Biomedical subjects
Preeti Shanbag
Publications and source records attributed to Preeti Shanbag.
Intravenous immunoglobulins in severe Guillian-Barre syndrome in childhood.
OBJECTIVE: This is a retrospective analysis of 25 children with severe Guillain-Barre syndrome admitted to our PICU. METHOD: All children were treated with intravenous immunoglobulins (IVIG) in a dose of 2 g/kg body weight over 2-5 days in addition to supportive and respiratory care. Seventeen children were elective admissions to the PICU whereas 8 children were transferred from other hospitals in a critical condition. Five of 8 of the late referrals died as compared to none of the elective admissions. RESULT: All 8 of the late referrals required mechanical ventilation as against 3 of the 17 elective admissions. Mean duration of PICU stay in the late referrals was 27 days as compared to 15 days in the elective admissions. CONCLUSION: The authors concur with previously published reports, that early use of IVIG could reduce the mortality and the need for intubation and mechanical ventilation.
Achalasia--alacrima--ACTH insensitivity syndrome (Triple A syndrome).
Triple A syndrome is characterized by achalasia of the cardia, alacrima, adrenocorticotrophic hormone (ACTH) resistant adrenal insufficiency and progressive neurological abnormalities including autonomic nervous dysfunction. An 8-year-old girl presented to the pediatric intensive care unit with sudden loss of consciousness and was diagnosed subsequently to have this condition. The authors present this condition since it is easily treatable and can be fatal if undiagnosed.
Medullary sponge kidney.
Medullary sponge kidney is a benign asymptomatic developmental anomaly of the kidney mostly seen in adult females. Presentation in childhood is uncommon. Urinary tract infection, nephrolithiasis, hematuria and hypercalciuria are the common complications. We report a eleven-year-old female child who presented with recurrent urinary tract infection and nephrolithiasis and was found to have bilateral medullary sponge kidney.
Persistent hyperinsulinemic hypoglycemia of infancy--successful therapy with nifedipine.
Recent studies have demonstrated a role for calcium channel blocking agents in the treatment of persistent hyperinsulinemic hypoglycemia of infancy. We report a 30 day old infant with PHHI whom we successfully treated with oral nifedipine alone.
The congenital long QT syndrome.
OBJECTIVE: The long QT syndrome (LQTS) is a disorder of the electrical system of the heart, due to dysfunction of the ion channels and involving the repolarisation process. The inherited form occurs when there is a mutation in one of the genes which encode the making of a channel. Prolongation of the QT interval renders the patient vulnerable to an arrythmia called torsade de pointes, resulting in syncope and sudden death. METHODS: Three children with the congenital long QT syndrome presented to the pediatric department, one of them also having a 2:1 atrio-ventricular block. The parents and siblings of these children were screened for the long QT syndrome with an electrocardiogram. 2D echocardiography was done to rule out structural abnormalities and audiometry for deafness. RESULTS: Four family members were identified on screening to have LQTS. Propranolol was started on all children with LQTS. The child with heart block also received a pacemaker. LQTS must be considered in all patients presenting with syncope especially if associated with deafness and/or a family history of sudden deaths in infancy or childhood. CONCLUSION: The corrected QT interval must be determined in all children with heart block since the two conditions are often associated.