Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan (99mTc RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.
Abdominal Pain ; Adult ; Arthritis ; Biopsy ; Cyclosporine ; Diagnosis ; Emergencies ; Erythrocytes ; Exanthema ; Follow-Up Studies ; Hemorrhage ; Humans ; Intestinal Perforation ; Intestines ; Intussusception ; Kidney ; Laparotomy ; Lower Extremity ; Nephritis ; Purpura ; Skin ; Systemic Vasculitis

We made a mistake in our recently published article.

We investigated the efficacy and tolerability of various anticholinergics in Korean children with non-neurogenic overactive bladder (OAB). A total of 326 children (males:females= 157:169) aged under 18 yr (mean age 7.3+/-2.6 yr) who were diagnosed with OAB from 2008 to 2011 were retrospectively reviewed. The mean duration of OAB symptoms before anticholinergic treatment was 16.9+/-19.0 months. The mean duration of medication was 5.6+/-7.3 months. Urgency urinary incontinence episodes per week decreased from 1.9+/-3.1 to 0.4+/-1.5 times (P<0.001). The median voiding frequency during daytime was decreased from 9.2+/-5.4 to 6.3+/-4.2 times (P<0.001). According to 3-day voiding diaries, the maximum and average bladder capacity were increased from 145.5+/-66.9 to 196.8+/-80.3 mL and from 80.8+/-39.6 to 121.8+/-56.5 mL, respectively (P<0.001). On uroflowmetry, maximum flow rate was increased from 17.6+/-8.4 to 20.5+/-8.2 mL/sec (P<0.001). Adverse effects were reported in 14 (4.3%) children and six children (1.8%) discontinued medication due to adverse effects. Our results indicate that anticholinergics are effective to improve OAB symptoms and tolerability was acceptable without severe complications in children.
Child ; Child, Preschool ; Cholinergic Antagonists/adverse effects/*therapeutic use ; Constipation/etiology ; Dizziness/etiology ; Female ; Humans ; Male ; Retrospective Studies ; Treatment Outcome ; Urinary Bladder, Overactive/*drug therapy

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea.
Adolescent ; Agammaglobulinemia/congenital/epidemiology ; Age Distribution ; Child ; Child, Preschool ; Common Variable Immunodeficiency/epidemiology ; Female ; Genetic Diseases, X-Linked/epidemiology ; Humans ; IgA Deficiency/epidemiology ; IgG Deficiency/epidemiology ; Immunologic Deficiency Syndromes/*epidemiology ; Infant ; Infant, Newborn ; Job's Syndrome/epidemiology ; Male ; Prevalence ; Questionnaires ; Registries ; Republic of Korea/epidemiology ; Severe Combined Immunodeficiency/epidemiology ; Sex Distribution ; Wiskott-Aldrich Syndrome/epidemiology ; Young Adult

PURPOSE: We wanted to estimate the prevalence and risk factors of overactive bladder(OAB) in Korean children who were 5-13 years of age, according to the definition of OAB. MATERIALS AND METHODS: A randomly selected cross-sectional study was conducted on 26 kindergartens and 27 elementary schools nationwide in Korea. There were 19,240 children; a parent was asked to complete the questionnaires, which included items about OAB and the children's voiding and defecating habits. OAB was defined as urgency with or without urge incontinence, and usually with an increased daytime frequency and nocturia(ICCS 2006, group A) or an increased daytime frequency(>8 times/day) and/or urge urinary incontinence with or without urgency (group B); its prevalence and associated factors were investigated. RESULTS: The response rate for the questionnaires was 85.84%. The overall prevalence of OAB was 16.59%(group A) and 18.79%(group B). For groups A and B, the prevalence of OAB decreased with age from 22.89% to 12.16% and from 40.44% to 9.60%, respectively(p=0.0001). The overall rate of wet and dry OAB was 26.97% and 73.03%, respectively. Compared to the normal group, the children with OAB had a higher prevalence of nocturnal enuresis(NE), constipation, fecal incontinence, a history of urinary tract infection and delayed bladder control in both groups A and B(p<0.05). The rate of increased daytime frequency and urge incontinence were 3.69% and 2.31%(p=0.009), and 26.97% and 14.78%(p=0.0001) in group A and for the non-OAB children, respectively; their prevalence in group A decreased with age from 5.04% to 3.06% and from 45.74% to 18.50%, respectively(p=0.0001). CONCLUSIONS: The overall prevalence of OAB in group A for Korean children 5-13 years of age was similar to that in group B. However, the range of prevalence in group B was much more variable than that in group A. NE, constipation, fecal incontinence, a history of urinary tract infection and delayed bladder control may be risk factors for OAB in children.
Child ; Constipation ; Cross-Sectional Studies ; Fecal Incontinence ; Humans ; Korea ; Parents ; Prevalence ; Risk Factors ; Urinary Bladder ; Urinary Bladder, Overactive ; Urinary Incontinence ; Urinary Incontinence, Urge ; Urinary Tract Infections

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

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

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*

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