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Biomedical subjects

L Monnens

Publications and source records attributed to L Monnens.

At least 73 records · Page 4Linked to original sources

Response to indomethacin and hydrochlorothiazide in nephrogenic diabetes insipidus.

Four boys with classical nephrogenic diabetes insipidus have been treated by indomethacin, by hydrochlorothiazide, and by the combination of indomethacin and hydrochlorothiazide. Hydrochlorothiazide treatment was slightly more effective than indomethacin treatment in reducing the urine volume and increasing the urine osmolality. The combination of indomethacin and hydrochlorothiazide appeared to be additive and especially helpful in infants and young children before the autonomy of drinking.

Child↗

The basal levels of active and inactive plasma renin concentration in infancy and childhood.

Basal plasma renin activity, active and inactive plasma renin concentration were measured in 89 healthy recumbent children aged between 1 week and 16 years. A significant (P less than 0.001) age-related decrease for active (r = -0.60), inactive (r = -0.59) and total renin concentration (r = -0.66) was observed. After correction for the influence of age, active renin concentration correlated with plasma renin activity (r = 0.81), but not with inactive renin concentration (r = 0.18). The proportions of active and inactive renin were not related to age, and the overall percentage of inactive renin was 79%.

Adolescent↗

Renin disappearance rate in puppies.

The disappearance rate of endogenous renin from the circulating blood after bilateral nephrectomy was studied in 4 puppies aged 16-21 days. Mean half-times of the fast and slow component of the disappearance curves of renin were 9.15 min +/- 0.87 (SD) and 84.0 min +/- 16.8 (SD), respectively. These values are not different from the values reported for adult dogs in the literature. The metabolic degradation of renin is not different between young and adult dogs and does not contribute to the elevated plasma renin activity in the puppy.

Aging↗

[Role of intravascular coagulation in the pathophysiology of the hemolytic-uremic syndrome].

Endothelial cell injury of capillaries and arterioles is considered to be the primary event in the hemolytic uremic syndrome. Intravascular coagulation increases the damaging processes. This intravascular coagulation can still be active after admission to the clinic. Several new data are available: prostacyclin deficiency can be present possibly due to the absence of a stimulating factor in plasma; the platelets are exhausted; an inhibitor of glomerular fibrinolysis has been demonstrated. A generally accepted therapy for HUS is still lacking.

Antithrombin III↗

[Pseudohypoaldosteronism: a special form of sodium loss in infancy].

An infant with urinary sodium wasting is described. The plasma renin activity and plasma aldosterone concentration were distinctly raised. Administering DOCA was without effect. The condition pseudohypoaldosteronism is due to renal tubular unresponsiveness to endogenous aldosterone and can be adequately treated by salt replacement.

Aldosterone↗

Levels of renin, angiotensin I and II, angiotensin-converting enzyme and aldosterone in infancy and childhood.

Basal plasma renin activity (PRA), angiotensin I and II (AI, AII), angiotensin-converting enzyme (ACE) activity and plasma aldosterone (PA) and sodium and potassium concentration were simultaneously measured in 55 healthy recumbent children aged between 1 week and 13 years. A significant (P less than 0.001) age-related decrease for PRA (r = -0.73), AI (r = -0.72), AII (r = -0.51) and PA (r = -0.71) was observed but not for ACE (r = 0.26, P = 0.06). After correction for age the correlation between PRA or PA and AI or AII was still significant (P less than 0.005). The strong correlation between AI and AII in the group as a whole (r = 0.82, P less than 0.001) and also in separate age groups, and an AI to AII ratio which was not different between the various age groups suggest that ACE activity in this age range is not rate-limiting for AII generation.

Adolescent↗

Hyporeninemic hypoaldosteronism in infancy.

Hyporeninemic hypoaldosteronism was observed in an infant at the age of 3 months. Persistent hyperkalemia, hyperchloremic acidosis and salt wasting were present. All abnormal electrolyte values were corrected by the administration of fludrocortisone. Both active and inactive renin were lowered. This infant had also an unexplained spastic quadriplegia and psychomotor retardation. Hyporeninemic hypoaldosteronism should be considered to be one of the causes of salt-losing in infancy.

Acidosis↗

Influence of the stress of venepuncture on basal levels of plasma renin activity in infants and children.

The influence of venepuncture on plasma renin activity (PRA), cortisol, and serum noradrenaline and adrenaline concentration in infants and children was evaluated by comparing values obtained immediately on insertion of an indwelling intravenous line (venepuncture value) and 5 and 60 minutes later. PRA values 5 min after venepuncture in the total group of infants and children were significantly higher (P less than 0.001) than venepuncture values. There was no significant difference between PRA values at 60 min compared to venepuncture or 5 min values. A well performed venepuncture does not systematically affect the PRA values obtained. A significant difference between values for noradrenaline, adrenaline and cortisol at the various points of time could not be demonstrated. The number of lower values for noradrenaline and adrenaline at 60 min, however, prevailed. The stress of a well performed venepuncture is not an explanation for the greater scatter of basal PRA levels in infants and young children.

Age Factors↗

Renal disorders in the branchio-oto-renal syndrome.

Intravenous urography and measurements of renal function were performed in 16 patients suffering from branchio-oto-renal syndrome. Malformations were visible by intravenous urography in all patients. Four out of 16 patients had a diminished glomerular filtration rate. Renal histology available in two patients revealed oligomeganephronic renal hypoplasia and multicystic dysplasia, respectively. Without renal agenesis or severe renal hypoplasia or dysplasia present in early infancy, renal abnormality does not seem to be a progressive disorder.

Abnormalities, Multiple↗

[A child with Zellweger's cerebrohepatorenal syndrome].

A child suffering from the cerebro-hepato-renal syndrome of Zellweger is presented. The clinical features are hypotonia, slow or absent feeding, profound psychomotor retardation, severe impairment of hearing, disturbed visual acuity and a characteristic facial appearance including high forehead and large fontanels. The biochemical abnormalities are disturbed liver function tests, increased concentration of pipecolic acid in serum, cerebrospinal fluid and urine, di- and trihydroxycoprostanic acid detectable in bile, serum and urine and an increased excretion of para-hydroxy-phenyllactate in urine.

Abnormalities, Multiple↗

Cerebro-hepato-renal syndrome of Zellweger: clinical symptoms and relevant laboratory findings in 16 patients.

The clinical features of 16 patients suffering from cerebro-hepato-renal syndrome are presented. Five of these children lived beyond 2 years. Four of them are still alive. The increase of pipecolic acid in serum and cerebrospinal fluid (CSF), the abnormality of the bile acids and the increased excretion of p-OH-phenyl lactate were a consistent finding. The concentration of pipecolic acid in urine was not always distinctly elevated. A loading test with DL-pipecolic acid was always abnormal.

Bile Acids and Salts↗

Malignant hypertension in a child with hemolytic-uremic syndrome treated with captopril.

A 5-year-old boy is presented suffering from malignant hypertension due to hemolytic-uremic syndrome. Captopril, an oral angiotensin-1-converting enzyme inhibitor, was able to curb the natural course of the malignant hypertension. Haemodialysis could be discontinued after 2 months. The glomerular filtration rate recovered to 63 ml/min/1.73 m2 11 months after the acute phase.

Captopril↗

Defect in bile acid concentrating ability of the gallbladder in congenital chloride diarrhoea.

Congenital chloride diarrhoea is assumed to be caused by a defect in the coupled NaCl influx mechanism in the ileum. As a similar coupled NaCl transport mechanism has been postulated in the gallbladder, the concentrating ability of the gallbladder was studied in a patient with congenital chloride diarrhoea. Bile acid concentrations were measured in the duodenal fluid before and after stimulation of gallbladder contraction by cholecystokinin. In the chloride-diarrhoea patient no increase in bile acid concentration was established after cholecystokinin injection, in contrast to a pronounced increase in three control children, suggesting that the absorption of salt and water by the gallbladder may be disturbed in the patient. The results support the postulated similarity of the NaCl transport mechanisms in the ileum and gallbladder. In congenital chloride diarrhoea one defect in a NaCl transport protein could explain the disturbances in electrolyte absorption.

Bile Acids and Salts↗

DDAVP test for assessment of renal concentrating capacity in infants and children.

The renal concentration capacity was tested by intranasal administration of DDAVP in 97 infants and children. The test was simple and reproducible. The renal concentrating capacity increased during approximately the first year of life. At the ages between 1 and 16 years the mean maximal osmolality of 991 mosm/kg was reached. The urine osmolality obtained in the DDAVP test was significantly lower than during the water deprivation test.

Adolescent↗

Amino acid solutions: composition and suitability for intravenous feeding in infants.

The amino acid composition, the electrolyte content, the titratable acidity, the osmolality and the pH of nine crystalline amino acid mixtures and one protein hydrolysate was analyzed and the results were compared with the data provided by the manufacturer. The amino acid composition of the protein hydrolysate (Aminosol 10%) showed a marked discrepancy between the concentration of amino acids measured and the concentration given by the manufacturer. The sodium, chloride and especially the ammonia content is high as compared to the synthetic amino acid solutions. The amino acid composition of the crystalline amino acid solutions was generally in reasonable good agreement with the composition given by the manufacturers. In DL-Trophysan an unknown amino acid, probably alloisoleucine, was found. Only in two (Vamin 7% and Amino acid solution 4200) out of eleven crystalline amino acid mixtures cystine and tyrosine could be detected; therefore only Amino acid solution 4200 and Vamin 7% might be suited for infants. However, infants fed parenterally with Vamin 7% often showed elevated levels of several amino acids. Amino acid solution 4200 with a composition similar to human milk seems to be more adapted and needs further investigation.

Amino Acids↗