Mar 252014
 
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Myelodysplastic syndromes.

Lancet. 2014 Mar 20;

Authors: Adès L, Itzykson R, Fenaux P

Abstract
Myelodysplastic syndromes are clonal marrow stem-cell disorders, characterised by ineffective haemopoiesis leading to blood cytopenias, and by progression to acute myeloid leukaemia in a third of patients. 15% of cases occur after chemotherapy or radiotherapy for a previous cancer; the syndromes are most common in elderly people. The pathophysiology involves cytogenetic changes with or without gene mutations and widespread gene hypermethylation at advanced stages. Clinical manifestations result from cytopenias (anaemia, infection, and bleeding). Diagnosis is based on examination of blood and bone marrow showing blood cytopenias and hypercellular marrow with dysplasia, with or without excess of blasts. Prognosis depends largely on the marrow blast percentage, number and extent of cytopenias, and cytogenetic abnormalities. Treatment of patients with lower-risk myelodysplastic syndromes, especially for anaemia, includes growth factors, lenalidomide, and transfusions. Treatment of higher-risk patients is with hypomethylating agents and, whenever possible, allogeneic stem-cell transplantation.

PMID: 24656536 [PubMed - as supplied by publisher]

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Mar 132014
 
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Factitious disorders and malingering: challenges for clinical assessment and management.

Lancet. 2014 Mar 5;

Authors: Bass C, Halligan P

Abstract
Compared with other psychiatric disorders, diagnosis of factitious disorders is rare, with identification largely dependent on the systematic collection of relevant information, including a detailed chronology and scrutiny of the patient's medical record. Management of such disorders ideally requires a team-based approach and close involvement of the primary care doctor. As deception is a key defining component of factitious disorders, diagnosis has important implications for young children, particularly when identified in women and health-care workers. Malingering is considered to be rare in clinical practice, whereas simulation of symptoms, motivated by financial rewards, is regarded as more common in medicolegal settings. Although psychometric investigations (eg, symptom validity testing) can inform the detection of illness deception, such tests need support from converging evidence sources, including detailed interview assessments, medical notes, and relevant non-medical investigations. A key challenge in any discussion of abnormal health-care-seeking behaviour is the extent to which a person's reported symptoms are considered to be a product of choice, or psychopathology beyond volitional control, or perhaps both. Clinical skills alone are not typically sufficient for diagnosis or to detect malingering. Medical education needs to provide doctors with the conceptual, developmental, and management frameworks to understand and deal with patients whose symptoms appear to be simulated. Central to the understanding of factitious disorders and malingering are the explanatory models and beliefs used to provide meaning for both patients and doctors. Future progress in management will benefit from an increased appreciation of the contribution of non-medical factors and a greater awareness of the conceptual and clinical findings from social neuroscience, occupational health, and clinical psychology.

PMID: 24612861 [PubMed - as supplied by publisher]

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Feb 082014
 
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Adrenal insufficiency.

Lancet. 2014 Feb 3;

Authors: Charmandari E, Nicolaides NC, Chrousos GP

Abstract
Adrenal insufficiency is the clinical manifestation of deficient production or action of glucocorticoids, with or without deficiency also in mineralocorticoids and adrenal androgens. It is a life-threatening disorder that can result from primary adrenal failure or secondary adrenal disease due to impairment of the hypothalamic-pituitary axis. Prompt diagnosis and management are essential. The clinical manifestations of primary adrenal insufficiency result from deficiency of all adrenocortical hormones, but they can also include signs of other concurrent autoimmune conditions. In secondary or tertiary adrenal insufficiency, the clinical picture results from glucocorticoid deficiency only, but manifestations of the primary pathological disorder can also be present. The diagnostic investigation, although well established, can be challenging, especially in patients with secondary or tertiary adrenal insufficiency. We summarise knowledge at this time on the epidemiology, causal mechanisms, pathophysiology, clinical manifestations, diagnosis, and management of this disorder.

PMID: 24503135 [PubMed - as supplied by publisher]

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Feb 012014
 

Liver cirrhosis.

Lancet. 2014 Jan 27;

Authors: Tsochatzis EA, Bosch J, Burroughs AK

Abstract
Cirrhosis is an increasing cause of morbidity and mortality in more developed countries, being the 14th most common cause of death worldwide but fourth in central Europe. Increasingly, cirrhosis has been seen to be not a single disease entity, but one that can be subclassified into distinct clinical prognostic stages, with 1-year mortality ranging from 1% to 57% depending on the stage. We review the current understanding of cirrhosis as a dynamic process and outline current therapeutic options for prevention and treatment of complications of cirrhosis, on the basis of the subclassification in clinical stages. The new concept in management of patients with cirrhosis should be prevention and early intervention to stabilise disease progression and to avoid or delay clinical decompensation and the need for liver transplantation. The challenge in the 21st century is to prevent the need for liver transplantation in as many patients with cirrhosis as possible.

PMID: 24480518 [PubMed - as supplied by publisher]

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Dec 032013
 
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Reduction of adverse effects from intravenous acetylcysteine treatment for paracetamol poisoning: a randomised controlled trial.

Lancet. 2013 Nov 27;

Authors: Bateman DN, Dear JW, Thanacoody HK, Thomas SH, Eddleston M, Sandilands EA, Coyle J, Cooper JG, Rodriguez A, Butcher I, Lewis SC, Vliegenthart AD, Veiraiah A, Webb DJ, Gray A

Abstract
BACKGROUND: Paracetamol poisoning is common worldwide. It is treated with intravenous acetylcysteine, but the standard regimen is complex and associated with frequent adverse effects related to concentration, which can cause treatment interruption. We aimed to ascertain whether adverse effects could be reduced with either a shorter modified acetylcysteine schedule, antiemetic pretreatment, or both.
METHODS: We undertook a double-blind, randomised factorial study at three UK hospitals, between Sept 6, 2010, and Dec 31, 2012. We randomly allocated patients with acute paracetamol overdose to either the standard intravenous acetylcysteine regimen (duration 20·25 h) or a shorter (12 h) modified protocol, with or without intravenous ondansetron pretreatment (4 mg). Masking was achieved by infusion of 5% dextrose (during acetylcysteine delivery) or saline (for antiemetic pretreatment). Randomisation was done via the internet and included a minimisation procedure by prognostic factors. The primary outcome was absence of vomiting, retching, or need for rescue antiemetic treatment at 2 h. Prespecified secondary outcomes included a greater than 50% increase in alanine aminotransferase activity over the admission value. Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov (identifier NCT01050270).
FINDINGS: Of 222 patients who underwent randomisation, 217 were assessable 2 h after the start of acetylcysteine treatment. Vomiting, retching, or need for rescue antiemetic treatment at 2 h was reported in 39 of 108 patients assigned to the shorter modified protocol compared with 71 of 109 allocated to the standard acetylcysteine regimen (adjusted odds ratio 0·26, 97·5% CI 0·13-0·52; p<0·0001), and in 45 of 109 patients who received ondansetron compared with 65 of 108 allocated placebo (0·41, 0·20-0·80; p=0·003). Severe anaphylactoid reactions were recorded in five patients assigned to the shorter modified acetylcysteine regimen versus 31 who were allocated to the standard protocol (adjusted common odds ratio 0·23, 97·5% CI 0·12-0·43; p<0·0001). The proportion of patients with a 50% increase in alanine aminotransferase activity did not differ between the standard (9/110) and shorter modified (13/112) regimens (adjusted odds ratio 0·60, 97·5% CI 0·20-1·83); however, the proportion was higher with ondansetron (16/111) than with placebo (6/111; 3·30, 1·01-10·72; p=0·024).
INTERPRETATION: In patients with paracetamol poisoning, a 12 h modified acetylcysteine regimen resulted in less vomiting, fewer anaphylactoid reactions, and reduced need for treatment interruption. This study was not powered to detect non-inferiority of the shorter protocol versus the standard approach; therefore, further research is needed to confirm the efficacy of the 12 h modified acetylcysteine regimen.
FUNDING: Chief Scientist Office of the Scottish Government.

PMID: 24290406 [PubMed - as supplied by publisher]

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Dec 032013
 
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Adult haemophagocytic syndrome.

Lancet. 2013 Nov 26;

Authors: Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X

Abstract
Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage-mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder.

PMID: 24290661 [PubMed - as supplied by publisher]

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