Serveur d'exploration Chloroquine

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Lysinuric protein intolerance with chronic interstitial lung disease and pulmonary cholesterol granulomas at onset

Identifieur interne : 002D87 ( Main/Exploration ); précédent : 002D86; suivant : 002D88

Lysinuric protein intolerance with chronic interstitial lung disease and pulmonary cholesterol granulomas at onset

Auteurs : Eitan Kerem [Israël] ; Orly N. Elpelg [Israël] ; Ruth S. Shalev [Israël] ; Eliezer Rosenman [Israël] ; Yaacov Bar Ziv [Israël] ; David Branski [Israël]

Source :

RBID : ISTEX:C5A26619F5A679562DF32C827FB733E8DC235279

English descriptors

Abstract

Hyperammonemia and encephalopathy developed in an 11-year-old girl with chronic interstitial lung disease and cholesterol casts in her lung biopsy specimen. She had decreased plasma levels of ornithine, lysine, and arginine and excessive urinary excretion of lysine and arginine, consistent with the diagnosis of lysinuric protein intolerance. Analysis of plasma and urinary amino acids should be considered in the diagnostic evaluation of patients with interstitial lung disease of uncertain origin.

Url:
DOI: 10.1016/S0022-3476(05)81703-2


Affiliations:


Links toward previous steps (curation, corpus...)


Le document en format XML

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<term>Acid analysis</term>
<term>Alveolar proteinosis</term>
<term>Arginine</term>
<term>Biopsy</term>
<term>Chest radiographs</term>
<term>Cholesterol crystals</term>
<term>Cholesterol granuloma</term>
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<term>Heart rate</term>
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<term>Histologic</term>
<term>Hypertension</term>
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<term>Interstitial</term>
<term>Interstitial lung disease</term>
<term>Interstitial process</term>
<term>Interstitial reticulomicronodular pattern</term>
<term>Intolerance</term>
<term>Lipoprotein</term>
<term>Lipoprotein value</term>
<term>Lung biopsy specimen</term>
<term>Lung disease</term>
<term>Lung volume</term>
<term>Lysine</term>
<term>Lysinuric</term>
<term>Lysinuric protein intolerance</term>
<term>Ornithine</term>
<term>Pediatrics</term>
<term>Pediatrics volume</term>
<term>Perheentupa</term>
<term>Plasma levels</term>
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<div type="abstract" xml:lang="en">Hyperammonemia and encephalopathy developed in an 11-year-old girl with chronic interstitial lung disease and cholesterol casts in her lung biopsy specimen. She had decreased plasma levels of ornithine, lysine, and arginine and excessive urinary excretion of lysine and arginine, consistent with the diagnosis of lysinuric protein intolerance. Analysis of plasma and urinary amino acids should be considered in the diagnostic evaluation of patients with interstitial lung disease of uncertain origin.</div>
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