PURPOSE: Recently, many studies on febrile convulsions again suggest that the degree of pyrexia may be related to the recurrence of febrile convulsions. In a previous study, we advocated that a low body temperature during the initial febrile convulsions is associated with an increase of recurrent febrile convulsions. Therefore, we have expanded the study by including 246 febrile convulsions during 6 years and investigated risk factors and especially the relationship between pyrexia and the recurrence rates. METHODS: Children with febrile convulsions were divided into three groups according to the degree of fever. Group I showed body temperatures higher than 39.5 degrees, group II from 38.5 to 39.4 degrees, and group III lower than 38.4 degrees. Then, we analyzed the recurrence rates of febrile convulsions. RESULTS: There occurred recurrent febrile convulsions in 19(41.3%) children with family history of febrile convulsion and 5(35.7%) children whose first-degree relatives diagnosed epilepsy. In group I, 5(13.5%) infants aged 6-18 months and 5(19.2%) aged 19-30 months had recurrent febrile convulsions. In group II, 22(36.1%) infants aged 6-18 months and 8(24.2%) aged 19-30 months had recurrent febrile convulsions. In group III, 21(42.0%) infants aged 6-18 months and 8(38.1%) aged 19-30 months had recurrent febrile convulsions. CONCLUSION: Children with a lower degree of pyrexia and also younger age at the onset of the first febrile convulsion were more susceptible to recurrent febrile convulsios than otherwise.
Body Temperature ; Child ; Epilepsy ; Fever* ; Humans ; Infant ; Recurrence* ; Risk Factors ; Seizures, Febrile*

PURPOSE: Our objective was to investigate the role of CD8+T cells in pathogenesis of wheezing in children with atopic nature. METHODS: Twelve atopic wheezers, 8 nonatopic wheezers, 8 disease controls and 8 healthy controls were enrolled in the study. We isolated CD8+ T cells from peripheral blood samples, incubated them for 72 hours either in the absence or presence of phytohemagglutinin (PHA) and compared the concentrations of interleukin (IL)-5, IL-13, and interferon (IFN)-gamma in the cell culture supernatants. RESULTS: In the atopic wheezer group, the IL-5 concentration was significantly higher after PHA stimulation than after non-stimulated incubation. This difference was not observed in the nonatopic wheezer, disease control and healthy control groups. IL-13 was undetectable in all of the cell culture supernatants. There was no significant difference in the IFN-gamma concentration between the PHA-stimulated and non-stimulated conditions in all 4 groups. CONCLUSION: The results of this study suggest that CD8+ T cells may play a role in the pathogenesis of wheezing in children with atopic nature through the production of IL-5.
Cell Culture Techniques ; Child ; Humans ; Interferon-gamma ; Interferons ; Interleukin-13 ; Interleukin-5 ; Interleukins ; Respiratory Sounds ; T-Lymphocytes

Hajdu-Cheney syndrome is a rare, autosomal dominant skeletal dysplasia marked by acro-osteolysis of the distal phalanges and severe osteoporosis. Although there are more than 60 reports published to date, proper treatment and subsequent outcome have been scarce. Herein, we report a progress of anti-resorptive therapy with zoledronic acid, in a woman with Hajdu-Cheney syndrome. Results suggest that anti-resorptive therapy may be important in delaying the progress of osteoporosis and preventing fractures, but not necessarily acro-osteolysis itself.
Acro-Osteolysis/complications/*drug therapy ; Adult ; Bone Density Conservation Agents/*therapeutic use ; Diphosphonates/*therapeutic use ; Female ; Hajdu-Cheney Syndrome/complications/*drug therapy ; Humans ; Imidazoles/*therapeutic use ; Osteoporosis/complications/*drug therapy

BACKGROUND AND OBJECTIVES: Survivors of pediatric hematopoietic stem cell transplantation (HSCT) are at risk for developing hypertension. The objectives of this study are to evaluate the prevalence and risk factors of early onset hypertension during the engraftment period after HSCT. SUBJECTS AND METHODS: This is a retrospective study of 157 consecutive patients (mean age at HSCT: 9.1+/-5.1 years) who underwent HSCT for acute myeloid leukemia (n=47), acute lymphoblastic leukemia (n=43), severe aplastic anemia (n=41), and other reasons (n=26). Blood pressure data were collected at five time points: 0, 7, 14, 21, and 28 days after HSCT. Hypertension was defined as having systolic and/or diastolic blood pressure > or =95th percentile according to age, gender, and height. To analyze the risk factors related to hypertension, data, including patients' demographic and transplant characteristics, were reviewed. RESULTS: Hypertension developed in 59 patients (38%), among whom 12 (7.6%) required long term therapy. Thirty-two (54%) patients had systolic and diastolic, 8 (14%) had only systolic, and 19 (32%) had only diastolic hypertension. Younger age, acute graft-versus-host disease, sinusoidal obstruction syndrome, treatment with antifungal agent, and greater increase in serum creatinine (Cr) levels were associated with hypertension. Multivariate analysis showed that younger age at HSCT and greater increase in serum Cr level were independent risk factors for hypertension. CONCLUSION: Prevalence of hypertension during immediate post-HSCT period is high, especially in younger children. A greater increase in Cr after HSCT was significantly associated with hypertension. Further study is needed to elucidate long-term cardiovascular complications in pediatric HSCT survivors.
Anemia, Aplastic ; Blood Pressure ; Child ; Creatinine ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Hepatic Veno-Occlusive Disease ; Humans ; Hypertension* ; Incidence* ; Leukemia, Myeloid, Acute ; Multivariate Analysis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prevalence ; Retrospective Studies ; Risk Factors* ; Survivors

BACKGROUND AND OBJECTIVES: Cardiovascular complications are the leading cause of morbidity and mortality in childhood cancer survivors. Hematopoietic stem cell transplantation (HSCT) is a curable therapy for pediatric cancer. However, changes in cardiac function in children after HSCT are not well known. We assessed left ventricular (LV) function in children after HSCT using speckle tracking echocardiography (STE). SUBJECTS AND METHODS: Forty consecutive patients with median age of 11.9 years (range, 1.5-16 years) who received HSCT for acute leukemia and had comprehensive echocardiography before and after (median 9.2 month) HSCT were included in this study. The LV function parameters including conventional tissue Doppler imaging (TDI) and STE data were collected from pre- and post-HSCT echocardiography. These data were compared to those of 39 age-matched normal controls. RESULTS: Compared to normal controls, post HSCT patients had similar (p=0.06) LV ejection fraction. However, the following three LV function parameters were significantly decreased in post HSCT patients: rate-corrected velocity of circumferential fiber shortening (p=0.04), mitral inflow E velocity (p<0.001), and mitral septal annular E' velocity (p=0.03). The following four STE parameters were also significantly decreased in post HSCT patients: LV global circumferential systolic strain (p<0.01), strain rate (SR, p=0.01), circumferential diastolic SR (p<0.01), and longitudinal diastolic SR (p<0.001). There was no significant change in TDI or STE parameters after HSCT compared to pre-HSCT. Patients with anthracycline cumulative dose >400 mg/m2 showed significantly (p<0.05) lower circumferential systolic strain and circumferential diastolic SR. CONCLUSION: Subclinical cardiac dysfunction is evident in children after HSCT. It might be associated with pre-HSCT anthracycline exposure with little effect of conditioning regimens. Serial monitoring of cardiac function is mandatory for all children following HSCT.
Case-Control Studies* ; Child* ; Echocardiography* ; Hematopoietic Stem Cell Transplantation* ; Humans ; Leukemia* ; Mortality ; Stem Cell Transplantation ; Survivors ; Ventricular Dysfunction, Left*

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.
Adolescent ; Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Child ; Cyclophosphamide ; Diagnosis ; Dyspnea ; Emergency Service, Hospital ; Female ; Glomerulonephritis ; Hematuria ; Hemoptysis ; Hemorrhage ; Hepatolenticular Degeneration ; Humans ; Penicillamine ; Peroxidase ; Plasmapheresis ; Proteinuria ; Trientine ; Vasculitis

Purpose: The aim of this study was to show that bariatric surgery (BS) is more effective than medical therapy (MT) in Asian obese patients. Methods: In this prospective, multicenter, nonrandomized, controlled trial, obese patients with body mass index of ≥35 kg/m2 or 30.0–34.9 kg/m2 with obesity-related comorbidities were assigned to undergo BS, such as laparoscopic sleeve gastrectomy and Roux-en-Y gastric bypass, or MT. Patients who underwent BS were evaluated 4, 12, 24, and 48 weeks after surgery, whereas patients who received MT were monitored at a hospital every 6 weeks for 1 year. At each visit, weight, waist and hip circumference, and blood pressure were measured, and patients underwent physical examination and laboratory testing. Health-related quality of life (HQOL) was investigated using Euro QOL-5 Dimension, Impact of Weight on Quality of Life questionnaire-Lite and Obesity-related Problems scale. Results: The study included 264 patients from 13 institutions; of these, 64 underwent BS and 200 received MT. Of the patients who underwent BS, 6.3% experienced early complications. Relative weight changes from baseline to 48 weeks were significantly greater in the BS than in the MT group (26.9% vs. 2.1%, P < 0.001), as were the rates of remission of diabetes (47.8% vs. 16.7%, P = 0.014), hypertension (60.0% vs. 26.1%, P < 0.001), and dyslipidemia (63.2% vs. 22.0%, P < 0.001). HQOL was better in the BS than in the MT group at 48 weeks. Conclusion BS was safe and effective in Korean obese patients, with greater weight reduction, remission of comorbidities, and quality of life improvement than MT.