Long‐term results of disodium etidronate treatment in pulmonary alveolar microlithiasis

Özçeli̇k, Uğur; Yalçın, Ebru; Arıyürek, M.; Doğru, Deniz; Cinel, Güzin; Gülhan, Bora; Ki̇per, Nural

doi:10.1002/ppul.21209

Cited by 60 publications

(54 citation statements)

References 14 publications

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“…The use of diphosphonates has also been proposed to reduce calcium phosphate precipitation in PAM. However, this therapy remains controversial given the limited number of reports in the literature [38,39,[60][61][62]. In 1988, the Toronto Lung Transplantation Group reported a successful double lung transplantation in humans.…”

Section: Therapy and Lung Transplantationmentioning

confidence: 99%

Pulmonary alveolar microlithiasis: review of the 1022 cases reported worldwide

et al. 2015

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Pulmonary alveolar microlithiasis (PAM) is a rare disease characterised by the widespread intra-alveolar accumulation of minute calculi called microliths. It is caused by mutation of the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells. The present study explores the epidemiological, familial, genetic, clinical, diagnostic, radiological and therapeutic aspects with the aim of contributing to a better understanding of this uncommon disease.We searched articles on PAM published up to December 2014 and 544 papers were found, accounting for 1022 cases.PAM is present in all continents and in many nations, in particular in Turkey, China, Japan, India, Italy and the USA. Familiality is frequent. The clinical course is not uniform and the causes of this clinical variability seem to be largely nongenetic. The optimal diagnostic procedure is the association of chest high-resolution computed tomography (HRCT) with bronchoalveolar lavage, but a chest radiograph may suffice in families in which a case has already been diagnosed. Moreover, chest radiography and HRCT allow the classification of the evolutionary phase of the disease and its severity. At present lung transplantation is the only effective therapy. However, better knowledge of the gene responsible offers hope for new therapies. @ERSpublications Familial, genetic, clinical, radiological and therapeutic aspects of pulmonary alveolar microlithiasis epidemiology http://ow.ly/St3Mw

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Section: Therapy and Lung Transplantationmentioning

confidence: 99%

Pulmonary alveolar microlithiasis: review of the 1022 cases reported worldwide

et al. 2015

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“…Currently, no clearly defined treatment options are available with exception of lung transplantation. In addition, clinical and radiological improvements were reported in two patients with PAM who were treated with bisphosphonates 6. The reported mutations in the SCL34A2 ‐gene most commonly occur in one of the exons with a predicted protein truncating effect 7.…”

Section: Discussionmentioning

confidence: 97%

A case of pulmonary alveolar microlithiasis associated with a homozygous 195 kb deletion encompassing the entire SLC34A2 gene

Stokman

Nossent

Grünberg

et al. 2016

Clinical Case Reports

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“…Systemic corticosteroids, calcium-chelating agents and bronchoalveoler lavage (BAL) are palliative solutions 10 . Ozçelik et al have described the positive effects of the long term use of sodium etidronate which is effective by inhibiting the hydroxyapatite microcrystal formation in pediatric patients 11 . However, there are also some studies showing that the sodium etidronate treatment is ineffective 7 .…”

Section: Discussionmentioning

confidence: 99%