Familial juvenile hypertrophy of the breast.

OBJECTIVE: Juvenile (virginal) hypertrophy of the breast (JHB) is a relatively rare condition leading to gigantomastia in peripubertal females. The pathology is limited usually to the breast, with otherwise normal growth and development and without any other deformities. The rapid growth of the breast (bilateral or unilateral) in adolescent girls leads to significant physical and psychological difficulties. This gigantomastia is treated surgically by breast reduction or mastectomy and its modification. Familial JHB was described only once in the literature, and its etiology is unknown. RESULTS: We report here on a familial pattern of juvenile hypertrophy of the breast accompanied by congenital anonychia. To the best of our knowledge, this is the first report of such a presentation. Our study dealt with four members of the same family, related through their fathers, enduring congenital anonychia of hands and feet with no functional limitation and who showed rapid uncontrolled breast enlargement in prepubertal age. This was severe enough to cause the curtailment of their social activity and cessation of schooling. The mothers of all four patients had normal breasts and nails, whereas their fathers had anonychia. The genetic basis for the association between the two clinical findings is yet to be determined. CONCLUSION: The four girls underwent breast reduction surgery.     

Long-term outcomes among localized prostate cancer survivors: health-related quality-of-life changes after radical prostatectomy, external radiation, and brachytherapy.

PURPOSE: We sought to elucidate long-term changes in health-related quality-of-life (HRQOL) outcomes by prospectively re-evaluating a well-characterized cohort of prostate cancer (PC) survivors 4 to 8 years after primary treatment. PATIENTS AND METHODS: Patients who had been evaluated previously at a median of 2.6 years after radical prostatectomy (RP), external radiation (three-dimensional conformal radiation therapy [3-D CRT]), or brachytherapy (BT) were recontacted at a median of 6.2 years after treatment. The clinical relevance of long-term HRQOL impairment among survivors was established by comparison with controls of similar age. Factors associated with HRQOL changes during this interval were evaluated. RESULTS: Of the 964 eligible men, 709 (73.5%) completed measurable questionnaires. In four domains (urinary irritative-obstructive, urinary incontinence, bowel, and sexual), significant HRQOL differences were detected for at least one of the therapy groups, compared with controls (all P < .05). During the 4-year interval, significant improvement was observed for the urinary irritative-obstructive (P < .0001) and bowel (P < .0001) domains among BT patients, whereas urinary incontinence HRQOL worsened for both the BT (P = .0017) and 3-D CRT (P = .0008) treatment groups. Overall sexual HRQOL deteriorated for the 3-D CRT cohort (P = .0017), as well as for controls (P = .0136). Among RP patients, significant HRQOL changes were not observed. CONCLUSION: During a 4-year interval from earlier to longer-term phases of PC treatment survivorship, sexual, urinary, and bowel dysfunction remain significant concerns among early-stage PC treatment survivors, compared with control men. Although postprostatectomy HRQOL remains relatively stable during this interval, disease-specific HRQOL continues to evolve among men treated with BT and 3-D CRT.     

Portal Hypertension: Concepts in Diagnosis and the Role of Transjugular Intrahepatic Portosystemic Shunts in the Management of Complications

Portal hypertension is responsible for many of the manifestations of chronic liver disease. Some of these complications are the direct consequences of portal hypertension, such as gastrointestinal bleeding from either ruptured gastroesophageal varices or portal hypertensive gastropathy, ascites, and portosystemic encephalopathy.In the last 15 years, new endovascular procedures such as transjugular liver biopsy with its hemodynamic evaluation have been playing an important role in the diagnosis and management of this entity. Also, in cases where complications of severe portal hypertension such as acute or refractory bleeding or ascites are present, a transjugular portosystemic shunt procedure can decompress the portal system with a success rate greater than 90% of the cases. This review article provides the basic concepts of portal hypertension and its management using endovascular approach.     

Initial survival data of kidney transplant patients with pre-transplant monoclonal gammopathy

Soltero L, Carbajal H, Xu J, McCarthy J, Suki WN, Gaber AO, Adrogué HE. Initial survival data of kidney transplant patients with pre‐transplant monoclonal gammopathy.
Clin Transplant 2011 DOI: 10.1111/j.1399‐0012.2011.01539.x.
© 2011 John Wiley & Sons A/S. Abstract: Background: Monoclonal gammopathy of undetermined significance (MGUS) is the presence of a serum monoclonal protein at a concentration of <3 g/dL without evidence of lymphoproliferative disease or organ damage. The prevalence of MGUS in kidney transplantation (KT) candidates is unknown. The present is a retrospective report of patients who underwent evaluation for a KT and were found to have MGUS at our center. Methods: All transplant candidates found to have MGUS between the years 2000 and 2007 were included. Variables were collected. Patients with MGUS that received a KT were compared with patients with MGUS that were not transplanted. Results: Of a total of 1215 KT candidates, 34 were found to have MGUS during the KT evaluation. Nine patients with MGUS were transplanted. Myeloma or lymphoproliferative disease was not observed. Following transplantation, the MGUS group had a lower survival than the non‐transplanted group. However, survival from the time of MGUS diagnosis was not different between the transplanted and non‐transplanted MGUS groups. Conclusions: In this group, transplantation did not confer a survival benefit. It is our hope that these initial data will serve as a platform for future studies. We suggest MGUS screening in all patients older than 50 yr of age undergoing evaluation for transplantation.     

Hepatitis B vaccination in human immunodeficiency virus-infected adults receiving hemodialysis

BACKGROUND: The Centers for Disease Control and Prevention (CDC) recommends hepatitis B virus (HBV) immunization for all hemodialysis (HD) patients because they are at high risk of infection. Several studies have shown that the development of protective antibody titers after HBV vaccination is much lower in HD patients. We hypothesized that human immunodeficiency virus (HIV) infection in patients with end-stage renal disease (ESRD) would further impair the immune response to hepatitis B vaccination. METHODS: We performed a retrospective cohort study of patients undergoing long-term hemodialysis from 1990 to 2002 at the United States-based dialysis facilities of Gambro Corporation, North America. The response rate defined as an increase in anti-HBs levels >/=10 mIU/L after a month of the third dose of HBV vaccination was determined in HIV-infected and a randomly selected group of ESRD patients. The demographic information, laboratory data, and hepatitis B surface antibody (anti-HBs) titers were recorded from the Gambro Corporation database on these patients. RESULTS: Of the 347 adult HIV ESRD patients, 116 received three doses of recombinant hepatitis B vaccination. Seventy percent were male, and the majority (86%) were black. Of the 116 patients who received three doses of HBV vaccination, 62 (53.4%) developed protective antibody titers. This was comparable to the response rate of 50.4% in the randomly selected 220 non-HIV hemodialysis patients. Among HIV ESRD patients, the mean hemoglobin (Hgb) was higher in patients who developed protective antibody titers (Hgb 11.61 +/- 2 vs. 10.55 +/- 1.86, P value <0.01). On multivariate logistic regression analysis, higher Hgb was associated with protective antibody titers (odds ratio: 1.34, 95% CI 0.99-1.72). Seventy percent of the HIV-infected responders maintained protective antibody titers 6 months after vaccination. CONCLUSION: Hepatitis B vaccination should be offered to all HIV-infected ESRD patients because over half of the patients with HIV and ESRD can develop protective antibodies.     

Laparoscopic Cardial Calibration and Gastropexy for Treatment of Patients with Reflux Esophagitis: Pathophysiological basis and Result

Laparoscopic antireflux surgery is the gold standard procedure for treatment of patients with reflux esophagitis. The current results of the laparoscopic approach are absolutely comparables with the results obtained during the open surgery era. The Nissen, Nissen-Rossetti, or Toupet techniques are the more frequently used. We have performed cardial calibration and posterior gastropexy or Nissen fundoplication by the open approach with similar results. The purpose of this article is to present the anatomo-physiological basis for employing cardial calibration and posterior gastropexy in patients with reflux esophagitis. This study includes 108 symptomatic patients, 12 of them with associated extraesophageal manifestations ( posterior laryngitis). Endoscopic mild or moderate esophagitis was confirmed in 83 patients, Barrett’s esophagus in 12 patients, and type I or II hiatal hernia in 13 patients. All patients were also submitted to manometry, 24 hour intraesophageal pH monitoring, and barium swallow before and after surgery. Follow-up ranged from 12 to 36 months. There were no conversion, major intraoperative, or postoperative complications; nor were there any deaths. Postoperative dysphagia was present in 5% of cases. Symptomatic recurrence of reflux was observed in 10.3% and endoscopic presence of esophagitis in 12.3% of cases . Lower esophageal sphincter pressure increased significantly after surgery, even in patients with endoscopic recurrence. 24-hour intraesophageal monitoring improved after surgery, except in patients with objective recurrence of esophagitis. In conclusion, laparoscopic cardial calibration with posterior gastropexy presents comparable results to those reported after Nissen fundoplication and therefore could be another excellent therapeutic option in patients with reflux esophagitisThis paper was accepted for presentation as a free paper oral presentation for the last World Congress of ISS-SIC (cancelled).     

Remifentanil as a single drug for extracorporeal shock wave lithotripsy: a comparison of infusion doses in terms of analgesic potency and side effects

This randomized, double-blind study was designed to evaluate analgesic effectiveness and side effects of two remifentanil infusion rates in patients undergoing extracorporeal shock wave lithotripsy (ESWL) for renal stones. We included 200 patients who were administered remifentanil either 0.05 microg x kg(-1) x min(-1) (n = 100) or 0.1 microg x kg(-1) x min(-1) (n = 100) plus demand bolus of 10 microg of remifentanil via a patient-controlled analgesia (PCA) device. No other sedating drugs were given. The frequencies of PCA demands and deliveries were recorded. Arterial blood pressure, oxygen saturation, and respiratory rate were recorded throughout the procedure; postoperative nausea and vomiting (PONV), dizziness, itching, agitation, and respiratory depression were measured posttreatment. Visual analog scale (VAS) scores were taken preoperatively, directly postoperatively, and 30 min after finishing the procedure. There were no statistically significant differences in the frequency of PCA demands and delivered boluses or among perioperative VAS scores. The extent of PONV and frequency of dizziness and itching immediately after and dizziness 30 min after the end of treatment were significantly reduced in the smaller dose group. We conclude that a remifentanil regimen of 0.05 microg x kg(-1) x min(-1) plus 10 microg demands is superior to 0.1 microg x kg(-1) x min(-1) plus demands, as there was no difference in the VAS scores recorded between groups and it has a less frequent incidence of side effects in patients receiving ESWL. IMPLICATIONS: Remifentanil is an appropriate analgesic choice for patients undergoing extracorporeal shock wave lithotripsy (ESWL) therapy, as it has both fast onset and offset times. We studied remifentanil as a sole drug for ESWL and have shown that an infusion rate of 0.05 microg x kg-1 x min-1 plus patient-controlled analgesia demands of 10 microg provides adequate analgesia and has significantly less side effects than a dose of 0.1 microg x kg-1 x min-1 plus 10 microg demands.     

Transcatheter closure of residual atrial septal defect after cardiac transplantation.

A 55-year-old male patient experienced 2 acute neurologic events 3 weeks after orthotopic cardiac transplantation. Transesophageal echocardiography demonstrated a patent foramen ovale in the native portion of the interatrial septum with bidirectional shunting by Doppler and microbubble contrast. The defect was closed successfully with a CardioSeal transcatheter septal closure device. This case demonstrates the advantages of the percutaneous approach for closure of residual defects in the post-operative patient.