History Of Recurrent Pneumothorax Research Articles

Acute Pneumonitis after instillation of Povidone iodine for Chemical Pleurodesis in Secondary Spontaneous Pneumothorax: A case report

Secondary spontaneous pneumothorax is common in adult and is often life threatening. Surgical intervention is preferred, however chemical pleurodesis is also widely recommended in the treatment of recurrent pneumothorax of different etiologies. Variety of agents such as erythromycin, tetracycline, autologous blood and talc slurry are available for chemical pleurodesis among which povidone iodine is cheap, easily available, effective and safe. In this paper, we report the case of acute pneumonitis after pleurodesis using povidone iodine in a 33 years old female with a known case of lymphangioleiomyomatosis with a history of recurrent pneumothorax.
 Chemical pleurodesis was performed using povidone iodine after complete expansion of lung and absence of persistent air leak which was confirmed clinically and radiologically. Post procedure, the patient complained of right sided chest pain and cough. Fever, sputum production and shortness of breath was absent. Chest X-ray revealed new consolidation and infiltrations in the right middle zone. Subsequent CT chest showed extensive parenchymal consolidation and ground glass opacities in the right middle lobe, representing pneumonia with acute lung injury.
 The acute pneumonitis spontaneously resolved with supportive care and the patient was discharged after seven days. Though drug induced pneumonitis is not a usual complication of povidone iodine, it is a possibility and has to be considered.

September 2021 Pulmonary Case of the Month: A 45­-Year-Old Woman with Multiple Lung Cysts

No abstract available. Article truncated after the first 150 words. History of Present Illness A 45-year-old woman presented with increasing dyspnea on exertion and a history of recurrent pneumothoraces. In March 2018 she had laparoscopic ovarian cyst removal and noted some subsequent shortness of breath. In August 2018 she developed a right pneumothorax requiring chest tube placement. In September 2018 she had recurrent right pneumothorax and had video-assisted thoracoscopic surgery (VATS) with a right pleurodesis. The operative note from the outside VATS indicates a RUL bleb was removed and a wedge biopsy was done from posterior segment of the RUL. Pathology from the wedge biopsy reported “minimal emphysematous disease without other diagnostic abnormality”. She continued to be short of breath after the operation. PMH, SH, and FH • In 1975 she reportedly had pulmonary tuberculosis. • In 2018 the pneumothoraces, pleurodesis and the right ovarian cyst resection noted above. She is a never smoker and has no family history…

Characterization of a splice-site mutation in the tumor suppressor gene FLCN associated with renal cancer

BackgroundRenal cell carcinoma is among the most prevalent malignancies. It is generally sporadic. However, genetic studies of rare familial forms have led to the identification of mutations in causative genes such as VHL and FLCN. Mutations in the FLCN gene are the cause of Birt-Hogg-Dubé syndrome, a rare tumor syndrome which is characterized by the combination of renal cell carcinoma, pneumothorax and skin tumors.MethodsUsing Sanger sequencing we identify a heterozygous splice-site mutation in FLCN in lymphocyte DNA of a patient suffering from renal cell carcinoma. Furthermore, both tumor DNA and DNA from a metastasis are analyzed regarding this mutation. The pathogenic effect of the sequence alteration is confirmed by minigene assays and the biochemical consequences on the protein are examined using TALEN-mediated transgenesis in cultured cells.ResultsHere we describe an FLCN mutation in a 55-year-old patient who presented himself with progressive weight loss, bilateral kidney cysts and renal tumors. He and members of his family had a history of recurrent pneumothorax during the last few decades. Histology after tumor nephrectomy showed a mixed kidney cancer consisting of elements of a chromophobe renal cell carcinoma and dedifferentiated small cell carcinoma component. Subsequent FLCN sequencing identified an intronic c.1177-5_-3delCTC alteration that most likely affected the correct splicing of exon 11 of the FLCN gene. We demonstrate skipping of exon 11 to be the consequence of this mutation leading to a shift in the reading frame and the insertion of a premature stop codon. Interestingly, the truncated protein was still expressed both in cell culture and in tumor tissue, though it was strongly destabilized and its subcellular localization differed from wild-type FLCN. Both, altered protein stability and subcellular localization could be partly reversed by blocking proteasomal and lysosomal degradation.ConclusionsIdentification of disease-causing mutations in BHD syndrome requires the analysis of intronic sequences. However, biochemical validation of the consecutive alterations of the resulting protein is especially important in these cases. Functional characterization of the disease-causing mutations in BHD syndrome may guide further research for the development of novel diagnostic and therapeutic strategies.

An 18-year-old man with recurrent pneumothorax since he was 10-year-old

An 18-year-old male patient was referred to the department of pediatric pulmonology with a history of recurrent pneumothorax. Initial pneumothorax occurred at the age of 10. Following diagnosis of congenital lobar emphysema, he had five episodes of pneumothorax and subsequently underwent right-lower lobe anterobasal segmentectomy. Based on thoracic computed tomography (CT) and clinical manifestation, Birt-Hogg-Dube (BHD) syndrome was suspected and confirmed following genetic testing. BHD syndrome is a rare tumor predisposition syndrome first described in 1977. The syndrome is characterized by skin fibrofolliculomas, lung cysts, recurrent spontaneous pneumothorax, and renal cell cancer. The underlying cause is a germline mutation in the folliculin (FLCN) gene located on chromosome 17p11.2. Clinical manifestation usually appears after the age of 20 years. In this case, we report a case of BHD with episodes of recurrent pneumothorax, the first of which occurred at the age of 10 years. Pulmonologists should be aware of this syndrome in patients with a personal and family history of pneumothoraces and CT findings of multiple pulmonary cysts as additional evaluation and testing may be warranted. Pediatr Pulmonol. 2016;51:E41-E43. © 2016 Wiley Periodicals, Inc.

A Case of Multisystem Endometriosis

Catamenial pneumothorax is a rare complication secondary to pleural endometriosis. We present a case of a 37-year-old-female with a history of recurrent pneumothoraces with an associated temporal relationship to the onset of her menses. In addition to her recurrent pneumothoraces, on further evaluation, she was found to have multiple nodular masses within the omentum. A thoracoscopic biopsy was subsequently performed, which showed endometrial implants within the pleural space and within the omental cavity. The radiological features and pathogenesis of this rare disease are reviewed and discussed with reference to relevant literature.

Lung Cysts as Indicator for Birt–Hogg–Dubé Syndrome

In this case, we describe a patient with a history of recurrent pneumothorax. Based on CT-thorax and histopathology of the lung tissue, the Birt-Hogg-Dubé syndrome was suspected and confirmed after genetic testing. Recognizing this syndrome by pulmonologists and radiologists is very important, because the risk on developing of renal cell cancer is high.

Pulmonary features of Birt-Hogg-Dubé syndrome: Cystic lesions and pulmonary histiocytoma

to describe clinical, radiologic and pathologic features of lung lesions in Birt-Hogg-Dubè syndrome (BHDS) (MIM 135150). review of 12 patients of BHDS from 3 unrelated Italian families evaluated at GB Morgagni Hospital, Forlì, from 2005 to 2010. mean age (±SD) at diagnosis was 44.6 (±16) years, 8 (66%) were male. All three index cases presented with a history of recurrent pneumothorax and/or cystic lung lesions evaluated by CT scan request by referring pulmonary physicians, none were diagnosed to have BHDS at the time of initial pulmonary evaluation. One of the three cases was a middle-aged female patient with a clinical phenotype indistinguishable from lymphangioleiomyomatosis (LAM), characterized by cystic lung lesions and kidney angiomyolipoma. In one case of BHDS presenting with recurrent pneumothorax and a solitary lung nodule, surgical lung resection revealed a pulmonary histiocytoma. In one case a novel mutation of BHD gene was detected (c.771 del, exon 7). BHDS is associated with cystic lung disease largely under-recognized by pulmonary physicians and can mimic LAM and may be associated with lung tumor, pulmonary histiocytoma. In one case we found a novel mutation in exon 7, c.771 del (ref.seq. NM_144997.5) never reported before.

Birt-Hogg-Dubé Syndrome, a Rare Case in Korea Confirmed by Genetic Analysis

Simple benign tumors can present as part of a syndrome with substantial mortality. Fibrofolliculomas are benign skin tumors most often associated with the Birt-Hogg-Dubé syndrome (BHDS). The most life-threatening complication of this syndrome is renal cancer and other major features include multiple lung cysts and spontaneous pneumothorax. We present the case of a 54 year-old man with multiple flesh-colored papules on his face confirmed histologically as fibrofolliculomas. He had a history of recurrent pneumothorax and chest computed tomography showed multiple lung cysts. To confirm the diagnosis of BHDS, we conducted gene analysis that revealed a single nucleotide duplication in the folliculin (FLCN) gene (Exon 11, C.1285dupC). BHDS confirmed by the FLCN gene mutation is rarely reported in Korea. Appropriate investigation is recommended whenever a patient with benign skin tumors is encountered.

"Bong lung" in cystic fibrosis: a case report

IntroductionMarijuana or "bong" lung has been recently described. Subjects typically develop large peripheral paraseptal lung bullae and are predisposed to spontaneous pneumothoraces. The underlying mechanism for bullae formation is uncertain, but probably relates to direct lung toxicity and repeated barotrauma as the smoker performs frequent valsalva manoeuvres in an attempt to derive a greater drug effect.Case presentationWe describe a case of probable "bong lung" occurring in a 23-year-old Caucasian man with cystic fibrosis who had a history of recurrent pneumothoraces and unusual findings on sputum cytology.ConclusionOur case highlights the importance of questioning young adult cystic fibrosis patients about illicit drug use and the utility of sputum cytology and computed tomography scanning when patients present with pneumothoraces and deteriorations in clinical status.

Linfangioleiomiomatosis pulmonar: Caso clínico

Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease, of unknown etiology, affecting almost exclusively women. Microscopically LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other diseases (sporadic LAM) or in conjunction with tuberous sclerosis complex (TSC). It presents with progressive breathlessness or with recurrent pneumothorax or chylothorax. We report a 33 year-old woman with a history of recurrent pneumothorax. Computed tomography (CT) scans showed numerous thin-walled cysts throughout the lungs, a characteristic finding in LAM. A pulmonary biopsy was compatible with the diagnosis and HMB-45 monoclonal antibodies were positive. Treatment with Sirolimus was started).

Cystic Lung Disease in Birt-Hogg-Dubé Syndrome

To describe the clinical, radiologic, and histopathologic aspects of cystic lung disease occurring in patients with Birt-Hogg-Dubé (BHD) syndrome, a rare, inheritable, multisystem disorder. We retrospectively reviewed five patients with BHD syndrome evaluated at the Mayo Clinic Rochester from 1998 through 2005. Mean age (+/- SD) at the time of pulmonary evaluation was 56.4 +/- 4.8 years; four patients were men. Three patients had not received a diagnosis of BHD syndrome at the time of initial CT of the chest. Three patients had a smoking history, and two were nonsmokers. Two patients had a history of recurrent pneumothoraces. Pulmonary function tests available in four patients revealed normal results in one patient and mild airflow obstruction or nonspecific pattern of abnormalities in three patients. CT of the chest revealed cystic lung disease in all five patients; cysts were round to oval in shape, ranged widely in size, and were randomly distributed throughout the lungs, except for a predilection to involve the lung bases more extensively. Three patients with a smoking history had more severe cystic changes compared to nonsmokers and included both patients with recurrent pneumothoraces. Surgical lung biopsy available in one patient revealed emphysema-like changes. Follow-up CT scans available in four patients revealed relative stability over a median interval of 20 months (range, 3 to 66 months). We conclude that cystic lung disease in BHD syndrome varies widely in severity, mimics pulmonary lymphangioleiomyomatosis, and may be worsened by smoking.

Thoracoscopic Lung Resection

Recent advances in rigid endoscopic imaging capabilities, light sources, and instrumentation have dramatically expanded the potential role of laparoscopic and thoracoscopic surgery. The recent introduction of an endoscopic linear stapling device has made possible thoracoscopic pulmonary resection. A 28-year-old woman with a history of recurrent pneumothorax, diffuse interstitial fibrosis, and left apical microblebs underwent thoracoscopic lung resection using this new stapling device. A 3 x 5 cm segment of involved lung tissue was removed. Postoperatively, the patient manifested no evidence of air leak and was discharged 48 h after surgery.

Value of computed tomography of the lung in the management of primary spontaneous pneumothorax

The treatment of patients with primary spontaneous pneumothorax remains controversial, since recurrence and the ultimate need for thoracostomy cannot be predicted. In the current study, computed tomography (CT) of the lung was performed prospectively on 26 consecutive patients with primary spontaneous pneumothorax to determine whether the size and/or the number of apical blebs would correlate with recurrence and/or need for thoracostomy. Both the number of blebs and the bleb score (calculated by the number of blebs multiplied by a numeric value assigned a range of bleb sizes) of the affected lung was significantly greater in patients with a history of recurrent pneumothorax and/or ultimate need for thoracostomy as treatment. CT of the lung may be useful in predicting the natural history of pneumothorax.

Unilateral Bronchospasm during Pleurodesis in an Asthmatic Patient

A case of unilateral bronchospasm during pleurodesis in a patient with a history of recurrent pneumothorax and asthma is presented. The etiology of this event is not clear; however, it likely includes reflex parasympathetic bronchoconstriction due to physical stimulation of the pleural surface during pleurodesis. Treatment involved independent lung ventilation, corticosteroids, and aminophylline.