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N Galie

Publications and source records attributed to N Galie.

17 recordsLinked to original sources
PubMed2006

Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil.

Pulmonary arterial hypertension (PAH) is fatal if untreated. Intravenous epoprostenol improves exercise capacity and haemodynamics in PAH, and increases survival in idiopathic PAH (IPAH). To evaluate the effects of subcutaneous (SC) treprostinil, a longer-acting prostacyclin analogue, followed by the addition of other PAH therapies if needed, 860 PAH patients treated with SC treprostinil for up to 4 yrs were followed. Survival is reported as Kaplan-Meier estimates. For 332 IPAH patients with baseline haemodynamics, observed survival is also compared with predicted survival using the National Institute of Health formula. Out of the 860 patients, 199 (23%) discontinued due to adverse events, 136 (16%) died, 117 (14%) discontinued due to deterioration, 29 (3%) withdrew consent and 11 (1%) underwent transplantation. In total, 97 patients (11%) switched from SC treprostinil to an alternative prostacyclin analogue; bosentan was added in 105 patients (12%) and sildenafil in 25 (3%). In conclusion, survival was 87-68% over 1-4 yrs for all 860 patients and 88-70% over 1-4 yrs with subcutaneous treprostinil monotherapy. For the idiopathic pulmonary arterial hypertension subset with baseline haemodynamics (n = 332), survival was 91-72% over 1-4 yrs. In contrast, predicted survival was 69-38% over 1-4 yrs. The safety profile for long-term subcutaneous treprostinil was consistent with previous short-term trials with no unexpected adverse events.

Adolescent↗
PubMed2001

Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia.

BACKGROUND: Most patients with familial primary pulmonary hypertension have defects in the gene for bone morphogenetic protein receptor II (BMPR2), a member of the transforming growth factor beta (TGF-beta) superfamily of receptors. Because patients with hereditary hemorrhagic telangiectasia may have lung disease that is indistinguishable from primary pulmonary hypertension, we investigated the genetic basis of lung disease in these patients. METHODS: We evaluated members of five kindreds plus one individual patient with hereditary hemorrhagic telangiectasia and identified 10 cases of pulmonary hypertension. In the two largest families, we used microsatellite markers to test for linkage to genes encoding TGF-beta-receptor proteins, including endoglin and activin-receptor-like kinase 1 (ALK1), and BMPR2. In subjects with hereditary hemorrhagic telangiectasia and pulmonary hypertension, we also scanned ALK1 and BMPR2 for mutations. RESULTS: We identified suggestive linkage of pulmonary hypertension with hereditary hemorrhagic telangiectasia on chromosome 12q13, a region that includes ALK1. We identified amino acid changes in activin-receptor-like kinase 1 that were inherited in subjects who had a disorder with clinical and histologic features indistinguishable from those of primary pulmonary hypertension. Immunohistochemical analysis in four subjects and one control showed pulmonary vascular endothelial expression of activin-receptor-like kinase 1 in normal and diseased pulmonary arteries. CONCLUSIONS: Pulmonary hypertension in association with hereditary hemorrhagic telangiectasia can involve mutations in ALK1. These mutations are associated with diverse effects, including the vascular dilatation characteristic of hereditary hemorrhagic telangiectasia and the occlusion of small pulmonary arteries that is typical of primary pulmonary hypertension.

Activin Receptors↗
PubMed1993

Effect of enoximone alone and in combination with metoprolol on myocardial function and energetics in severe congestive heart failure: improvement in hemodynamic and metabolic profile.

The hemodynamic and myocardial metabolic effects of enoximone (phosphodiesterase III inhibitor), alone or in combination with metoprolol (beta-adrenergic blocker), were studied in patients with congestive heart failure. Ten patients (New York Heart Association Class III-IV) underwent right heart and coronary sinus catheterization, and parameters were assessed at basal condition, at peak enoximone response (mean intravenous loading dose = 2.2 mg/kg), and after the combination with metoprolol (mean intravenous dose = 8.5 mg). Heart rate tended to increase during enoximone administration (from 102 +/- 16 to 107 +/- 16 min-1, ns) and was reduced during enoximone plus metoprolol (to 88 +/- 15 min-1, p < 0.05 vs. basal). Cardiac index was increased during enoximone (from 2.2 +/- 0.2 to 3.8 +/- 0.5 1/min/m2, p < 0.05) and decreased during enoximone plus metoprolol (to 2.8 +/- 0.5 1/min/m2, p < 0.05 vs. enoximone). Mean pulmonary wedge pressure fell during enoximone and remained reduced during enoximone plus metoprolol (from 27 +/- 9 to 9 +/- 3 and to 13 +/- 4 mmHg, respectively, both p < 0.05). Myocardial oxygen consumption did not change during enoximone (from 27 +/- 8 to 25 +/- 13 ml/min, ns) and was reduced during enoximone plus metoprolol (to 19 +/- 8 ml/min, p < 0.05 vs. basal). Myocardial lactate extraction tended to be lower during enoximone and during enoximone plus metoprolol conditions (from 38 +/- 17% to 26 +/- 20% and to 29 +/- 24%, respectively), but no statistical significance was found. Myocardial efficiency was increased during enoximone and during enoximone plus metoprolol (from 9 +/- 3% to 15 +/- 6% and to 14 +/- 6%, respectively, both p < 0.05). Thus in patients with congestive heart failure enoximone improves hemodynamics and, in most cases, it does not influence energetics. The addition of metoprolol to enoximone reduces heart rate, cardiac index, and myocardial oxygen consumption without any other major changes, producing a more physiologic hemodynamic and metabolic profile.

Adult↗
PubMedDate not supplied

[Thyroid tuberculosis--a clinical case].

We present the case of a 38-year-old male patient with tuberculosis of the thyroid; the diagnosis was established on histological examination after surgery.

Adult↗
PubMedDate not supplied

[Treatment methods in multiple hepatic, pulmonary and pleural hydatidosis].

The modern treatment of hepato-pleural-pulmonary hydatidosis is based on surgical excision and medical treatment with drugs like: Mebendazol, Albendazol or Praziquantel. There are presented 23 patients with multiple hepato-pleuro-pulmonary hydatidosis, operated in the last 4 years. Surgical excision in pleuro-pulmonary hydatidosis is adapted to cysts topography, aiming to eliminate the intact cysts and to close the remaining cavities.

Adult↗
PubMedDate not supplied

[Multiple, paravertebral Pott's abscesses].

The authors describe a case of vertebral tuberculosis with multiple localizations, high thoracical (T2-T6) and of the sacrum (S1-S4) at a young woman with nonspecific clinical symptoms and with CT examination suggestive of the diagnosis, where the diagnostical certainty was obtained by the bacteriological examination for bK of the pus (purulent liquid) extracted by CT guided fine needle biopsy performed in the posterior mediastinal tumor.

Abscess↗
PubMedDate not supplied

[Pulmonary benign lymphocytic angiitis and granulomatosis--a benign lymphoproliferative condition].

BLAG--benign lymphocytic angiitis and granulomatosis is a granulomatosis disease which histologically presents a dense infiltrate of lympnoid cells that disturbes the normal alveolar architecture. Faint granuloma formation occur within the lympnoid infiltrate. A 32 years female patient was admitted for dry cough, exertional dispnea, low fever during last 11 month. Histological diagnosis was BLAG. The treatment was a combination of cyclophosphamide and prednisone. Follow up information after 9 month of treatment show a generally favorable evolution (clinical and radiological).

Adult↗
PubMedDate not supplied

[Multiple primary cancers with different localizations and histologic types. Clinical case].

The authors describe the case of male patient of 46 years old, which was diagnosticated with 4 cancers of different localization and histopathological types in the course of 6 years. In chronological order these 4 neoplazias were: the Hodgkin's disease with mixed cellularity, the malign parieto-frontal right meningioma, a Grawitz renal tumor and a lung adenocarcinoma with bilateral lung and pleural metastasis. For both cerebral and renal tumors the patient was given a surgical treatment and for the Hodgkin's disease and lung cancer given chemotherapy and cobalt therapy.

Adenocarcinoma↗