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2.
Respir Med Case Rep ; 31: 101250, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33294353

RESUMO

Diffuse idiopathic pulmonary neuroendocrine cell (DIPNECH syndrome) remains unfamiliar to most clinicians even though it was first described almost 30 years ago. Diagnosis is usually confirmed histopathologically after lung biopsy, but often, a diagnosis or suspected diagnosis can be made radiographically. In this paper, we present a case report of a 68-year-old female with shortness of breath and fleeting pulmonary nodules observed on chest CT scan. She was initially misdiagnosed with asthma based on an abnormal pulmonary function test which revealed an obstructive ventilatory defect. The classic radiographic findings of DIPNECH syndrome and the typical patient demographics that should arouse suspicion of a DIPNECH diagnosis were also illustrated. DIPNECH syndrome is a clinicopathological syndrome whereas focal NECH is a pathological diagnosis that is often made incidentally on histological examination and is encountered in a variety of settings, including in resected carcinoid tumors, in the context of reactive changes concomitant with infection, in metastatic cancer, radiation pneumonitis, intra-lobar sequestration, smokers, interstitial lung disease, and lung adenocarcinoma. There are no proven treatments for DIPNECH syndrome. In patients with obstructive ventilatory symptoms, bronchodilators with inhaled steroids are usually prescribed. Some severe cases may require parenteral steroids. Somatostatin analogs (SSA) have also been used in some cases with mixed results. Rapamycin has been used in several cases based on the purported activation of the mammalian target of rapamycin (mTOR) in DIPNECH. Some patients with large carcinoid tumors may benefit from resection.

3.
Case Rep Vasc Med ; 2013: 538259, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24367736

RESUMO

Cor triatriatum sinistrum is a rare congenital disorder defined as a division of the left atrium by a diaphragmatic membrane resulting in two left atrial chambers. The membranous division of the atrium can be partial or complete and can affect either atrium, with involvement of the right atrium referred to as cor triatriatum dexter. The presence of fenestrations within the membrane allows for communication and forward passage of blood into the true atrium. Absence of fenestrations leads to early symptomatic engorgement of the lungs. We report the case of a young adult male presenting with recurrent hematemesis due to variceal bleeding. On CT imaging the patient was found to have cor triatriatum sinistrum, with a vertical membrane resulting in total obstruction of the pulmonary venous drainage on the right, with normal pulmonary venous drainage on the left. There was extensive pulmonary-systemic arterial collateralization to the right lung suggesting retrograde filling of the right pulmonary artery with effective flow reversal in the right lung.

4.
Case Rep Med ; 2013: 352128, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24454399

RESUMO

Scimitar syndrome, a rare congenital cardiopulmonary condition, presents in both pediatric and adult populations as an anomalous pulmonary venous return of most of the right lung to the inferior vena cava. Recently, asymptomatic adult cases have been diagnosed with advances in imaging studies. We report the case of an asymptomatic 43-year-old male, with a complex variant scimitar syndrome diagnosed by computed tomographic angiography.

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