Lung involvement in the multisystem syndrome CHARGE association.
نویسندگان
چکیده
The CHARGE association is a multisystem syndrome, with a wide range of phenotypic expression, causing mortality, especially in childhood. We performed a hospital audit, in order to quantify the pulmonary implications, in 28 boys and 19 girls aged 0.02-23 yrs, with a definite diagnosis of CHARGE. A review of the records of these children with CHARGE association revealed that aspiration was common during infancy, as a result of inco-ordination of swallowing and gastro-oesophageal reflux. Aspiration was suspected in 22 of the 47 cases (47%), recurrent chest infections occurred in 22 cases (47%), and lung involvement contributed to 7 out of 17 deaths (41%). We conclude that respiratory morbidity and mortality is common in CHARGE, and decreases with age. Early diagnosis and treatment affords the best prognosis.
منابع مشابه
A case report of Churg-Strauss syndrome in peripartum period
Allergic granulomatous angiitis (Churg-Strauss syndrome) is a variant of systemic vasculitis characterized by pulmonary and systemic small vessel vasculitis, extra-vascular granulomas and eosinophilia. Clinical features include presence of asthma and/or allergic rhinitis with constitutional symptoms such as fever and weight loss. Multisystem involvement includes pulmonary infiltrations, m...
متن کاملدرگیری پارانشیم ریه در بیماران سیستمیک اسکلروزیس در بیمارستان نور و الزهرای اصفهان
Background & Aim:Systemic sclerosis is a multisystem autoimmune disorder with multiple organ involvements characterized by inflammation and fibrosis involving the skin as well as internal organs. Pulmonary involvement consists of interstitial fibrosis and pulmonary vascular disease has an important effect on the course and survival of these patients. In this study, we investigated the prev...
متن کاملAssociation between atherosclerosis and parenchymal lung involvement in systemic sclerosis
Background: Systemic sclerosis is an autoimmune disease affecting connective tissues, (including epidermal, subepidermal, microvasculature, etc.), leading to various extent of end-organ damage. The leading cause of mortality among these patients is lung involvement. The cardiovascular events happen more frequently in patients suffering systemic scleroderma, comparing to healthy population. This...
متن کاملCombined pulmonary involvement in hereditary lysozyme amyloidosis with associated pulmonary sarcoidosis: a case report.
Sarcoidosis is a multisystem inflammatory disorder of unknown cause which can affect any organ system. Autosomal dominant lysozyme amyloidosis is a very rare form of hereditary amyloidosis. The Arg64 variant is extraordinarily rare with each family showing a particular pattern of organ involvement, however while Sicca syndrome, gastrointestinal involvement and renal failure are common, lymph no...
متن کاملCarpal Tunnel Syndrome in Association with Klippel-Trénaunay Syndrome: A Case Report
Klippel-Trénaunay syndrome is a rare congenital condition that rarely affects peripheral nerves. Median nerve involvement at carpal tunnel level has only been reported in a few occasions in the medial literature. A 61 years old Caucasian female patient with Klippel-Trénaunay syndrome presented with a 10 months history of paraesthesiae and numbness affecting the median nerve distribution area...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- The European respiratory journal
دوره 10 6 شماره
صفحات -
تاریخ انتشار 1997