No abstract available.
Blood Pressure* ; Humans ; Infant, Newborn*

No abstract available.
Caroli Disease*

No abstract available.
Corneal Opacity ; Ehlers-Danlos Syndrome*

No abstract available.
Allopurinol* ; Animals ; Liver* ; Rats* ; Reperfusion Injury* ; Verapamil*

No abstract available.
Poland Syndrome* ; Poland*

No abstract available.
Mucous Membrane* ; Prolapse* ; Vitelline Duct*

We report a case of severe(gradeV) unilateral vesicoureteral reflux(VUR) without any renal damage in a 6-month-old boy through the early sibling screening test for VUR, whose old brother had reflux nephropathy. The early detection of VUR aroused us to take special precautions to prevent urinary tract infection in this patient. We believe that this approach was helpful to reduce the risk of renal damage in this patient and are reporting the case with a brief review of related literatures. It is our firm suggestion that screening for vesicoureteral reflux should be carried out in every child who has siblings with reflux nephropathy.
Child ; Humans ; Infant ; Male ; Mass Screening* ; Siblings* ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

PURPOSE: This study was performed to elucidate the clinical pictures of acute focal bacterial nephritis(nephronia) in children. METHODS: We reviewed 9 children with nephronia diagnosed by ultrasonography or computed tomography of kidneys from September 1994 to August 2004. RESULTS: The overall male to female ratio was 2:1, and the age distribution ranged from 0.1 to 6 years(mean 2.8+/-2.2). The cardinal symptoms were fever, chills, abdominal pain and dysuria/frequency. The initial leukocyte count was 21,000+/-5,600/uL; ESR, 60+/-23 mm/hr; CRP, 17+/-10 mg/dl. Pyuria was noted in every patient and persisted for 10.5+/-7.8 days after antimicrobial treatment. Abdominal sonography demonstrated focal lesion of ill-defined margin and low echogenicity in 5 of 9 patients(55.6%), while computed tomography revealed nonenhancing low density area in all patients(100%). Three of 9 patients(33.3%) had vesicoureteral reflux, greater than grade III. The initial (99m)Tc-DMSA scan showed one or multiple cortical defects in every patient, and improvements were noted in 2(33.3%) of 6 patients who received follow up scan after 4 months. Intravenous antibiotics was given in every patient under admission. Total febrile period was 11.8+/-6.3 days(pre-admission, 4.0+/-3.0; post-admission, 7.8+/-5.5 days) and the patients needed hospitalization for 17.2+/-8.1 days. CONCLUSION: For the early diagnosis of 'acute focal bacterial nephritis' we should perform renal computed tomography first rather than ultrasonography, when the child has toxic symptoms and severe inflammatory responses in blood and urine.
Abdominal Pain ; Age Distribution ; Anti-Bacterial Agents ; Child* ; Chills ; Early Diagnosis ; Female ; Fever ; Follow-Up Studies ; Hospitalization ; Humans ; Kidney ; Leukocyte Count ; Male ; Nephritis* ; Pyuria ; Ultrasonography ; Vesico-Ureteral Reflux

A 4-day-old patient with Down syndrome (DS) visited out patient department (OPD) because of jaundice and VSD. Peripheral blood smear showed 21% of myeloblast. After 4 weeks of observation, WBC count was 55,100/mm3 (blast 90%). BM aspirate showed AML (M7) and treatment was started with low dose Ara-C (20 mg/m2 for 21 days). After remission, maintenance therapy was done with low dose Ara-C (16 mg/m2 for 21 days), 6-TG (40 mg/m2 for 21 days) and low dose IL-2 (0.5 106U/m2 for 21 days) alternatively for 2 years. The patient remained in complete remission and VSD was corrected at 9 months of age. This case shows that remission can be achieved with low dose Ara-C and it can be maintained thereafter with low dose Ara-C, 6-TG and IL-2. Low dose IL-2 has the advantage of selectively activating immune cells with high affinity receptors, low treatment related morbidity, good compliance which can be injected at OPD. As the patients with DS have defect in IL-2 secretion, IL-2 may have an beneficial effects on treating AML in DS.
Compliance ; Cytarabine ; Down Syndrome* ; Drug Therapy* ; Granulocyte Precursor Cells ; Humans ; Interleukin-2* ; Jaundice ; Leukemia, Myeloid, Acute*

Bartter syndrome is a rare disorder characterized by the association of hypokalemic hypochloremic metabolic alkalosis, hyperreninemia, hyperaldosteronemia, short stature and nephrocalcinosis. This disorder presents with hyperplasia of juxtaglomerular apparatus on renal biopsy. We experienced a case of late-onset Bartter syndrome with nephrocalcinosis in a 9-year-old boy, whose chief pictures were muscle weakness, short stature, persistent sterile pyuria and microscopic hematuria. We report this case with a brief review of related literatures.
Alkalosis ; Bartter Syndrome* ; Biopsy ; Child ; Hematuria ; Humans ; Hyperplasia ; Juxtaglomerular Apparatus ; Male ; Muscle Weakness* ; Nephrocalcinosis ; Pyuria