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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/28643460/periodic-limb-movement-in-sleep-and-sickle-cell-disease-a-neglected-association
#2
Ana C Cabañas-Pedro, Suely Roizenblatt, Altay A L de Souza, Sérgio Tufik, Maria S Figueiredo
High frequency of periodic limb movements in sleep (PLMS) has been described among children with sickle cell disease (SCD), but there is little information about PLMS among adults with SCD. We aim to determine the frequency of PLMS among adults with SCD and to identify possible associations with iron status and haemolytic parameters. We analysed polysomnography on 99 adults: 74 with sickle cell anaemia (HbSS), 19 with HbSC (double heterozygosis HbS and HbC) and 6 with HbS-beta thalassaemia. Laboratory data were collected close to the time of the polysomnography examination...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643430/platinum-containing-regimens-for-metastatic-breast-cancer
#3
REVIEW
Sam J Egger, Melina L Willson, Jenna Morgan, Harriet S Walker, Sue Carrick, Davina Ghersi, Nicholas Wilcken
BACKGROUND: Studies have reported high tumour response rates for platinum-containing regimens in the treatment of women with metastatic breast cancer. Most of these studies were conducted prior to the 'intrinsic subtype' era, and did not specifically focus on metastatic triple-negative breast cancers (mTNBCs). OBJECTIVES: To identify and review the evidence from randomised trials comparing platinum-containing chemotherapy regimens with regimens not containing platinum in the management of women with metastatic breast cancer...
June 23, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28643364/development-of-clinical-paroxysmal-nocturnal-haemoglobinuria-in-children-with-aplastic-anaemia
#4
Atsushi Narita, Hideki Muramatsu, Yusuke Okuno, Yuko Sekiya, Kyogo Suzuki, Motoharu Hamada, Shinsuke Kataoka, Daisuke Ichikawa, Rieko Taniguchi, Norihiro Murakami, Daiei Kojima, Eri Nishikawa, Nozomu Kawashima, Nobuhiro Nishio, Asahito Hama, Yoshiyuki Takahashi, Seiji Kojima
The clinical significance of paroxysmal nocturnal haemoglobinuria (PNH) in children with aplastic anaemia (AA) remains unclear. We retrospectively studied 57 children with AA between 1992 and 2010. During the follow-up, five patients developed clinical PNH, in whom somatic PIGA mutations were detected by targeted sequencing. The 10-year probability of clinical PNH development was 10·2% (95% confidence interval, 3·6-20·7%). Furthermore, the detection of minor PNH clones by flow cytometry at AA diagnosis was a risk factor for the subsequent development of clinical PNH...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28641647/-clinical-significance-of-serum-thrombopoietin-levels-in-patients-with-aplastic-anaemia
#5
Hai Cheng, Jiang Cao, Wei Chen, Kun-Ming Qi, Zhi-Ling Yan, Kai-Lin Xu
OBJECTIVE: To evaluate the clinical significance of thrombopoietin (TPO) level in diagnosis of pateints with aplastic anaemia (AA). METHODS: The TPO levels in sera from 54 AA patients and 119 healthy controls were examined. A total of 92 samples were collected from AA patients including 43 samples harvested at diagnosis, 23 samples in the cytopenic period after treatment, and 26 samples of patients in partial (n=10) or complete remission (n=16) following immunosuppressive treatment...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641483/good-performance-of-platinum-based-chemotherapy-for-high-grade-gastroenteropancreatic-and-unknown-primary-neuroendocrine-neoplasms
#6
Giovanni Brandi, Marco Paragona, Davide Campana, Nicole Brighi, Arrigo Bondi, Maria Abbondanza Pantaleo, Jody Corbelli, Maria Aurelia Barbera, Guido Biasco
To evaluate efficacy and safety of platinum and etoposide combination in the treatment of advanced gastroenteropancreatic (GEP) and unknown primary (CUP) neuroendocrine carcinomas (NEC), we analysed the records of 21 consecutive patients treated with this regimen from 1999 to 2012. Objective responses were obtained in 11 patients (52%) and disease stability (DS) in 5 (24%). Median progression-free survival (PFS) was 7 months (95% CI, 5.33-8.66). Median overall survival (OS) was 16 months (95% CI, 14.97-17...
June 22, 2017: Journal of Chemotherapy
https://www.readbyqxmd.com/read/28638625/early-eculizumab-use-in-atypical-haemolytic-uraemic-syndrome-in-a-jehovah-s-witness-refusing-blood-products
#7
May Al-Shaghana, Andrew Bentall, Mark D Jesky, William Lester, Graham Lipkin
Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah's Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28637764/roth-s-spot-in-megaloblastic-anaemia
#8
Deba Prasad Dhibar, Kamal Kant Sahu, Jitender Jinagal, Sanjay Jain, Subhash Chander Varma
No abstract text is available yet for this article.
June 21, 2017: Postgraduate Medical Journal
https://www.readbyqxmd.com/read/28636166/could-intradialytic-nutrition-improve-refractory-anaemia-in-patients-undergoing-haemodialysis
#9
Ahmad F Thabet, Sawsan M Moeen, Mohammed O Labiqe, Medhat A Saleh
BACKGROUND: This prospective randomised study was designed to evaluate the efficacy of intradialytic parenteral nutrition (IDPN) therapy in malnourished patients with refractory anaemia. METHODS: Forty patients who were malnourished with a BMI not greater than 23 (17-23) kg/m(2) , undergoing regular HD were included. Of those, 20 patients received 500-1000 ml of IDPN at a rate of 250-300 ml/h at each HD session three days per week for six consecutive months...
June 21, 2017: Journal of Renal Care
https://www.readbyqxmd.com/read/28635634/despite-inflammation-supplemented-essential-amino-acids-may-improve-circulating-levels-of-albumin-and-haemoglobin-in-patients-after-hip-fractures
#10
Roberto Aquilani, Ginetto Carlo Zuccarelli, Anna Maria Condino, Michele Catani, Carla Rutili, Consiglia Del Vecchio, Pietro Pisano, Manuela Verri, Paolo Iadarola, Simona Viglio, Federica Boschi
Essential amino acids (EAAs) are nutritional substrates that promote body protein synthesis; thus we hypothesised that their supplementation may improve circulating albumin (Alb) and haemoglobin (Hb) in rehabilitative elderly patients following hip fractures (HF). Out of the 145 HF patients originally enrolled in our study, 112 completed the protocol. These subjects were divided into two randomised groups, each containing 56 patients. For a period of two months, one group (age 81.4 ± 8.1 years; male/female 27/29) received a placebo, and the other (age 83...
June 21, 2017: Nutrients
https://www.readbyqxmd.com/read/28632072/maternal-obesity-obstetric-interventions-and-post-partum-anaemia-increase-the-risk-of-post-partum-sepsis-a-population-based-cohort-study-based-on-swedish-medical-health-registers
#11
Daniel Axelsson, Marie Blomberg
BACKGROUND: The objective was to estimate whether maternal obesity and/or obstetric interventions are associated with diagnosed maternal post-partum sepsis. METHODS: A retrospective observational cohort study including all deliveries in Sweden between 1997 and 2012 (N = 1,558,752). Cases of sepsis (n = 376) were identified by International Classification of Diseases, (ICD-10) codes A40, A41 and O 85 in the Medical Birth Register and the National Patient Register...
June 20, 2017: Infectious Diseases
https://www.readbyqxmd.com/read/28630222/acute-pneumatosis-cystoides-intestinalis-with-atrophic-desmosis-of-the-colon-in-a-child
#12
Blanca Schuster, Johannes Mayr
Acute pneumatosis cystoides intestinalis (PCI) has been described after bone marrow transplantation (BMT). Several case series have demonstrated successful conservative treatment of PCI in children. We present a child with Fanconi anaemia, who developed severe graft versus host disease of the gastrointestinal tract, skin and liver after BMT and an acute, severe form of PCI. Our case report illustrates the complexity of diagnostic and therapeutic procedures in PCI in immunocompromised children.
June 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28630218/mistaken-identity-haemoglobinuria-secondary-to-paravalvular-leak-masking-as-haematuria
#13
Sam Meadows, Patrick Gordon, Richard Inman
Haemolytic anaemia caused by a paravalvular leak presenting as progressively worsening red urine. Haemoglobinuria was easily mistaken for gross haematuria, resulting in extensive invasive urological investigation that proved to be futile. Further investigation following an emergency admission led to the realisation that intravascular haemolysis secondary to a paravalvular leakâ€"presenting 43 years following metallic valve insertionâ€"was the cause of discoloured urine and newly presenting symptomatic anaemia...
June 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#14
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28627939/off-label-prescribing-for-children-with-chronic-diseases-in-nigeria-findings-and-implications
#15
Kazeem Adeola Oshikoya, Ibrahim Adekunle Oreagba, Brian Godman, Joseph Fadare, Samuel Orubu, Amos Massele, Idowu Odunayo Senbanjo
BACKGROUND AND AIMS: Prescribing medicines in an off-label manner for children with chronic conditions is sparsely documented, even more so among developing countries. This needs addressing. The objective was to investigate the extent of this prescribing among children with epilepsy, asthma, and sickle cell anaemia in Nigeria. METHODS: Prescriptions for children ≤16 years documented in their case files that attended paediatric clinics in Lagos, Nigeria, for these three conditions between January and October 2015, were reviewed retrospectively to extract data on the medicines prescribed...
June 19, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28625299/managing-major-obstetric-haemorrhage-pharmacotherapy-and-transfusion
#16
REVIEW
Rachel Collis, Emilia Guasch
Major obstetric haemorrhage is a leading cause of maternal mortality. A prescriptive approach to early recognition and management is critical to improving outcomes. Uterine atony is the primary cause of post-partum haemorrhage. First-line prevention and treatment include the administration of uterine tonic agents; other conservative measures include uterine cavity tamponade and uterine compression sutures. Interventional radiology procedures have been used for both prophylaxis and treatment, but a hysterectomy may be necessary if conservative measures fail...
March 2017: Best Practice & Research. Clinical Anaesthesiology
https://www.readbyqxmd.com/read/28625177/iron-deficiency-without-anaemia-is-a-potential-cause-of-fatigue-meta-analyses-of-randomised-controlled-trials-and-cross-sectional-studies
#17
Katsuhiko Yokoi, Aki Konomi
Fe deficiency is a prevalent nutritional disease, and fatigue is a common complaint in the general and patient population. The association between Fe deficiency without anaemia (IDNA) and fatigue is unclear. Here, we performed a meta-analysis to evaluate the therapeutic effect of Fe on fatigue in patients with IDNA and the association between IDNA and fatigue in the population. Articles from the PubMed database up to 19 January 2016 were systematically searched. A total of six relevant randomised controlled trials (RCT) and six relevant cross-sectional studies were identified...
June 19, 2017: British Journal of Nutrition
https://www.readbyqxmd.com/read/28620089/development-and-validation-of-risk-prediction-equations-to-estimate-survival-in-patients-with-colorectal-cancer-cohort-study
#18
Julia Hippisley-Cox, Carol Coupland
Objective To develop and externally validate risk prediction equations to estimate absolute and conditional survival in patients with colorectal cancer. Design Cohort study.Setting General practices in England providing data for the QResearch database linked to the national cancer registry.Participants 44 145 patients aged 15-99 with colorectal cancer from 947 practices to derive the equations. The equations were validated in 15 214 patients with colorectal cancer from 305 different QResearch practices and 437 821 patients with colorectal cancer from the national cancer registry...
June 15, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28619120/g6pd-deficiency-alleles-in-a-malaria-endemic-region-in-the-western-brazilian-amazon
#19
Jamille G Dombrowski, Rodrigo M Souza, Jonathan Curry, Laura Hinton, Natercia R M Silva, Lynn Grignard, Ligia A Gonçalves, Ana Rita Gomes, Sabrina Epiphanio, Chris Drakeley, Jim Huggett, Taane G Clark, Susana Campino, Claudio R F Marinho
BACKGROUND: Plasmodium vivax parasites are the predominant cause of malaria infections in the Brazilian Amazon. Infected individuals are treated with primaquine, which can induce haemolytic anaemia in glucose-6-phosphate dehydrogenase (G6PD)-deficient individuals and may lead to severe and fatal complications. This X-linked disorder is distributed globally and is caused by allelic variants with a geographical distribution that closely reflects populations exposed historically to endemic malaria...
June 15, 2017: Malaria Journal
https://www.readbyqxmd.com/read/28618058/detection-of-iron-restriction-in-anaemic-and-non-anaemic-patients-new-diagnostic-approaches
#20
Lothar Thomas, Christian Thomas
OBJECTIVE: The aim of this study was to detect iron restriction in non-anaemic patients and iron-restricted erythropoiesis (IRE) in patients with anaemia. METHOD: Haematologic indices and biochemical markers of iron deficiency (ID) were determined using the clinically accepted cut-off level for serum ferritin of ≤30 μg/l as reference of ID. To evaluate the prevalence of iron restriction and IRE in patients with higher ferritin levels we used the thresholds of the markers of ID as reference...
June 15, 2017: European Journal of Haematology
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