Alpha-1-antitrypsin deficiency (AATD) and spontaneous pneumothorax: Guidelines do not recommend screening for AATD in patients with pneumothorax – What did we find in 10 years of clinical evidence?

Dias, Eliane Pedra; Farinha, Inês; Costa, Fátima

doi:10.1016/j.pulmoe.2020.05.014

Cited by 3 publications

(1 citation statement)

References 5 publications

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“…Genetic influence is supported by mutations of the FLCN gene found both in family cases -a family history of pneumothorax can cause in 10% of cases the appearance of familial pneumothorax disease [10,11] and in several genetic diseases -Birt-Hogg-Dubé syndrome, pulmonary lymphangiomyomatosis, Marfan syndrome, Ehlers-Danlos vascular syndrome, Loeys Dietz syndrome, cystic fibrosis. [12] Frequently affects tall and weak men [13].…”

Section: Introductionmentioning

confidence: 99%

Spontaneous Pneumothorax - Therapeutic Attitude - Brief Review

Nistor¹,

Ciuche²,

Stanciu-Gavan³

2020

IJCTS

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Background: Spontaneous pneumothorax is a type of air collection in the pleural cavity that it develops in the absence of trauma or an iatrogenic cause. It may become an emergency in thoracic surgery. The anatomical substrate of spontaneous pneumothorax is represented by blisters. But we must not minimize the emphysema bubbles that appear in the lung parenchyma, which may also be a common cause of spontaneous pneumothorax. In order to appear the spontaneous pneumothorax, it is considered necessary to have an area of minimal resistance in the visceral pleura and also a triggering element. For decades, the therapeutic options are been discussed. Objectives: literature review and updating of scientific data Method: The management of conservative treatment is known, ambulatory air aspiration, drain tube installation, video-assisted thoracoscopic surgery The use of chemical pleurodesis-betadine versus talc and mechanical-pleural abrasion, resection of blisters or ligature at their base, apical pleurectomy, are therapeutic options that are custom applied for each case. The current diagnostic and treatment guidelines have been designed about 10 years ago and with a little change. Results: We support the use as a chemical agent of betadine to prevent recurrences with a similar effect to that of talc. We concluded that the introduction of betadine into the drain tube after minimal pleurotomy for symphysis, can sometimes lead to local pain, sometimes atrocious, unbearable, even simulating the myocardial infarction pain. Therefore, before applying the chemical, we recommend the systemic analgesics and local analgesic from the amide group. Video-assisted thoracic surgery is the main surgical technique for treating pneumothorax in order to prevent recurrences. Conclusions-We considered necessary a mini-revision of the literature in order to update the scientific data and the effective treatment methods with the mention of some own amendments regarding the therapeutic options.

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Section: Introductionmentioning

confidence: 99%

Spontaneous Pneumothorax - Therapeutic Attitude - Brief Review

Nistor¹,

Ciuche²,

Stanciu-Gavan³

2020

IJCTS

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Recurrent spontaneous pneumothorax in a patient with wilson disease who is on long-term D-penicillamine: Case report

MDN,

LMH,

et al. 2024

JLPRR

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Introduction: Recurrent spontaneous pneumothoraces (PSP) in association with liver disease is well known to occur with alpha 1 antitrypsin deficiency (AATD). Wilson disease (WD) association with recurrent PSP is not a known entity. Case presentation: A 42-year-old nonsmoker man with a history of recurrent PSP was admitted with a large right pneumothorax. Following intercostal chest drain (ICD) patient’s breathing improved and the lung expanded. Despite that, he continued to have a small residual pneumothorax (<2cm). Twelve years ago, he was diagnosed with WD. The patient was on treatment with D-penicillamine. After 3 years of the diagnosis of WD, the patient developed left-sided recurrent PSPs where he ended up with a left-pleurectomy in 2015. For the current event, initially expectant management was done for residual pneumothorax. All the possible causes for PSP were excluded. On follow-up, he continued to be symptomatic. A thoracic surgical referral was arranged for Video-Assisted Thoracoscopic Surgery (VATS) and redo-pleurectomy. The safety of future treatment with D-penicillamine is not concluded as there is no convincing evidence to prove it as a culprit agent for PSPs. Multidisciplinary discussion was arranged and consideration of treatment with alternative copper chelating therapy was emphasized. Conclusion: Though rare, it is important to observe for occurrence of PSP in WD patients. This case report will be eye-opening for the association of lung disease and WD and related treatment.

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Recurrent spontaneous pneumothorax in a patient Wilson disease who is on long term D-penicillamine. Case report

Gunathilaka,

LMH,

Handagala

et al. 2024

Preprint

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Background Recurrent spontaneous pneumothoraces (PSP) in association with liver disease is well known to occur with alpha 1 antitrypsin deficiency (AATD). Wilson disease (WD) association to recurrent PSP is not a known entity. Case presentation A 42-year-old nonsmoker man with a history of recurrent PSP admitted with large right pneumothorax. Following intercostal chest drain (ICD) patient’s breathing improved and the lung expanded. Despite that, he continued to have a small residual pneumothorax (< 2cm). Twelve years ago, he was diagnosed to have WD. Patient was on treatment with D-penicillamine. After 3 years to the diagnosis of WD, patient had developed left sided recurrent PSPs where he ended up with left-pleurectomy in 2015. For the current event, initially expectant management was done for residual pneumothorax. All the possible causes for PSP were excluded. On follow up, he continued to be symptomatic. Thoracic surgical referral was arranged for Video-Assisted Thoracoscopic Surgery (VATS) and redo-pleurectomy. Safety of future treatment with D-penicillamine is not concluded as there is no convincing evidence to prove it as a culprit agent for PSPs. Multidisciplinary discussion arranged and consideration of treatment with alternative copper chelating therapy was emphasized. Conclusions Though rare, it is important to observe for occurrence of PSP in WD patients. This case report will be an eye opening for association of lung disease and WD and related treatment.

show abstract

Alpha-1-antitrypsin deficiency (AATD) and spontaneous pneumothorax: Guidelines do not recommend screening for AATD in patients with pneumothorax – What did we find in 10 years of clinical evidence?

Cited by 3 publications

References 5 publications

Spontaneous Pneumothorax - Therapeutic Attitude - Brief Review

Spontaneous Pneumothorax - Therapeutic Attitude - Brief Review

Recurrent spontaneous pneumothorax in a patient with wilson disease who is on long-term D-penicillamine: Case report

Recurrent spontaneous pneumothorax in a patient Wilson disease who is on long term D-penicillamine. Case report

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