No abstract available.
Extracorporeal Circulation* ; Heart* ; Potassium* ; Thoracic Surgery*

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

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 7 day old female baby suffered from perinatal asphyxia and trauma at birth. Subsequently, she had a violeceous subcutaneous plaque on back. The clinical setting, the skin manifestation and the histologic findings of the lesion were diagnostic of subcutaneous fat necrosis of the newborn. Histologic findings revealed fat necrosis with infiltration of inflammatory cells, foreign body giant cells and several fat cells contain needle shaped cleft in radial arrangement. Four weeks latter, the skin lesion was healed spontaneously.
Adipocytes ; Asphyxia ; Fat Necrosis ; Female ; Giant Cells, Foreign-Body ; Humans ; Infant, Newborn* ; Necrosis* ; Needles ; Parturition ; Skin ; Skin Manifestations ; Subcutaneous Fat*

The author reviewed the effectiveness of triple procedure in terms of the visual acuity and the intraocular pressure control after 6 months follow up of twenty-four consecutive glaucoma patients who was treated with triple procedure: trabeculectomy with simultaneous lens extraction and intraocular lens implanation. At 6 months after operation, the mean intraocular pressure was 7.0mmHg lower than the preoperative level, and the kind of glaucoma medication was decreased from 2.1 to 0.6. The visual acuity improved in 67% at two months and 93% at six months after operation, respectively. The several complications such as hyphema, shallow anterior chamber with wound leaking, posterior capsular opacity and uncontrolled intraocular pressure were appeared. Effectiveness of triple procedure in terms of intraocular pressure control and visual acuity improvement seems to be similar to that of the operation done separately.
Anterior Chamber ; Cataract ; Follow-Up Studies ; Glaucoma* ; Humans ; Hyphema ; Intraocular Pressure ; Lenses, Intraocular ; Trabeculectomy ; Visual Acuity ; Wounds and Injuries

VURD syndrome is a disorder characterized by a posterior urethral valve, unilateral reflux, and ipsilateral renal dysplasia. This syndrome is not uncommon, with an incidence in the range of 15 to 20% in patients with posterior urethral valve. Etiology is not clear, but there are a few reports that renal dysplasia is either a result of hydrostatic pressure transmitted to the developing kidney or a common teratogenic factor resulting in urethral obstruction, renal dysplasia, and vesicoureteral reflux. Infants with posterior urethral valves and persistent unilateral reflux after valve resection often have an associated nonfunctioning, dysplastic kidney. Misinterpretation of initial radiographic studies performed without coinciding bladder drainage results in a missed diagnosis of this syndrome and misguided surgical management. Functional assessment by a renal scan with bladder catheter drainage will avoid unnecessary staged or reconstructive procedures. Nephoureterectomy is recommended at about 1 year of age to improve voiding dynamics and to avoid infection. We experienced a case of VURD syndrome, first presenting bilateral hydronephrosis by ultrasonography during the prenatal period. Voiding cystourethrography revealed dilated posterior urethra and left vesicoureteral reflux, and DMSA scan showed a nonfunctioning left kidney.
Catheters ; Diagnosis ; Drainage ; Humans ; Hydronephrosis ; Hydrostatic Pressure ; Incidence ; Infant ; Kidney ; Succimer ; Ultrasonography ; Urethra ; Urethral Obstruction ; Urinary Bladder ; Vesico-Ureteral Reflux

BACKGROUND AND OBJECTIVES: The pathological findings of Non-Q wave myocardial infarction(NQMI) on ECG did not always correspond to subendocardial infarction(SEMI). The purpose of this study was to evaluate the diagnostic validity of ECG for SEMI by myocardial contrast echocardiography (MCE) in the patients with acute myocardial infarction(AMI). MATERIALS AND METHODS: The study population was 84 patients who underwent MCE under the diagnosis of AMI. MCE was performed by intracoronary injection of sonicated Hexabrix into the infarct related artery and SEMI was diagnosed by inspecting endocardial defect with epicardial enhancement on MCE. RESULTS: (1) Among 19 NQMI cases, 7 cases showed SEMI with MCE score 0.5, 11 cases with score 1, and 1 case with score 0. Among 65 Q-wave MI(QMI) cases, only 5 cases showed SEMI. (2) 7 cases who had NQMI with SEMI showed LV wall motion recovery at follow-up echocardiography except 1 case. Whereas, of 5 QMI cases who had SEMI, only 1 case improve LV wall motion. CONCLUSION: NQMI on ECG does not always imply SEMI on MCE, but the absence of pathologic Q wave in the patients with SEMI is thought to be a predictive factor of the recovery of LV wall motion.
Arteries ; Diagnosis ; Echocardiography* ; Electrocardiography* ; Follow-Up Studies ; Humans ; Infarction* ; Ioxaglic Acid

BACKGROUND AND OBJECTIVES: The pathological findings of Non-Q wave myocardial infarction(NQMI) on ECG did not always correspond to subendocardial infarction(SEMI). The purpose of this study was to evaluate the diagnostic validity of ECG for SEMI by myocardial contrast echocardiography (MCE) in the patients with acute myocardial infarction(AMI). MATERIALS AND METHODS: The study population was 84 patients who underwent MCE under the diagnosis of AMI. MCE was performed by intracoronary injection of sonicated Hexabrix into the infarct related artery and SEMI was diagnosed by inspecting endocardial defect with epicardial enhancement on MCE. RESULTS: (1) Among 19 NQMI cases, 7 cases showed SEMI with MCE score 0.5, 11 cases with score 1, and 1 case with score 0. Among 65 Q-wave MI(QMI) cases, only 5 cases showed SEMI. (2) 7 cases who had NQMI with SEMI showed LV wall motion recovery at follow-up echocardiography except 1 case. Whereas, of 5 QMI cases who had SEMI, only 1 case improve LV wall motion. CONCLUSION: NQMI on ECG does not always imply SEMI on MCE, but the absence of pathologic Q wave in the patients with SEMI is thought to be a predictive factor of the recovery of LV wall motion.
Arteries ; Diagnosis ; Echocardiography* ; Electrocardiography* ; Follow-Up Studies ; Humans ; Infarction* ; Ioxaglic Acid

Neurofibromatosis(Von Recklinghausen's disease) is an autosomal dominant disease that affects one in 2,500-3,000 births. The clinical presentation is characterized by multiple pigmented lesions of the skin, known as cafe-au-lait spots, and neurofibromas of the skin. The criteria for diagnosis are at least six cafe-au-lait spots, each greater than 1.5cm in diameter and positive family history and biopsy of a neurofibroma. Management of benign neurofibromatosis must be individualized for each patient and ranges from incisional biopsy to wide local excision. Genetic counseling is advised for all patients with this disease, since no cure is available. We report the case of multiple neurofibromatosis with oral, parapharyngeal space and whole body lesions that was treated with surgical excision.
Biopsy ; Cafe-au-Lait Spots ; Diagnosis ; Genetic Counseling ; Humans ; Neurofibroma ; Neurofibromatoses* ; Parturition ; Skin

No abstract available.
Animals ; Mice* ; Mycobacterium bovis*