Turkish J Pediatr
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Factor VII deficiency is a rare congenital coagulopathy. Prolonged prothrombin time with normal partial thromboplastin time indicates factor VII deficiency. For the definitive diagnosis, the specific factor VII level should be investigated. ⋯ After antibiotic therapy and fresh frozen plasma replacement his clinical status improved but the prothrombin time continued to be prolonged. On the 14th day, just before the end of antibiotic therapy, the infant died of sudden intracerebral hemorrhage. In this article, the clinical features and management of factor VII deficiency are discussed.
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Polyarteritis Nodosa (PAN) is a rare disease in childhood. No single pattern of clinical presentation characterizes this disease, but abdominal pain, central or peripheral nervous system disease, arthritis, myalgia and skin lesions occur at some time during the course of the illness. In this case a 16-year-old boy who presented with abdominal pain, elevated sedimentation rate associated with hypertension, and a high level of renin, all of which were detected during his hospitalization, suggested the diagnosis of PAN, and renal angiography was performed. Characteristic renal aneurysms were visualized and the diagnosis was confirmed.
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A retrospective analysis of the computerized data of patients admitted to our Emergency Unit Inpatient Service in 1991 was conducted to obtain data about age, sex, referred sources, admission period, monthly admission rates, diagnoses and eventual outcome. More than 47% of patients were younger than one year of age. The most common causes for hospital admission were infectious, respiratory and neurological diseases. ⋯ More than 60% of patients were treated by the Emergency Unit staff. The net mortality rate was 2.9%, infectious diseases being the most common cause of mortality. We conclude that demographic and diagnostic data regarding admissions to the Emergency Unit can be utilized to develop new strategies for patient care and to reorganize education programs for pediatric residents.
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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.