Biomedical subjects
W Proesmans
Publications and source records attributed to W Proesmans.
D-penicillamine-induced IgA deficiency in Wilson's disease.
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Different effects of exogenous cyclic adenosine monophosphate and its dibutryl derivative on plasma growth hormone, glucose, insulin and cortisol.
The effects of the infusion in four different dosages (0.001, 0.005, 0.02 and 0.2 mg/kg/min during 60 min) of cyclic 3',5'-adenosine monophosphate and of its dibutyryl derivative on plasma growth hormone and on glucose, immunoreactive insulin and cortisol were studied in 38 normal subjects and in 10 patients with idiopathic hypopituitarism. In normal subjects cyclic 3',5'-adenosine monophosphate provokes an increase in plasma growth hormone levels (only when a dosage of 0.2 mg/kg/min is used) without any changes in plasma glucose, insulin and cortisol. The maximal value of the means is observed 75 min after starting the infusion. Dibutyryl cyclic 3',5'-adenosine monophosphate (0.2 and 0.02 mg/kg/min) provokes a dose-related rise in plasma growth hormone levels which is always preceded by hyperglycaemia and hyperinsulinaemia. The peak of the mean growth hormone levels occurs at 135 min after initiation of the infusion. In all but one hypopituitary patients the nucleotides do not promote growth hormone secretion. It is concluded that exogenous cyclic 3',5'-adenosine monophosphate and its dibutyryl derivative may not be considered as analogous and that both compounds may contribute to study growth hormone release in normal subjects and in patients with growth abnormalities.
Fatal tubulo-intestitial nephropathy with chronic cholestatic liver disease.
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Retinal involvement in a case of nephronophthisis associated with liver fibrosis Senior-Boichis syndrome.
Electroretinographic and electroencephalographic studies were conducted in a 12-year-old boy with nephronophthisis, chronic hepatic fibrosis, mental retardation and tapetoretinal degeneration (Senior-Boichis syndrome). Markedly reduced ERG amplitudes and flat oscillatory potentials were found in the proband. Delayed scotopic implicit time and reduced amplitudes of the beta-wave were found in the mother's ERG. ERG may identify the carrier state of the Senior-Boichio syndrome.
The effect of exogenous 3':5'-adenosine monophosphate on urinary output in children with vasopressin-resistant diabetes insipidus.
The administration of c-AMP and DB c-AMP to six children with NDI has failed to yield an antidiuretic effect. From the present study it may be concluded that, at the doses used, neither c-AMP nor its dibutyryl derivative can mimic the action of ADH in NDI as they do in normal subjects. On the contrary, DB c-AMP increased urine volume and Cosm in a very marked way, without changing the creatinine excretion.
Nephronophthisis and tapetoretinal degeneration associated with liver fibrosis.
A 12 year-old boy was referred because of general weakness, enuresis and pallor which had been present for at least six months. Previously, the child had been hospitalized at the age of five, because of mental retardation and hepatosplenomegaly, for which no cause could be found. He had severe renal insufficiency, with all the hallmarks of nephronophthisis. In addition his vision was very poor and fundoscopy revealed tapetoretinal degeneration. The liver and spleen were grossly enlarged. Liver function was almost completely normal, but histology showed diffuse periportal febrosis with profiferation of the bile ducts. This observation seems to confirm the existence of a new syndrome, associating nephronophthisis and liver fibrosis as described by Boichis and coworkers (1973).
Dibutyryl cyclic 3':5'-adenosine monphosphate in hypopituitarism and Silver-Russell syndrome.
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Pseudohypoaldosteronism.
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Antidiuretic therapy in Bartter's syndrome.
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Down's anomaly (21 trisomy) and Turner's syndrome (46, XXqi) in the same sibship.
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Prenatal indomethacin toxicity in one member of monozygous twins; a case report.
A mother, treated with indomethacin because of premature labour, delivered a pair of monozygous twins at a gestational age of 33 weeks. Because of foetal transfusion syndrome, the first twin was polycythaemic and the second anaemic. The latter was also hydropic, suffered from anuria requiring peritoneal dialysis, and showed signs of severe pulmonary hypertension, probably as a consequence of intra-uterine constriction of the ductus arteriosus. The possible interaction between foetal transfusion syndrome, anuria, premature constriction of the ductus arteriosus and the prenatal administration of indomethacin is discussed. In general, indomethacin should cautiously be used as a tocolytic agent.
The best game is the waiting game.
Chronic dialysis and renal transplantation have been developed over the last three decades of the 20th century. These two forms of renal replacement therapy have revolutionized the fate of children in established renal failure. Yet, chronic dialysis is a serious burden to both the patient and the family and the long-term results of renal transplantation are far from excellent. Moreover, both forms of treatment have serious complications, some of them fatal. It is therefore important to highlight what has been achieved in terms of conservative treatment of chronic renal failure. This paper describes in detail the progress made in this field with special emphasis on the great opportunity to slow down progression from chronic renal disease to end-stage renal failure. Renal replacement therapy can wait in many children and should be postponed as long as possible, ideally until they have reached adulthood.
Surgical correction of renovascular hypertension in children.
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Somatostatin and the secretion of growth hormone induced by exogenous cyclic 3':5'-adenosine monophosphate in man.
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Cyclic AMP and the kidney: from a clinical point of view.
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[The hemolytic-uremic syndrome. Reflections on its definition, etiology and pathogenesis].
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Association of bilateral renal dysplasia and congenital hepatic fibrosis.
A young baby with jaundice and failure to thrive was found to have severe chronic renal failure and chronic cholestatic liver disease. She was the first child of healthy unrelated parents and died at the age of three months. Autopsy revealed bilateral renal dysplasia and congenital hepatic fibrosis. This particular association of kidney and liver disease has only rarely been observed. It must be differentiated from autosomal recessive polycystic liver and kidney disease.