Idiopathic pulmonary hemorrhage. Ultrastructural and mineralogic study.

A nine-year-old girl was hospitalized because of hypoxemia and anemia. Antibasement-membrane antibodies were present in the serum, but there was no renal involvement suggestive of Goodpasture's glomerulonephritis. Biopsy of the lung showed pulmonary hemosiderosis. In ultrastructural analysis, no lesion of the alveolar basement membrane or capillary endothelial cells was found; however, ferritin-looking deposits were present on alveolar basement membranes and the elastic lamina of arterioles and venules. Mineralogic studies showed these deposits to be made of iron and calcium (this latter element was probably precipitated on altered basement membranes or elastic lamina). Hemosiderin inclusions in the macrophages contained iron but no detectable calcium.

[Treatment of cardiac failure with refractory edema using extracorporeal ultrafiltration]. / Traitement des insuffisances cardiaques avec oedèmes réfractaires par ultrafiltration extracorporelle.

Thirteen patients with chronic congestive cardiac failure and refractory oedema were treated by haemodialysis. All patients had severe cardiac failure (Class IV NYHA) due to primary myocardial disease (5 cases), ischaemic heart disease (4 cases) or valvular heart disease (4 cases). Haemodialysis was performed via a Shaldon Y-shaped catheter in the internal jugular vein, with input and output through the same catheter using an alternating clamp. Filtration was carried out through a highly permeable membrane by a simple hydrostatic pressure gradient without a dialysis bath. The sessions were monitored haemodynamically by measuring the pulmonary artery pressures. The mean weight loss was 4.9 +/- 0.4 kg obtained after 3 three hour dialysis sessions with 24 hour intervals between each session (mean filtration flow = 12 ml/min). Hypotension was observed in one patient with low initial pulmonary artery pressures; two patients with severe valvular stenosis (1 mitral stenosis; 1 aortic stenosis) failed to lose weight. In the 10 remaining cases, there was a clearcut symptomatic improvement (5 patients Class III; 5 patients Class II NYHA) with total regression of oedema. There was a significant fall in pulmonary artery (mean PAP 40.5 +/- 6.5 mmHg to 34.6 +/- 6.5 mmHg; p less than 0.001) and pulmonary capillary pressure (27.6 +/- 6.9 mmHg to 22.5 +/- 5.8 mmHg; p less than 0.05) in these 10 cases. Cardiac output did not change significantly (cardiac index 2.2 +/- 0.5 l/m2/min compared to 2.3 +/- 0.4 l/m2/min after dialysis).(ABSTRACT TRUNCATED AT 250 WORDS)

[Data from a study of variations of arterial pressure during exercise in boys aged 11 to 16]. / Données de l'étude des variations de la tension artérielle à l'effort chez 106 garçons âgés de 11 à 16 ans.

Systolic, diastolic BP and heart rate were measured in 106 boys aged between 11 and 16 years old, at rest and during exercise on a bicycle ergometer. The results show: -- at rest: an increase in systolic and diastolic BP with age, and a decrease in heart rate; -- during exercise: an increase in systolic BP without an increase, or with a decrease in diastolic BP. The blood pressure readings were higher in overweight and post pubertal subjects. The value of this study lies in the high number of reproducible readings obtained and in the suppression of the effects of emotivity. It completes the observations made at rest in subjects suspected of having hypertension.

[Primary microvascular lesions of the kidney or pre-hypertensive nephroangiosclerosis. 25 cases]. / Lésions microvasculaires primitives du rein ou néphroangiosclérose pré-hypertensive. 25 observations.

Isolated non inflammatory lesions of renal microarteries (eventually with mild thickening of tubular basement membranes, but with negative immunofluorescent glomerular studies) were observed in 25 patients (22 males) in whom renal biopsy have been performed for proteinuria (P). Selection criteria were: pathological lesions by definition; absence of hypertension (HT) in clinical and at the time of biopsy; minimum follow up of 4 years after the first statement of the proteinuria (4 to 29 years; mean 14 years). Three groups have been isolated: 1. 3 patients have had an acute glomerulonephritis followed by disappearance of proteinuria. It reappears 1 to 5 years later. HT was discovered 2, 8 and 11 years after the proteinuria. Renal failure occurred 1 and 3 years after HT. 2. 14 patients had hereditary or acquired vascular risk factors (obesity, smoking, ethylism). In 7, HT occurred 3 to 15 years after P. In 2, renal failure occurred 4 to 8 years later. 3. 8 patients had no vascular risk factor; in 3 of them Ht developed 7, 13 and 20 years after the first statement. A positive immunofluorescence with IgM or C3 on renal arterioles had been found in only 3 of the 10 patients who in group 2 and 3 became hypertensive. A proteinuria may precede the occurrence of HT without being induced by glomerulonephritis. Group 2 and 3 suggest that these renal lesions of arterial sclerosis precede and may be a factor of HT. Indeed, this entity may be considered as a prehypertensive condition.

[Biological activity of enoxaprine (PK 10169) in hemodialysis. A dose study]. / Activités biologiques de l'éjnoxaparine (PK 10,169) en hémodialyse. Etude de dose.

Anti-Xa and anti-IIa activity were evaluated in 42 patients with chronic renal insufficiency, in an open randomized trial, to determine optimal dose of PK 10169 for prevention of coagulation in extracorporeal circuit during hemodialysis sessions. PK 10169 was given as single doses of 0.75, 1 and 1.25 mg/kg at start of dialysis, into the arterial line. All dialysis sessions were continued over the 4 hours provided for without the need for further injections. A linear relation existed between anti-Xa and anti-IIa activity measured at end of dialysis and the dose injected (p less than 0.001). Efficacy and tolerance were assessed clinically and biologically and were rated excellent at the 3 dose levels, the best tolerance/efficacy ratio being at the 1 mg/kg dosage.

[The use of enoxaparine for anticoagulation in extracorporeal circulation in hemodialysis at high risk for hemorrhage]. / Utilisation de l'énoxaparine pour l'anticoagulation du circuit extracorporel des hémodialyses à haut risque hémorragique.

After evaluation of efficacy of a low molecular weight heparin (LMWH), enoxaparine (Lovenox), in patients on continuous hemodialysis without a particular known hemorrhagic risk, this same LMWH was administered during 493 dialysis sessions to 46 patients presenting various degrees of risk of hemorrhage. Lower doses of 0.5 mg/kg or 0.75 mg/kg as bolus injections were administered at the start of the 4 or 5 hourly session. Clotting in the extracorporeal circulation (ECC) was noted in 0.6% treatments, the product being effective in all other sessions. Only one case of bleeding can be imputed to the LMWH injected during hemodialysis (0.2% of sessions). Although an open trial, the superiority of enoxaparine both for antithrombotic activity in ECC, and its simple management, as well as the small number of hemorrhages noted, has led to the routine use of this method in all patients at hemorrhagic risk.

[Enoxaparin in the prevention of thrombosis of extracorporeal circulation during dialysis of patients with chronic renal failure]. / L'enoxaparine dans la prévention de la thrombose du circuit extracorporel de dialyse des insuffisants rénaux chroniques.

Chronic renal failure patients under haemodialysis are exposed to two dangers: haemorrhage and clotting of the extracorporeal circulation circuit. This problem can be solved by using low molecular weight heparins. Two studies were conducted to evaluate the effectiveness and safety of a low molecular weight heparin: enoxaparin. The first study, which involved 42 chronic renal failure patients under haemodialysis without any particular risk, enabled the optimum dosage (1 mg/kg bodyweight) to be determined. The second study, which concerned 46 patients at high risk of haemorrhage who received enoxaparin 0.5 mg/kg or 0.75 mg/kg depending on the vascular approach, confirmed that enoxaparin was highly effective (clotting of the extracorporeal circuit in only 0.6 p. 100 of the cases) and well tolerated (bleeding in only 0.2 p. 100 of the cases).

[Mixed cryoglobulinemia with necrotizing angiitis. Apropos of 2 cases]. / Cryoglobulinémies mixtes avec angéites nécrosantes. A propos de deux observations.

Out of a series of 26 personal cases, 2 cases of mixed IgM-IgG cryoglobulinemia, one type II the other type III, are reported because they were associated with histologically proven necrotizing vasculitis. In both cases the numerous symptoms were due to renal damage (the vasculitis was discovered in the kidney) and to peripheral neuropathy. One of the patients died; the other had severely deteriorated general condition and required substitution hemodialysis. Cases of vasculitis associated with mixed cryoglobulinemia have often been published, but there are few reports mentioning necrotizing vasculitis; a search in the literature yielded only 9 cases. This small number does not mean that mixed cryoglobulinemia should not be listed among the causes of necrotizing vasculitis, but it makes it difficult to extract those specific features that would enable to predict which case of mixed cryoglobulinemia is associated or not with necrotizing vasculitis.

[Humoral immunity in an idiopathic nephrotic syndrome with minimal glomerular lesions and focal and segmental hyalinosis in children]. / Etude de l'immunité humorale des syndromes néphrotiques idiopathiques à lésions glomérulaires minimes et à hyalinose segmentaire et focale, chez l'enfant.

Humoral immunity was studied in 40 children presenting with idiopathic nephrotic syndrome (INS), of which 30 with minimal supposed or proven glomerular changes (MGC) and 10 with focal and segmental hyalinosis (FSH). Such patients are capable of producing in vivo active antibodies, in response to viral or bacterial infections and after anti-poliomyelitis immunisation. They present with decreased immunoglobulins G and increased immunoglobulins M during exacerbation of the disease. These abnormalities often persist during remission as well as in patients off-therapy. IgE are often increased during periods or exacerbation as well as in remission. On the contrary, the number of B lymphocytes and their distribution according to surface Ig are normal.

[Cellular immunity in idiopathic nephrotic syndrome with minimal glomerular lesions and focal and segmental hyalinosis in children]. / Etude de l'immunité cellulaire des syndromes néphrotiques idiopathiques à lésions glomérulaires minimes et à hyalinose segmentaire et focale, chez l'enfant.

Cellular immunity was studied in 31 children presenting with idiopathic nephrotic syndrome (INS), of which 23 with minimal supposed or proven glomerular changes (MGC) and 8 with focal and segmental hyalinosis (FSH). In vivo, a clear hyporeactivity to the delayed hypersensitivity tests and decreased blood T lymphocytes, with a great dispersion of the values were found. Furthermore, such patients' sera display a factor inhibiting the proliferative response of the lymphocytes of patients and of control subjects, to non specific mitogens (PHA), both during exacerbation and remission periods. The hypotheses of an abnormality of cellular immunity and of the existence of an inhibitory factor in the serum of INS with MGC and FSH are discussed.

[Polycystic kidney and Bourneville's tuberous sclerosis. Apropos of 2 cases]. / Polykystose rénale et sclérose tubéreuse de Bourneville. A propos de 2 observations.

Abdominal tumor is discovered in a 4 months old boy and a 2 years old another. The paraclinical exploration leads to the diagnosis of polycystic disease. But the clinical course permits to precise that the renal lesions are those of tuberous sclerosis. Indeed the children exhibit adenoma sebaceum at ages of 4 and 7 years.

[Pharmacokinetic of cyclosporin A]. / Pharmacocinétique de la ciclosporine A.

Pharmacokinetic studies of 13 children treated with cyclosporine A (CyA) were retrospectively analysed (9 renal transplants, 1 combined liver-kidney transplant, 1 heart transplant; 2 were treated for a nephrotic syndrome). The patients were separated into 2 groups: patients 0-15 years (group 1), patients 15-18 years (group 2). In comparison with adults (group 3), children of the 2 groups required higher CyA doses, related either to body weight or to body surface area. CyA dosage was performed by high performance liquid chromatography on whole blood samples. Despite higher doses, trough CyA levels were lower in groups 1 and 2 compared with group 3. Nephrotoxicity occurred in the only 2 children treated with CyA doses higher than 10 mg/kg/d.

[Acute renal insufficiency following endourethral surgery with glycine irrigation]. / Insuffisance rénale aiguë au décours de la chirurgie endo-uréthrale sous irrigation de glycocolle.

The authors analyse a case of acute renal failure in an old patient after a TUR of the prostate. The involved mechanism seems to be a toxicity of the glycine solution. In case of severe "TUR syndrome" with important resorption of glycine, the glycine could be catabolysed in oxalate, creating an hyperoxalemia and hyperoxaluria. The high blood level of oxalates can be responsible of acute renal failure and anuria. The treatment is simple: hemodialysis is able to eliminate quickly (in one session) the oxalates from the renal tubules and to restore the renal function. Prospective studies will give us an idea of the propitious conditions for this pathology and for the prevention.

[Thrombocytosis and reduced levels of factor VIII in rheumatoid purpura in children]. / L'hyperplaquettose et la baisse du facteur XIII dans le purpura rhumatoïde de l'enfant.

Thirty three patients with Henoch-Schoenlein purpura were studied at various developmental stages of this disease: specially platelet counts and factors XIII and VIII. During the development phases: 40,6% of the patients have a slight but regressive thrombocytosis (greater than 400 G/l); and 75% a reduced factor XIII, well correlated with the severity of the clinical status (level as low as 60% can be considered as a "gravity threshold"), and corrected during the improvement of the disease. This reduced factor XIII is probably linked to the local inflammation in the vessels. Factor VIII studies (specially VIII A: Ag) were normal.

Acute anaphylactoid reactions during hemodialysis in France.

A retrospective survey of anaphylactoid reactions during dialysis in France was conducted. In 52 of 112 hemodialysis units surveyed 111 patients who had suffered one or more anaphylactoid reactions during dialysis were identified. According to the Hamilton/Adkinson classification, in 31 patients reactions were minor, in 54 patients moderate, and in 26 patients severe. Four patients died of their reactions. A preponderance of reactions (75 and 11%) occurred with cuprammonium cellulose hollow-fiber and plate dialyzers, respectively. Severe dialyzer reactions were found to occur more frequently after the long (weekend) interdialytic interval. In an in vitro study, six brands of cuprammonium cellulose hollow-fiber dialyzers were rinsed with water and the eluates analyzed by size exclusion chromatography for contaminant particles. Substantial variation in the amount of extractable material was found between dialyzers of different brands, despite the fact that all dialyzers used membranes from the same manufacturer. Previous data by others has suggested that this extractable material is a derivative of cellulose. Results of our epidemiologic survey in France are similar to those previously reported in the United States and suggest an increased incidence of dialyzer reactions with ethylene oxide-sterilized cuprammonium cellulose dialyzers. The presence of cellulose-derived particles in the rinsing fluid of such dialyzers and the possible increased incidence of reactions after the long (weekend) interdialytic interval suggest that allergy to cellulose-derived particles eluted from cellulosic dialyzers may contribute to dialyzer hypersensitivity reactions.

[HLA and rheumatoid purpura with or without nephropathy]. / HLA et purpura rhumatoide avec ou sans néphropathie.

HLA profile was determined in 121 caucasoid children with rheumatoid purpura: out of those 55 developed nephropathy, of variable severity. HLA A and B were studied in all children, DR in 87. HLA BW 35 was more frequently found in total group of rheumatoid purpura (28,9%) and especially in nephropathies: 38,2% vs 20,4% in controls. However the increase is not significative even in nephropathy. On the other hand BW 35-DR 4 association is significatively higher in rheumatoid purpura with or without nephropathy than in controls. Analysis of 3 families with 2 affected sibs and 7 families with 1 affected child does not show any linkage with HLA. Determination of HLA profile does not allow to predict that the disease will be complicated by a nephropathy.