[Renal vascular lesions and the occurrence of hypertension in patients with IgA nephropathy]. / Lésions vasculaires rénales et développement d'une HTA chez les patients atteints d'une glomérulonéphrite à dépôts mésangiaux d'IgA. Une histoire familiale d'hypertension artérielle.

Worse prognosis of IgA nephropathy (IgAN) is associated to hypertension, high proteinuria, glomerular and vascular sclerosis. A family story of hypertension (FHT) in relatives could be a strong predictor of the occurrence of hypertension (HT) in children. Renal vascular lesions (RVL) are often observed in normotensive patients with IgAN. In order to evaluate a possible association between FHT and LVR in patients with IgAN, we investigated two groups of 73 IgAN patients, sex (56 males and 17 females) and age matched, according to the presence or not of FHT. FHT was diagnosed if relatives and/or at least one child under 60 years of age had treatment for HT or systolic and diastolic BP over 140/90 mmHg at the time of the survey. Patients entering into the study were followed during an average period of 5 to 8 years. At the end of the study, all patients were explored for HT and renal function. Creatinine clearance (CrCl) was evaluated by Cockcroft and Gault formula and renal failure was defined as CrCl<60mL/min. The results were as follow: at the time of renal biopsy, RVL were observed in 73% of males with FHT vs 16% of males without FHT (p<0.0001) and 70.6% of females with FHT vs 29.4% of females without FHT (p<0.001); at the end of the study period, HT was significantly associated to FHT in 89.6% of patients group with FHT vs 22.6% of HT patients in the group without FHT (p<.0001). Renal failure was present in 45.2% of patients with FHT vs 4.1% of patients without FHT (p<0.0001). These data suggest: VRL could be dependent of genetic factors; FHT should be an early predictor of VRL in patients with IgAN; FHT might be a risk factor for renal failure in patients with this renal disease.

[Polyradiculoneuropathy in an adult with primitive hyperoxaluria]. / Polyradiculoneuropathie au cours d'une hyperoxalurie primitive de l'adulte.

From the age of 31 a patient began to suffer from recurrent calcium oxalate urolithiasis. Liver biopsy showed a decrease in catalytic activity of the hepatic peroxisomal enzyme alanine: glyoxilate aminotransferase (AGT), which was mistargeted from peroxisomes to mitochondria. The genetic analysis revealed a mutation of the AGT gene. At age 47 he developed end-stage renal failure and underwent hemodialysis. After 12 months of hemodialysis he presented a rapidly declining clinical condition, a decrease of the residual renal function, a livedo reticularis with painful of extremities, and shortly thereafter a general weakness, which predominated on lower limbs. Apart from renal failure, routine biological examination and CSF were normal. Nerve conduction studies and electromyography supported the diagnosis of polyradiculoneuropathy. Pathological studies revealed mixed demyelinating-axonal lesions and deposits of calcium oxalate crystals within the media and the intima of epineural arterioles. A combined liver-kidney transplant was rapidly performed. The patient's condition improved in a few months and motor signs completely disappeared.

[Arterial hypertension in the hemodialysis patient. A model of salt-sensitive hypertension in man]. / L'hypertension artérielle du patient hémodialysé. Un modèle d'hypertension sensible au sel chez l'homme.

In uremic patient treated by hemodialysis (HD), a low potassium intake and a salt load due to diet and or a high sodium concentration in dialysate are often associated to refractory hypertension. Numerous reports in general population, based on epidemiologic and demographic data, have pointed to the relationship between sodium intake and hypertension. The degree of blood pressure fall in patients who have evidence of salt-sensitivity varies directly with the severity of the hypertension, being most prominent in those with higher pressures. Recent studies have suggested that a reduction of dialysate sodium can control hypertension in maintenance haemodialysis patients. In this study, five hypertensive haemodialysis patients were assigned to a regime of lowering the dialysate sodium concentration from 142 to 135 mmol/L in combination with an attempt to lower salt intake by advising the patients to eat a NaCl-restricted diet of no more than 6-8 g/day. During the period under study, dialysis time was kept constant. A significant increase of ultrafiltrate sodium concentration was observed during the first week after lowering the dialysate sodium concentration. Post dialysis systolic and diastolic pressures showed a clear trend to fall (systolic pressure 174 +/- 18 vs 118 +/- 13 mmHg, diastolic pressure 96 +/- 7 vs 75 +/- 13 mmHg) without a change of dry weight. The reduction of the mean arterial pressure on 48 h was demonstrated with ambulatory blood pressure recording. The results of this study suggest that reducing the dialysate sodium concentration lead to a decrease in peripheral resistance. A link between sympathetic overactivity as it is found in haemodialysis patients and sodium load could be a stimulating hypothesis. It is concluded that increasing dialysate sodium in short dialysis is responsible for the high prevalence of arterial hypertension often insufficiently controlled by antihypertensive medication. In hemodialysis patients with refractory hypertension, the lowering of the dialysate sodium concentration is indicated.

[Ischemic renal diseases have become the most frequent causes of end stage renal disease in the elderly]. / La néphroangiosclérose et la néphropathie ischémique athéromateuse sont devenues les causes les plus fréquentes d'insuffisance rénale terminale chez le sujet âgé de plus de 60 ans.

In the 80s it was established that atherosclerotic renal artery stenosis (ARAS) is a leading cause of renal insufficiency and that this condition ranks among the rare etiologies of chronic renal failure amenable to improvement or stabilization particularly in the white. Nephroangiosclerosis (NAS) is an increasing cause of ESRD in the western countries, especially in blacks. Epidemiological data on the vascular nephropathies leading to ESRD are still rare. In this study, we compare annual incidence of ESRD due to ARAS and NAS during two five-year periods: period A = 1982-1986, period B = 1992-1996. The region of the survey comprised 410,664 inhabitants (99.6% of Caucasians), of whom 100,230 were aged over 60 years. Diagnosis of ARAS required arteriography and that of NAS a renal biopsy. Undetermined vascular nephropathy was diagnosed when ESRD patients had had previously no arteriography or no histological examination. Major results were as follow (A vs B, incidence = n/million inhabitants): 1) Increasing incidence of ESRD due to all causes: 76 vs 95 per million, mean age at ESRD 56 vs 62 yrs, percentage of patients over 65 yrs 28 to 59% (p < 0.001). 2) Increasing incidence of ESRD due to vascular nephropathies: 5.5 vs 27.5 per million (p < 0.0001) in general population and 22 vs 110 per million (p < 0.0001) in population aged over 60 years, mean age at ESRD 68 vs 73 yrs. 3) Increasing incidence of different types of ischemic renal diseases leading to ESRD: ARAS 2.5 vs 15 per million (p < 0.0001) in general population and 10 vs 60 per million (p < 0.001) in those aged over 60 yrs, mean age 69 vs 74 yrs, NAS: 1 vs 8 and 4 vs 32 per million (p < 0.001), mean age 67 vs 72 yrs, undetermined VN 0.5 vs 2.5 and 2 vs 10 per million, 65 vs 73 yrs. Our study demonstrates that ischemic renal diseases 1) have become the most frequent causes of ESRD (27% of all patients and 43% of those aged over 6C years) in the Caucasian elderly. 2) are the only cause of increasing incidence of ESRD in this French region.

[Intravascular malignant B-cell lymphomatosis with renal glomerular involvement: a case]. / Lymphomatose maligne intravasculaire de type B avec atteinte rénale glomérulaire: une observation.

We report a case of intravascular malignant lymphomatosis observed in a 71 year-old male and characterised by the presence of a proteinuria in relation to the specific intraglomerular localisation. This malignant lymphoma, usually of the B phenotype, is rare and affects predominantly the central nervous system and the skin. Neoplastic cells home selectively to endothelium. Histological renal infiltration is frequent but a glomerular localisation, with proteinuria, is rare. The mechanism whereby lymphocytes home to endothelium cells is unclear but it could be related to the expression of lymphocyte-endothelium adhesion molecules. When present the nephrotic syndrome is associated with minimal change disease.

[A familial history of hypertension is associated with the development of hypertension and nephroangiosclerosis in patients with glomerulonephritis caused by mesangial IgA deposits]. / Une histoire familiale d'hypertension artérielle est associée à la survenue d'une hypertension artérielle et d'une néphro-angiosclérose chez les patients atteints de glomérulonéphrite à dépôts mésangiaux d'IgA.

The progress of IgA Nephropathy (IgAN) is correlated with glomerular and vascular sclerosis. Renal vascular lesions, i.e. nephrosclerosis, often precede the onset of hypertension (HBP) in young patients with IgAN. It is also recognized that a family history of HBP (FHBP) is strongly predictive of future onset of HBP in family members, when two or more first-degree relatives with HBP are identified. In order to examine the possible link between FHBP and nephrosclerosis, we compared 2 groups of 29 pts each (23 M and 6 F) with IgAN, matched for age and sex, according to the presence or absence of FHBP. FHBP was considered present if at least 2 or more 1st degree relatives under 60 years of age received antihypertensive Rx. Parents and siblings of patients were examined at home by two investigators. Patients with FHBP (+) and FHBP (-) were aged 36 +/- 12 and 35 +/- 12, respectively, at the time of renal biopsy and the follow-up was conducted for an average of 4.6 years. At the end of this survey, HBP and renal failure (Cr Cl < 80 ml/min) were reevaluated in all patients. At the time of renal biopsy, nephrosclerosis was significantly associated with FHBP: FHBP (+): 96.5% versus FHBP(-): 10.3%; p < 0.0001. At the end of the follow-up, FHBP was found to be associated with HBP (89.6% versus 10.3%; p < 0.001) and with renal failure (44.8% versus 3.4%; p < 0.001). These data suggest that nephrosclerosis has a strong genetic component in patients with IgAN, FHBP is an early clinical indicator of nephrosclerosis in these patients and that FHBP is a strong indicator of unfavorable prognosis in IgAN.

Calcium free hemodialysis: experience in the treatment of 33 patients with severe hypercalcemia.

OBJECTIVE: To assess the efficacy, adverse effects and relevance of calcium-free hemodialysis (CFHD) in the treatment of major hypercalcemia. DESIGN: Retrospective chart review. SETTING: Medical ICU. PATIENTS: All patients admitted over a 9-year period for hypercalcemia requiring urgent treatment and who underwent hemodialysis. INTERVENTIONS: CFHD with an acetate dialysate. MAIN RESULTS: Thirty-three patients with severe hypercalcemia from various etiologies received CFHD. Marked and rapid decrease of serum total calcium was obtained during all sessions (mean decrease: 1.71 +/- 0.54 mmol/l). Calcium rebound within 24 h after CFHD was observed in all evaluable cases (1 +/- 0.45 mmol/l; mean delay 13.7 +/- 5.8 h). Adverse cardiovascular effects occurred in 17 of 48 sessions (35%) and in 13 of 30 evaluable patients (43%). CONCLUSIONS: Adverse effects are frequent during CFHD. After correction of hypovolemia, its use should be restricted to patients with severe clinical symptoms or advanced renal impairment.

[Epidemiologic data, clinical and prognostic features of autosomal dominant polycystic kidney disease in a French region]. / Données épidémiologiques, cliniques et pronostiques de la maladie polykystique autosomique dominante dans une région française.

Epidemiological study on autosomal dominant polycystic kidney disease (ADPKD) was undertaken in a French region from 1988 to 1993. This survey was led in a population of 410,000 inhabitants and 84 kindreds with ADPKD and 296 affected members were studied. Prevalence of ADPKD in the studied region was calculated to 1/1111 inh. Renal prognosis was evaluated according to the Kaplan-Meier method in 296 affected subjects of whom 212 were members of propositus kindreds. In our region 17% of patients had ESRD by the age of 50 years, 47% by the age of 60 years and 70% by the age of 70. No significance difference was found between males and females. The influence of the sex of the parent from whom ADPKD was received on the renal prognosis of the disease in affected descendants was evaluated. Anticipation of ESRD for at least one offspring inheriting ADPKD from parent was found in 15 (38%) out of 39 families, without genetic imprinting linked to gender. Mean survival to ESRD for fathers transmitting ADPKD to offspring (52 +/- 10 years) was significantly earlier compared to that for mothers transmitting ADPKD (61 +/- 10 years, p < 0.001), therefore that for siblings inheriting the disease from their fathers (sons: 49 +/- 7 years, daughters: 51 +/- 9 years) and for those inheriting ADPKD from their mothers (sons: 57 +/- 10 yrs, p < 0.01, daughters: 55 +/- 6 yrs, p < 0.02). Prevalence of de novo mutations was evaluated to 1/135,000 inh. Adult polycystic disease of the liver (APLD) was studied in 82 kindreds with 158 ADPKD affected members by ultrasonography and/or CT. In patients with APLD, 49/84 (58.3%) were females compared to 46/74 (62.2%) in those without APLD. Familial APLD (at least 2 affected members and all with APLD) was demonstrated in 22/27 APLD kindreds (81.5%). Familial ADPKD without APLD (at least 2 affected members and all without APLD) was demonstrated in 12/12 kindreds (100%). Renal prognosis of ADPKD in 84 APLD pts was compared to that in 71 non-APLD pts, in whom mean age was not different at the time of the study. In APLD pts 28/84 (33.3%) had reached ESRD compared to 23/71 (32.3%) non-APLD pts (ns). The occurrence of stroke in ADPKD patients was documented in 24/231 pts (10.4%) from 11/82 kindreds (13.4%). Family history of cerebro-vascular event was found in 4/11 kindreds (36%).

[Epidemiology of primary glomerulopathies in a French region. Variations as a function of age in patients]. / Epidémiologie des glomérulopathies primitives dans une région française. Variations en fonction des périodes et de l'âge des sujets.

Between January 1st, 1976 and December 31st, 1990, histological diagnosis of primary glomerular diseases (PGD) was made in 480 patients born and living at the time of diagnosis in a region of France comprising 410,664 inhabitants, of whom 390,574 were aged from 10 to 80 years. The prevalence of PGD during a 70-year exposure to risk (10-80 years of age) was evaluated to 5.7 in 1000 (7.6 in 1000 males and 3,8 in 1000 females). The most common PGD was IgA nephropathy with a prevalence of 1.9 in 1000 (3.3 in 1000 males, 1 in 1000 females). The annual incidence of the disease was evaluated separately for 3 consecutive 5-year periods: period A (1976-80), period B (1981-85), and period C (1986-90). Within each of these 3 periods the number of patients with PGD was 179, 170 and 131 respectively, and annual incidence was 9.3, 8.8 and 6.7 in 100,000. The incidence of IgA nephropathy remained the same throughout the 3 periods: 2.6, 3.1 and 2.5 in 100,000. The incidence of membranoproliferative glomerulonephritis decreased from 1981 onward (0.9, 0.5 and 0.15/100,000). Acute streptococcal glomerulonephritis virtually disappeared during periods B and C. Lipoid nephrosis was less frequent in period C and idiopathic proliferative glomerulonephritis with crescents slightly increased (0.3, 0.3 and 0.6 in 100,000). There was no significant difference between the 3 periods regarding the incidence of other PGD. Incidence of IgA nephropathy was 3 to 4-fold higher in the adult aged from 20 to 60 years than in the elderly. In contrast, membranous nephropathy was 3 fold more frequent in the elderly than in the adult. Therefore only some histopathological forms have a different incidence according to age, but the major information furnished by this study is that the risk of occurrence of a PGD is similar in the population living in the area, whatever the age group (10-19 years: 6.4/10(5) inhabitants, 20-39: 7.1/10(5), 40-59: 8.4/10(5), 60-79: 8.4/10(5)). Finally, we confirm that the most common PGD going to end stage renal disease was IgA nephropathy, particularly under 60 years of age (0.8/10(5)). In contrast, membranous nephropathy was a less frequent cause of ESRD (0.2/10(5)).

Epidemiology of primary glomerular diseases in a French region. Variations according to period and age.

Between January 1, 1976 and December 31, 1990, histological diagnosis of primary glomerular diseases (PGD) was made in 480 patients born and living at the time of diagnosis in a region of France, comprising 410,664 inhabitants, of whom 390,574 were aged from 10 to 80 years. The prevalence of PGD during a 70 year exposure to risk (10 to 80 years of age) was evaluated to 5.7 in 1000 (7.6 in 1000 males and 3.8 in 1000 females). The most common PGD was IgA nephropathy with a prevalence of 1.9 in 1000 (3.3 in 1000 males, 1 in 1000 females). The annual incidence of the disease was evaluated separately for three consecutive five-year periods: period A (1976-80), period B (1981-85), and period C (1986-90). Within each of these three periods the number of patients with PGD was 179, 170 and 131, respectively, and annual incidence was 9.3, 8.8 and 6.7 in 100,000. The incidence of IgA nephropathy remained the same throughout the three periods: 2.6, 3.1 and 2.5 in 100,000. The incidence of membranoproliferative glomerulonephritis decreased from 1981 onward (0.9, 0.5 and 0.15 in 100,000), while that of membranous nephropathy increased slightly (1.2, 1.6 and 1.7 in 100,000). Acute streptococcal glomerulonephritis virtually disappeared during periods B and C. Lipoid nephrosis was less frequent in period C and idiopathic proliferative glomerulonephritis with crescents slightly increased (0.3, 0.4 and 0.6 in 100,000). There was no significant difference between the three periods regarding the incidence of other PGD.(ABSTRACT TRUNCATED AT 250 WORDS)

[Hepatic polycystic disease is not always associated with polycystic kidney: epidemiological data]. / La polykystose hépatique n'est pas toujours associée à la polykystose rénale: données épidémiologiques.

Adult polycystic disease of the liver (APLD) and of the kidney (ADPKD) is considered to represent one entity. In 75 ADPKD kindreds with 259 affected members, ultrasonography and/or CT were performed in 186 (71.8%) from 64 kindreds (85.3%). We demonstrated that ADPKD with or without APLD are two separate phenotypes and suggest genetic heterogeneity of these two entities.

Fatal co-infection with pulmonary tuberculosis and herpes simplex virus 1 pneumonia under steroid treatment.

We report a case of adult respiratory distress syndrome in a 65 yr old man under inappropriate steroid therapy who developed both Herpes simplex virus 1 (HSV) and Mycobacterium tuberculosis pneumonia; we conclude from clinical and histological data that HSV 1 played a major role in the fatal outcome of this immunocompromised patient, in spite of acyclovir treatment.