Pulmonary langerhans cell histiocytosis presenting as simultaneous bilateral spontaneous pneumothorax in a non-smoker patient

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20 of patients present with or later develop pneumothorax. It is striking that more than 90 of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax. © 2008 Tehran University of Medical Sciences. All rights reserved

Pulmonary metastases: Results of surgical resection in 60 cases: Treatment modalities other than surgery are needed

Pulmonary metastases occur in 30 of all oncology patients. Surgical resection of lung metastases is a widely accepted procedure but long-term results are disappointing with a 5-year survival rate of 20-40 and the results vary with the histologic type of the primary tumor. Due to unavailability of any study regarding pulmonary metastasectomy in Iran and emergence of new treatment modalities, reassessment of our current practices is essential. We performed a retrospective study of 60 cases of pulmonary metastasectomy during a 5-year period in one of the major thoracic surgery centers in Tehran (Imam Khomeini Hospital). Bilateral metastases were present in 23 of cases, number of metastases in each patient ranged from 1-12. Average disease-free interval was 12 months, pneumonectomy rate was 21.7 mostly as a second or third attempt and finally, recurrence or death following initial metastasectomy occurred in 12-18 months in most patients. These results confirm that surgery remains unsuccessful in obtaining long-term survival or cure in most patients with pulmonary metastases and treatment strategies other than surgery, such as radiofrequency ablation are needed to avoid performing multiple operations in these patients and improving their quality of life. © 2007 Tehran University of Medical Sciences. All rights reserved

The first inherited retinal disease registry in Iran: Research protocol and results of a pilot study

Background: To describe the protocol for developing a national inherited retinal disease (IRD) registry in Iran and present its initial report. Methods: This community-based participatory research was approved by the Ministry of Health and Medical Education of Iran in 2016. To provide the minimum data set (MDS), several focus group meetings were held. The final MDS was handed over to an engineering team to develop a web-based software. In the pilot phase, the software was set up in two referral centers in Iran. Final IRD diagnosis was made based on clinical manifestations and genetic findings. Ultimately, patient registration was done based on all clinical and non-clinical manifestations. Results: Initially, a total of 151 data elements were approved with Delphi technique. The registry software went live at www.IRDReg.org based on DHIS2 open source license agreement since February 2016. So far, a total of 1001 patients have been registered with a mean age of 32.41±15.60 years (range, 3 months to 74 years). The majority of the registered patients had retinitis pigmentosa (42, 95 CI: 38.9 to 45). Genetic testing was done for approximately 20 of the registered individuals. Conclusion: Our study shows successful web-based software design and data collection as a proof of concept for the first IRD registry in Iran. Multicenter integration of the IRD registry in medical centers throughout the country is well underway as planned. These data will assist researchers to rapidly access information about the distribution and genetic patterns of this disease. © 2020 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons. org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax

"PULMONARY METASTASES: RESULTS OF SURGICAL RESECTION IN 60 CASES: TREATMENT MODALITIES OTHER THAN SURGERY ARE NEEDED "

Pulmonary metastases occur in 30% of all oncology patients. Surgical resection of lung metastases is a widely accepted procedure but long-term results are disappointing with a 5-year survival rate of 20-40% and the results vary with the histologic type of the primary tumor. Due to unavailability of any study regarding pulmonary metastasectomy in Iran and emergence of new treatment modalities, reassessment of our current practices is essential. We performed a retrospective study of 60 cases of pulmonary metastasectomy during a 5-year period in one of the major thoracic surgery centers in Tehran (Imam Khomeini Hospital). Bilateral metastases were present in 23% of cases, number of metastases in each patient ranged from 1-12. Average disease-free interval was 12 months, pneumonectomy rate was 21.7% mostly as a second or third attempt and finally, recurrence or death following initial metastasectomy occurred in 12-18 months in most patients. These results confirm that surgery remains unsuccessful in obtaining long-term survival or cure in most patients with pulmonary metastases and treatment strategies other than surgery, such as radiofrequency ablation are needed to avoid performing multiple operations in these patients and improving their quality of life