Résumé
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.
Résumé
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.