We report a case of 15 days old newborn presenting with hypergalactosemia detected by newborn screening who had intrahepatic arterio-venous shunts with multiple pin-head sized cutaneous hemangiomas. Plasma level of galactose was elevated to 11.3 mg/dL at age of 7 days, but the activity of galactose-metabolizing enzymes including galactose-1- phosphate uridyltransferase, galactokinase, and uridine diphosphate galactose-4-epimerase were all normal. Intrahepatic arterio-venous shunts were diagnosed by abdominal ultrasonography with color doppler ultrasonography and abdominal computed tomography. At age of 3 months, the plasma level of galactose further elevated to 14.73 mg/dL, at which time lactose-free cows milk formula was started. At age of 6 months, the plasma level of galactose decreased to within normal range with disappearance of previously noted multiple cutaneous hemangiomas. In hypergalactosemia of the newborn, the intrahepatic shunts should be considered as a possible cause, once hereditary enzyme deficiencies have been ruled out.
Galactokinase ; Galactose ; Hemangioma ; Humans ; Infant, Newborn ; Mass Screening ; Milk ; Neonatal Screening* ; Plasma ; Reference Values ; Ultrasonography ; Ultrasonography, Doppler, Color ; Uridine Diphosphate

Although gastrointestinal manifestations are very common in patients with Henoch-Sch nlein purpura, protein losing enteropathy is a rare complication. We here report a case of protein losing enteropathy in a patient with Henoch-Sch nlein purpura. A 52-year old woman presented with lower abdominal pain, purpura and edema on lower extremity. Serum albumin was 1.9g/dL and 24 hour urine protein was 4.7g/ day. Skin and kidney biopsy revealed leukocytoclastic vasculitis and mesangial proliferative glomerulonephritis consistent with Henoch-Sch nlein purpura, respectively. Colonoscopy showed diffuse mucosal erosion at right colon. 99mTc-human serum albumin scintigraphy and fecal alpha-1-antitrypsin clearance confirmed protein losing enteropathy. The protein losing enteropathy improved with steroid treatment.
Abdominal Pain ; Biopsy ; Colon ; Colonoscopy ; Edema ; Female ; Glomerulonephritis ; Humans ; Hypoalbuminemia ; Kidney ; Lower Extremity ; Middle Aged ; Protein-Losing Enteropathies* ; Purpura* ; Radionuclide Imaging ; Serum Albumin ; Skin ; Technetium Tc 99m Aggregated Albumin ; Vasculitis

Acyclovir is a remarkably safe drug with potent antiviral effect against herpes virus. The two most serious adverse effects are neurotoxicity and nephrotoxicity. We here report the case of a 64-year old woman with acyclovir induced acute interstitial nephritis. She developed non-oliguric acute renal failure following the administration of oral acyclovir(800mg five times per day) to treat herpes zoster of left 2nd and 3rd thoracic nerves. We documented a normal serum creatinine level just before exposure to the drug. On admission, serum creatinine level was 2.4 mg/dL(baseline level; 0.8mg/dL). Percutaneous renal biopsy revealed interstitial infiltration of lymphocyte and eosinophil with interstitial edema, but there was no crystal formation in the tubules or collecting ducts. After withdrawal of the acyclovir, renal function returned to normal within 1 week.
Acute Kidney Injury ; Acyclovir* ; Biopsy ; Creatinine ; Edema ; Eosinophils ; Female ; Herpes Zoster ; Humans ; Lymphocytes ; Middle Aged ; Nephritis, Interstitial* ; Thoracic Nerves

BACKGROUND: Flow cytometric analysis for CD34 has been widely used for hematopoietic stem cell enumeration. The procedure is simple and rapid for clinical use but the lack of standardization resulted in great intralaboratory variations. In 1995, a guideline for CD34 analysis was established by International Society of Hematotherapy and Gene Engineering (ISHAGE) for reliable testing. We performed CD34 analysis using the ISHAGE guideline in umbilical cord blood (UCB), mobilized peripheral blood (MPB) and leukapheresis product (LP) and compared the results with those of in-house method. METHODS: CD34 analyses were performed in thirty units each of UCB, MPB and LP according to the ISHAGE guideline and in-house method and the results were analyzed by the t-test. Both methods used CD45FITC/CD34PE and its isotype controls. In ISHAGE guideline, among CD34+/ CD45+ cells, only those with low forward scattering, low to intermediate side scattering and low to intermediate CD45 fluorescent intensity were identified as stem cells, and the percentage of those cells among CD45+ cells was calculated. In in-house method, cells expressing both CD34 and CD45 antigens were selected by isotype control and the percentage of CD34+/CD45+ cells among CD45+ cells were calculated. RESULTS: Significant differences were observed in the percentages of CD34+ cells in UCB, MPB and LP between ISHAGE guideline (0.25%, 0.42%, 0.80%) and in-house method (0.40%, 0.55%, 1.20%) (P<0.001). So were the CD34+ cell counts : mean values of CD34+ cells in microliter of UCB, MPB and LP were 20, 40, 1,392 by ISHAGE guideline, and 35, 62, 2,079 by in-house method (P<0.001). CONCLUSION: ISHAGE guideline for CD34 enumaration was considered as a simple, rapid and reliable method for clinical setting and to have economic benefits because no additionalmonoclonal antibodies were required.
Antibodies ; Antigens, CD45 ; Cell Count ; Fetal Blood ; Hematopoietic Stem Cells ; Leukapheresis ; Stem Cells

PURPOSE: To evaluate the minimum 10-year follow up results of primary total hip arthroplasty (THA) performed using a sandwich-type (alumina-polyethylene-titanium) ceramic bearing. MATERIALS AND METHODS: Thirty four patients (40 hips) who underwent a THA with sandwich typed liners from November 1998 to December 2000 were analyzed. Among the 34 patients, 25 were men and nine were women. Mean follow-up was 134 months (range, 120~145 months) and mean patient age at the time of THA was 47.1 years (range, 24~65 years). The clinical results were evaluated using the Harris hip score and level of thigh pain with a limping gait. The radiographic evaluation was done in terms of the endosteal new bone formation, radiolucent line, subsidence of the stem, migration of the acetabular cup, and proof of loosening. RESULTS: The mean preoperative Harris hip score of 50.2 points (range, 31~87 points) was improved to 90.9 points (range, 75~99 points) at the final follow-up and thigh pain with limping gait in one case. All cases had fixation by bony ingrowth. No radiographically detectable loosening was observed in any hip. Fracture of ceramic liner in one case required change of the polyethylene liner. CONCLUSION: At the minimum 10-year follow up, survival rate as the end-point was favorable. However, fracture of the ceramic is still a major problem. Satisfactory results can be obtained by more precise surgical technique for acerabular inclincation and anteversion, and by improving the quality of the ceramic.
Arthroplasty ; Ceramics ; Female ; Follow-Up Studies ; Gait ; Hip ; Humans ; Male ; Osteogenesis ; Polyethylene ; Survival Rate ; Tacrine ; Thigh