Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia / 소아과

Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were 10x6 cm and 7.5x4.5 cm, respectively. His height was 155.1 cm (standard deviation score [SDS], -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and 9alpha-fludrocortisone; thereafter, the reduced testis size has been maintained.

Ethnic Coefficients for Glomerular Filtration Rate Estimation by the Modification of Diet in Renal Disease Study Equations in the Korean Population

Race and ethnicity are influential in estimating glomerular filtration rate (GFR). We aimed to find the Korean coefficients for the Modification of Diet in Renal Disease (MDRD) study equations and to obtain novel proper estimation equations. Reference GFR was measured by systemic inulin clearance. Serum creatinine (SCr) values were measured by the alkaline picrate Jaffe kinetic method, then, recalibrated to CX3 analyzer and to isotope dilution mass spectrometry (IDMS). The Korean coefficients for the 4 and 6 variable MDRD and IDMS MDRD study equations based on the SCr recalibrated to CX3 and to IDMS were 0.73989/0.74254 and 0.99096/0.9554, respectively. Coefficients for the 4 and 6 variable MDRD equations based on the SCr measured by Jaffe method were 1.09825 and 1.04334, respectively. The modified equations showed better performances than the original equations. The novel 4 variable equations for Korean based on the SCr measured and recalibrated to IDMS were 107.904xSCr-1.009xage-0.02 (x0.667, if woman) and 87.832xSCr-0.882xage0.01 (x0.653, if woman), respectively. Modified estimations of the MDRD and IDMS MDRD study equations with ethnic coefficients and the novel equations improve the performance of GFR estimation for the overall renal function.

Quinolone Resistance in Community-Acquired Acute Pyelonephritis / 대한신장학회잡지

BACKGROUND: Quinolone antibiotics are now frequently used for the empirical therapy of acute pyelonephritis (APN). However, failure of this empirical therapy is often encountered in clinical practice, and the emergence of quinolone resistance should be concerned. This study was undertaken to investigate the prevalence of quinolone resistance in community-acquired APN and to analyze the clinical characteristics of quinolone-resistant APN. METHODS: Clinical data were retrospectively analyzed from 367 patients who were admitted to Hanyang University Hospital for APN from January 2002 through December 2004. According to the result of urine culture, the patients were divided into quinolone-susceptible and quinolone-resistant groups, and clinical characteristics were compared. RESULTS: Urine culture was positive in 241 out of 367 patients, and E. coli was the most common (90%) isolate. The prevalence of quinolone resistance based on the in vitro ciprofloxacin susceptibility test was found to be 19.5%, and among the E. coli isolates the prevalence of quinolone resistance was 19.8 %. 91 patients had underlying diseases and they were more frequently complicated by urosepsis. The ages and the prevalence of azotemia were not significantly different between two groups. Quinolone-resistant group had a lesser frequency of urosepsis compared with quinolone-susceptible group. Diabetic patients were more frequently quinolone-susceptible than quinolone-resistant. CONCLUSION: Although the prevalence of quinolone resistance is rather high in community-acquired APN based on the in vitro susceptibility test, the choice of quinolone antibiotics seems to be valid for the empirical therapy.

Outcomes of Kidney Transplantation from Spousal Donors: Comparison with Kidney Transplantation from Living-related Donors / 대한신장학회잡지

BACKGROUND: Although transplantation is the best treatment for many people with end-stage renal disease, the gap between the number of organs and the number of potential recipients continues to widen. In addition to living-related individuals, the primary source of donor kidney, the severe organ shortage has led to consideration of genetically unrelated but emotionally related persons as donor candidates. The aim of this study was to compare the results of spousal kidney transplantation with those of living-related kidney transplantation and to analyze the characteristics of spousal kidney transplantation. METHODS: Clinical data were retrospectively analyzed from 21 patients with spousal kidney transplantation and 205 patients with living-related kidney transplantation. Cumulative renal allograft survival was compared between the two groups using Kaplan-Meier curve and log-rank test. Subgroup analysis was done within the patients with spousal kidney transplantation. RESULTS: The patients were significantly older in spousal group (43.7+/-7.8 years) than in living-related group (36.2+/-10.8 years). Donor age was also significantly higher in spousal group (43.0+/-8.4 years) than in living-related group (39.8+/-13.9 years). The number of HLA mismatch was significantly larger in spousal group (3.79+/-1.03) than in living-related group (2.60+/-1.21). The episodes of acute rejection occurring within a year after the transplantation were more frequent in spousal group (5/21) than in living-related group (13/205). Kaplan-Meier curves for cumulative survival of renal allograft revealed no difference between spousal group and living-related group. Renal allograft survival rates in spousal group were 85.2% at 1 year, 75.2% at 5 years, and 67.7% at 10 years after the transplantation. In living-related group, renal allograft survival rates were 96.6% at 1 year, 85.9% at 5 years, and 69.9% at 10 years after the transplantation. Within the patients with spousal kidney transplantation, cumulative renal allograft survival was superior in cases with absent acute rejection, husband-to-wife transplantation, and the number of HLA mismatch less than 5. CONCLUSION: Spousal kidney transplantation shares comparable results with living-related kidney transplantation despite older age, poorer HLA matching and a higher rate of acute rejection. Spousal donor transplants could be a real alternative especially when the donors are husband and the number of HLA mismatch is less than 5.

A Case of Transplant Glomerulopathy Complicated by Crescent Formation / 대한이식학회지

Transplant glomerulopathy (TGP) is specified as thickening of capillary wall of glomerulus and clinically presented with proteinuria and progressive graft dysfunction. In contrast, crescent formation represents an extracapillary proliferative glomerular change and is clinically presented with rapidly progressive renal failure. Previously, in transplant kidneys, crescent formation was reported only in anti-GBM disease and ANCA- associated vasculitis. Here we report a case with a very unusual combination of transplant glomerulopathy and crescent formation. Ten years after the renal transplantation the patient was admitted due to proteinuria and progressive azotemia. Although his underlying renal disease was IgA nephropathy, the transplant kidney biopsy revealed typical findings of transplant glomerulopathy without specific immune deposits, but with extensive cellular crescents. Methylprednisolone pulse therapy was not successful, and he was switched to maintenance hemodialysis.

Tumor Lysis Syndrome in Lymphoblastic Crisis of CML

Tumor lysis syndrome (TLS) defines the metabolic derangements that occur with tumor breakdown following the initiation of cytotoxic therapy. TLS results from the rapid destruction of malignant cells and the abrupt release of intracellular materials and their metabolites into the extracellular space. The syndrome causes hyperuricemia, hyperkalemia, hyperphosphatemia, secondary hypocalcemia and uremia. It can result in acute renal failure and be fatal. Early recognition of patient at risk and preventive measures are important. There is a high incidence of TLS in tumors with high proliferative rates and large burden such as acute lymphoblastic leukemia and Burkitt's lymphoma. It less commonly occurs in solid tumors such as testicular cancer, breast cancer and small cell lung cancer. There are only a few reports on TLS complicated in CML in blast crisis. So we report a 45-yr-old woman presenting with TLS associated with CML in lymphoblastic crisis after the initiation of cytotoxic chemotherapy.

Relationship between serum cystatin C and glomerular filtration rate in renal transplant patients / 대한내과학회지

BACKGROUND: Serum cystatin C has been proposed to be a sensitive glomerular filtration rate (GFR) marker. This study was undertaken to evaluate correlation between serum cystatin C and GFR estimates in renal transplant recipients. METHODS: Serum cystatin C, serum creatinine and 24-hour creatinine clearance were measured in 40 adult renal transplant recipients. Three different GFR estimates were used: 24-hour creatinine clearance, Modification of Diet in Renal Disease (MDRD) equation, and Cockcroft-Gault formula. Linear regression analysis was performed to evaluate correlations between GFR estimates and serum cystatin C, and nonparametric receiver operating characteristic (ROC) analysis was carried out to compare the diagnostic accuracy of serum cystatin C with that of serum creatinine. RESULTS: Significant correlations were noted between each of the 3 different GFR estimates (from 24-hour creatinine clearance, MDRD equation and Cockcroft-Gault formula) and serum cystatin C. Serum cystatin C also correlated significantly with serum creatinine. In the ROC analysis, the areas under the curve for cystatin C and creatinine were not significantly different when using a cut-off GFR estimate at 60 mL/min/1.73 m2. CONCLUSIONS: Serum cystatin C is a useful marker for the assessment of GFR in renal transplant recipients, but it is not clear whether cystatin C is superior to creatinine in detection of a mild to moderate renal impairment.

Immunocytochemical Study for Detection of Lactoglobulin in Alveolar Macrophage of Cow Milk Aspirated Mouse / 소아알레르기및호흡기학회지

PURPOSE: It is difficult to detect small amount of aspiration into the lungs due to the lack of safe, sensitive and specific diagnostic tool. Recently, in animal studies, it has been reported that immunocytochemistry for lactoglobulin can be used to detect the minimal aspiration of cow milk. So, we tried to determine the difference between immunocytochemistry for lactoglobulin and Oil Red O stain of alveolar macrophages in cow milk aspirated mice. METHODS: Fifty seven mice with 6-8 weeks old and 30-40 g weighing were used. Mice received either single or multiple intranasal instillation of 0.05 ml cow milk for study and saline for control under the anesthesia with ketamine and xylazine. The trachea of mouse was cannulated with 20G Jelco needle and then, mouse lungs were lavaged 3 times with 0.5 ml of phosphate buffer solution at 4 hours, 12 hours, 24 hours after the last milk or saline instillation. Cells in bronchoalveolar lavage fluid(BALF) were stained with Oil Red O and immunocytochemistry for beta-lactoglobulin. RESULTS: After single aspiration of milk, no cellular difference was found in bronchoalveolar lavage fluid(BALF) when compared with saline aspirated group at 4 hours. But after repeated aspiration of milk, significant change was observed in the number of alveolar macrophage, neutrophil, lymphocyte and eosinophil. Immunocytochemical reactivity was not observed in alveolar macrophages of saline aspirated group. Lipid-laden alveolar macrophages were recovered rarely in Oil Red O staining. Immunocytochemical staining displayed stain-positive alveolar macrophages for beta-lactoglobulin at 4 hours after milk aspiration, it had a peak at 12 hours and decreased markedly at 24 hours. Immunocytochemical stain positive alveolar macrophages appeared similarly in number between single and repeated aspiration group. CONCLUSION: These observations suggested that alveolar macrophages could be detected more easily on immunocytochemistry for lactoglobulin than Oil Red O stain and immunocytochemistry could be used as a sensitive & specific diagnostic method for the detection of milk aspiration.

Effects of Estrogen on Nitric Oxide and Aortic Atherosclerosis in Cholesterol-Fed Rabbits / 대한흉부외과학회지

BACKGROUND: Although estrogen is known to retard atherosclerosis, the mechanisms of which remain unclear. To examine the effect of estrogen on blood nitric oxide production and aortic atherosclerosis, we did an experiment with rabbits. MATERIAL AND METHOD: Forty male New Zealand White rabbits aging 5 weeks were randomly divided as 10 normal diet(ND), 10 normal diet(ND) + transdermal estradiol-2 patches(END; 0.02 microgram/d), 10 hypercholesterol diet(CD ; 0.5% of diet; w/w), and 10 CD + transdermal estradiol-2 patches(ECD) group, respectively. After 12 weeks, we measured the serum estradiol-2, nitric oxide and cholesterol and examined the atherosclerotic lesion of thoracic and abdominal aorta with light and scanning electron microscopy. RESULT: The concentration of estradiol-2 was increased in both ED and ECD groups(p<0.001). The serum nitric oxide production of END, CD, and ECD groups decreased significantly than that of ND group(p<0.001). The total, HDL and LDL cholesterol concentrations were increase in both CD and ECD groups(p<0.001). Aortic lesion was observed only in CD and ECD groups, without any difference. On light microscopic examination, the lesions were consists mainly of accumulation of lipid-laden macrophages (foam cell). On scanning electron microscopic examinations, CD group showed scattered areas of endothelial damage. However, estradiol-2 administration to the cholesterol-fed animals did not significantly change these pathologic findings. CONCLUSION: The results shows hyperlipidemia brings a decreased production of nitric oxide, probably due to endothelial dysfunction. Estradiol-2 patch administration decreased nitric oxide level in male cholesterol-fed rabbits with unknown mechanism. The transdermal estradiol-2 patch did not show any effect on blood cholesterol level. The estrogen did not cause any change on atherogenesis on both thoracic and abdominal aorta. The gender difference and administration method may offset the beneficial effect of estrogen on atherogenesis. To gain a complete understanding for the action of estrogen on atherogenesis, further detailed studies for both genders are needed.