Neurologic Outcomes of Periventricular Leukomalacia / 대한소아신경학회지

PURPOSE: Periventricular leukomalacia(PVL) is the most important cause of cerebral palsy in premature infants. However, there are relatively few studies demonstrating the correlation between ultrasound findings and neurologic outcomes of low-birth-weight infants. To clarify the situation, we analyzed ultrasound findings and neurologic outcomes of many infants with periventricular leukomalacia. METHODS: Our study includes 36 infants with PVL, born in Chungnam National University Hospital, from October 1998 to December 2001. 25 infants with bilateral PVL were compared with 11 infants with asymmetric PVL. For a period of 12 to 48 months, the children were evaluated with neurologic assessments. RESULTS: In infants with bilateral lesions, 88%(22/25) of them had evidence of cerebral palsy. 16 children had spastic quadriplegia and others had spastic diplegia. 7 children with unilateral lesions were free of motor sequele at follow up. Other neurologic handicaps(mental retardation, opthalmologic abnormality or epilepsy) were not related with the symmetry or sizes of the lesions. CONCLUSION: In this study, unfavorable neurologic outcomes of bilateral PVL are confirmed. Therefore, every effort should be made to prevent the development of periventricular leukomalacia as well as not to miss the diagnosis. In addition, if there are any small lesions, regular neurologic assessments and early start of rehabilitation programs should be done.

Protein C as a Differential Marker for Bacterial Infection among Pediatric Patients with Fever / 소아과

PURPOSE: This study was performed to find the clinical significance in protein C as a differential marker in the beginning stage of infection and prognosis factor in severe infection among pediatric patients who were admitted due to fever. METHODS: A total of 40 pediatric patients who had temperatures higher than 37.5degrees C on admission at the Department of Pediatrics, Chungnam National University between December, 2002 and August, 2003 were enrolled. Total white blood cell count(WBC), erythrocyte sedimentation rate(ESR), C-reactive protein(CRP) and protein C were performed for those patients on admission. Clinical progress, diagnosis and prognosis were reviewed for these patients. The 40 patients were divided into two groups based on the diagnosis of bacterial and nonbacterial infections. RESULTS: Twenty patients(50%) were suspected of bacterial infections that showed positive results in blood, sputum, urine, and spinal cord fluid. There were eight cases with bacterial pneumonia, five with urinary tract infection, four with bacterial meningitis, two with cellulitis, and one with typhoid fever. The remaining 20 patients were diagnosed with nonbacterial infections because they had negative results in blood cultures. ESR and CRP were increased beyond normal range in both groups. However, protein C was significantly decreased in the bacterial infection group and yet normal range in the nonbacterial infection group(P<0.05). CONCLUSION: Protein C can be used as a differential marker in order to distinguish between bacterial and nonbacterial infections. In addition, protein C can possibly be used as a prognostic factor that can predict severe infection.

A Case of Congenital Partial Nephrogenic Diabetes Insipidus

The most common form of genetic nephrogenic diabetes insipidus(NDI), a rare inherited disorder, is congenital and is transmitted in an X-linked recessive mode. It is refractory to the antidiuretic effect of normal to moderately increased levels of plasma arginine vasopressin(AVP) but, in some cases, may respond to high levels of the hormone or its analogue, deamino-D-arginine vasopressin(DDAVP). X-linked congenital NDI has now been linked to over 128 different mutations in diverse coding regions of the AVP receptor 2(AVPR2) gene. The functional effects of these mutations vary from complete loss of responsiveness to a simple shift to the right in the dose response curve. We report a case of congenital partial NDI, with transversion of A to G at codon 280 of the AVPR2 gene, resulting in a subsequent change of amino acid from tyrosine to cysteine, and that has been effective with hydrochlorothiazide and high dose of DDAVP.