Biomedical subjects
Publications and source records attributed to L Paunier.
Six patients with transient hyperphosphatasemia of infancy (THI) are described and compared to similar cases reported in the literature. THI appears to be more common than usually thought and may occur in healthy children as well as in patients with various clinical disorders. The evolution of such children seems uniformly normal. Invasive diagnostic procedures should therefore be avoided in this benign condition.
The relationship between renal prostaglandins (PG) and electrolyte transport in the thick ascending limb of Henle's loop (TALH) was examined in 8 normal subjects and in 7 patients with hypokalemia, the latter including 4 cases of Bartter's syndrome. Distal fractional delivery and distal reabsorption of chloride and sodium were determined using clearance techniques under maximal volume expansion, and urinary excretion rates of PGE2 and PGF2 alpha were measured. In the normal subjects, suppression of PG synthesis by indomethacin decreased distal delivery without changing distal reabsorption, suggesting that PG may influence solute transport at a site proximal to the diluting segment. In the subjects with Bartter's syndrome, however, distal reabsorption was impaired, this defect being apparently independent of PG as it was unaffected by inhibition of PG synthesis. In the patients with hypokalemia due to non-renal causes, distal reabsorption of chloride was increased, resulting in a decreased chloride clearance. These results suggest a link between renal PG and solute transport in normal man and also provide evidence that Bartter's syndrome is characterized by a prostaglandin-independent defect in distal reabsorption which is not found in hypokalemia due to other causes.
Mg, K and Na were measured in erythrocytes, lymphocytes and serum obtained from 20 normal subjects. The cells of 20 ml of blood were separated in Ficoll gradient, washed with a choline buffer and digested with H2SO4. The cations were measured by atomic absorption photometry, protein was determined by the Lowry method and the results were expressed as nmol/mg of protein. Intracellular lymphocyte concentrations (mean +/- SD, n = 20) were: Mg: 57.6 +/- 5.1, Na: 30.0 +/- 7.9, K: 614.3 +/- 69. In the red cells, ion concentrations were: Mg: 7.97 +/- 0.8, Na: 20.9 +/- 4.8, K: 343.0 +/- 42.0. There was a highly significant correlation between Mg and K concentration in erythrocytes (p less than 0.001) and lymphocytes (p less than 0.001). A negative correlation (p less than 0.05) was also found between lymphocyte and erythrocyte K concentration and K serum concentration.
The bacteriologic detection of group A Streptococcus in pharyngitis is vital in everyday practice to prevent serious potential sequelae. The purposes of this study were to determine whether throat cultures should be incubated in anaerobic atmosphere and whether an increased recovery rate could be obtained by stabbing of the plates (partial anaerobiosis) and by using a sulfamethoxazole-trimethoprim disk to enhance growth and identification. We examined 243 throat cultures, in duplicate, which were incubated in room air and in anaerobiosis (carbon dioxide, 10%). We found that, in aerobic incubation, the recovery rate of group A streptococci was 5.7%; in anaerobic incubation it was 19.8%. Stabbing of the agar to create a partial anaerobiosis was useless. When directly placed on the plate, the sulfamethoxazole-trimethoprim disk facilitated the identification of beta-hemolysis areas. To achieve maximum detection of group A streptococci in specimens obtained from the throats of infected children, we found that anaerobic incubation should be used.
Serum aluminum concentrations were measured in 16 children undergoing continuous ambulatory peritoneal dialysis after 7.9 +/- 2.1 (mean +/- SE) and 16.6 +/- 2.3 months of therapy, when the estimated simultaneous oral Al intake from Al hydroxide gels was 98 +/- 20 and 104 +/- 32 mg/kg/day, respectively. Serum Al concentrations were 55.2 +/- 11.4 and 59.8 +/- 10.4 micrograms/L, respectively, compared to 8.2 +/- 1.1 micrograms/L in normal children (P less than 0.001). Serum Al levels correlated with oral Al intake (r = 0.86, P less than 0.001) and inversely with body weight (r = -0.68, P less than 0.01) and age (r = -0.67, P less than 0.01). The youngest patient with the highest serum Al concentrations (208 and 174 micrograms/L) and greatest Al intake (310 and 192 mg/kg/day) had bone biopsy features characteristic of aluminum-related bone disease. Thus, higher aluminum intake per kilogram body weight given to young children is likely to raise the serum Al levels and increase the risk of osteomalacia. Aluminum-containing antacids should be used with caution in infants and young children with renal failure.
This paper describes a retrospective evaluation of the course of renal bone disease in 14 children undergoing treatment with continuous ambulatory peritoneal dialysis (CAPD) for an average of 11.9 +/- 1.5 months (mean +/- SE). The patients were divided in two groups according to the changes in serum alkaline phosphatase activity during the period of observation: five patients had alkaline phosphatase activity that decreased or was relatively stable (group I), and nine patients exhibited a rising serum alkaline phosphatase activity (group II). Serial radiological examinations showed adequate control of renal osteodystrophy in the patients of group I, whereas the patients of group II had no improvement or worsening of their bone disease. Group I had higher serum calcium and lower parathyroid hormone levels than group II at the end of period of observation despite similar dosage of vitamin D metabolite. The progression of bone disease was not related to the duration of CAPD or type of previous treatment for end stage renal disease. The observation that the radiological manifestations of secondary hyperparathyroidism were prevented in patients whose serum calcium levels were frequently above 2.62 mmol/liter (group I) while serum calcium levels between 2.25 and 2.50 mmol/liter in group II patients failed to lead to regression of secondary hyperparathyroidism is consistent with the existence of altered "set-point" regulation of the parathyroid gland in children undergoing CAPD.
The plasma profiles of 8 hormones were followed over the course of prepuberty and puberty in 30 adolescent males who developed gynecomastia and 24 who did not. Throughout puberty, ratios of delta 4-androstenedione to estrone (E1) and estradiol (E2) were significantly lower in the gynecomastia group than in the control group. Similarly, ratios of dehydroepiandrosterone-sulfate to E1 and E2 were significantly lower in the gynecomastia group. In contrast, ratios of plasma testosterone to E1 and E2 as well as plasma progesterone and PRL concentrations, were similar in both groups. Because of the adrenal origin of dehydroepiandrosterone and its sulfate, and of peripheral conversion of adrenal androgens to E1 and to E2, it appears that either decreased adrenal production of androgens and/or increased conversion of dehydroepiandrosterone-sulfate and delta 4-androstenedione to estrogens cause transient gynecomastia in adolescent boys.
The annual incidence of end-stage renal disease (ESRD: 1.32 patients aged 1/2-16 years per million population or 6.5 per million children of same age) and the causes have remained remarkably constant over the last 15 years in Switzerland. 58 patients (69%) were treated by dialysis and/or renal transplantation. The overall survival rate was 77% at 5 years and 65% at 10 years. 26 untreated patients (31%) died of uremia. They had not been treated despite existing facilities for the following reasons: Young age (7 of the 8 patients being less than 2 years old), psychosocial reasons (6), severe mental retardation (6) and associated problems (6). It is estimated that even in the future 20% of all pediatric patients with ESRD will be considered unsuitable for therapy by dialysis and renal transplantation.
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A case of primary hyperparathyroidism with clinical signs of rickets in a 15-year-old boy of South Morocco is presented. X-ray findings include a diffusely osteoporotic skeleton with areas of subperiostal resorption, cysts and metaphyseal rachitic changes. Hypercalcaemia, hypophosphataemia, increased alkaline phosphatase are found together with low calcidiol and high calcitriol plasma levels. The surgical removal of a chief-cell adenoma of a parathyroid gland leads to very rapid bone healing as well as normalization of blood chemistry. Reviewing the literature shows that 10 similar cases have been described. However, no correlation can be established between the occurrence of rachitic lesions and the Ca X P product. When limited calcium is available from the gut, elevated calcitriol then contributes to mobilize more mineral from bone, in conjunction with parathormone. In addition, the interaction of these 2 hormones on the renal tubule maintains a phosphate leak, creating proper conditions for the development of rachitic lesions.
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To study melatonin secretion by a gentle noninvasive method, a simple and quick RIA procedure to analyze melatonin in small urine volumes has been developed. Urinary extracts were prepurified by alkaline washes, and melatonin content was determined by RIA. The specificity of urinary melatonin determinations was confirmed by both thin layer chromatography and by gas chromatography-mass spectrometry. In the present study, we compared the amount of melatonin excreted in urine with plasma levels in 140 specimens from 13 adult volunteers. Comparisons showed a very good correlation between plasma levels at midnight and nocturnal excretion of urine, indicating the biological relevance of melatonin determination in urine. Our studies show further that urinary melatonin excretion displays the characteristic circadian rhythm usually observed in plasma. On the average, melatonin excretion is greatest between 2300--0300 h. The total 24-h excretion of melatonin varies considerably among different individuals.
The authors describe the case of a young girl with hemihypertrophy who developed a Wilms tumour in a kidney affected by nephroblastomatosis. The history and clinical findings are presented together with the histological results. The relationship between the congenital abnormality (hemihypertrophy), the dysgenesis (nephroblastomatosis) and the tumour is discussed.
The efficacy of a long-term prophylaxis with Co-trimoxazole was studied in 18 girls with recurrent urinary tract infections. SEven patients had neither anatomical nor functional anomalies of the urinary tract. Eight patients had a vesico-ureteral reflux, and three patients a complex urinary problem. The following therapeutic plan was applied: administration of a low dose of Co-trimoxazole (TMP: 0,6-1 mg/kg) given as a single evening dose during 3 months followed by a 3-month period without treatment. The children were followed in this way during 2 years. Seven children had no reinfection from the start of the prophylaxis during the 2 years of observation. Nine children had a significant decrease of the number of reinfections during the Co-trimoxazole treatment periods. Two children without anatomical anomaly had early reinfections with Co-trimoxazole resistant germs. No side effects related to Co-trimoxazole have been observed, and the tolerance of treatment was excellent. Co-trimoxazole administered as a single evening low dose efficiently prevented the recurrence of urinary tract infections.
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Plasma renin activity and one-dimensional echocardiographic measurements of the left atrium and left ventricle as well as function indices were studied repeatedly in 20 children with various cardiopathies (ages: 9 months to 15 years) before and after corrective surgery. Nine children had tetralogy of Fallot, four had pulmonary stenosis, four had rheumatic heart disease, two had ventricular septal defect, and one had atrial septal defect. Plasma renin activity was normal preoperatively, but increased significantly immediately after surgery, was still significantly higher on the 12th postoperative day and returned to normal six to eight weeks after surgery. Patients with tetralogy of Fallot and pulmonary stenosis had higher plasma renin activity values than the others. There was a positive correlation between plasma renin activity and postoperative percentage change of the left ventricular dimension. In patients with tetralogy of Fallot and pulmonary stenosis, this meant that plasma renin activity became normal when the preoperatively small left ventricle reached its normal dimension. This adjustment occurred slowly over a period of two months. In rheumatic heart disease and left-to-right shunt lesions, plasma renin activities became normal when the preoperatively dilated left ventricle decreased in size towards normal values; the plasma renin activities of these patients had reached normal levels by the fifth postoperative day. The renin secretion is modulated by various factors: of these, ventricular size and pulmonary venous return seem to be of importance.
The case of a 14 year old boy, suffering from severe bilateral nephrolithiasis, secondary to cystinuria, is presented. The progressive increase of the lithiasis, the dilatation of the excretory system of the right kidney and the painful passage of stones justified surgical removal of the staghorn stones on the right. The nephrolithotomy was performed under hypothermic perfusion of the kidney in situ, with section of the renal artery. This allowed an exsanguineous nephrotomy and the complete removal of all the stones. Anatomical repair of the calyces was possible, renal artery was re-anastomosed and the opening of the renal vein sutured without difficulty. No complication was encountered in the postoperative period, renal function remaining good. The intravenous pyelogram at the 15th postoperative day showed a marked improvement of the hydronephrosis. The authors review the literature describing the different methods of ischemic nephrotomy and compare the advantages of the procedure chosen in their case with the others : these advantages are the good preservation of the kidney, a bloodless nephrotomy, the long time allowed for the procedure and the removal of all the stones, and the possibility to continue the operation ex vivo when necessary, even using microsurgery.