Anaesthesia in sickle cell states.
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Biomedical subjects
Publications and source records attributed to R G Huntsman.
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The manifestations of the sickling disorders are becoming increasingly familiar to clinicians in Great Britain. One of these disorders, sickle-cell haemoglobin disease, has hitherto received little attention, being regarded as a relatively mild condition. This paper describes some of the distinctive clinical features of the disease as seen in a series of nine cases which have recently presented in London, two of which were fatal. The special hazards of the condition in relation to pregnancy, air travel, and general anaesthesia are discussed.
An automated technique is described which is capable of detecting sickle-cell haemoglobin and differentiating the sickle-cell trait from sickle-cell anaemia. The method is based upon the Itano solubility test and utilizes Technicon equipment.
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High altitude seemed to be responsible for seven recent cases of sickling crisis. People with sickle-cell trait are at risk if they fly in unpressurized aircraft, which are used for many local air services. Those with sickle-cell haemoglobin C disease should avoid air travel even in pressurized aircraft. Possibly as a result of "autosplenectomy," patients with sickle-cell anaemia seem to be able to fly in pressurized aircraft with little risk. All passengers and aircrew who might have some form of sickle-cell disease should be screened before flight.
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Victims of severe injuries frequently pass haemoglobin, myoglobin, or both proteins in their urine. If the kidneys of such persons are to be considered as donor material for transplantation, a pathology department may be requested to identify which of these pigments is present. If freshly passed urine is available, haemoglobinuria in the absence of myoglobinuria may be rapidly identified by spectroscopy. However, the rapid degradation of myoglobin to the met-myoglobin form will make spectroscopic recognition of this pigment in the urine unreliable. In the absence of variant haemoglobins, myoglobin may be easily distinguished from normal haemoglobin by routine electrophoresis on paper, starch gel, or cellulose acetate at alkaline pH. The electrophoretic method of choice in the presence of variant haemoglobins utilizes polyacrylamide gel at 12 g/100 ml as a supporting medium. At this concentration, the migration both of haemoglobin and its variants is sufficiently retarded to allow the easy recognition of myoglobin.
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