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https://www.readbyqxmd.com/read/28643569/iron-overload-directly-affecting-the-ovaries-in-a-patient-with-diamond-blackfan-anaemia-a-case-report
#1
Mariano Mascarenhas, Victoria Rawnsley, Adam Balen
Iron is a 'one-way' element and the primary point of regulation of body iron stores is at the level of intestinal iron absorption. Repeated blood transfusions for congenital anaemias bypass this regulatory checkpoint and inevitably lead to iron overload in the long-term. Iron overload causes multi-organ dysfunction of the heart, liver, pancreas and joints. It also causes reproductive toxicity primarily through its damaging effect on the anterior pituitary leading to hypogonadotrophic hypogonadism. Another less understood mechanism of reproductive toxicity is direct gonadal damage of excess free iron...
June 23, 2017: Human Fertility: Journal of the British Fertility Society
https://www.readbyqxmd.com/read/28643025/effects-of-bariatric-surgery-on-pelvic-floor-disorders-in-obese-women-a-meta-analysis
#2
REVIEW
Wenqing Lian, Yiqun Zheng, Hongxing Huang, Lianghua Chen, Bin Cao
PURPOSE: Obesity is an established risk factor for pelvic floor disorders (PFD) but the effects of bariatric surgery on PFD are uncertain. This meta-analysis was conducted to evaluate the effects of bariatric surgery on PFD in obese women. METHODS: A systematic search of PubMed, Cochrane Library, CNKI and CBM databases up to October 2016 was performed, and studies reporting pre-operative and post-operative outcomes in obese women undergoing bariatric surgery were included...
June 22, 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28637104/-treatment-options-in-non-alcoholic-fatty-liver-disease
#3
REVIEW
Won Kim
The prevalence of non-alcoholic fatty liver disease (NAFLD) has sharply increased over the past several decades in Korea. In most cases of NAFLD, metabolic stress and cellular apoptosis are often driven by metabolic abnormality, eventually leading to inflammation and fibrosis . Along with a dramatic surge in the obesity epidemic, 10-20% of NAFLD patients ultimately progress to non-alcoholic steatohepatitis (NASH), a precursor to cirrhosis and hepatocellular carcinoma, as well as multi-organ systemic diseases...
June 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28620491/pseudo-thrombotic-thrombocytopenic-purpura-presenting-as-multi-organ-dysfunction-syndrome-a-rare-complication-of-pernicious-anemia
#4
Saroj Kandel, Nibash Budhathoki, Shanta Pandey, Bikash Bhattarai, Aam Baqui, Ramesh Pandey, Divya Salhan, Danilo Enriquez, Joseph Quist, Frances M Schmidt
OBJECTIVE: We present a rare case of pernicious anemia presented as multi-organ dysfunction syndrome, later found to have pseudo-thrombotic thrombocytopenic purpura. METHODS: An 86-year-old female presented with respiratory distress, altered mental status, acute renal failure and was intubated in emergency room. She was found to have severe anemia, thrombocytopenia, high lactate, high lactate dehydrogenase and low haptoglobin. Peripheral smear revealed multilobulated neutrophils with schistocytes, poikilocytes and anisocytes...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28610912/characteristics-of-cardiomyopathy-in-alstr%C3%A3-m-syndrome-prospective-single-center-data-on-38-patients
#5
Alessandra Brofferio, Vandana Sachdev, Hwaida Hannoush, Jan D Marshall, Jürgen K Naggert, Stanislav Sidenko, Anna Noreuil, Arlene Sirajuddin, Joy Bryant, Joan C Han, Andrew E Arai, William A Gahl, Meral Gunay-Aygun
BACKGROUND: Alström syndrome (AS) is a rare monogenetic disorder with multi-organ involvement. Complex metabolic disturbances are common and cardiomyopathy is a well-recognized feature in infants as well as in older children and adults. Although the mechanism of cardiomyopathy is not known, previous reports suggest that individuals with infantile-onset cardiac disease recover completely. METHODS: In this single center prospective series of 38 children and adults (age range 1...
May 30, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28586613/hyperlactetemia-and-its-trends-in-critically-ill-children-admitted-in-pediatric-intensive-care-unit-of-a-developing-country
#6
Qalab Abbas, Muhammad Tariq Jamil, Leena Jafri, Anwar Ul Haque, Vivek Khetpal
OBJECTIVE: There is increasing evidence that in setting of critical ailments clinical signs lag biomarkers like Lactate and hyperlactetemia can be the only marker for this disorder. This study was conducted to describe the incidence of hyperlactatemia in critically ill children and its association with outcome. METHODS: Retrospective review of medical records of all children who had their lactic acid (LA) levels measured during their admission in PICU from January 2014 to December 2015 was done...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28582861/post-translational-oxidative-modifications-of-mitochondrial-complex-i-nadh-ubiquinone-oxidoreductase-implications-for-pathogenesis-and-therapeutics-in-human-diseases
#7
M M Srinivas Bharath
Mitochondrial complex I (NADH: ubiquinone oxidoreductase; CI) is central to the electron transfer chain (ETC), oxidative phosphorylation, and ATP production in eukaryotes. CI is a multi-subunit complex with a complicated yet organized structure that optimally connects electron transfer with proton translocation and forms higher-order supercomplexes with other ETC complexes. Efforts to understand the molecular genetics, expression profile of subunits, and structure-function relationship of CI have increased over the years due to the direct role of the complex in human diseases...
May 30, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28574114/venlafaxine-intoxication-with-development-of-takotsubo-cardiomyopathy-successful-use-of-extracorporeal-life-support-intravenous-lipid-emulsion-and-cytosorb%C3%A2
#8
Ines Schroeder, Michael Zoller, Matthias Angstwurm, Felix Kur, Lorenz Frey
We describe a young patient who ingested 18 g (240 times the daily therapeutic dose) of venlafaxine in a suicide attempt. She developed severe cardiomyopathy in a takotsubo distribution causing cardiogenic shock and multi-organ dysfunction syndrome (MODS). She was successfully treated with intravenous lipid emulsion (ILE), extracorporeal life support (ECLS) and CytoSorb®. This is remarkable as, to the best of the authors' knowledge, this is the highest amount of venlafaxine intake seen in the literature with a nonfatal outcome...
May 23, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28562132/burden-of-illness-associated-with-sinusoidal-obstruction-syndrome-veno-occlusive-disease-in-patients-with-hematopoietic-stem-cell-transplantation
#9
Zhun Cao, Kathleen F Villa, Craig B Lipkin, Scott B Robinson, Bijan Nejadnik, Christopher C Dvorak
AIMS: Sinusoidal obstruction syndrome (SOS) is a life-threatening complication of hematopoietic stem cell transplantation (HSCT) associated with significant morbidity and mortality. Healthcare utilization, costs, and mortality were assessed in HSCT patients diagnosed with SOS, with and without multi-organ dysfunction (MOD). MATERIALS AND METHODS: This retrospective observational study identified real-world patients undergoing HSCT between January 1, 2009 and May 31, 2014 using the Premier Healthcare Database...
June 14, 2017: Journal of Medical Economics
https://www.readbyqxmd.com/read/28554721/current-and-emerging-comorbidities-in-cystic-fibrosis
#10
Nicola J Ronan, Joseph Stuart Elborn, Barry J Plant
Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed ubiquitously throughout the body. Thus, while respiratory manifestations dominate much of cystic fibrosis (CF) care, there are prominent multi-organ manifestations and comorbidities. In the general population, the number of comorbidities increases with aging. Few illnesses have experienced such a dramatic improvement in survival as CF, which has been transformed from an illness of childhood death to one of adult survival. Hence, as longevity increases in CF, it is paralleled by an increasing number of patients with multicomplex comorbidities availing of care from adult CF multi-disciplinary teams...
May 26, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28553383/hemophagocytic-lymphohistiocytosis-a-diagnostic-conundrum
#11
Brittany Grzybowski, Vijay A Vishwanath
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the most common presenting signs and symptoms of HLH, including neurologic dysfunction, and having a high clinical suspicion for HLH in the setting of inflammatory/demyelinating diseases are important for prompt diagnosis and treatment...
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28540205/clinicopathological-spectrum-of-snake-bite-induced-acute-kidney-injury-from-india
#12
Sanjay Vikrant, Ajay Jaryal, Anupam Parashar
AIM: To study the clinico-pathological spectrum of snake bite-induced acute kidney injury (AKI). METHODS: A retrospective study of patients admitted at Indira Gandhi Medical College Hospital, Shimla with snake bite-induced AKI from July 2003 to June 2016. Medical records were evaluated for patient's information on demographic, clinical characteristics, complications and outcome. Outcomes of duration of hospital stay, requirement for intensive care unit support, treatment with dialysis, survival and mortality were analyzed...
May 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28536812/maternal-ascites-an-independent-prognostic-factor-in-severe-preeclampsia-a-matched-cohort-study
#13
J Yavana Suriya, Anish Keepanasseril, K Manikandan, Dilip Kumar Maurya, P Veena, S Soundara Raghavan
BACKGROUND: Preeclampsia is a multi-systemic, multi-organ dysfunction associated with increased maternal and perinatal complications. The presence of maternal ascites, a manifestation of endothelial dysfunction and increased capillary permeability, is shown to be associated with adverse outcomes. We aim to investigate the impact of maternal ascites on pregnancy outcome in women with severe preeclampsia. METHODS: A matched cohort study was conducted in a tertiary care teaching hospital in South India between March 2014 and March 2015...
May 23, 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28525674/continuous-hemoadsorption-with-a-cytokine-adsorber-during-sepsis-a-review-of-the-literature
#14
Khosrow S Houschyar, Malcolm N Pyles, Susanne Rein, Ina Nietzschmann, Dominik Duscher, Zeshaan N Maan, Kristian Weissenberg, Hubertus M Philipps, Catharina Strauss, Beate Reichelt, Frank Siemers
Sepsis is a well-recognized healthcare issue worldwide, ultimately resulting in significant mortality, morbidity and resource utilization during and after critical illness. In its most severe form, sepsis causes multi-organ dysfunction that produces a state of critical illness characterized by severe immune dysfunction and catabolism. Sepsis induces the activation of complement factor via 3 pathways and the release of inflammatory cytokines such as tumor necrosis factor alpha (TNF-α) and interleukin-1beta (IL-1β), resulting in a systemic inflammatory response...
May 29, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28507037/systemic-activation-of-nrf2-alleviates-lethal-autoimmune-inflammation-in-scurfy-mice
#15
Takuma Suzuki, Shohei Murakami, Shyam S Biswal, Shimon Sakaguchi, Hideo Harigae, Masayuki Yamamoto, Hozumi Motohashi
The transcription factor NRF2 (nuclear factor (erythroid-derived 2)-like 2) plays crucial roles in the defense mechanisms against oxidative stress and mediates anti-inflammatory actions in various pathological conditions. Recent studies have shown that the dysfunction of regulatory T cells (Tregs) is directly linked to the initiation and progression of various autoimmune diseases. To determine the Treg-independent impact of NRF2 activation on autoimmune inflammation, we examined Scurfy (Sf) mice that are deficient in Tregs and succumb to severe multi-organ inflammation by 4 weeks of age...
May 15, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28493820/deoxyguanosine-kinase-deficiency-a-report-of-four-patients
#16
Özlem Ünal, Burcu Hişmi, Mustafa Kılıç, Hayriye Hızarcıoğlu Gülşen, Turgay Coşkun, Serap Hatice Sivri, Ali Dursun, Aysel Yüce, Ayşegül Tokatlı
BACKGROUND: Hepatic involvement is a common feature in childhood mitochondrial disorders. Deoxyguanosine kinase (DGUOK) deficiency is one of the mitochondrial DNA depletion syndromes associated with hepatocerebral syndrome. Hepatic disease and neurologic dysfunction occurs within weeks after birth. Low birth weight is one of the common features. This study aims to describe the clinical and laboratory features of four infants carrying four different pathogenic variants in the DGUOK gene...
May 24, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28474627/scrub-typhus-in-uttarakhand-adjoining-uttar-pradesh-seasonality-clinical-presentations-predictors-of-mortality
#17
Anurag Bhargava, Reshma Kaushik, Rajeev Mohan Kaushik, Anita Sharma, Sohaib Ahmad, Minakshi Dhar, Garima Mittal, Sushant Khanduri, Priyannk Pant, Rajesh Kakkar
BACKGROUND & OBJECTIVES: Scrub typhus is a re-emerging mite-borne rickettsiosis, which continues to be underdiagnosed, with lethal consequences. The present study was conducted to determine the seasonality, clinical presentation and predictors of mortality in patients with scrub typhus at a tertiary care teaching hospital in northern India. METHODS: Scrub typhus was suspected in patients attending the hospital as per the standard case definition and serological evidence was obtained by performing an IgM ELISA...
December 2016: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/28458889/an-unusual-case-of-adult-onset-multi-systemic-langerhans-cell-histiocytosis-with-perianal-and-incident-thyroid-involvement
#18
Ozen Oz Gul, Pinar Sisman, Soner Cander, Erdem Gozden, Meral Kurt, Ozlem Saraydaroglu, Turkay Kirdak, Canan Ersoy, Erdinc Erturk
SUMMARY: Langerhans cell histiocytosis (LCH) is a rare sporadic disease characterized by histiocytic neoplastic infiltration of various organ systems and a wide spectrum of clinical manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal presentation and incidental finding of subsequent thyroid gland involvement in the follow-up period. The patient with a history of perianal LCH treated with surgical excision and local radiotherapy was referred to our Endocrinology Department upon detection of hypermetabolic nodular lesions in the left lateral lobe of thyroid gland on positron emission tomography-computed tomography (PET/CT) scan in the nineth month of follow-up...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28456874/pulmonary-artery-dimensions-as-a-prognosticator-of-transplant-free-survival-in-scleroderma-interstitial-lung-disease
#19
James Benjamin Gleason, Krunal B Patel, Felix Hernandez, Anas Hadeh, Kristin B Highland, Franck Rahaghi, Jinesh P Mehta
BACKGROUND: Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD...
April 29, 2017: Lung
https://www.readbyqxmd.com/read/28452684/dynamic-interactions-of-plasmodium-spp-with-vascular-endothelium
#20
Mark R Gillrie, May Ho
Plasmodial species are protozoan parasites that infect erythrocytes. As such, they are in close contact with microvascular endothelium for most of the life cycle in the mammalian host. The host-parasite interactions of this stage of the infection are responsible for the clinical manifestations of the disease that range from a mild febrile illness to severe and frequently fatal syndromes such as cerebral malaria and multi-organ failure. Plasmodium falciparum, the causative agent of the most severe form of malaria, is particularly predisposed to modulating endothelial function through either direct adhesion to endothelial receptor molecules, or by releasing potent host and parasite products that can stimulate endothelial activation and/or disrupt barrier function...
January 2, 2017: Tissue Barriers
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