ABSTRACT
INTRODUCTION: Light chain deposition disease is a rare anatomo-clinical disorder, which rarely leads to cystic lung destruction. CASE REPORT: We report the case of a 62years old female patient with a history of a monoclonal gammopathy of unknown significance who developed progressive dyspnea. Thoracic CT-scan demonstrated a diffuse pulmonary cystic disorder with predominance in the right lower lobe. Thoracoscopic surgical resection of that lobe led to a diagnosis of non-amyloid kappa light chain deposits. Surgery also resulted in a lung volume reduction effect with clinical and functional benefits related to improved ventilation of adjacent segments. CONCLUSION: This report of pulmonary cystic disorder related to a light chain deposition disease highlights the potential clinical and functional benefits observed after lung volume reduction surgery.
Subject(s)
Cysts/surgery , Immunoglobulin Light Chains/metabolism , Lung Diseases/surgery , Monoclonal Gammopathy of Undetermined Significance/surgery , Protein Aggregation, Pathological/pathology , Cysts/metabolism , Cysts/pathology , Diagnosis, Differential , Female , Humans , Immunoglobulin Light-chain Amyloidosis/diagnosis , Lung Diseases/metabolism , Lung Diseases/pathology , Middle Aged , Monoclonal Gammopathy of Undetermined Significance/complications , Monoclonal Gammopathy of Undetermined Significance/diagnosis , Monoclonal Gammopathy of Undetermined Significance/pathology , Pneumonectomy , Protein Aggregation, Pathological/complications , Protein Aggregation, Pathological/diagnosis , Protein Aggregation, Pathological/metabolism , Treatment OutcomeABSTRACT
The possible relative importance of the membrane and intracellular peptidases of the enterocytes in splitting dietary peptides to amino acids has been analysed. On the anoxic criterion, membrane hydrolysis was found to be predominant. Model experiments revealed cooperative interactions between the membrane enzyme and the coupled transport system. This cooperativity allows the main characteristics of oligomer transport to be described in terms of membrane digestion. Comparison of the behaviour of membrane and intracellular peptidase under different conditions has shown that the former are largely involved in digestion and the latter in intracellular metabolism. It is suggested that the efficiency of the membrane system is high enough to account for the hydrolysis of protein, especially taking into account the stimulation of enzyme processes in the brush border that occurs after ingestion of protein and carbohydrate. A sequential model based on the concept of three interacting enzyme layers (glycocalyx, lipoprotein membrane and cytosol) is presented.