Résumé
Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involve by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief of the literature.
Sujets)
Adulte , Femelle , Humains , Angiomyolipome , Épilepsie , Déficience intellectuelle , Poumon , Lymphangioléiomyomatose , Muscles lisses , Pénétrance , Maladies rares , Complexe de la sclérose tubéreuseRésumé
In order to assess the difference of autonomic dysfunction according to the absence or presence of nephropathy in patients with NIDDM, tests for cardiovascular autonomic function were performed in 23 NIDDM patients without nephropathy(M : F 14 : 9, mean age 50+/- 8) and in 21 patients with nephropathy (M : F 13 : 8, mean age 57+/-12). Tests for cardiovascular autonomic function included the R-R interval changes in response to respiration, Valsalva maneuver, and lying-standing, the blood pressure changes in response to lying-standing and sustained handgrip exercise. The prevalence of autonomic dysfunction was different between the patients without and with nephropathy(69.6% vs 85.7%). For patients without symptoms suggesting autonomic neuropathy, the significant differences were found in the change of R-R interval in response to respiration(22.16+/-21.30 vs 11.73+/-10.83; p=0.0390), in the change of lying-standing blood pressure(10.63+/-9.70 vs 22.00+/-13.67; p= 0.0044) and in the change of blood pressure during handgrip exercise(16.89+/-10.28 vs 8.40+/-7.24; p=0.0065). These results suggested that autonomic neuropathy may occur more frequently in patients with diabetic nephropathy than diabetes mellitus without neuropathy.
Sujets)
Humains , Pression sanguine , Diabète , Diabète de type 2 , Néphropathies diabétiques , Neuropathies diabétiques , Prévalence , Respiration , Manoeuvre de VasalvaRésumé
Neurilemmoma is a benign nerve sheath tumor derived from Schwann cells and occurs in the head, neck, flexor regions of the extremities, posterior mediastinum but extremely rare in the chest wall. They occur at all ages but are most common in persons between the ages of 20 and 50 years and develop spontaneously or possible as a result of trauma or irritation. The diagnosis of a neurilemmoma is confirmed histologically by the findings of Verocay bodies, Antoni A and B tissue patterns and the presence of S-100 protein. We experienced a case of chest wall neurilemmoma which was treated by local excision and report it with a brief review of the literature.
Sujets)
Humains , Diagnostic , Membres , Tête , Médiastin , Cou , Neurinome , Protéines S100 , Cellules de Schwann , Paroi thoracique , ThoraxRésumé
Neurilemmoma is a benign nerve sheath tumor derived from Schwann cells and occurs in the head, neck, flexor regions of the extremities, posterior mediastinum but extremely rare in the chest wall. They occur at all ages but are most common in persons between the ages of 20 and 50 years and develop spontaneously or possible as a result of trauma or irritation. The diagnosis of a neurilemmoma is confirmed histologically by the findings of Verocay bodies, Antoni A and B tissue patterns and the presence of S-100 protein. We experienced a case of chest wall neurilemmoma which was treated by local excision and report it with a brief review of the literature.