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Biomedical subjects

B Boudailliez

Publications and source records attributed to B Boudailliez.

At least 55 records · Page 3Linked to original sources

Diet, vitamin D and vertebral mineral density in hypercalciuric calcium stone formers.

To elucidate the pathophysiology of dietary calcium independent hypercalciuria, 42 calcium stone formers (Ca SF) were selected because they had on free diet a calciuria greater than 0.1 mmol/kg/day. For four days they were put on a diet restricted in calcium (Ca RD) by exclusion of the dairy products. They collected 24 hour urines on free diet and on day 4 of Ca RD as well as the two-hour fasting urines on the morning of the day 5 and the four-hour urines passed after an oral calcium load of 1 g, for measurement of creatinine, Ca, PO4, urea and total hydroxyprolinuria (THP). On day 5 fasting plasma concentrations of Ca, PO4, intact PTH, Gla protein, calcidiol and calcitriol were measured. The patients were firstly classified into dietary hypercalciuria (DH, 18 patients) and dietary calcium-independent hypercalciuria (IH, 24 patients) on the basis of the disappearance or not of hypercalciuria on Ca RD. Then the patients with IH were subclassified into absorptive hypercalciuria (AH) because of normal fasting calciuria (8 patients) and into fasting hypercalciuria (16 patients). Fasting hypercalciuric patients were subsequently divided according to the PTH levels into renal hypercalciuria (RH, 1 patient) with elevated fasting PTH becoming normal after the Ca load and undetermined hypercalciuria (UH, 15 patients) with normal PTH levels. Furthermore, their vertebral mineral density (VMD) was measured by quantitative computerized tomography which was normal in DH (91 +/- 6% of the normal mean for age and sex) but was decreased in IH to 69 +/- 4%. No difference in VMD was observed between AH and UH. Urinary excretions of urea, phosphate and THP was higher in IH than in DH and comparable in AH and UH. Sodium excretion Ca RD was the same in all groups and subgroups as well as the plasma parameters. Plasma calcitriol was increased in IH and DH comparatively to normal in spite of normal plasma calcidiol. Calciuria increase after oral calcium load, an index of Ca absorption, was higher in IH than in controls and comparable in IH and DH as well as in the three subgroups of IH. From these data and correlation studies in IH it is concluded: (1.) VMD is decreased in Ca stone formers with IH but not in those with DH, making the distinction of these two groups of hypercalciuria patients clinically relevant.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗

Comparison of intact, midregion, and carboxy terminal assays of parathyroid hormone for the diagnosis of bone disease in hemodialyzed patients.

The predictive value of three different RIAs of PTH for the diagnosis of the histological type of bone disease has been compared in 24 asymptomatic patients on chronic hemodialysis who had never been exposed to aluminum intoxication and who agreed to have a bone biopsy after double tetracycline labeling. The serum concentrations of PTH were measured using a two-site immunoradiometric assay for intact PTH(1-84) and region specific assays directed against the C-terminal (53-84) fragment or the midregion (44-68) of the molecule. The bone histomorphometric analysis showed that six patients had nonaluminic adynamic bone disease with low bone formation rate (BFR), eight had mild hyperparathyroidism characterized by increased bone resorption and normal BFR, nine had severe hyperparathyroidism with increased BFR, and only one had true osteomalacia with increased osteoid seam thickness. All PTH assays correlated with the various parameters of bone resorption and bone formation and were able to differentiate the histological type of bone disease only when groups of patients were considered. For classifying individual patients into severe hyperparathyroidism and adynamic bone disease groups, the intact PTH assay had the best predictive value with a sensitivity of 100% and a specificity of at least 70%. A nonaluminic adynamic bone disease was observed in more than 50% of the patients who had normal intact PTH levels (6/11). It is concluded that the intact PTH measurement is superior to C-terminal and midregion assays for the prediction of the histological type of bone disease in hemodialyzed patients and should be of considerable value to adapt their treatment in order to avoid the emergence of both severe hyperparathyroidism and adynamic bone disease. In the absence of aluminum intoxication it seems that maintaining intact PTH concentrations 1 to 1.5 times the upper limit of normal would correspond to the best bone histology.

Bone Diseases↗

Trichomegaly, pigmentary degeneration of the retina and growth disturbances. A probable autosomal recessive disorder.

Two brothers are described with trichomegaly, early pigmentary degeneration of the retina, growth retardation, anterior pituitary deficiencies and peripheral neuropathy. This syndrome, initially reported in a boy by Olivers and Mac Farlane in 1965 (6), and thereafter in six sporadic cases of both sexes, is not associated with a recognizable chromosomal defect. The present report of two brothers of healthy parents with negative familial history suggests an autosomal recessive mode of inheritance of this entity.

Adult↗

[Adrenoleukodystrophy: significance for genetic counseling of the determination of very long-chain fatty acids and of molecular biology].

Juvenile adrenoleukodystrophy (ADL) is a peroxisomal, X-linked, consistently fatal condition for which no treatment is currently available. Detection of heterozygote females and antenatal diagnosis by determination of very long chain fatty acid levels are therefore mandatory. We report the case of a family with two affected first cousins but six unaffected maternal uncles. Plasma levels confirmed the usual X-linked pattern with transmission by the grandmother and ruled out the hypothesis of delayed adrenomyeloleukodystrophy in the grandfather with heterozygosis of all the daughters. Although all six sons are unaffected, two certainly heterozygote females have normal plasma very long chain fatty acid levels. Heterozygosis in these women was confirmed by family restriction fragment length polymorphism studies (TAQ1) using the St 14 probe whose reliability for the diagnosis of heterozygotes with normal plasma very long chain fatty acid levels has previously been documented.

Adrenoleukodystrophy↗

[Prevalence of histological types of bone disease in a hemodialysis center limiting the oral intake of aluminum hydroxide].

UNLABELLED: To assess the prevalence of histologic bone disease in our center where Al(OH)3 intake is restricted, we reviewed 42 bone biopsies performed between 1975 and 1985 in patients dialyzed more than 29 months. Bone biopsies were performed systematically (2/3 of the cases) or because of a mild hypercalcemia (1/3 of the cases). Seventeen of these patients had been dialyzed before 1978 with softened water moderately contaminated by aluminum. Fifteen had always been dialyzed with reverse osmosis treated water and 10 had been exclusively treated by hemofiltration. The prevalence of osteitis fibrosa was 76%, that of osteomalacia null and that of adynamic bone disease 24% (but only 9.5% with positive Aluminon staining). When the 17 patients dialyzed with aluminum contaminated water before 1978 were excluded, only one patient among 25 had an aluminum adynamic bone disease (4%). This low prevalence can probably be explained by the restricted intake of Al(OH)3 thanks to the systematic administration of Ca CO3 and in a few cases of Mg (OH). The adynamic bone disease group has lower serum concentration of PTH and shorter duration on dialysis whereas the serum levels of calcium, phosphorus, magnesium and aluminum and daily dose of Ca CO3, Mg (OH)2 and Al(OH)3 do not differ. The frequency of the positivity of aluminum staining is not statistically different in the 2 groups. In 4 cases, adynamic bone disease without aluminum or iron intoxications is found, associated with a relative hypoparathyroidism. It is not explained by previous parathyroidectomy, diabetes or steroid therapy. CONCLUSIONS: 1) Restriction of aluminum intake and dialysis with reverse osmosis treated water lead to a low prevalence of aluminum bone disease. 2) A new bone disease in uremia is described: the idiopathic adynamic bone disease associated with a relative hypoparathyroidism.

Adult↗

Disappearance of aluminic bone disease in a long term asymptomatic dialysis population restricting A1(OH)3 intake: emergence of an idiopathic adynamic bone disease not related to aluminum.

In dialysis centers using reverse osmosis-treated water but not restricting A1(OH)3 administration, a high prevalence of histological aluminum bone disease has been reported. To assess whether this is also the case in our center where A1(OH)3 intake has always been restricted and even completely given up after 1980 thanks to high doses of CaCO3, we reviewed 42 bone biopsies performed between 1975 and 1985 in patients dialyzed for a mean duration of 56 months. Seventeen of these patients had been dialyzed before 1978 with softened water moderately contamined by aluminum, 15 had always been dialyzed with reverse osmosis-treated water and 10 had been exclusively treated by hemofiltration. The prevalence of aluminum bone disease in the whole population was 9.5% (4 patients) and consisted only of adynamic bone disease, osteomalacia being totally absent. When the patients dialyzed with aluminum-contaminated water were excluded as well as 1 diabetic patient who had taken A1(OH)3 for 1.5 years the prevalence of aluminum bone disease was null in this population. When the whole population is considered the prevalence of the other types of bone disease was 76% for osteitis fibrosa and 14.5% for a non-aluminic adynamic bone disease (6 cases). These latter cases differed from the osteitis fibrosa group only by a relative hypoparathyroidism not explained by higher plasma concentrations and higher oral cumulative doses of calcium, magnesium and aluminum or by lower plasma concentrations of phosphate and bicarbonate. None had previous parathyroidectomy, one had an unsuccessful transplantation and one was diabetic. Iron overload was excluded by negative Perls staining.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Prevention of osteitis fibrosa, aluminium bone disease and soft-tissue calcification in dialysis patients: a long-term comparison of moderate doses of oral calcium +/- Mg(OH)2 vs Al(OH)3 +/- 1 alpha OH vitamin D3.

Since 1980, moderately large doses of oral calcium (80 +/- 35 mmol/day as CaCO3 +/- calcium polystyrene sulphonate), in association if necessary with Mg(OH)2 (2.5 +/- 1 g/day), with a reduction in the dialysate Mg concentrations from 0.75 to 0.375 mmol/24 h, have replaced A1(OH)3 as phosphate binders in our centre. A1(OH)3 was previously given to our haemodialysis patients in association with small doses of Ca CO3 (less than or equal to 3 g/day) and if necessary with 1 alpha OH vitamin D3. To compare the long-term efficacy of this new approach with the former one in the prevention of renal osteodystrophy and soft-tissue calcification, 32 current patients were selected on the basis of at least 24 months of treatment in our centre and availability of a yearly bone survey (profile of lumbar spine and anteroposterior view of the pelvis, shoulders and hands). A group of 30 patients treated before 1980 were then selected on the same criteria and matched for age, sex, and duration on dialysis. Linear calcifications of the anterior and posterior walls of the aorta in front of L2, L3, L4 and on the lateral walls of the iliac and femoral arteries were measured and the para-articular calcifications and subperiosteal resorptions of the hands evaluated. The initial extent and the subsequent increase of the ocular and para-articular calcification were comparable in the two groups. Plasma alkaline phosphatase was stable in the normal range in both groups, as was plasma concentration of calcium. Plasma phosphate was slightly elevated (1.7 mmol/l) but stable and comparable in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aluminum↗

[Role of ultrasound in studying the first urinary infection in children].

A retrospective study comparing the findings of ultrasonography (5 MHz transducer) versus intravenous pyelography and voiding cystourethrography was conducted in 92 patients during the course of an initial urinary tract infection (greater than or equal to 10(5) bacteriae/ml, WBC greater than 25/mm3) gathered over a period of 5 years. The average interval of time between ultrasonography and intravenous pyelography was 8 days (range 1-70). False positive results for ultrasonography were noted in 11% (5 of 45 cases), while false negatives were observed in 36% (17 of 47). Of the 47 cases of obstructive uropathy diagnosed by conventional means, ultrasonography missed the diagnosis in 1 case of ureteral duplication as well as in 16 children with vesicoureteral reflux. In comparison, intravenous pyelography missed 8 of these 21 cases of vesicoureteral reflux. Overall sensitivity of ultrasonography was mediocre at 64% but excellent at 96% after excluding cases with vesicoureteral reflux. Specificity was poor for the group of patients with vesicoureteral reflux (33%) and did not improve when they were excluded (40%). Sensitivity and specificity were comparable whatever the age group. In conclusion, we would recommend that when both ultrasonography and voiding cystourethrography are normal in children with a urinary tract infection, intravenous pyelography could safely be deferred.

Child↗

[The importance of continuous ambulatory peritoneal dialysis technics in relation to intermittent peritoneal dialysis in acute renal insufficiency in children. Apropos of 7 cases].

We report seven cases of acute renal failure (ARF) treated by continuous ambulatory peritoneal dialysis (CAPD). ARF was caused by hemolytic uremic syndrome in six patients and acute fetal distress in one neonate. Mean age was 28 months (range 6 days-6 years). A flexible Tenckhoff catheter was inserted surgically under brief (10 minutes) general anesthesia in all patients. During the first 24 hours, in order to avoid clogging of the catheter, exchanges were done every 45 minutes with 20 ml/kg dialysate initially, gradually increased to 40-50 ml/kg. Thereafter, 5 to 8 exchanges were done every day (mean duration 9 days, range 4-21) until creatinine clearance reached 15 ml/mn/1.73 m2. No case of peritonitis or leakage was recorded. In two patients, migration of the straight catheter caused flow obstruction. None of the crook-shaped catheters migrated. Because dialysis was continuous and fluid balance could be controlled by using a hypertonic dialysate if called for, optimal protein (1-1.5 g/kg/d) and energy intake were possible. This intake was ensured mainly by continuous enteral nutrition. Improved patient comfort (no immobilization, no repeated punctures) and staff comfort (technical simplicity, independence from a cycler) are further non-negligeable advantages of CAPD.

Acute Kidney Injury↗

Indirect clinical evidence that 1 alpha OH vitamin D3 increases the intestinal absorption of aluminum.

In a previous study we showed that 1 alpha OH vitamin D3 [1 alpha (OH)3] given to 16 hemodialyzed patients taking Al(OH)3 at a constant dose increased their plasma concentrations of aluminum [Demontis et al. 1986]. In order to choose between 2 possible mechanisms explaining this increase (increased intestinal absorption or decreased tissue storage of aluminum), we gave, in the present study, 1 alpha (OH)3 the same dose (6 micrograms per week) for the same period (4 weeks) to 15 stable hemodialyzed patients after their Al(OH)3 had been discontinued for 6 weeks. Under Al(OH)3 treatment they had a mean plasma aluminum (2.33 +/- 2.36 mumol/l) which was not significantly different from that of the patients in our former study (1.23 +/- 0.25 mumol/l). After Al(OH)3 discontinuation, plasma aluminum (measured by inductively coupled plasma emission spectrometry) decreased significantly as early as the 2nd week of the control period (1.39 mumol/l). The decrease was maintained at a plateau throughout the 5 weeks of the control period (1.38 mumol/l), the 4 weeks of 1 alpha OH vitamin (vit) D3 administration (1.40 mumol/l) and the 8 weeks of the post 1 alpha (OH)3 period (1.22 mumol/l). Plasma calcium and phosphate concentrations increased significantly with 1 alpha (OH)3 and decreased thereafter whereas plasma PTH concentrations decreased during 1 alpha (OH)2 D3 and increased after its discontinuation suggesting biological activity of 1 alpha (OH)3. Since 1 alpha (OH)3 increases plasma aluminum in hemodialyzed patients only when they are simultaneously taking Al(OH)3, it is suggested that this increase is explained by an increase of intestinal absorption of aluminum and not by a tissue redistribution of aluminum.

Adolescent↗

[Treatment of acute renal failure by continuous hemofiltration in newborn infants and infants].

During acute oligoanuric renal failure in neonates and infants, arteriovenous or veno-venous continuous hemofiltration is a simple, useful technique when peritoneal dialysis or hemodialysis cannot be used. It allows fluid removal, optimal calorie intake and mild blood purification, when waiting for diuresis to stabilize. It does not give any control over hypercatabolism; the use of continuous dialysis increases epuration.

Acute Kidney Injury↗