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

PURPOSE: Serum alkaline phosphatase (sALP) levels show great variation with age and sex in children and adolescents. We aimed to study pediatric age- and sex-specific reference ranges for sALP and how the pattern changed according to age . METHODS: Cross-sectional results from 1366 healthy Korean children and adolescents (10-19.9 years) from the Korean National Health and Nutrition Examination Survey were analyzed. We constructed age- and sex-specific reference data for sALP with a Hitachi Automatic Analyzer 7600 using Pureauto S ALP. We assessed its peak both in relation to age. RESULTS: Reference values for sALP according to age were constructed. Peak sALP levels were found at 12-13 years in boys (P < 0.001). Total sALP levels decreased to the adult level at the age of 17 in boys and 15 in girls, respectively. CONCLUSION: This study provides reference values for sALP by age for Korean children and adolescents. This normative data that includes upper and lower limits will provide a basis from which to better evaluate sALP levels, especially for bone disorders such as rickets and vitamin D deficiency. However, caution should be made in interpreting sALP levels because different buffers might lead to different values in the same serum.
Adolescent ; Adult ; Alkaline Phosphatase ; Buffers ; Child ; Humans ; Nutrition Surveys ; Reference Values ; Rickets ; Sex Characteristics ; Vitamin D Deficiency

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*

OBJECTIVETo study the effects of BMP-2 gene therapy on vascularization in repairing bone defects.

METHODSThe isolated rabbit mesenchymal stem cells (rBMSC), after being transfected by adenovirus carrying BMP-2 gene (Ad-BMP-2) and seeded on xenogeneic bone scaffolds, were used to repair 1.5 cm-long radius bone defects. Five methods were in use in the experiments: Ad-BMP-2 infected rBMSC plus antigen-free bovine cancellous bone (BCB, Group A), rBMSC-BCB plus reconstructed hBMP-2 (Group B1), Ad-LacZ infected rBMSC-BCB (Group C), rBMSC-BCB (Group D) and only BCB scaffolds (Group E). After 4, 8, and 12 weeks of the operations, capillary vessel ink infusion, vascular endothelial growth factor ( VEGF) immunohistochemical staining and histological examination were conducted.

RESULTSAfter 4 weeks of the operations, usually in Group A one newly formed artery was found in every pore between the trabeculae of the BCB. The density of these intraosseous vessels was high in the periphery and decreasing towards the center of the grafts; by transmission electron microscopy, osteoblasts were always next to vascular endothelial cells and gradually developed into osteocytes with the increase of capillary vessel; VEGF expression were apparently enhanced in mesenchymocytes.

CONCLUSIONSBMP-2 gene therapy, by up-regulating VEGF expression, indirectly induces vascularization of grafts and is of great value to the treatment of bone in union and bone defects.

Animals ; Bone Marrow Cells ; cytology ; Bone Morphogenetic Protein 2 ; genetics ; therapeutic use ; Bone Regeneration ; Bone Substitutes ; Cattle ; Forelimb ; blood supply ; diagnostic imaging ; Genetic Therapy ; Mesenchymal Stromal Cells ; Rabbits ; Radiography ; Tissue Engineering ; Transfection ; Vascular Endothelial Growth Factor A ; metabolism

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