Nephrogenic diabetes insipidus is a rare genetic renal disease characterized by insensitivity of the kidney to the anti-diuretic effect of vasopressin in spite of elevated serum anti-diuretic hormone (ADH). Failure of the kidney to respond to ADH results in impaired osmoregulation and water reabsorption of the kidney, therefore, nephrogenic diabetes insipidus presents with a large amount of hypotonic polyuria, polydipsia, and dehydration. We report our experience of two familial cases of nephrogenic diabetes insipidus in brothers both having c.910+1delG in intron 2 of the AVPR2 gene with the brief review of related literatures.
Dehydration ; Diabetes Insipidus, Nephrogenic ; Humans ; Introns ; Kidney ; Polydipsia ; Polyuria ; Siblings ; Vasopressins ; Water ; Water-Electrolyte Balance

PURPOSE: The aim of this study was to observe clinical course including remission rate during antithyroid medication for Graves' disease in children and adolescents, and to evaluate factors related to remission. METHODS: This is a retrospective study of 42 patients (8 males), who were diagnosed at pediatric endocrine clinic in Chungbuk National University Hospital from January 1994 to December 2009. They were treated with antithyroid drugs only and were followed for at least 2 years. Their average age at diagnosis was 11.5 +/- 3.4 years, and average follow-up period was 4.5 +/- 2.2 years. RESULTS: At diagnosis, 64.3% of the subjects were in puberty, 87.8% showed goiter which was significantly prevalent in females (P < 0.05), and 38.9% manifested exophthalmos. Thyrotropin receptor antibody was positive in all the patients, whereas anti-microsomal antibody was positive in 88.1% and anti-thyroglobulin antibody in 81.0%. Twenty two (52.4%) patients remitted with 4.3 +/- 2.5 years' medication, and the others were continuously medicated at the last follow-up of 4.8 +/- 1.8 years. By quartile estimate for the remission time, 25 percentile was remitted at 3.7 years, 50 percentile at 7.1 years, and 75 percentile at 9.2 years. Among the factors for the remission, initial thyroid stimulating hormone level was significantly lower in non-remission group compared to remission group (P < 0.05). CONCLUSION: In pediatric Graves' disease treated only antithyroid drugs, the time required for remission is so long that it is necessary to establish fixed medication period and to choose definitive treatment modality as in adult Graves' disease through multicenter clinical study.
Adolescent ; Adult ; Antithyroid Agents ; Autoantibodies ; Child ; Exophthalmos ; Female ; Follow-Up Studies ; Goiter ; Graves Disease ; Humans ; Puberty ; Receptors, Thyrotropin ; Retrospective Studies ; Risk Factors ; Thyrotropin

INTRODUCTION: Persistent vesicoureteral reflux (VUR), a major cause of urinary tract infection (UTI) in children, can result in serious renal complications, such as reflux nephropathy and chronic renal failure. We evaluated the clinical characteristics and prognostic factors of VUR. METHODS: From December 1993 to May 2011, we examined 117 children with vesicoureteral reflux who were admitted to the Department of Pediatrics and Urology, Chungbuk National University hospital for a UTI. The patients were managed medically or surgically. RESULTS: Male patients had a slightly higher prevalence of VUR than female patients (55%). The degrees of the 161 refluxing ureters, as classified by the International Reflux Study Committee, were as follows: grade I, 15 ureters; grade II, 32 ureters; grade III, 54 ureters; grade IV, 26 ureters; grade V, 34 ureters. One hundred and sixty-one renal units (115 cases) underwent a 99m TC-DMSA renal scan, and 62% showed abnormal findings. The incidence of renal cortical defects showed a direct correlation with the severity of VUR. Ninety-four refluxing ureters were followed up medically, and 66 ureters (67%) either disappeared or improved. However, 9 refluxing ureters persisted. The spontaneous resolution rate of VUR seemed to be higher in younger patients with lower grades of reflux, and without renal cortical defects. Sixty-seven refluxing ureters (41%) were treated surgically, 62 refluxing ureters (92%) disappeared, and 5 refluxing ureters (8%) persisted. CONCLUSION: The incidence of renal cortical defects in patients with UTIs was 62% (in a 99m TC-DMSA renal scan), and showed a direct correlation with the severity of VUR. The spontaneous resolution rate seemed to be lower in the patients with higher grades of VUR, older age (over 4 years old) and diffuse renal cortical defects.
Child ; Chungcheongbuk-do ; Female ; Humans ; Incidence ; Kidney Failure, Chronic ; Male ; Pediatrics ; Prevalence ; Ureter ; Urinary Tract Infections ; Urology ; Vesico-Ureteral Reflux*

IgA nephropathy is the most common form of primary glomerulonephritis and chronic glomerular disease worldwide including Korea. Familial gathering of IgA nephropathy suggests that genetic factors contribute to the development of this disease. Although there have been many reports on familial IgA nephropathy with genetic analysis and their pedigrees, there has been few reports in Korea. We reported a partial familial IgA nephropathy pedigree with a brief review of the literatures.
Glomerulonephritis ; Glomerulonephritis, IGA ; Immunoglobulin A ; Korea ; Pedigree

PURPOSE: We studied the differences in the antibiotic susceptibilities of the microorganisms that causeing urinary tract infections (UTI) in children to obtain useful information on appropriate drug selection for childhood UTI. METHODS: We retrospectively analyzed the antibiotic susceptibilities of 429 microorganisms isolated from 900 patients diagnosed with UTI in the Department of Pediatrics, Chungbuk National University Hospital, from 2003 to 2008. RESULTS: The most common causative microorganisms for UTI were Escherichia coli (81.4%), Klebsiella pneumoniae (8.4%), Enterobacter spp. (1.7%), and Proteus spp. (0.4%). E. coli showed relatively high susceptibility as compared to imipenem (100%), amikacin (97.7%), aztreonam (97.9%), cefepime (97.7%), and ceftriaxone (97.1%), while it showed relatively low susceptibility to gentamicin (GM) (79.0%), trimethoprim/sulfamethoxazole (TMP/SMX) (68.7%), ampicillin/sulbactam (33.0%), and ampicillin (AMP) (28.6%). There were no significant differences in the image findings for causative microorganisms. CONCLUSION: Gram-negative organisms showed high susceptibility to amikacin and third-generation cephalosporins, and low susceptibility to AMP, GM, and TMP/SMX. Therefore, the use of AMP or TMP/SMX as the first choice in empirical and prophylactic treatment of childhood UTI in Korea should be reconsidered and investigated further.
Amikacin ; Ampicillin ; Aztreonam ; Ceftriaxone ; Cephalosporins ; Child ; Enterobacter ; Escherichia coli ; Gentamicins ; Humans ; Imipenem ; Klebsiella pneumoniae ; Korea ; Pediatrics ; Proteus ; Retrospective Studies ; Urinary Tract ; Urinary Tract Infections

PURPOSE: While the use of coupling material (CM) during defibrillation is known to decrease transthoracic impedance more so than other materials elsewhere, the situation in Korea is unclear. The study assessed the use of paddleskin CM during defibrillation in South Korean emergency institutes (EIs) and measured the impedances. METHODS: From October 15 to October 19, 2009, 112 EIs (all of regional emergency medical centers (EMCs), specialized care centers, and local EMCs) were surveyed. Institute members were queried about their current use of paddleskin CMs during defibrillation. Indications for use were studied by means of a World Wide Web search, descriptions on the CM packaging, and telephone conversations with CM manufacturers and related people. Impedance measurements were recorded from April 13 to August 3, 2009. RESULTS: During defibrillation, 27 different CMs were used in the 108 EIs. Only nine institutes used CMs directly for defibrillation. Impedence of nine CMs could not be measured. The impedance values were 1.69, 1.71, 2.00, and 6.12 ohm for four CMs used for defibrillation; 6.66 and 11.94 ohm for two CMs used for electrodes; 9.71, 9.82, 11.57, 13.68, 13.89, 14.36, 18.31, and 19.66 ohm for eight CMs used for ultrasonography; and 14.05, 17.71, 35.44, and 41.15 ohm for four other CMs. CONCLUSION: More education and public information about the use of CMs for defibrillation are needed.
Academies and Institutes ; Cardiography, Impedance ; Electric Countershock ; Electric Impedance ; Emergencies ; Emergency Service, Hospital ; Internet ; Korea ; Product Packaging ; Republic of Korea ; Telephone

PURPOSE: Due to the availability of serum prostate specific antigen (PSA) testing, the detection rate of insignificant prostate cancer (IPC) is increasing. To ensure better treatment decisions, we developed a nomogram to predict the probability of IPC. MATERIALS AND METHODS: The study population consisted of 1,471 patients who were treated at multiple institutions by radical prostatectomy without neoadjuvant therapy from 1995 to 2008. We obtained nonrandom samples of n = 1,031 for nomogram development, leaving n = 440 for nomogram validation. IPC was defined as pathologic organ-confined disease and a tumor volume of 0.5 cc or less without Gleason grade 4 or 5. Multivariate logistic regression model (MLRM) coefficients were used to construct a nomogram to predict IPC from five variables, including serum prostate specific antigen, clinical stage, biopsy Gleason score, positive cores ratio and maximum % of tumor in any core. The performance characteristics were internally validated from 200 bootstrap resamples to reduce overfit bias. External validation was also performed in another cohort. RESULTS: Overall, 67 (6.5%) patients had a so-called "insignificant" tumor in nomogram development cohort. PSA, clinical stage, biopsy Gleason score, positive core ratio and maximum % of biopsy tumor represented significant predictors of the presence of IPC. The resulting nomogram had excellent discrimination accuracy, with a bootstrapped concordance index of 0.827. CONCLUSION: Our current nomogram provides sufficiently accurate information in clinical practice that may be useful to patients and clinicians when various treatment options for screen-detected prostate cancer are considered.
Aged ; Asian Continental Ancestry Group ; Humans ; Logistic Models ; Male ; Middle Aged ; *Nomograms ; Prostatectomy ; Prostatic Neoplasms/*diagnosis/surgery

We investigated the clinical significance of large difference (> or = 2 points) between biopsy-derived (bGS) and post-prostatectomy Gleason scores (pGS). At 14 medical centers in Korea, 1,582 men who underwent radical prostatectomy for prostate cancer were included. According to the difference between bGS and pGS, the patients were divided into three groups: A (decreased in pGS > or = 2, n = 30), B (changed in pGS < or = 1, n = 1,361; control group), and C (increased in pGS > or = 2, n = 55). We evaluated various clinicopathological factors of prostate cancer and hazards for biochemical failure. Group A showed significantly higher mean maximal percentage of cancer in the positive cores (max%) and pathological T stage than control. In group C, the number of biopsy core was significantly smaller, however, tumor volume and max% were significantly higher and more positive biopsy cores were presented than control. Worse pathological stage and more margin-positive were observed in group A and C than in control. Hazard ratio for biochemical failure was also higher in group A and C (P = 0.001). However, the groups were not independent factors in multivariate analysis. In conclusion, large difference between bGS and pGS shows poor prognosis even in the decreased group. However it is not an independent prognostic factor for biochemical failure.
Age Factors ; Aged ; Biopsy ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Neoplasm Staging ; Prognosis ; *Prostatectomy ; Prostatic Neoplasms/*pathology/surgery ; Recurrence ; Severity of Illness Index