Medical management of porto-pulmonary hypertension and right heart failure prior to living-related liver transplantation

Porto-pulmonary hypertension and right heart failure are relatively prevalent complications of end-stage liver disease and may increase mortality of patients undergoing cadaveric orthotopic liver transplantation. Even with extensive pre-transplant evaluation, these complications are frequently diagnosed unexpectedly in the operating room and transplant procedure may need to be aborted due to high perioperative mortality from both right and left ventricular failure. Living-related liver transplantation is a new surgical alternative to cadaveric liver transplantation, but presence of porto-pulmonary hypertension increases its postoperative mortality as well. Due to inherent elective nature, however, living-related liver transplantation may allow for preoperative hemodynamic optimization and treatment of right ventricular failure. To the authors' knowledge, this is the first reported case of an adult patient with porto-pulmonary hypertension who underwent successful living-related liver transplantation. Favorable transplantation outcome was obtained in this case through good hemodynamic control with long-term IV epoprostenol therapy, preoperative right heart calcification, and perioperative administration of pulmonary vasodilators and inotropic agents.     

Recent advances in pediatric liver transplantation

Pediatric liver transplantation has matured into a wellestablished, highly successful treatment for advanced pediatric liver disease. Recent 1-year success rates range from 85% to 95%. This unprecedented achievement is the result of careful selection criteria and optimal timing of transplantation, technical advances in surgical technique, and improved treatment following transplant. This report highlights many recent published findings representing advances that have led to current successful approaches.     

Clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C) below age 1

Clinical Rheumatology -     

Loss of speech after orthotopic liver transplantation

Alteration of speech is a rare but distressing complication of orthotopic liver transplantation (OLT). We describe a characteristic speech disorder identified in a large series of consecutive patients undergoing OLT. Between 1988 and 1993, 525 adults underwent OLT. For all recipients with neurologic complications, we reviewed clinical findings, imaging and electrophysiologic test results, and perioperative laboratory data. Five patients (ages 23–52; UNOS status 3–4) exhibited a characteristic pattern of stuttering dysarthria, leading to complete loss of speech production, occasionally with elements of aphasia. In four of the five patients, right-sided focal seizures were subsequently noted. All cases presented within the first 10 postoperative days and improved within 1 month of cessation of cyclosporin (CyA), although halting, monotonous speech was evident to some degree in all five for up to 1 year. There was no correlation between onset of symptoms and CyA levels. None of the patients had clinical or radiologic findings suggestive of central pontine myelinolysis or akinetic mutism. EEGs and Spect scan results were consistent with dysfunction in the left frontotemporoparietal regions of the brain. A characteristic speech disorder, which may be described as cortical dysarthria or speech apraxia, occurs in approximately 1% of adults undergoing OLT. Prompt recognition of this syndrome and temporary cessation of CyA therapy may favorably affect the course.     

Microanatomy of milk ducts in the nipple

The aim of this study was to determine number and diameter of milk ducts in the nipple and to investigate the possible influences of age, breast weight, and diameter of the nipple on the number of ducts. Two hundred and twenty-six carcinoma mastectomy specimens were weighed and the nipple diameters measured. The number of ducts was counted in histological cross sections. Mean diameter of the nipple and mean breast weight were 13.9 mm and 844.6 g, respectively. There was a small but statistically significant positive correlation between nipple diameter and number of milk ducts (rho = 0.158; p = 0.01), but no correlation with breast weight. The mean number of ducts in the nipple duct bundle was 17.5. This is significantly higher than the number of ducts reported to open on the nipple surface. This discrepancy could reflect duct branching within the nipple or the presence of some ducts which do not reach the nipple surface. Smaller breast ducts (diameter < 0.5 mm) represent nearly 50% of the nipple ducts and could be a challenge to the ductoscopy technology.     

Portal hypertension in children: expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension

Complications of portal hypertension in children lead to significant morbidity and are a leading indication for consideration of liver transplantation. Approaches to the management of sequelae of portal hypertension are well described for adults and evidence-based approaches have been summarized in numerous meta-analyses and conferences. In contrast, there is a paucity of data to guide the management of complications of portal hypertension in children. An international panel of experts was convened on April 8, 2011 at The Children's Hospital of Pittsburgh of UPMC to review and adapt the recent report of the Baveno V Consensus Workshop on the Methodology of Diagnosis and Therapy in Portal Hypertension to the care of children. The opinions of that expert panel are reported.     

Protective effect of beta-glucan on contrast induced-nephropathy and a comparison of beta-glucan with nebivolol and N-acetylcysteine in rats

Background

It has been shown that beta-glucan (BG), which has antioxidant and immunomodulatory effects, attenuats renal ischemia–reperfusion injury. We aimed to investigate whether BG might have a preventive role against the development of contrast-induced nephropathy and to compare its effect with nebivolol (Nb) and N-acetylcysteine (NAC).

Methods

Thirty-six Wistar albino female rats were randomly divided into six groups (n?=?6 each): control, contrast media (CM), BG, BG?+?CM, Nb?+?CM, and NAC?+?CM. With the exception of control and CM groups, the others were given drugs orally once a day for 5?days. Kidney function parameters, inflammatory parameters, and serum and renal tissue oxidative stress markers were measured.

Results

Increases of serum creatinine and blood urea nitrogen levels were significantly higher (p?p?p?p?Conclusion This study suggests that BG protects or ameliorates against contrast-induced nephropathy. Its beneficial effects may be similar to or greater than those of Nb or NAC.