Lidocaine – It is used to reduce the sympathetic response in those who have suspected raised intracranial pressure (ICP) or those who received succinylcholine which also causes increase ICP or those with underlying asthma that have bronchospasm. Administration of lidocaine can causes reduction in mean arterial pressure (MAP). The dosage is 1.5 mg/kg. This drug is metabolised by liver. The side effects are: hypotension, arrythmia (irregular heart beat). Lidocaine can further interact with other drugs such as amiodarone and monoamine oxidase inhibitor to cause hypotension, and dronedarone to cause arrhythmia. Induction agents
Administration of induction agents followed by neuromuscular blockade agents helps to achieve optimal conditions for intubation. Etomidate – It is an imidazole-derivative that stimulates GABA receptors. The dosage is between 0.2 and 0.6 mg/kg (commonly 20 to 50 mg doses). Dose reduction may be required in those with hypotension. Etomidate has minimal cardiovascular side effects, reduces intracerebral pressure (by reducing cerebral blood flow), and does not cause histamine release. It has quick onset of action, short duration of action, and undergoes hepatic elimination. Myoclonus, pain at the site of the injection, post-operative nausea and vomiting are common. It can also suppresses the production of cortisol and aldosterone. Ketamine – It is highly lipophilic and crosses the blood-brain barrier. It inhibits the binding of glutamine to N-Methyl-D-aspartic acid (NMDA) receptors in Thalamocortical radiations and limbic system, causing amnesia. Through the same blockade of NMDA receptor, ketamine is also effective as a painkiller.
While cardiomyopathy can be life-threatening, it is commonly resolved or substantially improved in Barth Syndrome patients after puberty.Neutropenia, a granulocyte disorder that results in a low production of neutrophils, the bodys primary defenders against bacterial infections, is another severe manifestation of Barth Syndrome. In general, lower levels of neutrophils render a patient more vulnerable to bacterial infections; in Barth Syndrome patients, however, there are reports of relatively fewer bacterial infections as compared to non-Barth patients with neutropenia. Cause
The tafazzin gene (TAZ, also called G4.5 or NG_009634) is highly expressed in cardiac and skeletal muscle; its gene product, Taz1p, functions as an acyltransferase in complex lipid metabolism. Any type of mutation of TAZ (missense, nonsense, deletion, frameshift, and/or splicing) is closely associated with Barth syndrome.In 2008, Dr. Kulik found that every patient with Barth Syndrome that he tested had abnormalities in their cardiolipin, a lipid found inside the mitochondria of cells. Cardiolipin is intimately connected with the electron transport chain proteins and the membrane structure of the mitochondrion, the energy-producing organelle of the cell. iPLA2-VIA has been suggested as a target for treatment.The human tafazzin gene is over 10,000 base pairs in length, the full-length mRNA, NM_000116, being 1919 nucleotides long, encoding 11 exons with a predicted protein length of 292 amino acids and a molecular weight of 33.5 kDa. It is located at Xq28; the long arm of the X chromosome. This explains the X-linked nature of Barth Syndrome. There are some case reports of women who are asymptomatic carriers of the TAZ mutation.