Ponde V.,All India Institute of Physical Medicine and Rehabilitation Center |
Desai A.P.,Bai Jerbai Wadia Hospital for Children |
Shah D.,Bai Jerbai Wadia Hospital for Children
Paediatric Anaesthesia | Year: 2013
Background and objectives Arthrogryposis multiplex congenital is hallmarked with immobile joints and muscle fibrosis. The main objective of this study was to compare the success rate of ultrasound-guided sciatic and femoral nerve blocks with nerve stimulations in children diagnosed with distal arthrogryposis multiplex congenita. Method Sixty children aged 8 months to 2 years posted for foot surgery were randomly assigned to group NS and group US of 30 each. Under general anesthesia, femoro-sciatic block was performed with nerve stimulator guidance in group NS and ultrasound guidance in group US. Results Group NS: 23 of 30 (76.7%) children showed ankle movement with sciatic neurostimulation. In 7 (23.6%), distal motor response could not be elicited and the block was abandoned. Out of 23 children who could be given femoral block, in 12 (52%) patients quadriceps contractions were not elicited and fascia iliaca block was given. All 23 blocks were successful. CHIPPS score at 1, 4, 6, 8, and 10 h was 1.05 ± 0.90, 1.82 ± 1.18, 3.36 ± 1.65, 2.23 ± 2.02, and 1.18 ± 1.14, respectively. Group US: In 29 of 30 patients (96.6%), sciatic nerve was visualized with ultrasonography. All 29 children received femoral block, and they were successful. The odds of success in group US were 8.9 (95% confidence interval [1.0, 77.9]) as compared with NS group. The difference in success rate was statistically significant (P = 0.026). The analgesic duration difference in the US and NS groups was a mean 7.62 ± 0.57 h in group NS and 8.60 ± 0.66 h in group US (statistically significant [P < 0.001]). CHIPPS score at 1, 4, 6, 8, and 10 h was 0.79 ± 0.96, 1.61 ± 0.92, 2.96 ± 1.04, 2.36 ± 2.54, and 1.14 ± 1.01, respectively. The difference between the CHIPPS score was not statistically significant. Conclusion Ultrasonography significantly increases the success rate of sciatic and femoral block in arthrogryposis. © 2012 Blackwell Publishing Ltd.
Madkaikar M.,National Institute of Immunohematology ICMR |
Shabrish S.,National Institute of Immunohematology ICMR |
Desai M.,Bai Jerbai Wadia Hospital for Children
Indian Journal of Pediatrics | Year: 2016
Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. Severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes (macrophages) secreting high amounts of inflammatory cytokines threatens the life of the patient and may lead to death unless arrested by appropriate treatment. HLH can be caused either by certain underlying genetic diseases (familial HLH), or may also occur due to particular triggers in patients with no known inherited disorder (acquired HLH). Due to life threatening nature of the disease, early diagnosis and initiation of immunosuppressive therapy is extremely important. HLH diagnosis is based on constellation of clinical manifestations and laboratory parameters which often overlap with those of severe infection or sepsis. Identification of patients with familial HLH and their underlying genetic defects requires specialized laboratory tests and is important for predicting relapses and planning early therapeutic hematopoietic stem cell transplantation (HSCT). A high suspicion and thorough clinical, immunological and genetic work-up is required for diagnosis of HLH. Prompt initiation of adequate treatment is essential for the survival. Substantial progress has been made in exploring the complex cause and pathophysiology of HLH and also in management of HLH patients. © 2016, Dr. K C Chaudhuri Foundation.
Merchant R.H.,Dr Balabhai Nanavati Hospital |
Lala M.M.,Bai Jerbai Wadia Hospital for Children
Indian Journal of Pediatrics | Year: 2012
Clinical manifestations in children living with HIV/ AIDS differ from those in adults due to poorly developed immunity that allows greater dissemination throughout various organs. In developing countries, HIV-infected children have an increased frequency of malnutrition and common childhood infections such as ear infections, pneumonias, gastroenteritis and tuberculosis. The symptoms common to many treatable conditions, such as recurrent fever, diarrhea and generalized dermatitis, tend to be more persistent and severe and often do not respond as well to treatment. The use of Anti Retroviral Therapy (ART) has greatly increased the long term survival of perinatally infected children so that AIDS is becoming a manageable chronic illness. As the immunity is maintained, the incidence of infectious complications is declining while noninfectious complications of HIV are more frequently encountered. Regular clinical monitoring with immunological and virological monitoring and the introduction of genotypic and phenotypic resistance testing where resources are available have allowed for dramatically better clinical outcomes. However, these growing children are left facing the challenges of lifelong adherence with complex treatment regimens, compounded by complex psycho-social, mental and neuro-cognitive issues. These unique challenges must be recognized and understood in order to provide appropriate medical management. ©Dr. K C Chaudhuri Foundation 2012. © Dr. K C Chaudhuri Foundation 2011.
Otiv A.,G 202 |
Mehta K.,G 202 |
Ali U.,G 202 |
Nadkarni M.,Bai Jerbai Wadia Hospital for Children
Indian Pediatrics | Year: 2012
Objectives: To determine the renal size in normal Indian children by sonography. Settings: Pediatric teaching hospital, Mumbai, India. Duration: 1.5 years. Design: Cross-sectional observational study. Participants: 1000 normal Indian children aged 1 month - 12 years. Methods: Sonographic assessment of renal size (length, width and thickness) was performed using Philips real time mechanical sector scanner of 3.5-5 MHz frequency with electronic caliper. The mean renal dimensions and volume were calculated for each age group with ± 2SD. The renal length and calculated renal volume were correlated with somatic parameters like age, weight, height and body surface area. Regression equations were derived for each pair of dependent and independent variables. Results: No statistical difference was found in renal size between sexes and between right and left kidney. A strong correlation was seen between renal size with various somatic parameters, the best correlation was between renal size length and body height (coefficient of correlation=0.9). Conclusion: This study provides values of renal length (mean ± 2SD) in normal Indian children. Renal length can be easily calculated by derived linear regression equation. Key words: Anthropometry, India, Kidney, Size, Ultrasonography.
Shah I.,Bai Jerbai Wadia Hospital for Children |
Barot S.,Bai Jerbai Wadia Hospital for Children |
Madvariya M.,Bai Jerbai Wadia Hospital for Children
Indian Journal of Medical Microbiology | Year: 2015
Eosinophilic meningitis is defi ned as the presence of >10 eosinophils/μL in cerebrospinal fl uid (CSF) or at least 10% eosinophils in the total CSF leukocyte count. Eosinophilic meningitis has been reported in two case series and two case reports in India till date and has not been reported in children below 15 years of age. We present two children with eosinophilic meningitis with peripheral eosinophilia and the proposed etiologic agents based on the clinical setting and their response to antihelminthic agents.
Mhatre S.,13th floor KEM Hospital |
Madkaikar M.,13th floor KEM Hospital |
Desai M.,Bai Jerbai Wadia Hospital for Children |
Ghosh K.,13th floor KEM Hospital
Blood Cells, Molecules, and Diseases | Year: 2015
Inherited perforin deficiency is a rare autosomal recessive disorder that causes severe form of hemophagocytic lymphohistiocytosis (FHL2). The main aim of this study was to analyze the nature of gene mutations in a cohort of Indian patients with FHL2 and to utilize this knowledge for genetic counseling and prenatal diagnosis. Methods: 13 HLH patients with abnormal perforin expression on NK cells by flow cytometry were included in the study. The entire coding region and intronic splice sites of the PRF1 gene were sequenced from the genomic DNA of these patients. Results: 10 patients from the present series had an early presentation with severe clinical manifestations, while 3 had a delayed onset with unusual presenting features viz Hodgkin's lymphoma, tuberculosis and acute lymphoblastic leukemia. Sequence analysis revealed 11 different mutations (8 novel and 3 previously reported) spread over the entire coding region of PRF1 gene. Missense mutation Trp129Ser in heterozygous state was present in all the 3 patients with a delayed onset of the disease. Conclusion: A wide heterogeneity was observed in the nature of mutations in Indian FHL2 patients. Molecular characterization of PRF1 gene was not only used in the confirmation of diagnosis but also in genetic counseling and pre-natal diagnosis in affected families. © 2015 Elsevier Inc.
Rustagi T.,Bai Jerbai Wadia Hospital for Children
Journal of orthopaedic surgery (Hong Kong) | Year: 2013
Loeys-Dietz syndrome is characterised by vascular aneurysms, hypertelorism, and a bifid uvula. We report on an 11-year-old boy with Loeys-Dietz syndrome who presented with bilateral radial head dislocations and severe osteopaenia with changes of avascular necrosis in both hips causing an out-toeing, wide gait. Considering the poor prognosis for elbow movement and possible radial head dysplasia, surgical reduction of the radial heads was deferred. A subtrochanteric de-rotation osteotomy of the left hip was performed to improve the gait.
Sathe K.,Bai Jerbai Wadia Hospital for Children |
Ali U.,Bai Jerbai Wadia Hospital for Children |
Ohri A.,Bai Jerbai Wadia Hospital for Children
Indian Journal of Nephrology | Year: 2013
Several traditional medicines contain potentially toxic heavy metals. Heavy metal poisoning is not an uncommon cause of renal damage, although the diagnosis can be easily missed. We report a case of chronic ingestion of an ayurvedic medicine containing mercury in a 2-year-old girl, resulting in anuric renal failure due to acute interstitial nephritis.
Agashe M.V.,Bai Jerbai Wadia Hospital for Children |
Mehta R.,Bai Jerbai Wadia Hospital for Children |
Aroojis A.J.,Bai Jerbai Wadia Hospital for Children
Journal of Pediatric Orthopaedics Part B | Year: 2012
Habitual anterior dislocation of the hip joint is extremely rare. We believe this is the first case reported of a habitual anterior dislocation of the hip occurring in a child with cerebral palsy. The dislocation was painless, voluntary and used to occur every time the child would extend and externally rotate the limb. Operative intervention in the form of a varus derotation femoral osteotomy and anterior capsulorraphy was planned, but the caregivers of the patient were unwilling for the same. Hence, the patient was given an adduction and internal rotation brace and was counselled not to perform the activity needed to dislocate the hip. This case report calls attention to a unique problem previously not reported in cerebral palsy. © 2012 Lippincott Williams & Wilkins, Inc.
Shah I.,Bai Jerbai Wadia Hospital for Children |
Shah F.,Bai Jerbai Wadia Hospital for Children
Indian Journal of Gastroenterology | Year: 2016
We present four children with tyrosinemia and their response to NTBC [2-[2-nitro-4-trifluoromethylbenzoyl]-1, 3-cyclohexanedione]. One child received NTBC for only 3 months after which he was diagnosed to have hepatocellular carcinoma and underwent a living-related liver transplantation. The child is doing well post-transplant. Remaining three children have been on NTBC for almost 3 years, have normal liver functions, undetectable urine succinylacetone, and no portal hypertension or renal tubular acidosis. © 2016, Indian Society of Gastroenterology.