Response to three years of growth hormone therapy in girls with Turner syndrome

PURPOSE: Short stature is the most common finding in patients with Turner syndrome. Improving the final adult height in these patients is a challenge both for the patients and physicians. We investigated the clinical response of patients to growth hormone treatment for height improvement over the period of three years. METHODS: Review of medical records from 27 patients with Turner syndrome treated with recombinant human growth hormone for more than 3 years was done. Differences in the changes of height standard deviation scores according to karyotype were measured and factors influencing the height changes were analyzed. RESULTS: The response to recombinant human growth hormone was an increase in the height of the subjects to a mean value of 1.1 standard deviation for subjects with Turner syndrome at the end of the 3-year treatment. The height increment in the first year was highest. The height standard deviation score in the third year was negatively correlated with the age at the beginning of the recombinant human growth hormone treatment. Different karyotypes in subjects did not seem to affect the height changes. CONCLUSION: Early growth hormone administration in subjects with Turner syndrome is helpful to improve height response to the treatment.

Attenuated Renal Excretion in Response to Thiazide Diuretics in Gitelman's Syndrome: A Case Report

Gitelman's syndrome is a variant of Bartter's syndrome characterized by hypocalciuria and hypomagnesemia. The administration of thiazide diuretics may induce a subnormal increase of urinary Na+ and Cl- excretion in patients with Gitelman's syndrome, consistent with the hypothesis that less Na+ and Cl- than normal is reabsorbed by the thiazide-inhibitable transporter in Gitelman's syndrome. Specific mutations of NaCl cotransporter, coupled with mutant NaCl cotransporter expression studies clearly demonstrated that many of the characteristics of individuals with Gitelman's syndrome are explained by lack of function of NaCl cotransporter. We recently diagnosed a patient with Gitelman's syndrome by performing the thiazide and furosemide tests, and it is suggested that the clearance studies by diuretic administration may be of diagnostic help in Gitelman's syndrome.

Erectile Dysfunction in Hemodialysis Patients / 대한신장학회잡지

PURPOSE: Erectile dysfunction(ED) is a common problem in men with end-stage renal disease(ESRD). The present study was aimed at investigating the prevalence and risk factors of ED among patients receiving chronic hemodialysis. METHODS: The presence and severity of ED were assessed among 48 ESRD patients using the self-administered International Index of Erectile Function-5 (IIEF-5). Logistic regression was used to examine and test associations between ED and other medical conditions. RESULTS: The mean age was 57.9+/-11.9 years and the cause of ESRD was diabetes mellitus(47.9%), unknown cause(29.2%) and hypertension(16.7%). All subjects had ED. The proportions reporting mild ED, mild/moderate ED, moderate ED and severe ED were 8.3%, 18.8%, 18.8% and 54.2%, respectively. Age, diabetes mellitus, Karnofsky Index, serum albumin and creatinine levels were statistically correlated with ED. But, a multivariable logistic regression analysis demonstrated age(odds ratio [OR]=12.254, 95% confidence interval [CI] 2.02 to 74.32, p=0.006) and nitrates administration(OR=11.85, 95% CI 1.06 to 132.5, p=0.045) to be independently associated with the presence of severe ED. CONCLUSION: ED is extremely prevalent among hemodialysis patients. Increasing age and medical conditioning in which nitrates should be administered are associated with higher prevalence of severe ED.

Hyperhomocysteinemia in Patients with Chronic Renal Failure / 대한신장학회잡지

OBJEVTIVE: It has been reported that hyperhomocysteinemia is an independent risk factor for atherosclerotic complications, although the mechanisms remain unclear. The major determinents of total fasting plasma homocysteine(tHcy) concentrations have been recently reported but there are still conflicting data on the influence of those in peritoneal and hemodialysis patients. Therefore, we evaluated the prevalence and association of vascular complications and the determinents of hyperhomocysteinemia in chronic renal failure patients receiving conservative treatment(predialysis), peritoneal dialysis(PD) and hemodia- lysis (HD) patients. METHODS: We measured the factors, including fasting plasma vitamine levels(folate, vitamin B6 and vitamin B12), serum creatinine concentration, dialysis adequacy-related varibles as well as associated risk factors for vascular diseases that might affect tHcy concentrations in 37 predilysis, 30 PD, 34 HD patients and 21 normal persons. Continuous variables were compared using independent sample t-test. Spearman's correlation was used to determine the strength of association between tHcy and other predictive variables. Percentages were compared using Pearson's chi-square test or Fisher's exact test, depending on the frequencies. Independent determinents of tHcy concentration and atherosclerotic vascular complications were identified using multiple regression analysis. RESULTS: The prevalence of hyperhomocysteinemia was 83.8, 72, 88% among predialysis, PD and HD patients,respectively(Odds ratio was 103.33, 81.43, 150.0 vs. controls, respectively). tHcy values in predialysis, PD and HD patients are significantly higher than those in controls(24.68+/-9.01, 21.04+/-8.82, 23.62+/-9.46 vs. 8.80+/-2.07 mumoL/L, repectively, p<0.01). Predialysis, PD and HD patients with atherosclerotic vascular complications had higher tHcy concentrations than did predialysis, PD and HD patients without vascular complications(21.93+/-8.71 vs. 32.09+/-4.71 mumoL/L, p<0.01, 17.57+/-5.85 vs. 28.74+/-9.70 mumoL/L, p<0.01, 19.00+/-4.29 vs. 33.28+/-10.13, p<0.01 respectively). We also observed increasing odds ratios of vascular events with increasing tHcy concentrations. For predialysis, PD and HD patients, fasting plasma folate level had negative correlation with tHcy concentrations by spearman's simple correlaltion. And using muliple regression analysis, we recognized hyperhomocysteinemia is an independent risk factor for atherosclerosis and fasting plasma folate is a major determinent of tHcy concentrations in predialysis, PD and HD patients. CONCLUSIONS: Hyperhomocysteinemia in predialysis, PD and HD patients was more prevalent than that in normal controls. Risk of atherosclerotic vascular complications increased with increasing tHcy concentrations. Hyperhomocysteinemia is an independent risk factor for atherosclerosis and fasting plasma folate is a major determinent of tHcy concentrations in predialysis, PD and HD patients.

Percutaneous Catheterization of the Internal Jugular Vein for Hemodialysis

OBJECTIVES: The present study was aimed at evaluating the clinical experiences in the internal jugular venous catheterization for hemodialysis. METHODS: We retrospectively analyzed the data on internal jugular venous catheterization at Chonnam National University Hospital from May 2000 to Februrary 2001. RESULTS: There were 132 uremic patients with a total of 150 attempts of internal jugular cannulation. Overall success rate was 90.9% with average puncture trials of 2.3+/-2.1. 124 (82.7%) of the catheterization attempts were made on the right side and 26 (17.3%) were made on the left. The catheters were left in place from 2 to 87 days with an average of 19.5+/-15.3 days per catheter. The dialysis sessions per catheter were from 2 to 58 with an average of 11.3+/-6.8. The mean blood flow during hemodialysis immediately after catheterization was 213.4+/-42.2 ml/min. Thirty two (21.3%) patients had early complications. These included carotid artery puncture (11.3%), local bleeding (4.7%), local pain (3.3%), neck hematoma (0.7%) and malposition of the catheter (1.3%). Seventeen (11.3%) patients had late complications. These included fever or infection (11.3%), inadequate blood flow rate (3.3%) and inadvertent withdrawal (2.0%). There was no catheter-related mortality. CONCLUSIONS: Our experiences revealed that the internal jugular vein catheterization is relatively safe and efficient for temporary vascular access for hemodialysis.

Clinical Evaluations for Endoscopic Variceal Ligation in Esophageal Varices Bleeding / 대한소화기내시경학회지

Endoscopic injection sclerotherapy(EIS) has been shown to be the most effective simple method for control of bleeding and eradication of varices. This method has been aceepted widely as a standard treatment of bleeding esophageal varices. However, EIS may be associated with undesirable local and systemic complications. (continue...)

Endoscopic " 0 " Band Ligation Treatment for 3 Cases with Dieulafoy Lesion / 대한소화기내시경학회지

Dieulafoy lesion is very small and easily overlooked as a course of massive, often recurrent hemorrhage that results from the crosion of a submucosal artery, typically in the gastric cardia or fundus. The clinical picture of Dieulafoy lesion is quite uniform: patients commonly present with massive hemorrhage and melena without any relevant history. The diagnostic procedure of choice in patients with severe gastrointestinal bleeding is emergency endoscopy. The lesion is rare but potentially life threatening source of upper gastrointestinal bleeding. Before the endoscopic era, the prognosis for patients with these lesions was quite poor. However, recent reports have described the success of endoscopic therapy in the management of Dieulafoy lesion. We performed emergency endoscopy in 3 patients who had massive or recurrent episode of upper gastrointestinal bleeding, identified to the Dieulafoy lesion. We tried to Endoscopic "0" band ligation, successfully in hemostasis and prevention of recurrence.