Impaired systemic endothelial function in patients with pseudoexfoliation syndrome

Pseudoexfoliation syndrome (PEX) is the most common clinical precursor of open-angle glaucoma. Recent studies have shown that pseudoexfoliative material is widely distributed throughout the body, including blood vessels. The aim of our study was to evaluate endothelial function in the brachial artery of patients with pseudoexfoliation syndrome. We prospectively examined 23 patients with PEX (mean age, 70 +/- 8 years) and 20 healthy age- and sex-matched individuals (mean age, 68 +/- 9 years) as a control group. Brachial artery endothelial function was assessed by vascular response to reactive hyperemia (flow-mediated dilation (FMD) and sublingual nitroglycerin (NTG-mediated dilation) using high-resolution ultrasound. Flow-mediated and NTG-induced dilation were expressed as the percent change in diameter after reactive hyperemia and after NTG administration relative to the baseline value, respectively. Patients with cardiovascular disease and other conditions associated with endothelial dysfunction were excluded. When compared with controls, patients with PEX had significantly lower flow-mediated dilation (4.5 +/- 2.8 versus 8.2 +/- 3.7, P = 0.01) and NTG-mediated dilation (10.9 +/- 3.1 versus 15.8 +/- 3.8, P = 0.0001). Flow-mediated dilation and NTG-mediated dilation were similar in PEX patients with glaucoma (n = 11) and without glaucoma (n = 12). Flow-mediated and NTG-mediated dilation did not correlate with any measured parameter in any patient or control subject. The findings indicate that systemic endothelial function is impaired in PEX syndrome patients.     

Minimally invasive management of biliary complications after laparoscopic cholecystectomy

BackgroundWe conducted this prospective study to evaluate the efficacy of percutaneous catheter drainage as a minimally invasive treatment in the management of symptomatic bile leak following biliary injuries associated with laparoscopic cholecystectomy.MethodsTwenty two patients with symptomatic bile leak following laparoscopic cholecystectomy underwent percutaneous drainage of the bile collection under ultrasound control. In patients with jaundice and in those with persistent drainage, endoscopic retrograde cholecysto-pancreatography (ERCP) was performed immediately for diagnostic and for therapeutic intervention when appropriate. In other patients, ERCP was performed 4–6 weeks after the discharge from the hospital to document the healing of the leaking site.ResultsFive patients with jaundice were initially treated by a combination of endoscopic plus percutaneous drainage. One of them required surgical treatment following diagnosis of a major duct injury. The other 17 were treated by percutaneous drainage initially and for 14 of them it was definitive treatment. Three patients required sphincterotomy as additional treatment for stopping the leak. There were no complications related to the percutaneous drainage procedure.ConclusionsMost patients with bile leakage can be managed successfully by percutaneous drainage. If biliary output does not decrease, endoscopy is needed. In patients with jaundice endoscopic diagnostic and therapeutic procedures should be performed immediately.     

Analysis of C-reactive protein and biochemical parameters in pericardial fluid

This study was designed to examine the relationship between pericardial fluid and plasma CRP levels, and to alterations in other biochemical parameters in patients undergoing Coronary Artery Bypass Grafting (CABG). The study group consisted of 96 Coronary Artery Disease (CAD) patients who were referred to our clinic for a CABG procedure and from whom sufficient amount of pericardial fluid could be collected. The patients were classified into 3 groups: Stable Angina Pectoris (SAP) (n=27), Unstable Angina Pectoris (USAP) (n=36), and Post-Myocardial Infarction (PMI) (n=33). Levels of CRP, glucose, albumin, total protein, Creatine Kinase (CK), Creatine Kinase-MB (CK-MB), and Lactate Dehydrogenase (LDH) were determined in pericardial fluid samples and in simultaneously collected blood samples from radial artery. The pericardial CRP and LDH levels in the PMI group were higher than in the SAP (p=0.015 and p=0.000, respectively) and USAP (p=0.011, p=0.047) groups. Serum CRP levels in USAP (p=0.014) and PMI (p= 0.000) groups were higher than those in the SAP group. Pericardial albumin levels in the PMI group were higher than in the USAP group (p=0.038). In all groups, the pericardial fluid/serum protein ratio was > 0.5, the LDL ratio was > 0.6, and pericardial fluid LDH concentrations were > 300 mg/dl. CRP level of pericardial fluid was significantly higher in the PMI group than in other groups. However, pericardial fluid LDH levels were higher than blood LDH levels in this group and were also higher than pericardial fluid LDH levels of other groups.     

Klippel-Trenaunay syndrome associated with polydactyly

Klippel-Trenaunay syndrome (KTS) manifests cutaneous vascular nevus, superficial venous varicosities, and hypertrophy of the affected limb. KTS may be associated with other developmental anomalies such as polydactyly, syndactyly, and macrocephaly. We present a case with KTS associated with polydactyly.     

L-carnitine reduces cochlear damage induced by gamma irradiation in Guinea pigs

L-carnitine (LC) protects cells from peroxidative damage. In this study, we tested whether L-carnitine (LC) prevents radiation-induced cochlear damage after total cranial irradiation (radiotherapy; RT). Male albino guinea pigs were randomly distributed in 3 groups. The Control group (n = 11) received neither LC nor irradiation, but saline solution ip and sham irradiation for 5 days. The RT group (n = 32) received saline solution ip as placebo therapy and exposure to total cranial irradiation of 33 Gy in 5 fractions of 6.6 Gy/day on 5 successive days, with a calculated (alpha/beta = 3.5) biological effective dose of fractionated irradiation equal to 60 Gy conventional fractionation. The LC + RT group (n = 36) received total cranial irradiation, plus LC (100 mg/kg/day, ip) for 5 days. The guinea pigs were killed at 4, 24, or 96 hr after the last dose of RT and the cochleas were enucleated for histopathologic examination. There was no cochlear degeneration in the control group. In the RT group, total cranial irradiation caused degeneration in stria vascularis (SV), spiral ganglion (SG), outer hair cells (OHC), and inner hair cells (IHC) of cochleas at 4, 24, and 96 hr. In the LC + RT group, LC administration reduced radiation-induced cochlear degeneration in SV and SG at 4, 24, and 96 hr, and in OHC and IHC at 24 and 96 hr (p <0.05). Thus, this study shows that L-carnitine can ameliorate radiation-induced cochlear damage in guinea pigs.     

Haematological findings in children with inborn errors of metabolism

Summary Early detection and therapy of haematological abnormalities and/or diseases may improve the prognosis of metabolic disorders. Accordingly, we aimed to evaluate the frequency and types of haematological abnormalities in children[-31pc] with various inherited metabolic disorders. The study group comprised 46 children with metabolic disorders who were followed at the Pediatric Metabolism Unit and were referred to the Pediatric Hematology Unit for evaluation of anaemia between June 2000 and 2005. The mean age of the children was 55.2 ± 64.8 months at haematological evaluation (range 1 month–18 years, median 22.0 months); 16 were female and 30 were male. Of these 46 patients with anaemia, 25 of (54.3%) had anaemia of chronic disease (ACD), 9 (19.6%) had iron-deficiency anaemia (IDA), 7 (15.2%) had megaloblastic anaemia due to vitamin B₁₂ deficiency, 3 (6.5%) had chronic haemolytic anaemia, 2 (4.3%) had autoimmune haemolytic anaemia, 1 had β-thalassaemia major, and 1 had hereditary spherocytosis. In addition to the anaemia, bicytopenia or pancytopenia was found in 8 of 46 children (17.4%). The study indicated that in organic acidaemias including methylmalonic acidaemia, propionic acidaemia, isovaleric acidaemia, and argininosuccinic acidaemia, the majority of patients had ACD (75%), which was followed by vitamin B₁₂ deficiency anaemia and IDA (p < 0.001). In PKU, both nutritional anaemias and ACD were present at about same frequency: 46.7% and 40%, respectively (p > 0.05). This study suggested that congenital anaemias such as hereditary spherocytosis or thalassaemias should be kept in mind as a coexisting haematological diseases in young patients with inborn errors of metabolism. In conclusion, ACD and nutritional anaemias are the most prevalent anaemias seen in patients with inborn errors of metabolism. Early detection of the disease, early administration of specific diet, and close monitoring of the patients are very important factors to prevent the development of haematological diseases in patients with inborn errors of metabolism. Competing interests: None declared     

Transplantation of viral-positive hepatitis C-positive kidneys into uninfected recipients offers an opportunity to increase organ access

The advent of direct-acting antivirals (DAAs) has provided the impetus to transplant kidneys from hepatitis C virus-positive donors into uninfected recipients (D+/R−). Thirty D+/R− patients received DAA treatment. Sustained virologic response (SVR12) was defined as an undetectable viral load in 12 weeks after treatment. An age-matched cohort of uninfected donor and recipient pairs (D−/R−) transplanted during same time period was used for comparison. The median day of viral detection was postoperative day (POD) 2. The detection of viremia in D+/R− patients was 100%. The initial median viral load was 531 copies/μL (range: 10-1 × 10⁸ copies/μL) with a median peak viral load of 3.4 × 10⁵ copies/μL (range: 804-1.0 × 10⁸ copies/μL). DAAs were initiated on median POD 9 (range: 5-41 days). All 30 patients had confirmed SVR12. During a median follow-up of 10 months, patient and graft survival was 100%, and acute rejection was 6.6% with no major adverse events related to DAA treatment. Delayed graft function was significantly decreased in D+/R− patients as compared to the age-matched cohort (27% vs 60%; P = .01). D+/R− transplantation offers patients an alternative strategy to increase access.