Turkish J Pediatr
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Tay-Sachs disease (GM2 gangliosidosis I) is an autosomal recessive lysosomal-storage disorder confined to the central nervous system, resulting from deficiency of hexosaminidase A. The case presented is of a twelve-month-old girl brought to the hospital because of mental-motor deterioration and convulsions. ⋯ There was no organomegaly. The diagnosis of Tay-Sachs disease was made by means of absence of serum hexosaminidase A activity.
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Supraventricular tachycardia (SVT) is a life-threatening dysrhythmia in childhood. Initial treatment includes classic vagal maneuvers, some pharmacological agents, cardioversion and application of ice water to the face. In this paper we report our results of ice water application to the face in SVT. ⋯ There was no recurrence in six patients. No response was noted in only one patient. Application of ice water to the face is an easy, safe, effective and repeatable procedure in the initial treatment of supraventricular tachycardia.