Under normal circumstances, two α-globin chains are combined with two β-chains (designated α 2β 2) to form HbA, which is the most common type in the adult. All of them have a tetrameric structure, with two differing pairs of polypeptide chains (globins) joined to an iron-containing molecule (haem). Pathophysiologyĭifferent types of haemoglobin are produced by humans to cover the oxygen demands of pre- and postnatal life. In total, 72 articles were included in the review. We also used articles that provide information on genetics, clinical features, and the diagnostic and therapeutic approach to the disorder.
#Thalassemia minor series
As randomised prospective data are lacking, the articles we considered were mostly case reports or series and retrospective studies.
#Thalassemia minor manual
One further study was found by manual searching of the references in the electronically identified articles. Consequently, 22 articles were found suitable for inclusion in the review. Articles in languages other than English were used, provided they had a detailed English abstract. After removing those found in duplicate, 96 articles remained, 26 of which were found to be relevant.
#Thalassemia minor free
We conducted a PubMed ®, EMBASE and COHRANE LIBRARY literature search for all types of published articles up to May 2013 combining the free text and MeSH thesaurus terms: ‘Cooley's anaemia’, ‘thalassaemia’, ‘thalasaemia alpha’, ‘thalassaemia beta’ and ‘anaesthesia’ or ‘perioperative care’ in all possible combinations. Nowadays, thalassaemic patients are more likely to survive to adulthood and therefore present for surgery, and anaesthetists should be familiar with the disease to provide safe anaesthesia and peri-operative care. Moreover, advances in medical treatment, especially early and regular blood transfusion and iron chelation, have dramatically increased the survival of these patients. However, due to immigration and travelling, thalassaemia has become a disease of international interest. Cooley and Lee first reported the disorder in 1925 in children from Italy presenting with splenomegaly and bone deformities 2. Its etymology reveals the geographical association of the early reported cases with regions around the Mediterranean Sea 1. The term ‘thalassaemia’ originates from the Greek words ‘θάλασσα, thalassa’ and ‘αίμα, haema’ which mean ‘sea’ and ‘blood’, respectively. Thalassaemias comprise a heterogeneous group of inherited blood disorders characterised by defective synthesis of haemoglobin. A multidisciplinary pre-operative approach, clinical optimisation and a carefully planned strategy are mandatory. Neuraxial techniques may also be challenging due to spinal skeletal abnormalities and extramedullary haemopoiesis. Cardiovascular depression due to negative inotropic and vasodilating effects of general anaesthesia should be minimised.
When general anaesthesia is administered, the risk of difficult intubation due to oro-facial malformation should be considered. Thromboembolic events due to hypercoagulability should be prevented by simple measures, such as graduated compression stockings, intermittent pneumatic compression and early mobilisation, and possibly anticoagulant drugs. Susceptibility to infections should be managed with broad-spectrum antibiotics. Haemoglobin level should be optimised pre-operatively and maintained at adequate levels with transfusion and blood-saving strategies. Restrictive lung dysfunction is commonly encountered, especially in patients with splenomegaly. Patients may develop cardiomyopathy, pulmonary hypertension or heart failure requiring pre-operative echocardiography or cardiac catheterisation. In thalassaemic patients, multiple organ systems may be affected by the disease, blood transfusion, iron overload and chelating therapy.
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