Human cytomegalovirus DNA kinetics using a novel HCMV DNA quantitative assay in white blood cells of immunocompromised patients under Ganciclovir therapy.

The clearance of human cytomegalovirus (HCMV) was evaluated in infected patients under Ganciclovir (GCV) treatment, using a novel HCMV DNA quantitation assay (HCMV DNA hybrid capture system, Murex Diagnostics). Peripheral white blood cells (WBC) from whole blood specimens of seven AIDS patients, three kidney and two allogeneic bone marrow transplant (BMT) recipients suffering from HCMV disease, were assessed by this method. HCMV DNA 50 and 90% mean clearances were observed at 2.11 +/- 1.97 and 6.22 +/- 4.31 days, respectively, after initial GCV treatment. The viral DNA kinetics were correlated with positive and negative pp65 antigenaemia and viral blood culture. Two-fold higher clearances and initial DNA levels were observed in the AIDS group compared to the transplant group. Neither clinical nor virological relapses were observed under GCV treatment. HCMV DNA quantitation in WBC appears well adapted for a therapeutic follow up of patients with HCMV disease.

Progressive muscular dystrophy. Functional improvement after a renal allograft.

From his first years a child showed signs of a primary and rapidly developing muscular dystrophy. The diagnosis was established by an increased serum CK level and by electromyography and muscle biopsies. Afterwards this child developed a severe renal deficiency which needed binephrectomy and the graft of a normal kidney. During the few months just after the graft, the disability increased and the patient could not stand upright by himself. Later on, he gradually became able to walk on his own and without bracing. He could climb stairs and stand up from the floor. The CK activity returned to normal. At present, 4 years after the graft (the patient is 16 years), the improvement of his functional abilities is constant, although the CK activity has increased again. In this article we give evidence that this patient suffers from a primary muscular dystrophy. We discuss the type of dystrophy concerned. We believe that it is the graft of a normal kidney which was responsible for the improvement observed, and not the physiotherapy or the drugs administered after the graft.

Uptake of trihalomethanes by patients during hemodialysis.

Trihalomethanes (THM) present in tap water were also found in dialysis fluid because they were not eliminated by water treatment. THM, absorbed through the dialyser membranes, increased considerably in blood and in expired air of patients on hemodialysis during the dialysis sessions. The uptake of THM during each dialysis session was about 1 mg.

Senior-Loken syndrome (nephronophthisis and tapeto-retinal degeneration): a study of 8 cases from 5 families.

The association of nephronophthisis and tapeto-retional degeneration was described by both Senior and Loken in 1961, but prior to 1974 only 28 cases had been published. This report describes 8 new cases in 27 members of 5 families. The severe juvenile type produces blindness in infancy and death from renal failure before the age of ten. The adult type is characterized by later onset, slower progression of the renal disease and milder ocular manifestations. The eye disease may be congenital amaurosis of Leber type, pigmentary retinal degeneration or retinitis punctata albescens and the electroretinogram (ERG) is of value in the diagnosis of these varieties of hereditary tapeto-retinal degeneration. Renal involvement is often asymptomatic. Defective urinary concentration leading to polyuria and polydipsia is the earliest sign. Proteinuria is inconstant and urinary sediment is often normal. Two patients had aminoaciduria. The disease progresses inexorably to chronic renal failure. One patient has been successfully transplanted and two others are on chronic hemodialysis. Renal histological changes are those of nephronophthisis with tubulointerstitial lesions and multiple cysts. Senior-Loken syndrome appears to be transmitted by a single autosomal recessive pleotropic gene of variable expression. Degeneration of neuroepithelium and renal tubular epithelium, both tissues of ectodermal origin, may represent a genetically determined enzyme abnormality.

[Role of the titration of anti-neutrophil cytoplasmic antibodies in therapeutic follow-up of patients with systemic vasculitis]. / Place du titrage des anticorps anticytoplasme des polynucléaires neutrophiles dans le suivi thérapeutique des patients atteints de vascularite systémique.

We studied clinical and biological data of 18 patients presenting ANCA associated diseases for 16 months at least. Five relapses were preceded by ANCA elevation, 1 relapse was not. Four transient elevations were noted without any clinical event. We think ANCA level elevation by itself is not enough for deciding therapy intensification, clinical data are necessary for doing so.

[Branchio-oto-renal malformation syndrome (author's transl)]. / Le syndrome malformatif branchio-oto-rénal. A propos de 5 observations.

After a brief review of the dysgenetic abnormalities which may affect the ear, branchial arches and pouches and the kidneys, the authors summarise those syndromes associating abnormalities of the ear and kidney on the one hand, and secondly abnormalities of the ear and facial and cervical fistulae. However, they are specifically interested in the branchio-oto-renal syndrome, reporting 5 cases. The syndrome combines deafness (middle ear and inner ear), pre-auricular and cervical fistulae and renal abnormalities. They attempt to summarise the syndrome by combining these 5 cases with the 15 published previously (4 initial cases of Melnick in 1974, 4 cases of Fitch in 1976, 7 of Fraser in 1978). The genetically transmissible nature of the syndrome would appear to be beyond doubt. The branchio-oto-renal syndrome was described for the first time in 1974 by Melnick, Bixter and Silk, who reported its existence in a father and three of his children, with an association of a malformation of the middle ear (block of the stapes), of the inner ear (cochlear hypoplasia), bilateral pre-auricular fistulae, bilateral cervical fistulae and renal hypoplasia with or without displasia. In 1974, Fitch and Srolowitz reported 4 new cases, and Fraser, Ling, Ologe and Nogrady a further 7 in 1978. The 5 cases published here bring the total known number to 20.

Increase in blood silicon concentration in patients with renal failure.

Comparatively with aluminium, human blood silicon concentration was determined by plasma emission spectrometry. In 33 healthy subjects mean value of 110 micrograms/l (s = 34) was obtained. In 14 patients with renal failure, but non dialyzed, Si increased and showed a good correlation with creatinine suggesting that Si is a good indicator of renal failure. In 127 hemodialyzed patients Si was increased up to a mean blood concentration of 1,740 micrograms/l and sometimes to values as high as 5,000 micrograms/l. Possible pathological consequences of this elevation are still to be determined.

Cyanide and thiocyanate blood levels in patients with renal failure or respiratory disease.

Whole blood cyanide and plasma thiocyanate were measured by a headspace gas chromatographic method and a colorimetric method, respectively, in 16 healthy subjects, in 10 patients with respiratory disease and in 12 patients on chronic dialysis for renal failure. In healthy subjects, whole blood cyanide and plasma thiocyanate concentrations were significantly higher in smokers (1.8 +/- 0.4 mumol/l; 206 +/- 74 mumol/l) than in non-smokers (0.8 +/- 0.4 mumol/l; 74 +/- 19 mumol/l). In renal failure patients on hemodialysis, no difference was noted in cyanide levels (0.6 +/- 0.4 mumol/l), but there was a significant increase in plasma thiocyanate levels during the interdialysis period (62 +/- 24 mumol/l; 91 +/- 24 mumol/l). No difference in cyanide and thiocyanate levels of patients with respiratory disease was seen, in agreement with a weak pulmonary elimination of cyanide.

[Prognostic impact of renal manifestations of necrotizing angeitis. Apropos of 24 cases]. / Incidence pronostique des manifestations rénales des angéites nécrosantes. A propos de vingt-quatre observations.

To analyze the prognostic significance of renal involvement, data from 24 cases of necrotizing angeitis, with renal involvement in 16, is studied. Associated disorders are described. Patients are divided into four groups: acute renal failure, progressive renal failure, renal involvement with normal renal function, and no renal involvement. Acute renal failure et onset appears to be a poor prognosis factor in necrotizing angeitis. Progressive renal failure seems to have no bearing on the short-term and medium-term vital prognosis. The value of plasma exchange therapy in these indications is discussed.

[Glomerular lesions renal transplant patients excluding recurrence of glomerulopathy]. / Lésions glomérulaires du transplant rénal en dehors des récidives de glomérulopathie.

The authors report their experience with glomerular lesions after renal transplantation, but exclude recurrent glomerulonephritis. Fourteen cases (3 membranous glomerulopathies and eleven allograft glomerulopathies) are discussed in relation to 98 recipients of 105 renal allografts. Two patients with membranous glomerulopathy had HBs antigen in their serum. A poor graft prognosis is observed in 2 of 3 patients. Among eleven recipients who presented with allograft glomerulopathy, two groups are clearly defined with very different graft prognosis. The severity of the vascular lesions was the sole difference between these two groups. The possible physiopathologic mechanisms of allograft glomerulopathy are discussed. It is worth noting that the role of vesico-ureteric reflux is not a prominent feature of this study.

[Specific renal lesions revealing the presence of sarcoidosis (author's transl)]. / L'atteinte rénale spécifique révélatrice de la sarcoïdose.

Three cases of granulomatous nephropathy are reported. In two patients this was definitely related to a true renal sarcoidosis, while the third case raised the problem of the significance of an "isolated sarcoidosis nodule". The authors discuss the frequency, clinical symptoms, and pathological appearances of these renal lesions which reveal the presence of sarcoidosis, as well as the relationship between renal insufficiency and the parenchymatous lesions observed, and therapy.