Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia

Background/Aims: Congenital adrenal hyperplasia (CAH) is characterized by adrenal insufficiency with or without salt wasting. It is also accompanied by adrenomedullary hypofunction. The aim of the present study was to investigate adrenomedullary function in patients with CAH due to 21-hydroxylase and 11 beta-hydroxylase deficiencies and in age-matched normal subjects. Methods: We measured plasma catecholamines (epinephrine and norepinephrine) and urine metanephrine in 44 patients with CAH, 32 due to 21-hydroxylase deficiency (17 patients with the salt-wasting form and 15 patients with the simple virilizing form), and 12 due to 11 beta-hydroxylase deficiency, and in 25 healthy controls. Results: Plasma epinephrine and urine metanephrine levels were significantly higher in the controls than in patients with CAH (p = 0.02 and p < 0.001, respectively). Plasma norepinephrine levels were significantly lower in the controls than in patients with CAH (p < 0.001). Interestingly, patients with the salt-wasting form had lower norepinephrine levels in comparison to the other subgroups of CAH. Conclusion: Despite the fact that CAH patients have insufficient epinephrine secretion, these patients have the ability to increase compensatory norepinephrine. However, this increase is much lower in patients with the salt-wasting form. These findings need to be confirmed by other studies. Copyright (C) 2009 S. Karger AG, Base

A new screening tool for self-neglect in community-dwelling older adults: IMSelf-neglect questionnaire

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Default Mode Network Connectivity Is Linked to Cognitive Functioning and Csf A Beta(1-42) Levels In Alzheimer'S Disease

Background: Changes in the default mode network (DMN) activity are early features of Alzheimer's disease (AD) and may be linked to AD-specific A beta pathology. Methods: Cognitive profiles; DMN connectivity alterations; and cerebrospinal fluid (CSF) amyloid beta (A beta)(1-42), total tau, phosphorylated tau 181, and alpha-synuclein levels were studied in 21 patients with AD and 10 controls. Results: DMN activity is altered in AD. Posterior cingulate cortex (PCC) functional connectivity with other parts of DMN was related to cognitive function scores. The reduction of connectivity of the dorsal PCC with the retrosplenial cortex on the right side was closely related to decreased CSF A beta(1-42) levels in patients with AD. Conclusions: The dorsal PCC and retrosplenial cortex may have special importance in the pathogenesis and cognitive findings of AD. (C) 2015 Elsevier Ireland Ltd. All rights reserved.Wo

Successful Results of Pamidronate Treatment in Children With Osteogenesis Imperfecta With Emphasis on the Interpretation of Bone Mineral Density for Local Standards

Aim: To assess the long-term effect of pamidronate therapy on bone mineral metabolism and bone mineral density (BMD) in children with osteogenesis imperfecta (OI) and to evaluate BMD results with respect to national standards

Indications, effectiveness and safety of percutaneous endoscopic gastrostomy: A single center experience and literature review.

Background and Objectives: Percutaneous endoscopic gastrostomy (PEG) has been widely used since 1980 in enteral feeding of patients that are not able to be fed orally for a long time. The aim of this study is to evaluate the PEG indications, effectiveness and PEG related complications from a single center in Istanbul, Turkey. Methods and Study Design: 265 patients with PEG who were followed up by the clinical nutrition team of a university hospital between 2010-2018 were evaluated retrospectively. Nutritional Risk Screening-2002 (NRS-2002) test, anthropometric measurements, bioelectrical impedance analysis and laboratory data were used to evaluate the patients' nutritional status. Results: The most common indications for PEG were dementia (35.1%), amyotrophic lateral sclerosis (22.6%), stroke (15.8%), and cancer (14%). The mean body weight of the patients was increased after PEG (63.5 +/- 12.2 vs 62.0 +/- 12.7 kg). Mid upper arm circumference and calf circumference of the patients increased after PEG (27.5 +/- 2.5 vs 25.4 +/- 3.1 cm and 32.2 +/- 7.9 vs 29.6 +/- 5.9 cm, respectively). Serum albumin of the patients was increased significantly after PEG (3.34 +/- 0.69 g/dL to 3.64 +/- 0.65 g/dL) without any significant change in serum CRP. Subgroup analyses showed a significant increase in the mean serum albumin of patients with dementia after PEG (3.23 +/- 0.67 g/dL to 3.54 +/- 0.58 g/dL). Local insertion site infection occurred in 15 patients (5.6%) and only 3 patients had systemic inflammatory symptoms after local infection (1.1%). Conclusions: The results of our study showed that long-term enteral feeding with PEG is an effective and safe method that provides improvement in nutritional status