Obstructive Sleep Apnea in Amyloidosis Treated With Nasal Continuous Positive Airway Pressure

Lesser, Barry A.; Leeper, Kenneth V.; Conway, William

doi:10.1001/archinte.1988.00380100125028

Cited by 10 publications

(5 citation statements)

References 19 publications

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“…This study shows that OSAS, reported earlier in immunoglobulin‐related AL amyloidosis ( Lesser et al . 1988 ), b‐amyloid‐related Alzheimer's disease ( Reynolds et al .…”

Section: Discussionsupporting

confidence: 83%

“…Correlating pathological data are not available but amyloid infiltration, already present in young asymptomatic patients ( Kiuru 1992), may contribute to the hypertrophy and abnormal laxity of the pharyngeal soft tissues and the tongue. Obstructing macroglossia occurs also in AL‐amyloidosis ( Lesser et al . 1988 ), acid maltase deficiency ( Margolis et al .…”

Section: Discussionmentioning

confidence: 99%

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Obstructive sleep apnoea syndrome in hereditarygelsolin‐related amyloidosis

Kiuru

Nieminen

Partinen

1999

Journal of Sleep Research

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SUMMARY Gelsolin-related amyloidosis (AGel amyloidosis) is a rare autosomal dominant disorder, reported worldwide in kindreds carrying a G654A or G654T gelsolin gene mutation. The main clinical signs are cutis laxa, cranial and peripheral neuropathy, and corneal lattice dystrophy but heavy intermittent snoring also occurs. To evaluate whether sleep apnoea is present we performed nocturnal sleep recordings, cephalometric and spirometric analyses and multiple sleep latency tests (MSLT) in five snoring patients with a G654A gelsolin gene mutation. Four patients had obstructive sleep apnoea syndrome (OSAS) with redundant oropharyngeal and hypopharyngeal soft tissues, macroglossia and cranial neuromuscular dysfunction. The fifth patient had hypersomnia without obstructive sleep apnoea. Nasal continuous positive airway pressure (CPAP) was an effective treatment. This study presents the first evidence in favour of an association between AGel amyloidosis and OSAS, but further studies are needed to define the prevalence of OSAS and the pathogenetic roles of amyloid and variant gelsolin in its evolution.

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“…This study shows that OSAS, reported earlier in immunoglobulin‐related AL amyloidosis ( Lesser et al . 1988 ), b‐amyloid‐related Alzheimer's disease ( Reynolds et al .…”

Section: Discussionsupporting

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Obstructive sleep apnoea syndrome in hereditarygelsolin‐related amyloidosis

Kiuru

Nieminen

Partinen

1999

Journal of Sleep Research

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“…However, there are very few data on SDB in amyloidosis, mostly case reports which focused on patients with macroglossia and redundant oropharyngeal and hypopharyngeal soft tissues. [31][32][33] To our knowledge, this is the first study to evaluate SDB in the specific context of CA including the three main types. The first major finding is the very high prevalence of SDB (90%), with 27% of CSA.…”

Section: Prevalence Of Sleep-disordered Breathing In Cardiac Amyloidosismentioning

confidence: 99%

Prevalence, Severity, and Prognostic Value of Sleep Apnea Syndromes in Cardiac Amyloidosis

Bodez

Guellich

Kharoubi

et al. 2016

Sleep

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“…Amyloid-Ablagerungen im Bereich der oberen Atemwege, typischerweise in Zunge und Rachen, können schwere schlafbezogene Atemstörungen zur Folge haben. Ursächlich sind in der Literatur sowohl erworbene als auch hereditäre Amyloidoseformen beschrieben [30,31] angesehen. Möglicherweise kann durch eine ergänzende Bestrahlung die Rezidivneigung reduziert werden [33,34].…”

Section: Schlafbezogene Atemstörungenunclassified

Amyloidose in der Pneumologie

et al. 2012

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Amyloidosis is a rare disorder within the field of pneumology, however, it is estimated that there are a relevant number of unreported cases. In routine clinical practice, the disease is seldom considered in the diagnostic work-up of unclear respiratory symptoms and radiological findings. For amyloidosis to be considered as a differential diagnosis, the clinician must be aware of the broad variety of its clinical manifestations. In cases where amyloidosis is suspected, it is important to obtain an early tissue biopsy. If the diagnosis can be confirmed by Congo red staining, further subtyping of the amyloid protein is necessary in order to identify a treatable cause of the disease. The amyloidoses are classified according to the type of amyloid protein, with clinical subclassifications distinguishing hereditary from acquired forms and localised from generalised manifestations. Apart from causal therapy of the primary disease, the treatment of generalised amyloidosis includes specific systemic therapy. The majority of localised forms are treated with specific local interventions showing good long-term results. This review outlines the pathophysiology, classification, diagnostic pathways and therapeutic modalities in amyloidosis. Furthermore, typical manifestations of amyloidosis of the lung and the specific treatment options are discussed.

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Obstructive Sleep Apnea in Amyloidosis Treated With Nasal Continuous Positive Airway Pressure

Cited by 10 publications

References 19 publications

Obstructive sleep apnoea syndrome in hereditarygelsolin‐related amyloidosis

Obstructive sleep apnoea syndrome in hereditarygelsolin‐related amyloidosis

Prevalence, Severity, and Prognostic Value of Sleep Apnea Syndromes in Cardiac Amyloidosis

Amyloidose in der Pneumologie

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