Maternal Overt Hypothyroidism and Neurobehavioral Outcome of Neonates: A Cohort Study from an Iodine-deficient Area of Northern India.

Objective: To study the relation between maternal overt hypothyroidism and neurodevelopmental outcome of neonates in iodine-deficient region of Northern India (Kashmir Valley). Design: Prospective cohort study. Setting: Endocrinology department of a tertiary-care hospital. Participants: 82 hypothyroid pregnant women were enrolled and followed up till delivery. The neonates born to this group represented the case neonates. 51 euthyroid healthy pregnant women were selected as control group. The neonates born to these mothers served as controls. Main outcome measures: Early neonatal behavioral assessment at 3-4 weeks of age. Results: The mean TSH and free T4 in neonates of mothers with well controlled hypothyroidism was significantly different from those born to mothers with poorly controlled hypothyroidism and controls in 1st trimester, but the difference was statistically insignificant for 2nd and 3rd trimester values. Conclusion: Overt maternal hypothyroidism in iodine-deficient area constitutes a risk factor for an abnormal neurobehavioral development of affected child.

Acute Intermittent Porphyria with Transient Cortical Blindness.

Acute intermittent porphyria is a hereditary disorder characterized by deficient activity of the enzyme porphobilinogen deaminase. It manifests with occasional neurovisceral crises due to overproduction of porphyrin precursors. We report a 12 year old male child with acute intermittent porphyria, who presented with encephalopathy and transient blindness of cerebral origin.

Experience with oral mexiletine in primary erythromelalgia in children

Primary erythromelalgia is characterized by burning pain, redness, and warmth in the extremities. We present two cases of primary erythromelalgia both of whom presented with a history of several months of severe burning pain in both hands and feet. Both patients had received multiple pain medications with no improvement in symptoms. Pain was relieved by putting affected parts in ice cold water, which resulted in immersion injury of the affected parts. Both patients stopped taking part in school and social activities. We tried oral mexiletine, a class Ib antiarrythmic agent, in view of its reported role in various chronic painful conditions. Dramatic improvement was observed with its use. Both patients improved after several weeks of use, and there were fewer soaking episodes. We observed no adverse effects with mexilitine therapy

Nosocomial sepsis-induced late onset thrombocytopenia in a neonatal tertiary care unit: a prospective study

Late onset sepsis [LOS] [onset of sepsis >72 hours of age or nosocomial sepsis] is an important cause of morbidity and mortality in the neonatal intensive care unit [NICU]. Thrombocytopenia is an important complication of sepsis. We investigated the incidence of thrombocytopenia in LOS patients and studied the influence of various parameters on platelet response. Infants born in the level 3 neonatal intensive care unit between January 2002 and December 2006 with documented LOS were included in this prospective study. Multiple hemograms with platelet counts, bacterial blood culture and fungal blood culture were obtained in all patients. Demographic and clinical data were compared between patients without thrombocytopenia and with mild, moderate and severe thrombocytopenia. Duration of thrombocytopenia in relation to type of organism and mortality with respect to degree of thrombocytopenia were also studied. Of 200 patients with culture-proven nosocomial sepsis, 119 [59.5%] patients developed thrombocytopenia [platelet count <150x10[9]/L]. In our series Klebsiella pneumoniae was the most frequently isolated organism [125/200, 62.5%] and the incidence of thrombocytopenia was 60.0% [75/125]. However, the incidence of thrombocytopenia was highest among patients who had concurrent bacterial and fungal sepsis [28/31, 90.3%] Coagulasenegative staphylococcal [CoNS] sepsis was present in 21 [10.5%] patients and the incidence of thrombocytopenia was 33.3%. Isolated fungal sepsis was present only in 6 [3%] patients and the incidence of thrombocytopenia was 66.0%. The incidence of thrombocytopenia was highest among preterm babies and low-birth weight [LBW] babies. Twenty-seven percent [54/200] of babies presented with mild thrombocytopenia, 20% [40/200] presented with moderate thrombocytopenia, and 12.5% [25/200] developed severe thrombocytopenia. Severity of thrombocytopenia was also directly related to the presence of necrotizing enterocolitis [NEC] and disseminated intravascular coagulation [DIC]. The mortality rate was significantly associated with the degree of thrombocytopenia. LOS sepsis is an important risk factor for thrombocytopenia in the NICU. Fungal and gram-negative sepsis are frequently associated with a decreased platelet count. Sepsis-induced thrombocytopenia is more common among LBW babies and preterm babies. The mortality rate is significantly related to degree of thrombocytopenia