No abstract available.
Drug Therapy* ; Germ Cells* ; Neoplasms, Germ Cell and Embryonal*

A 59-year-old male farmer, an alcohol addict, has experienced repeated bullous skin lesions on the erythematous base over the exposed areas of tbe skin, especially on the face and hands, after sun-light exposure. The face showed hyperpigmenation, hypertrichosis and sclerodermoid changes. And also there were hypopigmented scars on the dorsum of the hands and cIaw-1ike fingers. Characteristic laboratory findings were as follows: Urine appeared portwine color and fluoresced under the Woods Iight. A 24-hour urine collection contamed 4l32ug of uroporphyrin and 3815ug of corproporphyrin. BSP retention(7% in 45 Min.) was increased, and A/G ratio(0. 8:1) was inverted. Gamma globulin(44, 9%) was markedly increased on serum electrophoresis. Roentgenograms revealed minimal tuberculosis on the chest P-A and superficial gastritis on the upper G-I series. Electrocardiogram showed left ventricular hypertrophy. Pathological findings of the specimen obtained from sclerodermoid lesion of the forehead revealed hypermelanosis in the basal cell layer and amorphous, basophilic collagen degeneration in the upper dermis, on the other hand loss of sinusoids due to swelling of hepatocytes and chomic inflammatory cell infiltrate in the interlobular septa were noticed on the specimen taken from the liver. For treatment, phlebotomy and metabolic alkalinization were trjed.
Basophils ; Cicatrix ; Collagen ; Dermis ; Electrocardiography ; Electrophoresis ; Fingers ; Forehead ; Gastritis ; Hand ; Hepatocytes ; Humans ; Hyperpigmentation ; Hypertrichosis ; Hypertrophy, Left Ventricular ; Liver ; Male ; Middle Aged ; Phlebotomy ; Skin ; Thorax ; Tuberculosis ; Urine Specimen Collection ; Wood

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.
Adrenal Glands ; Adrenalectomy* ; Biopsy ; Drug Therapy ; Humans ; Liver* ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neuroblastoma* ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography, Prenatal

No abstract available.
Humans ; Interferon-alpha* ; Interferons* ; Melanoma* ; Ulcer*

No abstract available.
Anterior Cruciate Ligament* ; Autografts* ; Tendons* ; Transplants*

Thin basement membrane nephropathy(TBMN) is defined histologically as follows: 1) By light rnicroscopy only minor abnormalities are detected in the glomeruli at most minor mesangial widening. 2) By electron microscopy, diffuse thinning of glomerular basement rnembrane is demonstrated. 3) By immunofluorescence, absence of immunoglobulins and complement components is demonstrated. 4) Alport's syndrome and systemic diseases that may affect the glomerular structure have been excluded. TBMN presented frequently with recurrent or persistent microscopic hematuria. Massive proteinuria such as in nephrotic syndrome rarely occurs in TBMN. We reported two cases of TBMN presented with typical minimal change nephrotic syndrome.
Basement Membrane* ; Complement System Proteins ; Fluorescent Antibody Technique ; Hematuria ; Immunoglobulins ; Microscopy, Electron ; Nephritis, Hereditary ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Proteinuria

No abstract available.
Fibronectins* ; Osteogenesis*

No abstract available.

The infantile polycystic kidney disease is rare fetal urinary tract anomaly. It is inherited with an autosomal recessive pattern and recurrence rate is 25%. The gene locus is on chromosome 6p. The pathogenesis of infantile polycystic kidney is the primary defect of the collecting ducts. The ultrasonographic finding of infantile polycystic kidney is oligohydramnios, bilaterally symmetrical enlarged kidneys with maintenance of their reinform shape. The differential diagnosis with adult polycystic kidney disease is important. The massive enlargement of the kidneys is rarely seen in adult polycystic kidney disease and the examination of the parents and other members of the family is helpful to confirm the adult polycystic kidney disease. If there is severe renal involvements, stillbirth or neonatal death secondary to pulmonary hypoplasia would be developed. If it were diagnosed before viability, termination of pregnancy is recommended. In a fetus at risk, diagnosed after viability, pregnancy termination is also recommended since this condition is uniformly fatal. We present a case of infantile polycystic kidney.
Diagnosis, Differential ; Female ; Fetus ; Humans ; Kidney ; Oligohydramnios ; Parents ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant ; Pregnancy ; Recurrence ; Stillbirth ; Urinary Tract

No abstract available.
Nuchal Cord* ; Ultrasonography*