A Clinical Study on Hereditary Spherocytosis / 대한소아혈액종양학회지

PURPOSE: The aim of this study was to investigate the clinical and laboratory findings of hereditary spherocytosis comparing those of different age groups. METHODS: The clinical and laboratory findings of hereditary spherocytosis from June 1989 to August 1998 at Asan Medical Center were analyzed retrospectively according to two different age groups, Group I (9 patients diagnosed under 10 years of age) and Group II (19 patients diagnosed at or over 10 years of age). RESULTS: 1) Mean age at diagnosis was 2.4+/-1.97 and 28.2+/-18.81 years, and family history was positive in 44% and 47% in Group I and II patients respectively. 2) Splenectomy was carried out in 33% and 79% of Group I and II patients respectively, and accessory spleen was found in 100% and 20% of splenectomized patients respectively. 3) Gallstone was found in 11% and 42% of Group I and II patients respectively, and aplastic crisis developed in 0% and 10% respectively. 4) Post-splenectomy hematological parameters improved as follows: Group I; from hemoglobin at diagnosis of 8.5+/-3.59 g/dL to post-splenectomy level of 12.6+/-0.86 g/dL, hematocrit 24.5+/-10.25% to 38.1+/-4.86%, corrected reticulocyte 9.0+/-4.16% to 1.2+/-0.84%, total bilirubin 3.2+/-1.53 mg/dL to 2.2+/-1.34 mg/dL. Group II ; from hemoglobin at diagnosis of 8.9+/-2.95 g/dL to post-splenectomy level of 12.6+/-1.27 g/dL, hematocrit 24.9+/-7.85% to 37.4+/-2.89%, corrected reticulocyte 4.8+/-2.74% to 2.0+/-1.12%, total bilirubin 5.2+/-5.05 mg/dL to 1.1+/-0.49 mg/dL. CONCLUSION: There were no age related differences in hematologic findings at diagnosis and many of the patients with milder form of the disease could be detected later in adult life. The frequency of gallstone was up to 42% in patients whose diagnosis was delayed after 10 years of age, and aplastic crisis was a rare complication. Splenectomy was an effective treatment leading to normal hemoglobin concentrations in all patients. Accessory spleen was found in 33% of splenectomized patients, which emphasizes the necessity of spleen scan before splenectomy.

A Clinical Study of Urolithiasis in Children

PURPOSE: The object of this study was to determine the clinical characteristics of Korean children with urolithiasis. METHODS: From August 1989 to August 1999, 50 patients diagnosed with urolithiasis in a radiologic study were studied retrospectively. We described age, sex distribution, family history, underlying cause, initial symptoms, location of stones, stone analysis and treatment. RESULTS: The total number of patients was 50 cases. Male to fernale ratio was 1.8 and mean age was 6.2+/-4.7 years. Symptoms were hematuria in 23(46%) patients, abdorninal pain in 10(20%), flank pain in 7(14%), fever in 8(16%), dysuria in 1(2%), and incidental in 1(2%). Stones were found in the kidney in 26(52%) patients, ureters in 21(42%), urethra in 1(2%), and kidney and ureter in 2(4%). Hydronephrosis and hydroureter was present in 14(28%) patients, hydronephrosis in 11(22%), vesicoureteral reflux in 2(4%), nonfunctioning kidney in 1(2%), ureterocele in 1(2%), and duplex kidney in 1(2%). Extensive studies, including 24hr urine chemical analysis, were done to find the cause of urolithiasis in 32 patients. Causes were hypercalciuria in 18(56.3%) patients, idiopathic in 9(28.1%), and urinary tract infection in 5(15.6%). Stones were analysed biochemically in 10 patients. Calcium oxalate stones were present in 7 patients, calcium phosphate stones in 1, and struvite stones in 2. Extracoporeal shock wave lithotripsy was performed in most(40%) of the cases, and 26% of the cases passed their stones spontaneously. CONCLUSION: Hematuria is the most common symptom of pediatric urolithiasis. Upper urinary tract is the main site of urolithiasis. The main cause of urolithiasis is hypercalciuria. The main composition of urolithiasis is calcium oxalate.