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1.
Journal of the Korean Society of Pediatric Nephrology ; : 119-124, 2006.
Article in Korean | WPRIM | ID: wpr-206566

ABSTRACT

PURPOSE: The prognosis of acute poststreptococcal glomerulonephritis(APSGN) has been reported to be favorable. However, several studies have reported that patients with nephrotic range proteinuria in the acute phase or persistent proteinuria may progress to chronic renal failure. To elucidate this further, we analyzed the features of proteinuria and its prognosis in pediatric patients with APSGN. METHODS: A total of 48 children with APSGN admitted to our hospital between Jan. 2000 and Dec. 2004 were included. After discharge from the hospital, patients were regularly followed up every month by clinical evaluations and laboratory tests including routine urinalysis and quantification of proteinuria. RESULTS: Age of the patients ranged from 3 to 15 years(median 5.8 years) at the time of disease onset. Proteinuria was present in 34(70.8%) patients and 5 of them showed heavy proteinuria. Proteinuria normalized within one month in most patients(82.3%) and there was no one with proteinuria after 6 months. Cyclosporine A(5 mg/kg/day in two divided doses) was given to 3 patients with massive proteinuria that lasted longer than 2 months and the result was complete remission within 4 months. CONCLUSIONS: Our data indicated that the prognosis of APSGN during childhood is excellent. Children with severe proteinuria or subnormal renal function in poststreptococcal glomerulonephritis had favorable prognosis without chronic renal failure, and children with crescentic formation also had favorable prognosis. Three patients who continued to have heavy proteinuria for more than 2 months received cyclosporine A and remission of proteinuria was achieved within a couple of months.


Subject(s)
Child , Humans , Cyclosporine , Glomerulonephritis , Kidney Failure, Chronic , Nephrotic Syndrome , Prognosis , Proteinuria , Urinalysis
2.
Journal of the Korean Child Neurology Society ; (4): 215-223, 2006.
Article in Korean | WPRIM | ID: wpr-163800

ABSTRACT

PURPOSE:Mitochondrial disorder is a progressive disease, but there are no specific treatment modalities to prevent the progression. Also, there has been little understanding on the pattern of disease progression nor natural history. The aim of this study was to elucidate the initial clinical phenotypes, patterns of the disease progression, and its natural history of the patients with mitochondrial A3243G mutation. METHODS:Among the patients with biochemically or genetically confirmed mitochondrial disorders, 7 patients with A3243G mutation were included in a 7 year follow-up observation(range: 3-11 years). We classified the patients into two groups by the initial clinical presentations:systemic and neurologic onset. They were clinically evaluated with serial brain MRI and MRS for the evaluation of the disease evolution patterns. RESULTS:The clinical manifestations of mitochondrial A3243G mutation were extremely variable; seizure, headache, dementia, myopathy, sensorineural hearing loss, external ophthalmoplegia, diabetes mellitus, cardiomyopathy, easy fatigability, and short stature. Among the 7 patients, 4 patients initially presented neurologic symptom such as seizure(3) and headache(1), and 3 patients systemic symptoms such as DM(2) and easy fatigability(1). All the patients with neurologic onset showed relentless progression with recurrent stroke- like episodes and intractable seizures, and finally fell into be functionally dependent states or death. All the patients with systemic onset showed clinically silent periods for 3-10 years, and still they were in functionally independent states despite subsequent neurologic symptoms. CONCLUSION:We could find out the relationship between initial clinical phenotypes and final outcomes in mitochondrial A3243G mutation. However, the population is small in this study so that a larger scaled analysis is needed.


Subject(s)
Humans , Brain , Cardiomyopathies , Dementia , Diabetes Mellitus , Disease Progression , Follow-Up Studies , Headache , Hearing Loss, Sensorineural , Magnetic Resonance Imaging , Mitochondrial Diseases , Muscular Diseases , Natural History , Neurologic Manifestations , Ophthalmoplegia , Phenotype , RNA, Transfer , Seizures
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