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.
Bile Duct Neoplasms* ; Bile Ducts* ; Bile*

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*

No abstract available.
Thrombocytosis*

No abstract available.
Humans ; Tuberculosis*

No abstract available.
Colon*

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

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