improvement (>50% improvement), or no improvement. The final outcome was considered favorable when there was a complete cessation of spasms; with absence of relapse and no progression to other seizure types for at least 6 months. RESULTS Records of 148 children (120 boys) were analyzed. The mean (SD) age at onset and presentation was 5.3 (4.6) months, and 13.1 (7.3) months, respectively. Perinatal asphyxia (61.4%), neonatal sepsis/meningitis (10.6%), and postnatal meningitis (11.4%) were the predominant causes. The etiology could not be ascertained in 16.6% of children. Favorable outcome was observed in 45 (30.4%) children with spasm cessation rate of 25.4% with prednisolone. Age at onset, gender, time lag to treatment, presence of perinatal asphyxia, or co-morbid cerebral palsy did not affect the final outcome. CONCLUSION This study highlights the developing country perspective of children with West syndrome, including delayed presentation, adverse perinatal events as the predominant etiology, and modest response to oral steroids.

Benign Tertian Malaria: How Benign Is It Today?

Malaria is a disease of global importance and afflicts more than 90 countries and territories in the tropical and subtropical regions. Malaria imposes great socio-economic burden on humanity, and with six other diseases (diarrhea, HIV/AIDS, tuberculosis, measles, hepatitis B, and pneumonia), accounts for 85% of global infectious disease burden. TheWorld Health Organization (WHO) estimates 3.3 billion people at risk, 247 million malaria cases and one million estimated annual malarial mortality worldwide [1]. Approximately 2.48 million cases are reported annually from South-east Asia, of which 75% cases are contributed by India alone. WHO has reported malaria mortality rate 15000 per year from India; however, a recent study by million death collaborators suggest much higher annual malarial mortality in India (205,000 overall and 55000 in children <14 years age) [2]. Among cases with malaria, proportion of Plasmodium vivax (Pv) and P. falciparum (Pf) varies in different parts of India with 10–30% cases caused by Pf and remaining 70– 90% by Pv in most parts of the country. A continued rise in Pf has been reported recently and its proportion has gradually risen to nearly 50% of total cases in recent years [3]. Traditionally, infection with Pf is considered responsible for severe malaria and malarial mortality in literature. Very little information is available on the contribution of Pv to severe disease. As the term “benign tertian malaria” implies, Pv malaria has usually enjoyed the status of uncomplicated disease that runs a benign course and is rarely fatal. Earlier studies from Thailand and Vanuatu in last decade suggested a protective effect of Pv and suggested that Pv co-infection with Pf may attenuate severity of Pf malaria [4, 5]. However, there is growing evidence that Pv is responsible for a significant burden of disease worldwide. Recent studies from Papua New Guinea, Indonesia and India have reported all complications of severe malaria with Pv monoinfections as well [6– 8]. These studies have shown that 21–27% of patients with severe malaria have Pv monoinfection and clinical spectrum of these cases is broad with an overall mortality of 0.8–1.6% [9]. A study from Bikaner, in northwest India has reported a much higher proportion (63.1%) of severe malaria contributed by Pv mono-infection in children. In this study, the proportion of severe malaria attributable to P. vivax was 67.4% in children <5 years of age compared with 30.4% of P. falciparum and 2.2% (1/46) of mixed infection. Besides this, proportion of patients having severe manifestations, including severe anemia, thrombocytopenia, cerebral malaria, acute respiratory distress syndrome (ARDS), hepatic dysfunction, renal dysfunction and abnormal bleeding was also significantly higher in association with Pv monoinfection in 0–5 year age group. Also, Pv monoinfection was reported almost equally serious to cause significant mortality in comparison to Pf (case fatality rate of severe Pv was 3.9% versus 3.2% of severe Pf; P=1.0) [7]. This study further reaffirms the emergence of severe vivax malaria. Complications in severe malaria are either sequestration related, such as cerebral malaria, renal dysfunction, hepatic dysfunction and ARDS, or non-sequestration related, such as anemia and thrombocytopenia [10]. Sequestration related complications were earlier reported in Pf infection only; however, clinical data provided by Kochar, et al. indicates that Pv infection can cause both sequestration related and D. Yadav : J. Chandra :A. K. Dutta Department of Pediatrics, Lady Hardinge Medical College and associated Kalawati Saran Children’s Hospital, New Delhi, India

Immunization of HIV Infected Children

Immunization is one of the most effective approaches to reduce morbidity and mortality in HIV infected children. However, progressive immune suppression may lead to impaired responsiveness to most of the vaccines. Besides this, there are many other areas of concern in these children including risk of diseases and safety issues with live vaccines, need for increased amount or number of doses of a particular vaccine, shortened duration of protective efficacy, need for revaccination after immune reconstitution and effect of vaccines on HIV viral replication. Published literature on safety, effectiveness and immunogenicity of vaccines in HIV infected children and status of individual vaccines with existing guidelines has been discussed in present review.

Essential thrombocytosis and antiphospholipid antibody syndrome causing chronic Budd-Chiari syndrome.

Essential thrombocytosis is extremely rare in children. However, when present, it is associated with increased prevalence of antiphospholipid antibodies and thrombo-hemorrhagic complications. The authors report here a child with Budd-Chiari Syndrome resulting from essential thrombocytosis and associated antiphospholipid antibodies. A 13- y-old boy presented with microcytic hypochromic anemia, hepatosplenomegaly and thrombocytosis. CT scan demonstrated calcified thrombus in inferior vena cava (IVC). Diagnosis of essential thrombocytosis was considered in view of persistent thrombocytosis, antiphospholipid antibodies, bone marrow showing increased number, clusters and giant forms of megakaryocytes and IVC thrombosis. He was started on warfarin prophylaxis and did not have thrombotic recurrence on follow up.

Persistent hyperinsulinemic hypoglycemia of infancy.

Abstract Congenital hyperinsulinism (CHI) is the most frequent cause of severe, persistent hypoglycemia in infancy and childhood. We report a case of CHI with diffuse pancreatic abnormality diagnosed preoperatively using the 68Ga octreotide (DOTA NOC) positron emission tomography scan. Genetic analysis revealed homozygous ABCC8 splicing mutation. Subtotal (95%) pancreatectomy was done, and the infant remained euglycemic and was discharged on breast feeds. The patient is continuously followed up and is asymptomatic until 9 months.

Post-diphtheritic neuropathy: a clinical study in paediatric intensive care unit of a developing country.

Summary A retrospective study was done on 48 consecutive patients with clinical diagnosis of postdiphtheritic neuropathy admitted to the paediatric intensive care unit of tertiary care hospital in North India between January 2008 and December 2010 to study the clinical profile of post-diphtheritic neuropathy in children. The case records were reviewed and information regarding personal details, clinical features, recovery parameters and outcome was recorded using a predesigned proforma. Median age was 4.25 years. All cases were unimmunized. Median latency period was 15 days. Of the children, 52% had palatal palsy whereas 48% had limb weakness initially. Median duration of progression of weakness was five days. Limb muscle weakness was present in 94%. Respiratory muscles were involved in 85.4% cases and 60.4% required mechanical ventilation, while 14.6% had fatal outcome and 10.4% had hypoxic neurological injury. Boys were affected more. Median duration of latency was shorter; muscle weakness, progression and recovery were faster as compared with observational studies in adults.

Neonatal Kasabach-Merritt phenomenon

Kasabach-Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy in the presence of a rapidly enlarging vascular tumor. It usually presents in early infancy, but onset in early neonatal period, facial hemangioma, and vincristine use in neonates has rarely been reported. We, hereby, present a 6-day-old male child presenting with facial hemangioma and intracranial hemorrhage, and KMP responding well to steroids and vincristine. Pathophysiology of disease and various treatment options have been discussed.