Transcatheter Occlusion of a Giant Pulmonary Arteriovenous Malformation in a 1-year-old Child Using Amplatzer Vascular Plugs and Interlocking Detachable Coils / 전남의대학술지

No abstract available.

Transcatheter Occlusion of a Giant Pulmonary Arteriovenous Malformation in a 1-year-old Child Using Amplatzer Vascular Plugs and Interlocking Detachable Coils / 전남의대학술지

No abstract available.

Clinical Outcomes of Initial Dexamethasone Treatment Combined with a Single High Dose of Intravenous Immunoglobulin for Primary Treatment of Kawasaki Disease

PURPOSE: To investigate the clinical effects of a single high dose intravenous immunoglobulin (IVIG) combined with initial dexamethasone as a primary treatment on Kawasaki disease (KD). MATERIALS AND METHODS: Between January 2008 and December 2010, we reviewed the medical records of 216 patients with complete KD patients that were admitted to a single medical center. 106 patients were treated with a single high dose of IVIG (2 g/kg) alone and 110 patients received IVIG and dexamethasone (0.3 mg/kg per day for three days). RESULTS: The combined IVIG plus dexamethasone patient group had a significantly shorter febrile period and duration of hospital stay (1.4+/-0.7 days vs. 2.0+/-1.2 days, p<0.001; 5.8+/-1.7 days vs. 6.9+/-2.5 days, p<0.001, respectively) than the IVIG alone group. The combined IVIG plus dexamethasone group required IVIG retreatment significantly less than the IVIG only group (12.7% vs. 32%, p=0.003). After completion of the initial IVIG, C-reactive protein levels in the combined IVIG plus dexamethasone group were significantly lower than those in the IVIG only group (2.7+/-4.0 mg/dL vs. 4.6+/-8.7 mg/dL, p=0.03). In the combined IVIG plus dexamethasone group, the incidence of coronary artery lesions tended to be lower without worse outcomes at admission after initial infusion of IVIG and in follow-up at two months; however, the differences were not significant (8.2% vs. 11.3%, p=0.22; 0.9% vs. 2.8%, p=0.29). CONCLUSION: Initial combined therapy with dexamethasone and a single high-dose of IVIG resulted in an improved clinical course, in particular a shorter febrile period, less IVIG retreatment, and shorter hospital stay without worse coronary outcomes.

Clinical Manifestation of Ambulatory Blood Pressure Monitoring in Children and Adolescent with Hypertension

BACKGROUND: The use of ambulatory blood pressure monitoring (ABPM) in childhood hypertension has been in great advance. ABPM has been able to detect significantly high blood pressure (BP) and also helpful for identifying white coat hypertension. METHODS: Between March 2007 and December 2009, we evaluated individuals aged from 7 to 19 years who were referred as high BP with ABPM, echocardiogram, and abdominal computerized tomography, calculation of body mass index (BMI) at Ajou University Hospital. ABPM was performed using the Tonoport V monitors and SpaceLab 90217 monitors. Seventy were evaluated with 55 boys and 5 girls. The mean age of them was 13.1 +/- 2.8 years (mean +/- standard deviation). RESULTS: Forty of seventy (57.1%) were diagnosed as hypertension in ABPM and 8 of 40 were diagnosed as secondary hypertension with underline diseases such as hyperthyroidism, chronic renal disease, Takayasu's arteritis, coarctation of aorta. The mean BMI in 40 was 24.8 +/- 4.72 kg/m2. Twenty-three of seventy (32.8%) were obese with higher prevalence than in general population. White coat hypertension was diagnosed in 30 of 70 (42.9%). CONCLUSIONS: ABPM could provide more detailed data including mean values of BP, load, and night dip in assessment of children's BP. According to increasing children's obesity, use of ABPM was thought to be necessary for evaluation of their risk of hypertension and useful for diagnosis of masked hypertension and white coat hypertension. Further more study of ABPM in children and adolescents would be needed for absolute standards of ABPM.

A Case of an Addition of Chromosome 9 Short Arm Associated with Multiple Congenital Anomalies

Human chromosome 9 is characterized by a high degree of morphologic heteromorphisms, including variation in the size of the heterochromatin. We present a case of a de novo short arm addition of chromosome 9, [46, XY, add(9)(p13)], associated with multiple anomalies, including trigonocephaly, upward slant of the palpebral fissures, patent ductus arteriosus, pulmonary hypertension, hypertrophic cardiomyopathy, umbilical hernia, ambiguous genitalia, and sensorineural hearing and visual loss. This mutation affects the pericentric region of the heterochromatin. This patient exhibited a clinically important breakpoint of the heterochromatic region of chromosome 9 short arm and the associated anomalies.

Clinical Parameters Predicting Responsiveness to Treatment in Enuresis Patients

PURPOSE: We tried to find out the clinical parameters which predict the outcome of treatment in children with enuresis. METHODS: Enuresis patients who visited our hospital during 2003-2007 were included. Parameters such as age, gender, height, weight, minimal voided volume, maximal voided volume, maximum functional bladder capacity, frequency of voiding, urine S,G. before and after sleep were measured and an enuresis diary was also recorded. The reduction in wetting frequencies were classified into three groups; none( or =50%) during the 2 weeks of evaluation and behavioral therapy to the 'initial non-responders'. RESULTS: Parameters mentioned above showed no significant relation to the treatment outcome. The response rate during the 2 weeks of the evaluation period was 32%(49/151) [complete in 1.3%(2/151), partial in 29.6%(47/151)]. Two-months' treatment responses were complete in 14(40%), partial in 19(54.3%) and none in 2(5.9%) responders(n=35), while they were 10(13.5%), 46(62.2%) and 18(24.3%), respectively in the non-responders(n=73)(P<0.05). CONCLUSION: We suggest that initial 'responsiveness' can be used as a predictor for good treatment outcome in patients with enuresis.

The Effect of Gonadotropin Releasing Hormone Agonist and Growth Hormone on Predicted Adult Height in Girls with Precocious Puberty / 대한소아내분비학회지

PURPOSE: Gonadotropin releasing hormone agonist (GnRH agonist) and growth hormone (GH) treatment is widely used in precocious puberty to delay rapid pubertal growth. We studied the effect of these agents on the predicted adult height in girls with precocious puberty. METHODS: 41 girls were selected who had precocious sexual development, with onset from 5 to 8 years of age. They were divided into two groups. Twenty four girls treated only with GnRH agonist were compared with 17 girls treated with GnRH agonist and GH. We analyzed chronologic age (CA), bone age (BA) and predicted adult height (PAH) during and after treatment. RESULTS: Before treatment, there were no differences for CA, BA, body mass index (BMI), PAH and Tanner stage between two groups. After treatment, the PAH [153.7+/-6.85 cm (-1.31+/-1.25 SDS) vs 158.8+/-5.82 cm (-0.30+/-1.24 SDS)] for both age groups were significantly increased. The difference between initial PAH and follow up PAH was significantly increased when the initial PAH was low (P=0.015), and the duration of treatment was long (P=0.009). CONCLUSION: In girls with precocious puberty, GnRH agonist delayed bone maturation and increased PAH. PAH increased more when GnRH agonist and GH treatment was used as opposed to GnRH agonist treatment alone. GH combination therapy should be considered if the initial PAH was very low.

Clinical Characteristics and Renal Outcomes of Acute Focal Bacterial Nephritis in Children

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.