Fetal pulmonary fibrosis due to Hermanski-Pudlak syndrome (HPS): a rare case report with open lung biopsy findings

Hermanski-Pudlak Syndrome (HPS) is an extremely rare autosomal recessive disorder. Albinism, bleeding diathesis and other associated complications are the main manifestations of HSP. Here we report a 56-year-old woman who was referred with gradually increasing dyspnea. She had a past history of coughing, epistaxis, gums bleeding, easy bruising and severe sunburn in normal sun exposure. Her blood oxygen saturation was 87% in room air. Physical examination revealed oculocutaneous albinism, strabismus, horizontal nystagmus and fine inspiratory crackle. Laboratory studies showed prolonged partial thromboplastin time and PaO2 value of 39 mmHg. There was a reticulonodular pattern in chest radiography. Open lung biopsy confirmed the diagnosis of pulmonary fibrosis. Although the patient had been treated with Pirfenidone, she died because of respiratory failure. Although HPS is a rare syndrome, finding more about the pathophysiology of HPS and also developing new methods of treatment is indisputable

Statins in patients with COVID-19: a retrospective cohort study in Iranian COVID-19 patients.

Background: The coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has profoundly affected the lives of millions of people. To date, there is no approved vaccine or specific drug to prevent or treat COVID-19, while the infection is globally spreading at an alarming rate. Because the development of effective vaccines or novel drugs could take several months (if not years), repurposing existing drugs is considered a more efficient strategy that could save lives now. Statins constitute a class of lipid-lowering drugs with proven safety profiles and various known beneficial pleiotropic effects. Our previous investigations showed that statins have antiviral effects and are involved in the process of wound healing in the lung. This triggered us to evaluate if statin use reduces mortality in COVID-19 patients. Results: After initial recruitment of 459 patients with COVID-19 (Shiraz province, Iran) and careful consideration of the exclusion criteria, a total of 150 patients, of which 75 received statins, were included in our retrospective study. Cox proportional-hazards regression models were used to estimate the association between statin use and rate of death. After propensity score matching, we found that statin use appeared to be associated with a lower risk of morbidity [HR = 0.85, 95% CI = (0.02, 3.93), P = 0.762] and lower risk of death [(HR = 0.76; 95% CI = (0.16, 3.72), P = 0.735)]; however, these associations did not reach statistical significance. Furthermore, statin use reduced the chance of being subjected to mechanical ventilation [OR = 0.96, 95% CI = (0.61-2.99), P = 0.942] and patients on statins showed a more normal computed tomography (CT) scan result [OR = 0.41, 95% CI = (0.07-2.33), P = 0.312]. Conclusions: Although we could not demonstrate a significant association between statin use and a reduction in mortality in patients with COVID19, we do feel that our results are promising and of clinical relevance and warrant the need for prospective randomized controlled trials and extensive retrospective studies to further evaluate and validate the potential beneficial effects of statin treatment on clinical symptoms and mortality rates associated with COVID-19