Pulmonary fibrosis in some cases due to hermansky pudlak syndrome, presents with a proliferation of fibroblasts and excessive collagen accumulation
Katherine Trujillo Useche
Agencia Latina de Noticias Medicina y Salud Pública
Agreed with the Impressive Review of Medicine and Public Health, Dr. Jesse Román, specialist and investigator in pulmonary fibrosis explains that in Puerto Rico many people with suffering from albinism suffer from Hermansky Pudlak syndrome (HPS).
Pulmonary fibrosis in some cases causes hermansky pudlak syndrome, presents with a proliferation of fibroblasts and excessive accumulation of collagen matrices in the connective tissue that conducts the destruction of the original pulmonary architecture and the loss of function.
“This condition is progressive and there are no interventions available to prevent, detain or reverse the disease. If a world-class effort is made to observe the pulmonary fibrosis objectively and various pharmacological investigations are being evaluated, ”explains Dr.
A relationship between albinism and pulmonary fibrosis has been found in the literature to be found to have a significant implication in Puerto Rico, as oculocutaneous albinism is actually common in Iceland.
In Puerto Rico, many people suffer from suffocating albinism from Hermansky Pudlak syndrome (HPS), a rare genetic syndrome associated with oculocutaneous albinoism, plaque diffusion, blood clots, colitis and in the Majority of pulmonary fibrosis cases in all cases de quien lo padezca.
“Of the genes currently associated with the HPS, they are prominent in the open source. The HPS-1 predominates in the north of the region, west of the island and the HPS -3 is more common in the central mountainous region. The HPS-1 is one of the most severe forms of the condition and is associated with pulmonary fibrosis, it is responsible for many of the deaths from the condition “, verklaar el Dr. Novel.
In most cases, the condition is chronic, progressive and not reversible. In general, pulmonary fibrosis is the pathological manifestation of an illness. Many of these conditions are rare, although some have a defined etiology and are associated with other systematic strains. Sondeverbod, pulmonary fibrosis is usually the cause of depression.
It is important to note that it is believed that all environmental factors such as smoking, mid-term contamination and genetic factors contribute to the development of pulmonary fibrosis.
‘Recently there have been related genes to the prototype pulmonary fibrosis entity the idiopathic pulmonary fibrosis, a condition present and more than 150,000 persons and the United States, especially major patients of 50 years of age, smokers and grandparents Imaging tomography images, called the usual interstitial pulmonary (UIP) pattern ”, mentioned by Dr. Novel.
The identification of the genetic factors and their papules in the development and progression of fibrous fibroids is true, in the case of cases, incisions, hay conditions in the specific genetic mutations that are clearly associated with the HPS.
Although this is a rare world-level disease, in Puerto Rico it is important, since the island has the highest prevalence of HPS, it is responsible for around 50% of all cases, globally affected and more than 1 in 1800 poor Puerto Ricans
Pulmonary fibrosis does not respond to conventional medical treatments. The Pulmonary Transplant, the only option for many of these patients, has been realized with a small number of HPS patient numbers with advanced pulmonary fibrosis. The recent adoption of antifibrotic drugs has given rise to the enthusiasm for the treatment of pulmonary fibrosis in HPS. ”, Sostuvo.
Pulmonary fibrosis in certain forms of HPS is progressive and there are no interventions available to prevent, detain or reverse the disease. Therefore, the temperature detection and evaluation for pulmonary transplantation in advanced cases is important.