No abstract available.
Hemorrhage* ; Pregnancy* ; Round Ligament of Uterus*

We report 1 case of Fanconi's anemia which has severe pancytopenia, PDA, hypoplatic right thumb and cryptorchism with a brief review of related literature and references.
Cryptorchidism ; Fanconi Anemia* ; Male ; Pancytopenia ; Thumb

Authors experienced the cases like to Muco-Cutaneous Lymph node Syndrome (MCLS) admitted to the department of Pediatrics, St. Benedict Hospital from Jan. 1978 to Dec. 1979. Here Authors presented the clinical datas of twenty cases simulating this syndrome as reported by Kawasski. 1. The peak age group was from 1 to 2 years of age and the youngest case was 3 months old infant. Most of them (95%) was under 3 yesrs old. The ratio of male and female was 13 to 7. The seasonal incidence was higher in May to June and November to February. The annual incidence of 1979 was more than 2 times as that of 1978. 2. The clinical symptoms on admission were as follow : fever (100%), cough (70%), diarrhea (65%), vomiting (45%), irritability (40%), So, on admission, Authors had used to overlook as respiratory disease or acute gastroenteritis. 3. The 70% of them showed at least 5 items of the principal symptoms of MCLS. At acute phase, 14 cases (70%) also showed the erythematous desquamation of anogenital region, and 2 cases (10%) showed a transitory hydrocele. The appearance of rashes was observed in 10 cases (50%), and 7 cases of the remainder showed the desquamation of digits during convalescent phase. 7 of 15 cases (46.7%) showed only the desquamation of fingertips. 4. 8 cases (40%) had sinus tachycardia, and I case had gallop rhythm. Most of them showed normal EKG, and 4 cases (20%) showed low voltage of limb leads. 5. In the laboratory findings, most of them had leucocytosis, elevated ESR, positive CRP, normal ASO, and 5 cases (25%) had slight increase of serum transminase. Hepatomegaly was observed in 7 cases (35%). 6. In spite of the intensive treatment, fever was continued for about 5 days in 5 cases (25%).
Cough ; Diarrhea ; Electrocardiography ; Exanthema ; Extremities ; Female ; Fever ; Gastroenteritis ; Hepatomegaly ; Humans ; Incidence ; Infant ; Lymph Nodes* ; Male ; Pediatrics ; Seasons ; Tachycardia, Sinus ; Vomiting

Tow patients with severe glomerulonephitis and their serum creatinine clearance of 6.0ml/min/1.73m2 in case 2 were treated with high dosage intravenous methylprednisolone, 30mg/kg given over one hour period on each of six slternate days. Follwing the ?ulse therapy, both patients with rapidly deteriorathin renal funtion began to inprove and theit serum creatinine clearance levels showed over douvble by sixdays In both patients treated early in the curse of their disease, creatinine clearance increased to 80ml/min/1.73m2 in case 2 after one year follow-up and in recent study, clinical and laboratory findings are within normal limit.
Creatinine ; Follow-Up Studies ; Humans ; Methylprednisolone*

PURPOSE: To investigate the cut-off value of creatine kinase (CK) BB according to blood sampling time in predicting neonatal prognosis, we studied the time-related releasing pattern of CK isoenzymes after birth asphyxia. METHODS: CK was measured within 1 hour and at 6-10 hours after birth from 45 newborn infants who were suspected to be perinatal asphyxia or fetal distress. The infants were followed up for 1 year to evaluate neonatal prognosis. RESULTS: Total CK, CK-MM, CK-BB and CK-BB% from blood samples within 1 hour after birth were 758.6U/L, 588.7U/L, 105.7U/L and 17.5%, respectively, and those at 6-10 hours after birth were 1,298.0U/L, 1,127.1U/L, 57.7U/L and 6.6%, respectively. When the cut-off value of CK-BB measured within 1 hour after birth was set to 200U/L, positive predictive value (PP) was 0.38 and negative predictive value (NP) was 0.76 for infants who died within 28 days. For neurologic sequelae on 1-year-old PP and NP are 0.33 and 0.76 respectively, for neonatal seizures, 0.38 and 0.92, for abnormal brain sonogram cases 1.0 and 0.88, repectively. CONCLUSION: CK-BB and CK-BB% were measured significantly higher within 1 hour after birth than at 6-10 hours after birth. Because of different CK-BB levels between 2 separate sampling times, we have to use 2 different cut-off values. It is recommended that cut-off values for CK-BB and CK-BB%, measured within 1 hour after birth, are 200U/L and 20%, respectively and cut-off values for CK-BB and CK-BB% measured in 6-10 hours after birth are 100U/L and 10%, respectively.
Asphyxia ; Brain ; Creatine Kinase* ; Creatine* ; Fetal Distress ; Humans ; Infant ; Infant, Newborn ; Isoenzymes ; Parturition ; Prognosis* ; Seizures

No abstract available.
Conus Snail* ; Hemangioblastoma*

No abstract available.
Conus Snail* ; Hemangioblastoma*

BACKGROUND: Hematopoietic stem cells of the human fetal liver prior to 15 weeks gestation have remakable advantages for successful engraftment due to embryological immune immaturity, especially in-utero transplantation. This study was undertaken to obtain objective assessment data about the possibility of fetal liver hematopoietic stem cell transplantation in the future. METHODS: Six cases of the fetal liver tissue were obtained from therapeutic abortions at 12~20 weeks gestation. The fetal liver was collected in RPMI media containing 10% fetal calf serum and the cell suspensions were obtained by centrifugation following physical disruption. The number of nucleated cells in each case was counted and the colony numbers in methyl cellulose media were scored according to incubation period with or without growth factors. Some of the cells were cryopreserved in the liquid nitrogen tank, thereafter cell viability and colony numbers were evaluated according to cryopreservation period. RESULTS: The nucleated cell numbers obtained from each fetal liver increased with gestational age. The colony numbers after incubation increased with gestational age and the erythroid lineage was predominant in 3 cases which are under 15 weeks gestation. The colonogenic activity after incubation with combination of hematopoietic growth factors increased in only one case. The cell viability and the colony numbers after cryopreservation was decreased compare to the value before cryopreservation. CONCLUSION: The number of nucleated cells and hematopoietic stem cell colony formation were increased with gestational age and viability of the cells after cryopreservation was decreased. Further systematic studies using more cases would be needed to obtain objective assessment data for fetal liver transplantation program in the future.
Abortion, Therapeutic ; Cell Count ; Cell Survival ; Centrifugation ; Cryopreservation ; Female ; Gestational Age ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells* ; Humans ; Intercellular Signaling Peptides and Proteins ; Liver Transplantation ; Liver* ; Methylcellulose ; Nitrogen ; Pregnancy ; Suspensions

OBJECTIVE: Hemangiopericytoma is known as a malignant tumor originating from pericytes and rarely occurs in the central nervous system. We present 6 cases of pathologically confirmed meningeal hemangiopericytoma. METHODS: Retrospective study was done based on patient's recordings including radiological studies. Each case of tumors was treated surgically and postoperative radiotherapy was done. RESULTS: There were 5 cases of intracranial and 1 case of spinal hemangiopericytomas. Three of 5 intracranial hemangiopericytomas were located at tentorial region. Total tumor removal was done in 4 cases and postoperative local recurrence (or regrowth) was noted in 3 cases despite of postoperative external radiation therapy, 2 of which had died. CONCLUSION: Our cases show more frequent tentorial locations and poor clinical outcomes of hemangiopericytomas compared with meningiomas.
Central Nervous System ; Hemangiopericytoma* ; Meningioma ; Pericytes ; Radiotherapy ; Recurrence ; Retrospective Studies

No abstract available.
Embryonic Development* ; Female ; Humans* ; Pregnancy ; Serum Albumin*