Search results for "GAL"

Long-term effectiveness of agalsidase alfa enzyme replacement in Fabry disease: A Fabry Outcome Survey analysis

2015

Outcomes from 5 years of treatment with agalsidase alfa enzyme replacement therapy (ERT) for Fabry disease in patients enrolled in the Fabry Outcome Survey (FOS) were compared with published findings for untreated patients with Fabry disease. Data were extracted from FOS, a Shire-sponsored database, for comparison with data from three published studies. Outcomes evaluated were the annualized rate of change in estimated glomerular filtration rate (eGFR) and left ventricular mass indexed to height (LVMI) as well as time to and ages at a composite morbidity endpoint and at death. FOS data were extracted for 740 treated patients who were followed for a median of ~ 5 years. Compared with no trea…

Dissecting the molecular antifibrotic effect of the TGF-Beta(I) receptor kinase inhibitor galunisertib in precision-cut liver slices

2016

Splenic respiratory gas exchange and glucose uptake in patients with splenomegaly in hypersplenism and Hodgkin's disease.

1977

Blood samples are taken from the splenic artery, vein and pulp of patients suffering from Hodgkin's disease (n=10) or hypersplenism (n=7) and undergoing splenectomy. In these samples, the relevant parameters of the respiratory gas exchange as well as glucose and lactate concentrations are determined. In hypersplenism (mean splenic wet weight: 543 g) the mean oxygen consumption of the splenic tissue amounts to 0.9 ml O2/100 g/min taking into account a mean splenic blood flow of 80 ml/100 g/min. The glucose uptake and the lactate release are 9 mg/100 g/min and 5.5 mg/100 g/min, respectively. These values are in close agreement with the results obtained in the normal and undisturbed spleen in …

Ingesta nutricional en pacientes afectados de esclerosis lateral amiotrófica una consulta ambulatoria de nutrición artificial en Portugal

2021

Introducción: La Esclerosis Lateral Amiotrófica (ELA) es una patología neurodegenerativa crónica y progresiva, que produce la muerte de las motoneuronas. Deriva en pérdida de peso, de masa muscular e incremento de deficiencias nutricionales. Existe relación entre la ingesta nutricional y la patogénesis de la ELA. El objetivo de este análisis fue describir las características sociodemográficas, antropométricas y clínicas junto con la ingesta nutricional en una muestra de pacientes portugueses afectados de ELA. Material y Métodos: Se realizó una anamnesis clínica breve. El peso corporal fue evaluado siguiendo el protocolo de la International Society for the Advancement of Kinanthropometry (IS…

The Infanticide: Some Forensic and Ethical Issues

2013

Forensic investigation and clinical treatment of infanticide mother is occasion to reflect about this crime, related to social stigma. The forensic-psychiatric assessment must be anchored on authentic and meaningful clinical relationship to criminal mother, without misinterpretation and controtransferal dynamics, such as emotional reactions of mistrust and stigmatization toward the offender, which prevent the evaluator from a correct psychological and psychopathological comprehension. Otherwise, diachronic, multidisciplinary diagnostic evaluation may lead to strategies for treatment and rehabilitation, which can bring the patient to regain his dignity, his working capacities and social role…

2018

Objective: Acromegalic patients display a distinct neuropsychological profile and suffer from chronic physical complaints. We aimed to investigate in more detail these aspects in acromegalic patients, dependent on influencing factors like disease activity, age, sex, chronic medication, surgery, pituitary radiation, pituitary insufficiency and comorbidities. Design: Cross sectional, multicentric. Methods: 129 patients (M/W 65/64, 58.3 ± 12.7 years, 53/76 with active/controlled disease). Acromegalic patients completed the following inventories: NEO-FFI, IIP-D, and the Giessen Complaints List (GBB-24), after written informed consent. Age, sex, IGF-1 concentrations, comorbidities, treatment mod…

Utility of Periodontal exploration in patients with Fibromyalgia

2012

This is an open-access article distributed under the terms of the Creative Commons Attribution License.

New loci associated with kidney function and chronic kidney disease

2010

Chronic kidney disease (CKD) is a significant public health problem, and recent genetic studies have identified common CKD susceptibility variants. The CKDGen consortium performed a meta-analysis of genome-wide association data in 67,093 individuals of European ancestry from 20 predominantly population-based studies in order to identify new susceptibility loci for reduced renal function as estimated by serum creatinine (eGFRcrea), serum cystatin c (eGFRcys) and CKD (eGFRcrea 60 ml/min/1.73 m 2; n = 5,807 individuals with CKD (cases)). Follow-up of the 23 new genome-wide-significant loci (P 5 × 10 8) in 22,982 replication samples identified 13 new loci affecting renal function and CKD (in or…

Endocrinology and physiology of pseudocyesis

2013

This literature review on pseudocyesis or false pregnancy aims to find epidemiological, psychiatric/psychologic, gynecological and endocrine traits associated with this condition in order to propose neuroendocrine/endocrine mechanisms leading to the emergence of pseudocyetic traits. Ten women from 5 selected studies were analyzed after applying stringent criteria to discriminate between cases of true pseudocyesis (pseudocyesis vera) versus delusional, simulated or erroneous pseudocyesis. The analysis of the reviewed studies evidenced that pseudocyesis shares many endocrine traits with both polycystic ovarian syndrome and major depressive disorder, although the endocrine traits are more akin…

Malignant metastatic melanoma to the gallbladder: Report of a peculiar case

2020

Highlights • Melanoma is one of the most aggressive and one of the fastest growing types of cancer. The gallbladder envolvement from a metastatic cutaneous malignant melanoma is a highly uncommon finding, usually associated with diffuse metastatic disease and observed during autopsy. • We report a case of a 35-years old Caucasian woman with isolated metastasis of gallbladder form cutaneous primary malignant melanoma underwent to laparoscopic cholecystectomy. • Malignant cutaneous melanoma is a skin cancer characterized by the highest mortality. Isolated metastasis of gallbladder are barely described and mostly present in a widespread metastatic disease. Surgical treatment is indicated consi…