Optimal Timing of Surgery of Hydronephrosis Due to Ureteropelvic Junction Obstruction in Neonates and Infants

PURPOSE: We review our experience with pyeloplasty for unilateral ureteropelvic junction obstruction of moderate to severe hydronephrosis observed by prenatal ultrasonography to assess the appropriate timing of operation for recovery of renal function and obstruction. METHODS: We retrospectively reviewed the records of the total 28 patients who underwent pyeloplasty between 1995 and 2001 at Asan Medical Center. We compared pre and postoperative differentials in renal function and diuretic renogram as measured by technetium-99m-mercaptoacetyl- triglycerine scan and the degree of hydronephrosis by renal ultrasonography. RESULTS: In all 28 patients postoperative follow-up renal ultrasonography revealed significant improvement in hydronephrosis. In 10 poorly functioning hydronephrotic kidneys in which relative renal function function was less than 35%, renal function was improved postoperatively in 3 cases, but not improved in 7 cases. In all 28 patients postoperative follow-up diuretic renogram revealed significant improvement. CONCLUSION: We believe that the early pyeloplasty should be considered when ultrasonography and diuretic renography suggest obstruction because renal function does not improve significantly after pyeloplasty over preoperative value.

Clinical Study of Renal Cystic Diseases in Children

PURPOSE: Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. METHODS: This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. RESULTS: In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases(7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. CONCLUSION: Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.

A Case of Successful Danazol Therapy in Autoimmune Thrombocytopenia Associated with Systemic Lupus Erythematosus

Autoimmune thrombocytopenia and autoimmune hemolytic anemia occur in 10-26% of patients with systemic lupus erythematosus(SLE). These hematological manifestations may be the sole presenting sign and can precede the appearance of diagnosable SLE in 5 to 23% of cases. The conventional treatment for SLE associated with these disorders includes corticosteroid therapy and splenectomy, but autoimmune thrombocytopenia or autoimmune hemolytic anemia may be refractory to both treatments. We experienced a case of a SLE patient complicated by severe autoimmune thrombocytopenia. Therapy with intravenous immunoglobulin and corticosteroids failed. She responded to danazol and remission of thrombocytopenia(platelet >100,000/mm3) was observed 7 days after starting danazol. Danazol therapy seems to be a useful and well tolerated treatment for refractory autoimmune thrombocytopenia associated with SLE.

Diagnostic Utility of Tc-99m DISIDA Hepatobiliary Scintigraphy in the Diagnosis of Biliary Atresia / 대한소아소화기영양학회지

PURPOSE: Biliary atresia, one of the major causes of neonatal cholestais, is an idiopathic, serious disorder, affecting the newborn that results in complete obstruction of biliary tract. Successful reestablishment of bile flow is dependent on early surgical intervention, early diagnosis is imperative. The authors evaluate the utility of Tc-99m-labeled diisoprpyliminodiacetic acid (DISIDA) hepatobiliary scintigraphy in the diagnosis of biliary atresia. METHODS: From January, 1995 to August, 1999, total 60 patients with neonatal cholestasis underwent Tc-99m DISIDA hepatobiliary scintigraphy at Asan Medical Center. RESULTS: The undelying causes of neonatal cholestasis were biliary atresia in 14, neonatal hepatitis in 33, intrahepatic bile duct paucity in 9, and total parenteral nutrition induced cholestasis in 4. All patient with biliary atresia were interpreted correctely in DISIDA hepatobiliary scintigraphy, showing 100% sensitivity. Of the 46 patients with neonatal hepatitis and other causes, 37 patients had intestinal radioactivity showing 80% specificity. CONCLUSION: Visualization of DISIDA in the intestinal tract indicates patency of the biliary ducts and excludes the diagnosis of biliary atresia. But the absence of intestinal excretion on the DISIDA hepatobiliary scintigraphy dose not necessarily indicate biliary atresia.