Adding Branched-Chain Amino Acids and Vitamin D to Whey Protein Is More Effective than Protein Alone in Preserving Fat Free Mass and Muscle Strength in the First Month after Sleeve Gastrectomy.

Objectives: Sleeve gastrectomy (SG) is one of the most commonly performed weight loss (WL) bariatric procedures. The main goal of WL is reducing total body weight (TBW) and fat mass (FM). However, TBW loss is systematically accompanied by a decline in fat-free mass (FFM), predominantly in the first post-surgical month, despite protein supplementation.

Deleterious effects of interruption followed by reintroduction of enzyme replacement therapy on a lysosomal storage disorder.

Temporary interruption of enzyme replacement therapy (ERT) in patients with different lysosomal storage disorders may happen for different reasons (adverse reactions, issues with reimbursement, logistic difficulties, and so forth), and the impact of the interruption is still uncertain. In the present work, we studied the effects of the interruption of intravenous ERT (Laronidase, Genzyme) followed by its reintroduction in mice with the prototypical lysosomal storage disorder mucopolysaccharidosis type I, comparing to mice receiving continuous treatment, untreated mucopolysaccharidosis type I mice, and normal mice. In the animals which treatment was temporarily interrupted, we observed clear benefits of treatment in several organs (liver, lung, heart, kidney, and testis) after reintroduction, but a worsening in the thickness of the aortic wall was detected.

Adult onset of ganglioneuroblastoma of the adrenal gland: case report and review of the literature.

Ganglioneuroblastoma (GBN) is a malignant neoplasm of the autonomic nervous system. Adult onset of ganglioneuroblastoma is extremely rare. Only 16 cases have been reported in English literature, to date.

Characterization of joint disease in mucopolysaccharidosis type I mice.

Mucopolysaccharidoses (MPS) are lysosomal storage disorders characterized by mutations in enzymes that degrade glycosaminoglycans (GAGs). Joint disease is present in most forms of MPS, including MPS I. This work aimed to describe the joint disease progression in the murine model of MPS I.

Enzyme replacement therapy started at birth improves outcome in difficult-to-treat organs in mucopolysaccharidosis I mice.

Since we previously observed that in patients with mucopolysaccharidosis (MPS) the storage of undegraded glycosaminoglycans (GAG) occurs from birth, in the present study we aimed to compare normal, untreated MPS I mice (knockout for alpha-l-iduronidase-IDUA), and MPS I mice treated with enzyme replacement therapy (ERT, Laronidase, 1.2mg/kg every 2 weeks) started from birth (ERT-neo) or from 2 months of age (ERT-ad). All mice were sacrificed at 6 months.

Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice.

Mucopolysaccharidosis (MPS) type I (Hurler syndrome) is a lysosomal storage disorder characterized by deficiency of alpha-L-iduronidase (IDUA), intracellular storage of glycosaminoglycans (GAGs) and progressive neurological pathology. The MPS I mouse model provides an opportunity to study the pathophysiology of this disorder and to determine the efficacy of novel therapies. Previous work has demonstrated a series of abnormalities in MPS I mice behavior, but so far some important brain functions have not been addressed.

Intraperitoneal implant of recombinant encapsulated cells overexpressing alpha-L-iduronidase partially corrects visceral pathology in mucopolysaccharidosis type I mice.

Background Aims: Mucopolysaccharidosis type I (MPS I) is characterized by deficiency of the enzyme alpha-L-iduronidase (IDUA) and storage of glycosaminoglycans (GAG) in several tissues. Current available treatments present limitations, thus the search for new therapies. Encapsulation of recombinant cells within polymeric structures combines gene and cell therapy and is a promising approach for treating MPS I.

Training and recruiting future pharmacists through a hospital-based student internship program.

Purpose: A hospital-based pharmacy internship program is described.

Summary: The University of Pittsburgh Medical Center (UPMC) is a 19-hospital partnership affiliated with the University of Pittsburgh Schools of the Health Sciences, serving over 4 million patients per year through its community and teaching hospitals, community care programs, and managed care insurance product. UPMC created a structured pharmacy internship program that provides students with the skills to prepare them for future employment in a hospital or institutional pharmacy setting and creates a hiring and benefits infrastructure focused on student retention after graduation.

Gamma-glutamyltransferase activity in human atherosclerotic plaques--biochemical similarities with the circulating enzyme.

Background And Purpose: Serum gamma-glutamyltransferase (GGT) activity has been identified as a predictor of complications of atherosclerosis, with a prognostic value for cardiovascular diseases and stroke. Human atherosclerotic lesions contain active GGT, which can give rise to pro-oxidant molecular species; thus a direct contribution of GGT to atherosclerosis progression is conceivable. The relationship between plaque and serum GGT is however unclear.

Association of GIST, breast cancer and schwannoma in a 60-year-old woman affected by type-1 von Recklinghausen's neurofibromatosis.

Neurofibromatosis (NF1 or von Recklinghausen's disease) is a common autosomal dominant disease associated with a higher incidence of neoplasms than in the general population. We report the case of a 60-year-old woman affected by NF1 who was coincidentally diagnosed with a gastrointestinal stromal tumor, a breast carcinoma and a peripheral nervous system tumor.

Nationwide survey of hospital practices when compounding parenteral nutrition solutions in latex-allergic patients.

We surveyed 100 institutions in 50 states, varying in size from 50 to 1,000 beds. The purpose of this survey was to examine the policies and techniques hospitals used in confirming latex allergy (LA) in patients and preparing parenteral nutrition (PN) for LA patients. Our survey indicated that within the institutions in our study, many inpatient pharmacists do not use any defined method for confirming LA other than what is documented in the patient profile upon admission.

Somatization in primary care: a comparative survey of immigrants from various ethnic groups in Rome, Italy.

Objectives: Those responsible for interviewing immigrants in primary care settings often underestimate the importance of somatic symptoms arising from psychological distress. This study investigates the current prevalence of somatization in immigrants, and evaluates the comparative rates of somatic complaints in four ethnic groups (Caucasians, Asians, South/Center Americans, and Africans).

Methods: We studied the 301 consecutive outpatients (aged between 16 and 70 years) attending the "Caritas" primary care unit for immigrants in Rome (Italy) from January to December 2003, all of whom completed the 21-item version of the Bradford Somatic Inventory (BSI-21).