Shahram Seyfi, Nazli Farnoosh, Kayvan Latifi, Parviz Amri Male , Hamed Mehdinezhad Gorji , Asadollah Shakeri, Khadijeh Ezoji ,
Volume 80, Issue 11 (February 2023)
Abstract
Background: Sporadic lymphangioleiomyomatosis (S-LAM) is a rare disease that generally affects young women and involves the abnormal proliferation of smooth muscle cells (LAM cells) in the lungs (pulmonary LAM). There are two types of LAM, sporadic and LAM with tuberous sclerosis, which is an autosomal dominant genetic disease caused by mutations in the Tsc1 and Tsc2 genes. The most common manifestation of this disease is pneumothorax due to cyst rupture. Median transplant-free survival for pulmonary LAM is 23 years from diagnosis. Factors associated with a poor prognosis are often those associated with a more rapid decline in lung function.
Case Presentation: The patient was a 24-year-old woman who presented with pneumothorax and multiple bullae in both lungs. The patient had no family history of respiratory disease, and the patient herself had no symptoms other than mild shortness of breath following activity from a year ago. A lung CT scan was performed for the patient and multiple bullae were evident in both lungs, which confirmed the diagnosis. There was no evidence of involvement of other organs but the lung. For the patient in the ICU with the diagnosis of left pneumothorax, a chest tube was inserted and she underwent mechanical ventilation. Finally, after 24 days of hospitalization, oxygen therapy of the patient was performed with a normal mask, and she underwent left lung pleurodesis with talcum powder, and a few days later, right lung pleurodesis was performed on the patient. On the 36th day of hospitalization, her general condition improved and she was discharged from the ICU. The patient was discharged in good general condition and had no problem on her four months follow-up.
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Conclusion: Fortunately, with the diagnosis of LAM for the patient according to the CT scan of the patient's lung, she was treated with insertion of a chest tube and by performing pleurodesis, the recurrence of pneumothorax was prevented. In a young woman with spontaneous pneumothorax, sporadic LAM is one of the differential diagnoses.
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Nazli Farnoosh, Shahram Seyfi, Khadijeh Ezoji, Asadollah Shakeri, Kayvan Latifi, Parviz Amri Male ,
Volume 81, Issue 8 (November 2023)
Abstract
Background: Noninvasive ventilation (NIV) has been used as one of the most promising methods to treat hypoxic respiratory failure in COVID-19.
Methods: In this study, the clinical outcomes of 80 patients with acute respiratory failure caused by COVID-19 were evaluated. The disease was confirmed in two methods: clinical and radiographic confirmation of disease in patients with an acute respiratory infection (highly suspected) or in some cases with a positive PCR test. The rate of discharge from the ICU without the need for intubation was evaluated as the primary outcome and based on that, the patients were divided into two groups: NIV failure and NIV success. Mortality rate, length of stay in ICU, frequency of intubated patients and laboratory factors of patients as secondary outcomes in two groups were compared.
Results: Regarding the outcome of death and discharge, there was a statistically significant difference between the two groups. Therefore, all patients with corona who were admitted to ICU and received NIV and were intubated due to non-responsiveness to NIV died and the patients were discharged from the hospital without intubation. All the patients investigated in this study, had received antiviral, antibiotic, and glucocorticoid treatment during hospitalization. In this study, the patients who were in the NIV success group and were not intubated had a lower mean age compared to the group without NIV success (54 vs. 67 years, respectively). The number of white blood cells in the NIV success group was 3945.28±10011.21 and in the group without NIV success was 9242.38±17296.16, which was statistically significant (P=0.004). The number of blood neutrophils in the successful and unsuccessful NIV groups was 12.19±83.04 and 4.31±89.49, respectively (P=0.034). The mean APACHEII score in patients in the NIV success group (11.07±5.05) was significantly lower than the group without NIV success (20.16±4.96).
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Conclusion: Mortality was higher in the intubated group than in the NIV group. The combination of APACHEII score and respiratory rate one hour after receiving NIV can predict success with NIV.
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