To Evaluate the clinical characteristics of childhood-onset systemic lupus erythemoatosus (SLE) and analyse the factors related to outcome of renal function in lupus nephritis, we reviewed medical records of 18 cases of SLF diagnosed at the Department of Pediatrics. Pusan National University Hospital from January 1981 to December 1990. The results were as follow: 1)Male to female ratio was 1:2.6. 2) Nephropathy was the most common initial impression, and 8 cases were diagnosed as SLE at first. 3) The clinical menifestation. was ANA (+), malar rash, renal disorder, hematologic disorder in frequency. 4) Diffuse proliferative lupus nephritis was the most common in renal histopathologic studies. 5) Among the follow-up cases, active lupus was 39%, remission was 28%. 6) As factors related to outcome of lupus nephrits were, hypertension and proteinuria were siginificant statistically.
Busan ; Exanthema ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Medical Records ; Pediatrics ; Proteinuria

A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

No abstract available.
Humans ; Infant, Newborn*

Rupture of the sinuses of Valsalva is not common in childhood. It is frequently associated with other heart disease, most commonly with VSD. It is classified into four types, anatomically. The symptoms are differentiated as acute, gradual progression, and unruptured aneurysm. We experienced 2 cases of rupture of the sinuses of Valsala, which were corrected completely after operation. The patients were 14 year old boy with his chief complaint of dyspnea and chest pain, and an 11 year old girl with chief complaint of exertional dyspnea.
Adolescent ; Aneurysm ; Chest Pain ; Child ; Dyspnea ; Female ; Heart Diseases ; Humans ; Male ; Rupture*

No abstract available.
Bacteria*

Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

No abstract available.
Colon*

No abstract available.
Diabetes Mellitus, Type 1* ; Echocardiography*

BACKGROUNDS: Behavioral and psychological symptoms as well as cognitive impairement are very disturbing symptoms in dementia. It is important in managing dementia patient to control these behavioral and psychological symptoms. In this study, we examined the effect of risperidone and optimal therapeutic dosage on controlling these behavioral and psychological symptoms of dementia (BPSD). METHODS: 57 patients (male: 19, female: 38) with dementia, aged 65 and older in Buyeo geriatric hospital located in Buyeo-gun, chung-nam, were finally included in this study. risperidone was administrated to these subjects for 10 weeks to control BPSD. Behavioral Pathology in Alzheimer's Disease Rating Scale (BEHAVE-AD) was rated before administration of risperidone and after administration of risperidone for 10 weeks to evaluate the improvement of BPSD. Global Deterioration Scale (GDS) was rated before administration of risperidone. The correlation between baseline GDS score and the change of sum score of BEHAVE-AD was analyzed. RESULT: The mean dose of resperidone at the endpoint was 0.706+/-0.522 mg/d. The significant reduction of the sum score of BEHAVE-AD was observed in subjects after administration of risperidone for 10 weeks. Clinical improvement (> or =50% reduction of sum score of BEHAVE-AD from baseline sum score) was showed in 32 subjects (56.1%) among 57 subjects. Also there was weakly negative correlation between baseline GDS score and the change of sum score of BEHAVE0AD. CONCLUSION: Risperidone was effective in controlling BPSD of dementia patients at 0.706+/-0.522 mg/d.
Alzheimer Disease ; Dementia* ; Female ; Humans ; Pathology ; Risperidone*

PURPOSE: Our purpose was to deterrnine the efficacy of maternal corticosteroid therapy on the morbidity of premature infants between 26 and 31 weeks' gestation. METHOD: A total of 62 premature infants between 26 and 31 weeks gestation admitted to pediatric department of National Medical Center from Nov, 1990 to June 1996 were analyzed to evaluate the efficacy of prenatal corticosteroid therapy on the morbidity such as hyaline membrane disease, intreventricular hernorrhage, necrotizing enterocolitis, sepsis, neonatal death, days on ventilation and hospital days. RESULT: Among 62 women who delivered premature infants between 26 and 31 weeks, 22 received betamethasone before delivery and 40 did not. 1) The rate of hyaline membrane disease was less in the betamethasone group (41% vs. 70%, P<0.05). 2) The rate of intracranial hemorrhage was less in the betamethasone group (0 vs. 20%, P<0.05). 3) The days of ventilator care was less in the betamethasone group among survival cases (27+/-3.2 vs. 5.2+/-4.6, P<05). 4) Arnong 14 women who delivered at 26 to 28 weeks, 4 received betamethasone before delivery and 10 did not. The rate of neonatal death was less in the betamethasone group (o% vs. 80%, P<0.05). 5) Among 48 women who delivered at 29 to 31 weeks, 18 received betamethasone before delivery and 30 did not. The rate of hyaline membrane disease was less in the betamethasone group (39% vs. 73%, P<0.05). CONCLUSION: Betamethasone appears to reduce hyaline membrane disease, intraven- tricular hemorrhage, neonatal death and the morbidity significantly in premature infants between 26 and 31 weeks' gestation.
Betamethasone ; Enterocolitis, Necrotizing ; Female ; Hemorrhage ; Humans ; Hyaline Membrane Disease ; Infant, Newborn ; Infant, Premature* ; Intracranial Hemorrhages ; Pregnancy ; Sepsis ; Ventilation ; Ventilators, Mechanical