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1.
J Autoimmun ; 96: 134-141, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30236485

RESUMEN

OBJECTIVE: The aim of our study was to assess major cardiovascular event incidence, predictors, and mortality in ANCA-associated vasculitis (AAV). METHODS: We conducted a retrospective cohort study of all GPA or MPA, according to Chapel Hill Consensus Conference classification criteria, diagnosed between 1981 and 2015. Major cardiovascular event was defined as acute coronary artery disease, or ischemic stroke, or peripheral vascular disease requiring a revascularization procedure. We calculated the comparative morbidity/mortality figure (CMF) and we used Cox proportional hazards regression models to assess the risk of coronary artery disease, ischemic stroke associated with AAV, after adjusting for covariates. RESULTS: 125 patients, 99 GPA (79,2%) and 26 MPA (20,8%), were followed 88.4 ±â€¯78.3 months. Ischemic stroke incidence was four times higher than in the general population (CMF 4,65; 95% CI 4,06-5,31). Coronary artery disease incidence was four times higher than in the general population (CMF 4,22; 95% CI 1,52-11,68). Smoking habits and history of coronary artery disease were strongly associated with coronary artery disease occurrence (adjusted HR 8.8; 95% CI 2.12-36.56, and adjusted HR 10.3; 95% CI 1.02-104.5, respectively). ENT flare-up was an independent protective factor for coronary artery disease occurrence. We did not identify factors significantly associated with stroke occurrence. The age-adjusted mortality rate was 22.5 per 1000 person-years. Mortality in AAV was 1.5 times higher than in the general population (CMF 1.56; 95% CI 1.34-1.83). CONCLUSION: AAV have a significantly increased risk of mortality, ischemic stroke, and coronary artery disease.


Asunto(s)
Enfermedad de la Arteria Coronaria/epidemiología , Granulomatosis con Poliangitis/epidemiología , Isquemia/epidemiología , Poliangitis Microscópica/epidemiología , Accidente Cerebrovascular/epidemiología , Enfermedad Aguda , Anciano , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Estudios de Cohortes , Enfermedad de la Arteria Coronaria/mortalidad , Femenino , Estudios de Seguimiento , Granulomatosis con Poliangitis/mortalidad , Humanos , Isquemia/mortalidad , Masculino , Poliangitis Microscópica/mortalidad , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Riesgo , Accidente Cerebrovascular/mortalidad , Análisis de Supervivencia
2.
Lupus ; 28(4): 529-537, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30799679

RESUMEN

INTRODUCTION: Pregnancies in women with lupus nephritis are at high-risk of complications, while scarcity of scientific knowledge on prognostic factors impedes a fair medical counseling. We aimed to identify determinants associated with maternal and fetal complications. MATERIALS: We retrospectively reviewed medical charts of pregnancies that lasted more than 22 weeks in 66 patients with pre-existing lupus nephritis between 2004 and 2013 in France. Univariate and multivariate analyses were conducted to identify determinants for maternal complications, lupus renal flare and fetal prematurity or death. RESULTS: Eighty-four pregnancies were identified. A maternal complication occurred in 31 pregnancies (36.9%): mostly preeclampsia (17 pregnancies, 20.2%) and renal flares (12 pregnancies, 14.3%). Overall fetal survival was 94.0% (79/84). Maternal pregnancy complications were independently associated with prepregnancy body mass index >25 kg/m2 (OR 3.81, 95% CI 1.03-14.09) and immunological activity (positive anti-dsDNA antibodies or Farr assay lupus) (OR 4.95, 95% CI 1.33-18.43). Renal lupus flares were independently associated with maternal age (OR 1.50, 95% CI 1.12-2.01) and prepregnancy immunological activity (OR 15.99, 95% CI 1.57-162.68) while a remission time >12 months had a protective effect (OR 0.17, 95% CI 0.04-0.68). Three parameters were associated with a higher risk of fetal prematurity or death: a prepregnancy body mass index >25 kg/m2 (HR 3.58, 95% CI 1.45-8.83), hypertension (HR 8.97, 95% CI 3.32-24.25), and immunological activity (HR 3.34, 95% CI 1.30-8.63). CONCLUSION: Maternal age, prepregnancy hypertension, body mass index >25 kg/m2 and lupus immunological activity may be considered as the main determinants for fetal and maternal complications. A remission time above 12 months for patients with lupus nephritis could be associated with a reduced risk of renal flare during pregnancy.


Asunto(s)
Nefritis Lúpica/epidemiología , Sobrepeso/epidemiología , Preeclampsia/epidemiología , Complicaciones del Embarazo/epidemiología , Resultado del Embarazo/epidemiología , Adulto , Factores de Edad , Índice de Masa Corporal , Femenino , Francia/epidemiología , Humanos , Hipertensión Renal/epidemiología , Recién Nacido , Recien Nacido Prematuro , Estimación de Kaplan-Meier , Nefritis Lúpica/inmunología , Edad Materna , Análisis Multivariante , Muerte Perinatal/etiología , Embarazo , Pronóstico , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Mortinato/epidemiología , Adulto Joven
3.
Prog Urol ; 24(2): 138-41, 2014 Feb.
Artículo en Francés | MEDLINE | ID: mdl-24485085

RESUMEN

The renal oncocytoma tumors are rare (5% of renal tumors). These benign tumors are incidentally diagnosed most often in an asymptomatic form. Sometimes it is multiple bilateral tumors affecting the renal parenchyma and forming a renal oncocytosis. We report the case of a unilateral right renal oncocytosis, very rare situation, characterized by a right renal parenchymal nodules with oncocytoma and a normal left kidney.


Asunto(s)
Adenoma Oxifílico , Neoplasias Renales , Adenoma Oxifílico/diagnóstico , Adenoma Oxifílico/cirugía , Humanos , Neoplasias Renales/diagnóstico , Neoplasias Renales/cirugía , Masculino , Persona de Mediana Edad
4.
Clin Genet ; 77(3): 258-65, 2010 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-19817772

RESUMEN

The oral-facial-digital syndrome type I (OFD I) is characterized by multiple congenital malformations of the face, oral cavity and digits. A polycystic kidney disease (PKD) is found in about one-third of patients but long-term outcome and complications are not well described in the international literature. Renal findings have been retrospectively collected in a cohort of 34 females all carrying a pathogenic mutation in the OFD1 gene with ages ranging from 1 to 65 years. Twelve patients presented with PKD - 11/16 (69%) if only adults were considered -with a median age at diagnosis of 29 years [IQR (interquartile range) = (23.5-38)]. Among them, 10 also presented with renal impairment and 6 were grafted (median age = 38 years [IQR = (25-48)]. One grafted patient under immunosuppressive treatment died from a tumor originated from a native kidney. The probability to develop renal failure was estimated to be more than 50% after the age of 36 years. Besides, neither genotype-phenotype correlation nor clinical predictive association with renal failure could be evidenced. These data reveal an unsuspected high incidence rate of the renal impairment outcome in OFD I syndrome. A systematic ultrasound (US) and renal function follow-up is therefore highly recommended for all OFD I patients.


Asunto(s)
Envejecimiento , Síndromes Orofaciodigitales/complicaciones , Insuficiencia Renal/etiología , Adolescente , Adulto , Niño , Preescolar , Estudios de Cohortes , Femenino , Estudios de Asociación Genética , Humanos , Lactante , Riñón/patología , Persona de Mediana Edad , Síndromes Orofaciodigitales/genética , Síndromes Orofaciodigitales/patología , Síndromes Orofaciodigitales/fisiopatología , Proteínas/genética , Adulto Joven
5.
Ann Dermatol Venereol ; 134(8-9): 667-71, 2007.
Artículo en Francés | MEDLINE | ID: mdl-17925692

RESUMEN

BACKGROUND: Nephrogenic fibrosing dermopathy is a cutaneous and systemic sclerosis affecting patients with renal failure. CASE-REPORT: A 68-year-old man with renal insufficiency and on dialysis developed hardening of the skin and severe joint contractions. He had previously undergone angiography with gadolinium-containing contrast agents. A skin biopsy confirmed nephrogenic fibrosing dermopathy. The patient was treated by oral steroids followed by extracorporeal photopheresis. An improvement was seen after 12 cycles. DISCUSSION: Treatment of nephrogenic systemic fibrosis is not codified and is normally based on the methods used for other forms of systemic sclerosis. Six cases of patients showing improvement under extracorporeal photopheresis have been published. The physiopathology of the disease is unknown. Gadolinium could act as a triggering agent by attracting circulating fibrocytes in the dermis of patients. Medical authorities recommend avoidance of gadolinium in patients with advanced kidney failure unless strictly necessary.


Asunto(s)
Medios de Contraste/efectos adversos , Gadolinio DTPA/efectos adversos , Fotoféresis , Insuficiencia Renal/complicaciones , Esclerodermia Sistémica/tratamiento farmacológico , Anciano , Biopsia , Fibrosis , Humanos , Angiografía por Resonancia Magnética , Masculino , Diálisis Renal , Insuficiencia Renal/terapia , Esclerodermia Sistémica/etiología , Piel/patología , Resultado del Tratamiento
6.
J Med Genet ; 39(11): 796-803, 2002 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-12414817

RESUMEN

Autosomal recessive distal renal tubular acidosis (rdRTA) is characterised by severe hyperchloraemic metabolic acidosis in childhood, hypokalaemia, decreased urinary calcium solubility, and impaired bone physiology and growth. Two types of rdRTA have been differentiated by the presence or absence of sensorineural hearing loss, but appear otherwise clinically similar. Recently, we identified mutations in genes encoding two different subunits of the renal alpha-intercalated cell's apical H(+)-ATPase that cause rdRTA. Defects in the B1 subunit gene ATP6V1B1, and the a4 subunit gene ATP6V0A4, cause rdRTA with deafness and with preserved hearing, respectively. We have investigated 26 new rdRTA kindreds, of which 23 are consanguineous. Linkage analysis of seven novel SNPs and five polymorphic markers in, and tightly linked to, ATP6V1B1 and ATP6V0A4 suggested that four families do not link to either locus, providing strong evidence for additional genetic heterogeneity. In ATP6V1B1, one novel and five previously reported mutations were found in 10 kindreds. In 12 ATP6V0A4 kindreds, seven of 10 mutations were novel. A further nine novel ATP6V0A4 mutations were found in "sporadic" cases. The previously reported association between ATP6V1B1 defects and severe hearing loss in childhood was maintained. However, several patients with ATP6V0A4 mutations have developed hearing loss, usually in young adulthood. We show here that ATP6V0A4 is expressed within the human inner ear. These findings provide further evidence for genetic heterogeneity in rdRTA, extend the spectrum of disease causing mutations in ATP6V1B1 and ATP6V0A4, and show ATP6V0A4 expression within the cochlea for the first time.


Asunto(s)
Acidosis Tubular Renal/genética , Pérdida Auditiva Sensorineural/genética , ATPasas de Translocación de Protón/genética , Acidosis Tubular Renal/enzimología , Adolescente , Adulto , Niño , ADN/química , ADN/genética , Análisis Mutacional de ADN , Oído Interno/enzimología , Epitelio/enzimología , Femenino , Regulación Enzimológica de la Expresión Génica , Genes Recesivos/genética , Ligamiento Genético , Genotipo , Pérdida Auditiva Sensorineural/enzimología , Humanos , Masculino , Repeticiones de Microsatélite , Mutación , Polimorfismo de Nucleótido Simple , Polimorfismo Conformacional Retorcido-Simple
7.
Arch Intern Med ; 144(11): 2274-6, 1984 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-6093726

RESUMEN

Cholangiocellular carcinoma developed in two uremic patients with polycystic kidney and liver disease, who had been treated with intermittent hemodialysis for one and nine years. In one case, in situ transformation of the liver cyst epithelium into cholangiocellular carcinoma could be demonstrated. The incidence of cholangiocellular carcinoma in patients undergoing long-term dialysis for polycystic kidney and liver disease, however, has yet to be determined.


Asunto(s)
Adenoma de los Conductos Biliares/complicaciones , Hepatopatías/complicaciones , Neoplasias Hepáticas/complicaciones , Enfermedades Renales Poliquísticas/complicaciones , Transformación Celular Neoplásica , Quistes/complicaciones , Femenino , Humanos , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Diálisis Renal
8.
Presse Med ; 34(15): 1069-72, 2005 Sep 10.
Artículo en Francés | MEDLINE | ID: mdl-16334882

RESUMEN

INTRODUCTION: Glomerulonephritis, mainly membranoproliferative or membranous (MG), is observed much less often than interstitial involvement in Sjögren's syndrome (SS). CASE: We report a case of MG revealed by thrombosis of the inferior vena cava and of a renal vein in a 40-year-old woman with primary SS, which began with polyarthritis, immune-type lymphadenopathy, and Hashimoto thyroiditis and did not include obvious sicca syndrome. After failure of moderate-dose steroids and then azathioprine, each over separate 9-month periods, the MG responded well within a few weeks to monthly alternation of methylprednisolone and oral cyclophosphamide for 6 months. DISCUSSION: SS may be an underestimated cause of glomerulonephritis, especially membranoproliferative and membranous glomerulonephritis. They should be considered even in the absence of obvious sicca syndrome. Although the prognosis is usually good, renal insufficiency can occur. In cases of MG, if moderate-dose steroids fail, monthly alternation of methylprednisolone and cyclophosphamide for 6 months appears effective and well tolerated, with a low risk of carcinogenicity.


Asunto(s)
Glomerulonefritis Membranosa/etiología , Síndrome de Sjögren/complicaciones , Administración Oral , Adulto , Antiinflamatorios/administración & dosificación , Antiinflamatorios/uso terapéutico , Antirreumáticos/administración & dosificación , Antirreumáticos/uso terapéutico , Biopsia , Ciclofosfamida/administración & dosificación , Ciclofosfamida/uso terapéutico , Femenino , Estudios de Seguimiento , Glomerulonefritis Membranosa/tratamiento farmacológico , Glomerulonefritis Membranosa/patología , Humanos , Inmunosupresores/administración & dosificación , Inmunosupresores/uso terapéutico , Glomérulos Renales/patología , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Pronóstico , Inducción de Remisión , Síndrome de Sjögren/diagnóstico , Factores de Tiempo
9.
Diabetes Care ; 17(8): 906-8, 1994 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-7956642

RESUMEN

OBJECTIVE: Animal studies suggest that hyperlipidemia may play a direct role in glomerular damage. In patients with non-insulin-dependent diabetes mellitus (NIDDM), dyslipidemia occurs early in the course of nephropathy and may be involved in the progression of renal disease. CASES: We report on two young NIDDM patients with marked hyperlipidemia and proteinuria, in whom renal biopsy demonstrated nondiabetic glomerulopathy. In both cases, the decrease in blood lipid levels was associated with a major decrease in proteinuria. Episodes of hyperlipidemia were associated with a resumption of heavy proteinuria in one patient with serum triglyceride levels and proteinuria being closely correlated. CONCLUSIONS: These two cases suggest that hyperlipidemia has an important role in the pathogenesis of glomerular disease.


Asunto(s)
Diabetes Mellitus Tipo 2/fisiopatología , Glomerulonefritis/patología , Hiperlipidemias/complicaciones , Proteinuria , Adulto , Animales , Biopsia , Glucemia/metabolismo , Colesterol/sangre , Diabetes Mellitus Tipo 2/complicaciones , Diabetes Mellitus Tipo 2/metabolismo , Nefropatías Diabéticas/patología , Dieta con Restricción de Proteínas , Femenino , Glomerulonefritis/complicaciones , Humanos , Hiperlipidemias/metabolismo , Riñón/patología , Masculino , Triglicéridos/sangre
10.
Diabetes Metab ; 29(4 Pt 1): 352-61, 2003 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-14526263

RESUMEN

OBJECTIVES: Vasopressin (VP) is known to be elevated in patients with diabetes mellitus (DM). While the influence of acute hyperglycemia has been ruled out, the mechanism or the osmotically active compound responsible for the increase in VP secretion is still not elucidated. Because the plasma level of several amino acids (AAs) is increased in DM, we evaluated whether AAs could represent an effective osmotic stimulus for VP secretion. RESEARCH DESIGN AND METHODS: In a cross-over study, eight healthy volunteers randomly received an infusion of isotonic saline (control) or mixed AA solution, i.v., at a low or a high rate (2 or 4.5 mg/min/kg BW, respectively). Plasma VP (P(VP)) was measured for two hours before and three hours during AA or control infusion. RESULTS: AA infusion induced a dose-dependent elevation in plasma AA concentration but did not alter P(VP). However, effective plasma osmolality (P(osm)) (osmolality minus urea concentration) remained unchanged because a concommittant fall in plasma sodium concentration (P(Na)), likely due to sodium-linked uptake of AA in peripheral cells, compensated for the rise in plasma AA. CONCLUSION: The stability of effective P(osm) may explain the lack of change observed in P(VP). Because sodium is a very efficient stimulus for VP secretion, it may be assumed that the fall in P(Na) occurring during AA infusion should have reduced VP secretion and thus P(VP). In this setting, the stability of P(VP) suggests that AAs induced an increase in VP secretion which counterbalanced the fall attributable to the decrease in P(Na). In conclusion, in acute experiments, AAs seem to represent an effective stimulus for VP secretion, almost equally potent as sodium. Further studies are needed to evaluate their contribution to the high P(VP) seen in the chronic setting of DM.


Asunto(s)
Aminoácidos/sangre , Aminoácidos/farmacología , Vasopresinas/metabolismo , Adulto , Aminoácidos/administración & dosificación , Estudios Cruzados , Homeostasis , Humanos , Infusiones Intravenosas , Masculino , Concentración Osmolar , Sodio/sangre , Vasopresinas/sangre , Vasopresinas/orina
11.
J Hum Hypertens ; 2(4): 261-4, 1988 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-3070040

RESUMEN

The case of a young woman presenting with a renin-secreting soft tissue sarcoma is described. The primary extrarenal tumour as well as metastatic disease were associated with severe hypertension and both required surgical treatment. The location of these rare malignant tumours and their association with renin-dependent hypertension is discussed. In cases of this type, reappearance of hypertension suggests tumour recurrence.


Asunto(s)
Renina/metabolismo , Sarcoma/metabolismo , Neoplasias de los Tejidos Blandos/metabolismo , Adolescente , Neoplasias Encefálicas/secundario , Neoplasias Encefálicas/cirugía , Captopril/uso terapéutico , Femenino , Humanos , Hipertensión/tratamiento farmacológico , Hipertensión/etiología , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/cirugía , Sarcoma/patología , Sarcoma/cirugía , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/cirugía
12.
J Nephrol ; 12 Suppl 2: S42-6, 1999.
Artículo en Inglés | MEDLINE | ID: mdl-10688401

RESUMEN

Major progress has been achieved in autosomal dominant polycystic kidney disease in the last 30 years; Progress in imaging procedures has been decisive for diagnosis (by ultasonography), management of kidney and liver complications (by CT scan), and investigation and sometimes management of intracranial aneurysms (by MRI-angiography and endovascular treatment procedures). On the other hand, progress in molecular genetics has led to the identification of PKD1 and PDK2 genes, and their respective gene products, polycystin 1 and 2. A two-hit model for cyst formation has recently been put forward. The link between the gene defects and cyst fluid formation and progression is still unknown. In addition, cystic and non-cystic lesions coexist in the disease, underlining that the primary molecular defect is located upstream of the mechanism of cyst formation.


Asunto(s)
Enfermedades Renales Poliquísticas , Animales , Progresión de la Enfermedad , Humanos , Enfermedades Renales Poliquísticas/complicaciones , Enfermedades Renales Poliquísticas/diagnóstico , Enfermedades Renales Poliquísticas/genética , Enfermedades Renales Poliquísticas/terapia , Riñón Poliquístico Autosómico Dominante/complicaciones , Riñón Poliquístico Autosómico Dominante/genética , Riñón Poliquístico Autosómico Dominante/fisiopatología
13.
Clin Nephrol ; 39(3): 125-36, 1993 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-7681736

RESUMEN

UNLABELLED: From 1987 to 1991, 2500 sera were tested for presence of anti-neutrophil cytoplasmic antibodies (ANCA) by standard indirect immunofluorescence (IIF) and specific proteinase 3 (PR3) and myeloperoxydase (MPO) ELISA. Clinical and histological data leading to precise diagnosis were retrospectively obtained in 98 patients with ANCA positivity by IIF and then a comparative study based on ANCA specificity was performed. Vasculitis was present in all cases. Among patients with anti-PR3 (n = 38), 19 had Wegener's granulomatosis (WG), 15 microscopic polyarteritis (mPA), 2 idiopathic necrotizing and crescentic glomerulonephritis (NCGN) and 2 relapsing polychondritis (RP). Among patients with anti-MPO (n = 45), 26 had mPA, 3 classical polyarteritis nodosa (PAN), 5 WG, 8 NCGN, 2 systemic lupus erythematosus (SLE) and one Churg-Strauss syndrome (CSS). Negative MPO and PR3 specific ELISA despite positive IIF were observed in 15 patients (13 WG, 1 mPA, 1 PAN). In the PR3 group, males predominated (66%) and the mean age was 49 years (range 13-85); in the MPO group, females predominated (62%) and the mean age was 57 years (range 13-85). These differences were statistically significant (p < 0.05). Renal involvement was present in 92% of patients and renal biopsy showed pauci-immune necrotizing and crescentic glomerulonephritis in nearly all cases. PR3 specificity was associated with frequent eye involvement (32%) and presence of granulomas (45%), but was not associated with other autoantibodies. MPO specificity was associated with a higher prevalence of pulmonary hemorrhage (40%) and various autoimmune disorders, especially antinuclear antibodies. Cholestasis was observed in 50% of WG with negative MPO and PR3 ELISA. Renal and patient survival at the 75th percentile was 15 months with MPO-ANCA and 16 months with PR3, and was similar for patients with WG and mPA. Relapses occurred in 20% of patients with anti-MPO and 36% of patients with anti-PR3. Serological follow-up was obtained in 44 patients. With immunosuppressive treatment, ANCA disappeared in 66% of cases and this disappearance was always associated with absence of disease activity. IN CONCLUSION: 1. This study confirms that the presence of ANCA is a good marker of vasculitis. 2. Despite some clinical differences, MPO and PR3-associated vasculitis have a similar prognosis. 3. The titer of ANCA determined by ELISA is not correlated with the severity of vasculitis but disappearance of ANCA is always associated with absence of disease activity.


Asunto(s)
Autoanticuerpos/inmunología , Epítopos/inmunología , Glomerulonefritis/inmunología , Granulomatosis con Poliangitis/inmunología , Inmunoglobulina G/inmunología , Poliarteritis Nudosa/inmunología , Anticuerpos Anticitoplasma de Neutrófilos , Biomarcadores , Ensayo de Inmunoadsorción Enzimática , Femenino , Técnica del Anticuerpo Fluorescente , Granulomatosis con Poliangitis/diagnóstico , Humanos , Masculino , Persona de Mediana Edad , Mieloblastina , Peroxidasa/inmunología , Poliarteritis Nudosa/diagnóstico , Serina Endopeptidasas/inmunología
14.
Clin Nephrol ; 47(5): 281-8, 1997 May.
Artículo en Inglés | MEDLINE | ID: mdl-9181274

RESUMEN

The outcome and consequences of pregnancy in women with impaired renal function are still debated. To assess the benefit of recent advances in coordinated obstetrical and nephrologic management, we analyzed fetal and maternal outcome of 43 pregnancies in 30 women with various types of primary renal disease and moderate to severe renal failure at conception defined by serum creatinine concentration (Scr) ranging from 0.11 to 0.49 mmol/l. All pregnancies took place during the 20-year period from 1975 through 1994 and were prospectively followed jointly by our Nephrology Unit and Obstetric and Neonatology Units of University Hospitals. Of the 43 pregnancies (45 fetuses), 13 ended in fetal death (including 5 first-trimester abortions and 8 fetal deaths beyond the 20th gestational week). There were 32 live births, a success rate of 82% not considering first-trimester abortions. Successful pregnancies were significantly more frequent in the last decade than in the preceding one (91 vs 65%, p = 0.05). Overall live birth rate was higher in pregnancies started with Scr < 0.20 mmol/l than in those with Scr > 0.20 mmol/l (80% vs 53%, p = 0.02). The upper preconception Scr value associated with a successful fetal outcome was 0.27 mmol/l. Hypertension was the major factor of fetal prognosis, as the relative risk of fetal loss was 10.6 times higher when hypertension was present at conception or early in pregnancy than when blood pressure was spontaneously normal or well-controlled by therapy. An accelerated course toward end-stage renal failure was observed in 7 patients (23%), all of whom had severe hypertension and heavy proteinuria at conception. We conclude that fetal outcome in patients with impaired renal function has been improved in recent years, due to advances in obstetrics and neonatology, improved blood pressure control and close co-operation between nephrologists and obstetricians, but that a risk of fetal loss and of accelerated deterioration of maternal renal disease still persists when Ccr at conception is lower than 25-30 ml/ min/1.73 m2.


Asunto(s)
Muerte Fetal/etiología , Fallo Renal Crónico/fisiopatología , Complicaciones del Embarazo/fisiopatología , Adulto , Creatinina/metabolismo , Femenino , Humanos , Hipertensión/fisiopatología , Fallo Renal Crónico/complicaciones , Embarazo , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Resultado del Embarazo , Estudios Retrospectivos
15.
Adv Exp Med Biol ; 336: 273-9, 1993.
Artículo en Inglés | MEDLINE | ID: mdl-7905232

RESUMEN

Clinical and histological data leading to precise diagnosis were retrospectively obtained in 98 patients with antineutrophil cytoplasmic antibodies (ANCA) detected by indirect immunofluorescence (IIF). Specificity was determined by myeloperoxidase (MPO) and proteinase 3 (PR3) specific ELISA in all and a comparative study based on ANCA specificity was performed. Vasculitis was present in all cases. PR3-ANCA occurred predominantly in males (25/38) with WG (19/38). MPO-ANCA occurred predominantly in older women and were often associated with various autoimmune disorders. There was a high prevalence of lung hemorrhage (18/45) and mPA (26/45) in this group. Patients with negative MPO and PR3 specific ELISA despite positive IIF (n = 15) were almost exclusively WG (13/15) and were characterized by a high prevalence of hepatic and digestive manifestations. Renal and patient survival at the 75th percentile was 15 months with MPO-ANCA and 16 months with PR3, and was similar for patients with WG and mPA. With immunosuppressive treatment, ANCA disappeared in 66% of cases and this disappearance was always associated with absence of disease activity.


Asunto(s)
Autoanticuerpos/sangre , Vasculitis/inmunología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anticuerpos Anticitoplasma de Neutrófilos , Especificidad de Anticuerpos , Enfermedades Autoinmunes/inmunología , Ensayo de Inmunoadsorción Enzimática , Femenino , Técnica del Anticuerpo Fluorescente , Glomerulonefritis/inmunología , Granulomatosis con Poliangitis/inmunología , Humanos , Enfermedades Pulmonares/inmunología , Masculino , Persona de Mediana Edad , Mieloblastina , Peroxidasa/inmunología , Poliarteritis Nudosa/inmunología , Estudios Retrospectivos , Serina Endopeptidasas/inmunología
16.
Arch Mal Coeur Vaiss ; 84(8): 1171-5, 1991 Aug.
Artículo en Francés | MEDLINE | ID: mdl-1953265

RESUMEN

UNLABELLED: The diagnosis of remediable renovascular hypertension (RVH) requires demonstration of lateralization of renal vein renin (RVR). In order to increase the accuracy of RVR ratio, we investigated the acute effects of a bolus of Nicardipine (Nic.: 4 mg i.v.) on hemodynamics and RVR in 19 patients. 13 patients had an unilateral renal artery stenosis greater than 75% (RVH: 10 atheroma and 3 fibrodysplasia) and 6 patients had essential hypertension (EH). In 6 patients (5 RVH and 1 EH) treatment could not be discontinued and only a monotherapy by central alpha-agonists was prescribed. RVR samples were obtained 15 minutes after renal vein catheterization (baseline values: T0) and 10 minutes after Nic. injection (T10). During the whole procedure, mean blood pressure (MBP) and heart rate (HR) were monitored every 2 minutes by an automatic device. Active renin was measured by a new immunoradiometric assay. A RVR ratio (stenotic/contralateral side) greater than 1.5 was considered as a positive ischemic index. RESULTS: The relative changes in MBP and HR between T0 and T10 were of same magnitude in both groups. No patient suffered any untoward effect from the fall in MBP. Nic. increased RVR release from both sides in RVH group as well as in the EH group. At baseline, 6/13 of the RVH patients and none of the EH patients had a RVR greater than 1.5. After Nic. injection, all the RVH patients had a RVR greater than 1.5 and none among EH patients. We conclude that single i.v. Nic. bolus is a safe and a reliable procedure which increases diagnostic accuracy by enhancing RVR when there is an unilateral RVH disease.


Asunto(s)
Hipertensión Renovascular/diagnóstico , Nicardipino , Adulto , Anciano , Protocolos Clínicos , Femenino , Humanos , Hipertensión/diagnóstico , Hipertensión Renovascular/etiología , Inyecciones Intravenosas , Masculino , Persona de Mediana Edad , Nicardipino/administración & dosificación , Renina/sangre
17.
Gastroenterol Clin Biol ; 22(1): 50-4, 1998 Jan.
Artículo en Francés | MEDLINE | ID: mdl-9762166

RESUMEN

OBJECTIVES: Polycystic liver disease is sometimes responsible for chronic symptoms linked to hepatomegaly which can result in acute complications such hemorrhage or infection of cysts. The aim of this retrospective study was to evaluate the results of partial hepatic resection in patients with symptomatic or complicated polycystic liver disease. METHODS: Twelve patients (11 women and one man, mean age 49) with diffuse polycystic liver disease were treated by partial liver resection (left lateral lobectomy in 7, left hepatectomy in 4, and extended right hepatectomy in 1). Four patients had terminal renal failures and three had chronic haemodialysis. Median follow-up was 34 months. RESULTS: Ascites occurred postoperatively in 10 patients (83%) and was long-lasting (> 2 weeks) in 5; all patients with end-stage renal failure had long-lasting ascites. One of them died on the 40th postoperative day of ascites infection. Another patient with end-stage renal failure died two years postoperatively from chronic disabling ascites and malnutrition while awaiting kidney transplantation. The 10 other patients were markedly improved after partial liver resection, including a marked decrease in hepatomegaly, and the disappearance of chronic symptoms and cystic complications. This beneficial effect was incomplete in the two surviving patients with end-stage renal failure until kidney transplantation was performed. CONCLUSION: These results suggest that partial liver resection is a highly effective treatment in patients with symptomatic polycystic liver disease, preferably before the onset of end-stage renal failure.


Asunto(s)
Quistes/cirugía , Hepatopatías/cirugía , Adulto , Anciano , Femenino , Humanos , Complicaciones Intraoperatorias , Hepatopatías/complicaciones , Masculino , Persona de Mediana Edad , Enfermedades Renales Poliquísticas/complicaciones , Enfermedades Renales Poliquísticas/cirugía , Complicaciones Posoperatorias , Estudios Retrospectivos , Resultado del Tratamiento
18.
Gastroenterol Clin Biol ; 19(12): 1011-7, 1995 Dec.
Artículo en Francés | MEDLINE | ID: mdl-8729413

RESUMEN

Von Hippel-Lindau's disease is a rare genetic disease, with an autosomal dominant mode of inheritance, characterised by the development of several tumours, such as haemangioblastoma of the central nervous system and retina, renal cysts or carcinoma and pheochromocytoma. Several pancreatic lesions, mainly represented by multiple cysts, are also encountered. We report here 8 cases of pancreatic involvement in patients affected with von Hippel-Lindau's disease. It consisted of multiple cysts (3 cases), serous cystadenoma (2 cases), endocrine tumour (1 case), haemangioblastoma (1 case) and ductal adenocarcinoma (1 case). Diagnosis of a rare lesion of the pancreas (multiple cysts, serous cystadenoma or vascularized tumour) in a young patient may lead to search the other lesions of von Hippel-Lindau's disease and to undergo a familial inquiry in order to propose a multidisciplinary approach for patients affected by the disease.


Asunto(s)
Adenocarcinoma/etiología , Cistadenoma Seroso/etiología , Hemangioblastoma/etiología , Quiste Pancreático/etiología , Neoplasias Pancreáticas/etiología , Enfermedad de von Hippel-Lindau/complicaciones , Adulto , Cistadenoma Seroso/diagnóstico por imagen , Cistadenoma Seroso/cirugía , Endoscopía del Sistema Digestivo , Resultado Fatal , Femenino , Hemangioblastoma/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Quiste Pancreático/diagnóstico por imagen , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/patología , Neoplasias Pancreáticas/cirugía , Tomografía Computarizada por Rayos X , Ultrasonografía , Enfermedad de von Hippel-Lindau/genética
19.
J Radiol ; 75(10): 543-52, 1994 Oct.
Artículo en Francés | MEDLINE | ID: mdl-7799278

RESUMEN

The aim of this study was to assess the magnetic resonance imaging (MRI) characteristics of 13 benign complex renal cysts using T1 and T2-weighted images and contrast-enhanced images. The results have been compared to CT and ultrasonographic findings in all cases and correlated with histopathologic data in 12 cases. Five groups have been defined according to the MR features. Group 1: homogeneous low signal intensity on T1-weighted images and homogeneous high signal intensity on T2-weighted images mimicking simple cyst (n = 2); group 2: homogeneous high signal intensity on both T1 and T2-weighted images mimicking hemorrhagic cyst (n = 1); group 3: caracterised by high signal intensity on T1-weighted images and fluid-iron level on T2-weighted images (n = 3); group 4: characterised by fluid-iron level on both T1 and T2-weighted images (n = 3); group 5: pseudotumoral feature: heterogeneous signal intensity and/or wall contrast enhancement (n = 3). Among the 13 indeterminate lesions on ultrasonography and CT, MRI was of diagnostic value in 8 cases, whereas the 5 remaining cases remained indeterminate on MR images. Our results suggest that MRI can be useful in the diagnosis of benign complex cyst of the kidney presenting as indeterminate cystic lesion on other modalities.


Asunto(s)
Enfermedades Renales Quísticas/diagnóstico , Imagen por Resonancia Magnética , Adulto , Anciano , Femenino , Humanos , Enfermedades Renales Quísticas/clasificación , Enfermedades Renales Quísticas/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
20.
Presse Med ; 16(6): 295-8, 1987 Feb 21.
Artículo en Francés | MEDLINE | ID: mdl-2950465

RESUMEN

Thirty-one cases of post neurosurgical bacterial meningitis from one center were reviewed to determine their promoting factors and responsible organisms. Most cases occurred after a protracted operation (6 hours on average). 26% cases were related to a primary cutaneous infection. 58% organisms were Gram negative bacilli. The course of meningitis was often complicated or prolonged due to a scalp decubitus ulcer or cerebrospinal fluid leakage. Vancomycin is often requested during staphylococcal meningitis. Third generation cephalosporins have low MIC against many Gram negative bacilli and should be used. Pefloxacin, a new quinolone, may be valuable. Treatment of scalp wound infection is often requested.


Asunto(s)
Enfermedades del Sistema Nervioso Central/cirugía , Meningitis/etiología , Complicaciones Posoperatorias/etiología , Adolescente , Adulto , Anciano , Antibacterianos/uso terapéutico , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Meningitis/líquido cefalorraquídeo , Meningitis/tratamiento farmacológico , Persona de Mediana Edad , Estudios Retrospectivos
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