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Biomedical subjects

J C Piette

Publications and source records attributed to J C Piette.

At least 145 records · Page 8Linked to original sources

[Spinal cord involvement revealing systemic sarcoidosis].

Spinal neurosarcoidosis is rare (0.43 p. 100 of all sarcoidosis) and can be the initial manifestation of the disease. A 43-year-old right handed African man developed a progressive dorsal neck pain, slowly worsened paresthesia weakness in the legs and a gait disturbance. Magnetic resonance imaging (MRI) scan of the cervical and thoracic spinal cord (sagittal T(1)- weighted image) revealed diffuse enlargement of the cord from C2 to T7 with intense intramedullary enhancement from C2 to T3 after administration of contrast material on sagittal T(1)- weighted image. Cranial MRI scan was normal. Radiographs of the chest revealed bilateral symmetric hilar mediastinal lymphadenopathy with no pulmonary infiltrates. Bronchial biopsy demonstrated non caseating granulomas with langerhans giant cells. The serum angiotensin converting enzyme level was elevated. The patient received corticosteroid with good progressive response. His neurologic symptoms improved markedly and twenty months post-treatment MRI showed no abnormality on enhanced T(1)- weighted images. Two years later he had no relapse. Only 6 to 10 p. 100 of patients with neurosarcoidosis have spinal cord involvement, which may account for the first clinical manifestation of the disease. Most patients with neurosarcoidosis have associated extraneurologic abnormalities. The diagnosis is supported usually by extraneurologic biopsies. Spinal cord biopsy needs to be considered on case-by-case basis.

Adrenal Cortex Hormones↗

[Edematous syndrome revealing an exudative lymphocytic gastritis: efficacy of omeprazole].

Lymphocytic gastritis is characterized by intense lymphocytic infiltration of gastric epithelium. Excessive gastric protein loss is uncommon. We describe the case of a 49-year-old white woman suffering from generalized edema and abdominal pain. She had severe serum hypoproteinemia, hypoalbuminemia and hypogammaglobulinemia. There was no renal, cardiac or hepatic origin of protein loss, and no protein-losing enteropathy. Endoscopic examination showed diffuse varioliform gastritis and histology confirmed lymphocytic gastritis with > 30% intraepithelial lymphocytes without Helicobacter pylori. The protein loss stopped within two weeks of the beginning of omeprazole and extensive edema disappeared. Four years later, the patient was still free from edema. Inflammatory involvement of the gastric mucosa probably caused protein losing in this patient. Recognition of this exsudative gastropathy is important because long term remission is obtained with omeprazole.

Abdominal Pain↗

[Horton's disease and corticosteroid-responsive hearing loss].

BACKGROUND: Temporal arteritis (TA) is a inflammatory panarteritis of the elderly, which may diffuse to all large arteries. Five to 10% of the patients present nonclassical signs with respiratory, vascular, cardiologic, neurologic or hepatic manifestations. We reviewed eighteen cases of TA and deafness in the literature and report two new cases. CASE REPORTS: Our two patients developed sudden, bilateral and corticosteroid responsive hearing loss. One patient developed hearing loss before the diagnosis of TA. CONCLUSION: The mechanism of deafness is probably explained by vertebrobasilar or terminal cochleovestibular arteritis. While the diagnosis of TA is usually easy, it may be difficult in other circumstances. Sensorineural hearing loss may be the first symptom of TA. Prompt recognition and treatment may lead to partial or total improvement of hearing loss in 55% of the cases.

Aged↗

Amyloidosis and cardiac involvement.

BACKGROUND: Amyloidosis is a rare disease characterized by the extracellular accumulation of a protein polysaccharide complex: amyloid. Cardiac involvement may occur with or without clinical manifestations, and is considered as a major prognostic factor. AIM OF THE STUDY: Firstly, to analyze the clinical, electrocardiographic, radiological and echocardiographic features in a group of patients with extracardiac biopsy-proven amyloid infiltration and evidence of echocardiographic amyloid heart disease. Secondly, to compare the survival of amyloidosis patients, with or without cardiac involvement. PATIENTS AND METHODS: We retrospectively analyzed the main echocardiographic features of 47 patients with biopsy proven amyloidosis. No clinical, electrocardiographic, radiological or scintigraphic criterium were selective for cardiac involvement. Thirty patients with echographic features of amyloid heart disease were identified and compared to 17 patients without echographic features of amyloid heart disease. RESULTS: Amyloid disease with heart involvement was AL in 25/30 (83%) patients and occurred more commonly in middle age men (mean age: 53+/-11 years). The main clinical presentation was congestive heart failure (59%), but 37% of patients had no clinical cardiac features. The electrocardiogram was abnormal in 86% and the cardiac silhouette was enlarged on chest roentgenogram in 27% of patients. The main echocardiographic findings were: diffuse ventricular wall thickening in 21 patients (70%) and isolated septal wall thickening in 9 patients (30%); restrictive pattern of left ventricular (LV) diastolic function in 17 patients (57%); pericardial effusion in 12 patients (40%); impaired LV systolic function in 8 patients (27%); atrial enlargement in 8 patients (27%); characteristic granular sparkling of LV myocardium in 8 patients (27%); mitral and/or aortic valve thickening in 4 patients (13%). Cardiac symptoms developed in 72% of the non symptomatic patients having echocardiographic evidence of cardiac involvement. Twenty-five patients died during the study period and the death was due to cardiac disease in 76%. Median survival time was 36 months from time of amyloidosis diagnosis, and it was 23 months from time of amyloid myocardiopathy diagnosis. It shortened to 6 months when congestive heart failure appeared. CONCLUSION: Patients with a histologically proven amyloidosis should be examined by echocardiography, because cardiac involvement is frequently found in patients with no clinical symptoms, and non symptomatic patients having echocardiographic evidence of cardiac involvement will almost always develop cardiac symptoms. Survival actuarial study confirms the significant adverse influence of cardiac involvement in amyloidosis.

Amyloidosis↗

[Efficacy of colchicine in recurrent acute idiopathic pericarditis].

Acute idiopathic pericarditis is complicated by recurrence in 15 to 30% of cases. The preventive treatment of recurrences is not well codified. Aspirin, non-steroidal anti-inflammatory drugs and corticoids are the commonest prescribed treatments. The objective of this study was to assess the value of colchicine in the prevention of recurrences of acute idiopathic pericarditis. Twenty-eight cases of recurrent acute pericarditis (2 episodes, 1 month between each episode) admitted to a department of internal medicine between 1989 and 1999 were reviewed. Analysis was concentrated on the 13 idiopathic forms. The subjects were 7 women and 6 men with an average age of 41 years (10-62) at the time of the first episode of acute pericarditis. These 13 patients were treated with colchicine (1 to 2 mg/day) after failure of conventional treatment (aspirin 13/13, NSAID 13/13, steroids 9/13 and pericardiocentesis 3/13). Ten patients were followed up regularly (6 months after starting colchicine) and were improved with respect to number, duration and intensity of their recurrences. The average duration of colchicine therapy was 17 +/- 14 months (6-48). Progressive withdrawal of NSAID and steroids was obtained in 8/10 cases. The authors conclude that colchicine is useful in the prevention of recurrence of acute pericarditis. It may be proposed as treatment of choice, especially in the idiopathic forms.

Acute Disease↗

[Bone marrow transplantation in the treatment of autoimmune diseases. ISAMAIR Group].

EXPERIMENTAL BASIS AND CLINICAL OBSERVATIONS: Remission of an autoimmune disease has been observed in certain patients after bone marrow allograft from a healthy donor. Autoimmune disease in the donor can also be transmitted to an unaffected recipient. These phenomena would be hematopoietic-dependent. BONE MARROW ALLOGRAFTS: Indications for the treatment of refractory autoimmune diseases are exceptional due to the related mortality even in patients without malignant hematologic disease. A NEW THERAPEUTIC CONCEPT: Therapeutic intensification, followed with autologous peripheral stem cell grafts, a procedure with a mortality below 3% in 1997, constitutes a therapeutic alternative in these difficult indication concerning severe refractory autoimmune diseases including: sclerodermia, vasculitis, lupus, inflammatory myositis, autoimmune cyopenia.

Autoimmune Diseases↗

[Gougerot-Sjögren syndrome. Neurologic complications].

PERIPHERAL NERVE INVOLVEMENT: Peripheral nerve involvement is better known than central nervous system involvement. The dominant features are sensoromotor polyneuropathies or pure sensorial polyneuropathies. CENTRAL NERVOUS SYSTEM: The manifestations are polymorphous, generally presenting as encephalitis and/or focal or multifocal involvement of the spinal cord. OTHER LOCALIZATIONS: Diffuse encephalic involvement is less common: acute aseptic meningitis, intellectual deterioration. Psychiatric manifestations are also frequently observed. THERAPEUTIC OPTIONS: Response to corticosteroid therapy or immunosuppressor therapy can be spectacular but is unpredictable.

Central Nervous System Diseases↗

Octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases.

Chronic intestinal pseudoobstruction (CIPO) is a rare syndrome that may occur in association with connective tissue diseases (CTD). Effective management is a major challenge. We report 3 cases in which subcutaneous octreotide was efficacious in the treatment of digestive symptoms in CIPO. In 2 of the 3 cases, previous treatment with domperidone, cisapride, or erythromycin had been unsuccessful. All 3 patients underwent a regimen of oral antibiotics along with octreotide to stimulate small bowel motility. The effects of octreotide were evident within 48 hours after the first injection in all patients. In 2, the efficacy seemed to decrease after 1 week and 6 months respectively, but increasing the dosage led to another remission. CIPO in CTD is a severe condition that can evolve regardless of the underlying disease activity. Octreotide appears to be efficacious in improving both clinical symptoms and manometric patterns. When its therapeutic effect diminishes, increasing the dosage can be useful.

Adult↗