HOMOCYSTINURIA : #Anesthesia IMPLICATIONS

⏫ There is increased(⏫) levels of homocystine and methionine in descent and urine due to the lack of Cystathionine B synthetase which catalyses the employment of homocystine and serine into cystathionine

⏫ Raised cystine levels bring to poverty the resistance of endothelium against thrombosis, reduces the agility of the vasodilator nitric oxide (NO) and become greater platelet aggregation. So there is ostentatious incidence of thromboembolism. We have to render certain good hydration, good cardiac output,seasonable mobilisation and should provide mechanical +/- pharmacological thromboprophylaxis. Many patients order be on anticoagulation. If untreated 50% of patients resolution have thromboembolic complications and the destruction is about 20% before the period of 30 years.  So the one and the other modification of the dosing of anticoagulants ( especially admitting that regional anesthesia is planned) if resigned is receiving them and providing prophylaxis in anticipation of DVT are important elements of perioperative care. The incidence of thrombotic complications are greater quantity in pregnant patients. 

Blood ropiness and platelet adhesiveness can be reduced through dextran, and the prior administration of pyridoxine

⏫ Reduced cystine results in unsatisfactory collagen and fragmentation of elastic cloth of large arteries. There is done against the state incidence of vascular diseases like Cerebrovascular diseases, Coronary Artery Disease, Peripheral Vascular Diseases

⏫ Patients may regard increased insulin levels resulting in hypoglycemia. Dextrose macerating will prevent hypoglycaemia.

⏫ Acute psychiatritc symptoms, madness etc have been reported and the altered availability of homocysteine, methionine and cystiene that are having glutamate agonist properties, has been postulated while a factor which promotes this.

⏫ Regional anaesthesia has certain theoretical disadvantages. Penetration of a expanded epidural blood vessel might initiate thrombosis, during the time that may the accompanying venous stasis of the diminish limbs.

Reference: ANAESTHESIA DATABOOK, A Perioperative and Peripartum Manual, 3RD EDITION

Rosemary Mason

#unconsciousness of pain , #dvt , #biochemistry ,

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