Stargardt's disease is a heredo-macular dystrophy which evidences itself by reduced visual acuity, bilaterally, slowly progressive lesion, and by color blindness starting in youth. The diagnosis of Stargardt's disease is usually made by biomicroscopy and fluoresceine angiography which show many irregular shaped, small yellowish flecks. According to Fishman, the characteristic prolonged time to obtain adequate rod amplitude in dark-adapted electroretionography is advisable for differential diagnosis of Stargardt's disease. We didn't find the abnormal finding of dark-adapted electroretinography in two patients of Stargardt's disease. So, more studies will be needed for electroretinography of Stargardt's disease.
Adolescent ; Angiography ; Color Vision Defects ; Diagnosis ; Diagnosis, Differential ; Electroretinography ; Fluorescein ; Humans ; Visual Acuity

We report a case of an erythrodermic form of mycosis fungoides in a 68-year-old male, who showed generalized erythroderma with scales for 3 months. Lymphadenopathies in the inguinal and neck areas were present. Histopathological findings showed epidermotropism, perivascular atypical lymphocyte infilteration in the upper dermis, and dermatopathic lymphadenopathy in the inguinal lymph node. T cell markers were positively stained in the immunohistochemical study. These clinicopathological features were consistent with an erythrodermic form of mycosis fungoides(TNM IIl). The patient was treated with PUVA therapy and low dose chemotherapy.
Aged ; Dermatitis, Exfoliative ; Dermis ; Drug Therapy ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Male ; Mycosis Fungoides* ; Neck ; PUVA Therapy ; Weights and Measures

Pneumatic retinopexy is a recently described procedure to treat the uncomplicated retinal detachment. The procedure of pneumatic retinopexy is composed of cryotherapy (or laser photocoagulation), intraocular gas injection and postoperative positioning. The advantages of pneumatic retinopexy are less tissue trauma, no hospitalization and minimal complication. For these reasons it is more and more popular, but further studies are needed to assess its ultimate safety and efficacy. The authors experienced 7 cases of primary rhegmatogenous retinal detachment treated with pneumatic retinopexy, from September, 1987 to March, 1988. Four cases of the retinal detachment were successfully reattached after pneumatic retinopexy. In the other 3 cases, new retinal tear and detachment were found after pneumatic retinopexy, which were successfully repaired with the conventional scleral buckling procedure.
Cryotherapy ; Hospitalization ; Retinal Detachment* ; Retinal Perforations ; Retinaldehyde* ; Scleral Buckling

A 12-year-old female with histiocytosis X accompanied by autoimmune hemolytic anemia. During the episode of hemolysis, the hemoglobin level fell to 5.2 g/dL. The direct antiglobulin test was weakly positive. The anti-C and anti-e were identified in her serum. The Rh subgroup of her family(father, mother and brother) including the patient, were all same as DCe. The antibodies which showed anti-C and anti-e specificity were confirmed autoanti-Ce(non-separable) using the serum absorbed with various known Rh phenotyped RBCs. Two packed RBCs phenotyped as DeE were transfused for correction of anemia. Acute and delayed hemolytic transfusion reactions were not noted after transfusion. Identification of blood group specific autoantibodies may be benificial in such case for blood transfusion.
Anemia ; Anemia, Hemolytic, Autoimmune* ; Antibodies ; Autoantibodies ; Blood Group Incompatibility ; Blood Transfusion ; Child ; Coombs Test ; Female ; Hemolysis ; Histiocytosis, Langerhans-Cell ; Humans ; Mothers ; Sensitivity and Specificity

No abstract available.
Humans ; Male ; Nursing* ; Urinary Incontinence*

No abstract available.
Blood Transfusion* ; Humans ; Infant*

PURPOSE: In the present study, the effects of autologous peripheral blood mononuclear cells (PBMNC) transplantation after granulocyte colony stimulation factor (G-CSF)-induced mobilization was investigated in patient with severe peripheral arterial disease. METHOD: 4 cases were enrolled: 3 cases are atherosclerosis obliterance and 1 case is thromboangitis obliterance. Following administration of G-CSF (10microgram/kg, maximum 600microgram), 4 times for 4 days. PBMNC were harvest and injected via femoral artery to vascular foot bed. RESULT: No serious complication was observed during G-CSF injection, harvest and injection. Improvement in the ankle brachial pressure index was seen in all patients at 4 weeks and ischemic ulcer improved in 2 of 3 patients. Using of pain control decreased in all cases and increased blood flow at 99 m-Tc perfusion scan in 2 of 2 patients. CONCLUSION: These favorable outcomes strongly suggest a therapeutic impact of transplantation PBMNC in clinical setting.
Ankle ; Atherosclerosis ; Cell Transplantation* ; Extremities* ; Femoral Artery ; Foot ; Granulocyte Colony-Stimulating Factor ; Granulocytes ; Humans ; Perfusion ; Peripheral Arterial Disease ; Transplants* ; Ulcer

PURPOSE: We wished to compare the clinical effectiveness of cystatin C (CyC) and serum creatinine (sCr) to assess renal function in renal transplantation patients. METHODS: We compared the clinical effectiveness of CyC with that of the sCr to estimate 24-hour urine creatinine clearance (CrCl) in 72 adult recipients who underwent renal transplantation from January 2001 to December 2008. We analyzed the data in terms of accuracy, bias, precision and sensitivity as a function of length of time posttransplantation and CrCl value. RESULTS: The patients were divided into four groups according to CrCl value <30, <60, <90, and > or =90 mL/min/1.73 m2. The corresponding Cr-based glomerular filtration rate (GFR) estimates had accuracies of 0.71, 0.906, 0.963, and 1.00 within 50% of the reference, with biases (mean percentage errors) of 4.7, 5.32, -5.79, -31.33 mL/min/1.73 m2, and precisions (mean absolute percentage errors) of 7.57, 10.03, 14.52, and 31.33 mL/min/1.73 m2, respectively. The CyC-based GFR estimates had accuracies of 0.35, 0.79, 0.93, and 0.67 within 50% of the reference, respectively, with biases of 15.03, 13.37, -5.58, and -34.79 mL/min/1.73 m2 and precisions of 15.03, 14.80, 17.91, and 34.79 mL/min/1.73 m2. The sensitivity for detecting GFR below 60 mL/min/1.73 m2 was higher for CyC (0.96, 1, and 0.95) than for Cr (0.77, 0.75, and 0.82). CONCLUSION: CyC is a more sensitive indicator of low GFR (CrCl <60 mL/min/1.73 m2) than sCr. However, CyC-based GFR estimates are restrictive data, and are neither accurate nor specific. Therefore, to evaluate renal function, we may need a revised CyC-based GFR formula and close monitoring of sCr.
Adult ; Bias (Epidemiology) ; Creatinine ; Cystatin C ; Glomerular Filtration Rate ; Humans ; Kidney Transplantation ; Transplants

No abstract available.
Gestational Age* ; Humans ; Immunoglobulins* ; Infant, Newborn*

Congenital diaphragmatic hernias (CDH) usually cause respiratory distress soon after birth and are associated with a high mortality rate in the early postnatal period. However, there is a milder form of CDH that does not manifest during the neonatal period. The late presenting CDH is characterized by a variable clinical picture. We present the case of an otherwise healthy 5-month-old girl, who was referred for evaluation of an 1-day history of vomiting and irritability. Chest simple X-ray and CT showed bowel loops in the left thoracic cavity, which was consistent with diaphragmatic hernia. At operation, she was found to have a small left posterolateral diaphagmatic defect with viable small bowel loops in the left thoracic cavity. After surgical reposition of the hernia, the symptoms such as vomiting and irritability subsided. The lack of typical manifestation of CDH such as respiratory distress may lead to delayed diagnosis. The possibility of late presenting CDH should not be overlooked even after the neonatal period.
Delayed Diagnosis ; Hernia ; Hernia, Diaphragmatic ; Humans ; Infant ; Parturition ; Thoracic Cavity ; Thorax ; Vomiting