Primary pulmonary alveolar proteinosis

Gayani, Gga; Karunaratna, Wgsg; Dilhani, Pdw; Muhunthan, S; Hmm, Herath; Weerarathna, Thilak Priyantha

doi:10.4038/gmj.v18i2.6200

Cited by 2 publications

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References 2 publications

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“…Different pathophysiological processes have been described 5 . There is a strong association with inhaled industrial fumes, toxins and tobacco smoking 3,5,6 . Symptoms are often non-specific ranging from mild dyspnoea to type 2 respiratory failure 1,3,4,6 .…”

Section: Discussionmentioning

confidence: 99%

“…Symptoms are often non-specific ranging from mild dyspnoea to type 2 respiratory failure 1,3,4,6 . Prevalence and incidence vary depending on geographical location between 0.1 to 40 per million and 0.2 per million, respectively by several authors mainly amongst caucasians 3,5,6 . Majority of the patients are between 20-50 years of age with a male preponderance 3 .…”

Section: Discussionmentioning

confidence: 99%

“…The process of WLL has complex cardiopulmonary interactions and is a challenge for an anaesthetist. In Sri Lanka, only a few cases have been reported 3,4 but none have described the experience of anaesthetising for WLL. We report a case of a 32year-old female with severe PAP who underwent WLL in a resource-limited setting.…”

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confidence: 99%

“…Registrar in Anaesthesiology, National Hospital for Respiratory Diseases, Welisara 2 Consultant Anaesthetist, National Hospital for Respiratory Diseases, Welisara 3 Senior Registrar in Anaesthesiology, National Hospital for Respiratory Diseases, Welisara…”

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confidence: 99%

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Anaesthetic Management of Whole Lung Lavage in a Resource-Limited Setting: Case Report

Varun,

Dhammika,

Jayathilaka

et al. 2024

Sri Lankan J Anaesthesiol

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Pulmonary Alveolar Proteinosis (PAP) is a rare disease1 Whole lung lavage (WLL) is the gold standard treatment for pulmonary alveolar proteinosis (PAP)2. The process of WLL has complex cardiopulmonary interactions and is a challenge for an anaesthetist. In Sri Lanka, only a few cases have been reported3,4 but none have described the experience of anaesthetising for WLL. We report a case of a 32- year-old female with severe PAP who underwent WLL in a resource-limited setting.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Anaesthetic Management of Whole Lung Lavage in a Resource-Limited Setting: Case Report

Varun,

Dhammika,

Jayathilaka

et al. 2024

Sri Lankan J Anaesthesiol

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“…In Sri Lanka there are only two previously reported cases of PAP who were managed with therapeutic-limited bronchoalveolar lavages (BALs) [ 5 , 6 ]. We report the case of an adolescent girl with PAP who underwent therapeutic WLL, which was the first WLL in Sri Lanka.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary alveolar proteinosis and first successful whole lung lavage in Sri Lanka: a case report

et al. 2017

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BackgroundPulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for a patient with pulmonary alveolar proteinosis.Case presentationWe describe the case of a 15-year-old Sri Lankan girl who presented with symptoms of progressive shortness of breath and dry cough for 6 months’ duration. She had a history of exposure to silica in her household environment. High-resolution computed tomography revealed crazy paving appearance in both lungs suggestive of pulmonary alveolar proteinosis. An open lung biopsy revealed intra-alveolar granular amphophilic material which was strongly periodic acid–Schiff positive and diastase resistant, which is consistent with pulmonary alveolar proteinosis. She was followed up for 2 years with periodical segmental bronchoalveolar lavages which showed minimal improvement in her symptoms as well as in exercise desaturation. Due to severe dyspnea and hypoxemia on exertion, she underwent whole lung lavage. It resulted in a marked improvement in her symptoms, exercise desaturation, and chest X-ray results.ConclusionWhole lung lavage was successfully performed for the first time in Sri Lanka for a patient with pulmonary alveolar proteinosis.

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Primary pulmonary alveolar proteinosis

Cited by 2 publications

References 2 publications

Anaesthetic Management of Whole Lung Lavage in a Resource-Limited Setting: Case Report

Anaesthetic Management of Whole Lung Lavage in a Resource-Limited Setting: Case Report

Pulmonary alveolar proteinosis and first successful whole lung lavage in Sri Lanka: a case report

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