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

The global, prevalence, and risk factors of postoperative fever after percutaneous nephrolithotomy: A systematic review and meta-analysis

Objective: This study aimed to explore the global, prevalence, and risk factors of fever after percutaneous nephrolithotomy (PCNL) by conducting a systematic review and meta-analysis. Methods: The high-sensitivity searching was conducted without time limitation until December 30, 2020 in Web of Sciences, Scopus, and PubMed based on inclusion and exclusion criteria. Results: The prevalence rates of fever and sepsis among patient undergoing PCNL were estimated 9.5% (95% confidence interval [CI]: 9.3%–9.7%), and 4.5% (95% CI: 4.2%–4.8%), respectively. Nephrostomy tube was used in 9.96% (95% CI: 9.94%–9.97%) of patients. The mean preoperative white blood cells of patients were 6.401×109/L; 18.3% and 4.55% of patients were considered as the positive urinary culture and pyuria, respectively. About 20.4% of patients suffered from residual stones. The odds ratios (ORs) of fever in patients who suffering from diabetes mellitus, hydronephrosis, staghorn stones, and blood transfusion were 4.62 (95% CI: 2.95–7.26), 1.04 (95% CI: 0.81–1.34), 2.57 (95% CI: 0.93–7.11), and 2.65 (95% CI: 1.62–4.35), respectively. Patients who underwent PCNL in prone position were more likely to develop fever (OR: 1.23; 95% CI: 0.75–2.00) than patients in supine position. Conclusion: The current study showed that patients who suffer from diabetes mellitus, hydronephrosis, staghorn stones, nephrostomy tube or double-J stent, blood transfusion, and also patients who underwent PCNL in prone position surgery are more likely to develop a postoperative fever after PCNL