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Numerous clinical studies on the disease were made in Puerto Rico. There are eight classic forms of disorder, from a genetic mutation that gave rise to the disease. However, the general prognosis is even worse. The disease can cause functional disorders of the lungs, intestines, kidneys or the heart. The main complication most forms of disorder is pulmonary fibrosis, 40-50 years that age usually from the patient. It is a serious complication seen in many forms of HPS and the cause of death by disease. Syndrome Hermansky-Pudlak syndrome is autosomal recessive hereditary are. The defective gene, called HSP, responsible for the disease is located on the long arm of chromosome 10 (10q 2). Some research has shown that an abnormality of Lysosomal function for the development of the disease may be responsible for. HPS1, AP3B1, HPS3, HPS4, HPS5, HPS6, DTNBP1 and BLOC1S3 are associated with Hermansky-Pudlak syndrome syndrome. In autosomal recessive diseases, the State does not appear unless a person inherits two copies of the defective gene responsible for the disease, a copy from each parent. A person receives a normal gene, a gene of the disease that are carrier creatine e2 review of passengers for the disease, but usually no symptoms. The risk of transmission of the disease to the children of a couple who are both carriers of a recessive disease, is 25 percent. Fifty percent of their children are likely to be carriers of the disease, but usually show no symptoms of the disease. Twenty - five per cent of children receive two normal genes, one from each parent and are genetically normal (for this specific function). The risk is the same for each pregnancy. Albinism and eye problems: individuals have different amounts of pigment of the skin (melanin). As a result of albinism, there are eye problems such as sensitivity to light (photophobia), Strabismus (crossed eyes), and nystagmus (involuntary eye movements). Syndrome Hermansky-Pudlak syndrome affects vision. Bleeding disorders: people with the syndrome have platelet dysfunction. Since platelets are necessary for clotting, individuals will bruise and bleed easily. Cellular memory disorders: the syndrome causes a waxy substance (ceroid) accumulate in the tissues of the body and cause damage, particularly in the lungs and kidneys. A preoperative consultation of the pulmonologist is needed. The anesthesia team should be aware that patients may have postoperative pulmonary complications in the syndrome. Preoperative Hematology consultation is advisable prior to elective ocular operations. Given that patients with the syndrome have bleeding tendencies, intraoperative, perioperative and postoperative bleeding should prevent and treat. If the aggregation of platelets with desmopressin is improved, it can be administered before the operation. However, plasmapheresis is sometimes necessary in the perioperative period. Ophthalmologists should avoid blocks retrobulbar syndrome patients. If possible, the patients with HPS may benefit from General endotracheal anesthesia. Phacoemulsification may help prevent the intraoperative and postoperative bleeding in patients with the syndrome. Prolonged bleeding after strabismus surgery in patients with the syndrome has been reported. . . . .