No abstract available.
Child* ; Humans ; Nephrotic Syndrome*

No abstract available.
Thyroid Gland* ; Thyroid Nodule*

No abstract available.
Anemia ; Gaucher Disease* ; Humans ; Siblings* ; Thrombocytopenia

Biliary atresia is a surgical disease of infancy with a very poor prognosis usually attributed to the rarity of surgically correctable case. This paper presents a case of incorrectable type of biliary atresia 50 days old Direan female infant who were subjected to the Modified Kasai Operation She had shown good bile excretion after surgery but developed recurrent fever, severe hypocalcemia and roentgenographically prover rickets during the follow up periods. She died 7 months after the operation.
Bile ; Biliary Atresia* ; Female ; Fever ; Follow-Up Studies ; Humans ; Hypocalcemia ; Infant ; Prognosis ; Rickets

No abstract available.
Thyrotropin*

No abstract available.
Vitelline Duct*

OBJECTIVE: This study was proposed to evaluate the electrophysiologic changes in central motor conduction and in silent period (SP) after paraspinal transcutaneous electrical stimulation near caudal area of the spinal cord. METHOD: Conditioning stimulation was applied to T12 paraspinal area for 20 minutes using interferential current therapy (80~100 Hz) in 11 healthy subjects. The amplitude and latency of central motor conduction and duration of SP were measured in motor evoked potential (MEPs) by using magnetic stimulator, before and after the conditioning stimulation. These variables were recorded in both tibialis anterior muscle, innervated from stimulated spinal area, and both abductor pollicis brevis, innervated from cervical cord not directly stimulated by electrical stimulation. RESULTS: After conditioning stimulation, the amplitudes of central motor conduction decreased (p<0.01), and the latencies did not change in both cervical and lumbar muscles in transcranial and spinal MEP studies, and the duration of SP was decreased in same manner (p<0.01). CONCLUSION: These results mean that the excitability of anterior horn cells decreases and the supraspinal inhibitory mechanism of the central motor conduction is suppressed by a certain conditioned electrical cutaneous stimulation in entire spinal cord.
Anterior Horn Cells* ; Electric Stimulation* ; Evoked Potentials, Motor ; Muscles ; Spinal Cord ; Transcutaneous Electric Nerve Stimulation

PURPOSE: Many evidences about hyperuricemia as a risk factor to hypertension and renal progression in kidney diseases have been reported. We have analyzed the impact of uric acid on renal progression of IgA nephropathy while getting rid of possible confounding variables and revealed the possible pathophysiology of uric acid in terms of hypertension or renal vasculopathy provoked by uric acid. METHODS: We selected 172 patients with IgA nephropathy diagnosed by renal biopsy at Seoul National University Hospital. We adapted the criteria of renal progression as the final value of serum creatinine increased more then twice compared to the value at renal biopsy. RESULTS: Serum uric acid was correlated with the severity of interstitial fibrosis and tubular atrophy and the presence of artherosclerosis. The incidence of hypertension during follow-up period was not different between low uric acid group and high uric acid group. The high uric acid level was an independent risk factor to renal progression in IgA nephropathy with multivariate analysis regardless of the presence of hypertension or artherosclerosis of renal pathologic finding. The high uric acid level was also a risk factor to renal progression in patients with estimated GFR more than 60 mL/min/1.73m2. CONCLUSION: Serum uric acid was related to the presence of artherosclerosis and the renal progression of IgA nephropathy.
Atrophy ; Biopsy ; Confounding Factors (Epidemiology) ; Creatinine ; Fibrosis ; Follow-Up Studies ; Glomerulonephritis, IGA* ; Humans ; Hypertension ; Hyperuricemia ; Immunoglobulin A* ; Incidence ; Kidney Diseases ; Multivariate Analysis ; Risk Factors ; Seoul ; Uric Acid*

Endothelin derived from the endothelial cells of microvessel is a potent vasoactive peptide, which has various physiologic actions in many internal organs. The fact that endothelin receptors are present on the osteoblastic cells suggests that endothelin play a role in bone metabolism. This study was done to study the effect of endothelin-1 on osteoblast and the combined effect of dexamethasone and endothelin-1 on osteoblast. Human osteoblasts isolated from ilium were cultured in DME/F12 medium, and divided into 5 groups; Group 1 (control), Group 2(10(-7)M endothelin-1), Group 3(10(-7)M endothelin-1+1:2500 monoclonal antibody), Group 4(10(-7)M dexamethasone+10(-7)M endothelin-1), and Group 5(10(-7)M dexamethasone). [3H]-thymidine uptake in groups was 23373.2+/-2722.4 cpm/well, significantly higher than that of control (P<0.05), and the increase was blocked by the addition of monoclonal antibody to endothelin(group 3). [3H]-thymidine uptake in groups adding steroid with or without endothelin was significantly lower than that of other groups (P<0.05). Group 2 showed marked increase in type I procollagen mRNA compared with other groups, but group 3 and 4 showed no significant effect on the expression of type I procollagen mRNA. In histochemical staining for alkaline phosphatase activity, the cells in groups with steroid were strongly positive in staining, large in size and looked well differentiated. Osteocalcin synthesis was also increased in groups with steroid treatment compared with other groups. This study demonstrated that endothelin-1 stimulated DNA synthesis and the expression of type I procollagen mRNA in human osteoblasts, and inhibited alkaline phosphatase activity, but had no significant effect on osteocalcin. Dexamethasone stimulated alkaline phosphatase activity and osteocalcin synthesis, and inhibited DNA synthesis but had no significant effect on the expression of type I procollagen mRNA. Dexamethasone masked the effect of endothelin-1 on human osteoblastic cells, and the effect of dexamethasone was predominant in the group of a combination of endothelin-1 and dexamethasone.
Alkaline Phosphatase ; Collagen Type I ; Dexamethasone ; DNA ; Endothelial Cells ; Endothelin-1 ; Endothelins* ; Humans ; Ilium ; Masks ; Metabolism ; Microvessels ; Osteoblasts* ; Osteocalcin ; Receptors, Endothelin ; RNA, Messenger

CD4+/CD56+ hematodermic neoplasm is a rare and aggressive lesion that affects many organs, and skin involvement is highly characteristic. It is also termed blastic natural killer cell lymphoma in the World Health Organization classification. Several origins of tumor cells have been proposed, but recent studies have shown a relationship with plasmacytoid dendritic cells. A 2-year-old boy presented with multiple bruise-like violaceous subcutaneous nodules and plaques on the trunk, upper and lower extremities. Histological examination showed small-to-medium-sized blastoid cellular infiltration in the dermis and subcutaneous tissue. Tumor cells were positive for CD4, CD56 and TdT, and negative for CD8, CD20 and MPO. It primarily affects elderly patients, but, in this case, occurred in an infant. Due to its rarity, we present a case of CD4+/CD56+ hematodermic neoplasm affecting a pediatric patient.
Aged ; Dendritic Cells ; Dermis ; Humans ; Infant ; Killer Cells, Natural ; Lower Extremity ; Lymphoma ; Preschool Child ; Skin ; Subcutaneous Tissue ; World Health Organization