Adynamic bone disease, characterized by a low bone formation rate with normal or reduced amount of unmineralized osteoid, is supposed to be the consequence of aluminum intoxication in uremic ...patients. However, the emergence of adynamic bone disease has been recently reported in hemodialyzed patients in the total absence of aluminum overload. This study was aimed to assess whether such a histological pattern of adynamic bone disease was already present in uremic patients not yet on dialysis. Twenty-seven asymptomatic uremic patients (mean age +/- SD 43 +/- 10 years, mean creatinine clearance 19 +/- 3 ml/mm) were studied and bone biopsies were repeated in 16 of them after 18 +/- 10 months of treatment with oral calcium carbonate (1-3 g of elemental calcium/day) and calcidiol (21 +/- 14 micrograms/day). None of the patients received aluminum hydroxide, and the search for bone aluminum deposits was negative in all patients both before and after treatment. Two patients fulfilled the criteria of adynamic bone disease on their post-treatment biopsies. They originated from patients classified as having normal bone histology before treatment. Comparison with the other patients showed that they had comparable plasma C-terminal PTH but higher plasma creatinine than patients with normal bone histology and lower plasma C-terminal PTH than patients with osteitis fibrosa but comparable plasma creatinine. The plasma levels of 1,25(OH)2D reached values above normal after treatment in these two patients. It is suggested that adynamic bone disease not related to aluminum intoxication can develop in uremic patients independently of dialysis, and is favored by a relative hypoparathyroidism for the degree of renal failure, possibly induced by elevated plasma concentrations of calcitriol.
This study reports the antibody response and clinical follow-up of uraemic children awaiting kidney transplantation after administration of the Oka-strain varicella vaccine (Varilirix). ...Seroconversion was observed in 20 out of 23 patients found to be seronegative when tested by the fluorescent antibody to membrane antigen technique, and an antibody booster response was observed in 41 out of 47 seropositive patients. Mild clinical varicella occurred in 5 vaccinated patients and herpes zoster in 3 initially seropositive ones. Nevertheless, a dramatic decrease in the incidence of both varicella and herpes zoster was observed in a series of 330 consecutive transplantations after the introduction of the varicella vaccine.
Décrire la première famille française de syndrome H et ses principaux signes cliniques.
Nous rapportons une patiente de 12 ans atteinte de syndrome H. Elle présente un syndrome inflammatoire sévère, ...des poussées de fièvre, des douleurs articulaires, une péricardite fluctuante, une surdité progressive, un infléchissement pondéral, une microcéphalie, une fatigue chronique et des lésions cutanées infiltrantes hyperpigmentées du dos et de la face interne des cuisses. Un diabète insulinodépendant immunologique s’y associe rendant le traitement des poussées par la corticothérapie délicat.
Le diagnostic de syndrome H a été confirmé par la mise en évidence d’une mutation homozygote dans le gène du transporteur hENT3 de nucléosides. Curieusement, cette mutation est aussi retrouvée à l’état homozygote chez son frère aîné asymptomatique.
Il s’agit de la première description du syndrome H dans une famille française. Cette pathologie multi systémique est méconnue et a pour l’instant essentiellement été rapportée dans des familles arabes consanguines vivant près de Jérusalem.
Le syndrome H mérite d’être mieux reconnu, particulièrement chez des patients atteints d’inflammation chronique, de lésions cutanées hyper pigmentées, de surdité et de diabète insulinodépendant.
Une atteinte cardiaque peut survenir au cours du syndrome hémolytique et urémique (SHU) typique. Elle demeure cependant exceptionnelle, mais grève souvent le pronostic vital.
Cas 1. Charles, âgé de ...21 mois, avait un SHU typique; une insuffisance rénale aiguë nécessitait une dialyse péritonéale durant 20 jours et ces antibiotiques à cause d'une fièvre inexpliquée. Au 10
e jour de dialyse, on notait la survenue d'un infarctus du myocarde, vraisemblablement secondaire à une embolie coronaire liée à une endocardite. L'enfant était traité par héparine. L'évolution était favorable, avec cependant une nécrose antéro-apicale séquellaire et une insuffisance rénale modérée.
Cas 2. Une fille, âgée de 24 mois, avait un SHU typique, nécessitant une dialyse péritonéale durant 10 jours. Au 4
e jour de dialyse, apparaissait une myocardiopathie, découverte lors d'une décompensation cardiaque qui ne pouvait être imputée ni à un désordre hydroélectrolytique, ni à une hypertension artérielle, ni à une dialyse inefficace. Une atteinte neurologique apparaissait au 5
e jour de dialyse. L'évolution était favorable sur les plans neurologique, cardiaque et néphrologique.
Cas 3. Une fille, âgée de 25 mois, avait un SHU typique, oligoanurique, nécessitant une dialyse péritonéale pendant 25 jours. L'enfant n'avait pas de problème cardiovasculaire pendant la phase aiguë. Il persistait cependant une insuffisance rénale chronique postdialytique (clairance de la créatinine: 15 mL/min/1,73 m
2). Trois mois plus tard, apparaissait une myocardiopathie dilatée hypokinétique sans cause précise. L'évolution était favorable sous traitement digitalique.
Afin d'améliorer le pronostic du SHU, une atteinte cardiaque doit être recherchée au cours de la phase aiguë de la maladie et plusieurs mois après.
Cardiac involvement rarely occurs in classic hemolytic uremic syndrome (HUS); it is often fatal.
The first patient, a 21-month-old boy, developed classic HUS with acute renal failure. Peritoneal dialysis was performed for 20 days. On the 10th day of dialysis, myocardial infarction occurred, probably related to coronary thrombus. The patient was given heparin and antibiotics because of an unexplained fever. The outcome was favorable despite antero-apical cardiac necrosis, and moderated chronic renal failure. The second patient, a 24-month-old girl, also showed a classic HUS, which required peritoneal dialysis for 10 days. Dilated cardiomyopathy with cardiac failure appeared on the 4th day of dialysis, not related to the volume overload and metabolic consequences of the acute renal failure, such as systemic hypertension or ineffective dialysis. On the 5th day of dialysis neurological involvement appeared. Neurological, cardiac and renal outcome was favorable. The third patient, a 25-month-old girl, developed a classical HUS, requiring peritoneal dialysis for 25 days. No cardiac insult appeared during the acute phase of the disease. After dialysis, the child had chronic renal failure (creatinine clearance: 15 mL/min/1.73 m
2). Dilated cardiomyopathy appeared 3 months later, without definite etiology. The outcome was favorable with digoxin treatment.
A cardiac involvement should also be searched for in the acute phase of HUS and several months later.
Since Mai et al. found, with the intestinal lavage technique, that the same dose of elemental calcium given as acetate (Ca Ac) complexed in the gut of uremic patients twice as much phosphate as ...calcium carbonate (CaCO3) while inducing a rather low calcium absorption, we wanted to see if half the dose of elemental calcium given as Ca Ac could control, on medium term, the predialysis plasma phosphate as well as CaCO3 while inducing less frequent hypercalcemia. This was evaluated in a cross-over study of 3 periods of 10 weeks according to the sequence Ca Ac, CaCO3 and Ca Ac, in 12 compliant patients on chronic dialysis previously treated by CaCO3. Because of poor tolerance of Ca Ac during the first period, 4 patients were excluded and the results were assessed only on the 8 patients who completed the study. For half the doses of elemental calcium (620 +/- 250 mg versus 1,310 +/- 560 mg versus 710 +/- 200 mg/day), Ca Ac allowed the same control of predialytic hyperphosphatemia (1.67 +/- 0.34; 1.74 +/- 0.32; 1.75 +/- 0.38) with paradoxically comparable normal mean plasma calcium concentration (2.61 +/- 0.14; 2.56 +/- 0.13; 2.55 +/- 0.14 mmol/l). Plasma alkaline phosphatases and intact PTH concentrations remained also stable during the 3 periods. The frequency of hypercalcemia greater than 2.75 mmol/l (12; 9; 20%) and of hyperphosphatemia greater than 2 mmol/l (17; 22; 27%) were comparable with the 2 treatments. In conclusion, Ca Ac controls predialytic hyperphosphatemia as efficiently as CaCO3 for half the dose of elemental calcium without, however, decreasing the frequency of hypercalcemia.
