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Biomedical subjects

W Proesmans

Publications and source records attributed to W Proesmans.

At least 37 records · Page 2Linked to original sources

Heparin plus dipyridamole in childhood hemolytic-uremic syndrome: a prospective, randomized study.

From 1976 to 1985, a total of 58 infants and children with the hemolytic-uremic syndrome were randomly assigned to treatment either with heparin and dipyridamole or with supportive management only. In the treatment group, two patients died in the early weeks of the disease. Analysis of clinical and laboratory data showed no significant difference between either group of patients as to the evolution of their illness except for a significantly higher incidence of anuria and a significantly faster recovery from hypertension in the treated group. Renal biopsy studies showed no differences between the two groups in terms of incidence and severity of the histologic lesions. The long-term data on blood pressure and creatinine clearance values in the survivors were similar in both groups. This study indicates that treatment with heparin and dipyridamole has no benefit over symptomatic therapy alone in the typical form of childhood hemolytic-uremic syndrome.

Adolescent

Reversible arterial spasm in an adolescent with primary oxalosis.

A 16-year-old girl with primary oxalosis type I presented with progressive claudication soon after being treated with chronic intermittent hemodialysis. Arterial insufficiency of the lower limbs was confirmed clinically (purple discoloration of the skin and absence of arterial pulses) and with Doppler sonography. The arteriogram showed diffuse and symmetric narrowing with smooth vessel walls. Treatment with sodium nitroprusside had a spectacular effect; nifedipine was less effective. Renal transplantation with the father's kidney resulted in a rapid, complete and sustained reversal of the ischemic features. Magnesium withdrawal is assumed to be a pathogenic factor of the vascular spasm in this patient.

Adolescent

Hemolytic uremic syndrome in childhood: renal function ten years later.

Forty-six patients who developed a Hemolytic Uremic Syndrome (HUS) during the period 1970-1976, were examined ten years later. Thirty-two individuals had no signs of renal disease whereas fourteen showed at least one abnormality. In the latter group a urinary osmolality below 800 mosmole per kg water was the most frequent defect found (eight cases). Three adolescents had both hypertension and proteinuria, which are considered as important late sequelae.

Adolescent

Electrolyte composition of the amniotic fluid in Bartter syndrome.

In three patients with neonatal Bartter syndrome associated with polyhydramnios, analysis of the amniotic fluid showed normal sodium, normal-to-low potassium, but high chloride concentrations. This finding clearly suggests a renal chloride reabsorption defect as the primary cause of the neonatal form of Bartter syndrome. It is suggested that whenever polyhydramnios occurs, the electrolyte composition of the amniotic fluid should first be analysed in order to establish the diagnosis of Bartter syndrome.

Adult

Nephropathic cystinosis: effect of long-term cysteamine therapy.

Three children with nephropathic cystinosis received cysteamine therapy, mostly in the form of phosphocysteamine, for more than six years. The patients were between two and three years of age at the start of the study. The daily dose of cysteamine was 60 mg/kg as cysteamine base. In all three, rapidly progressive renal failure occurred before their 10th birthday. When comparing their evolution with data on the natural history of childhood cystinosis, no improvement was observed in terms of growth and glomerular function. It is concluded that cysteamine therapy did not provide clear benefit to the three patients reported here.

Body Height

Acute tubulo-interstitial nephritis and uveitis syndrome (TINU syndrome).

Acute renal failure due to tubulo-interstitial nephritis developed in a 15-year-old girl. The disease was accompanied by uveitis and an inflammatory syndrome, consisting of a markedly increased erythrocyte sedimentation rate and high serum gamma globulin levels. The nephropathy as well as the inflammatory syndrome subsided spontaneously. A topical antiphlogistic treatment healed the ocular disease, which has not relapsed so far. The association of acute tubulo-interstitial nephritis and acute uveitis observed in several patients has led to the identification of a specific syndrome with a very particular symptomatology and course, the so-called TINU syndrome, the interest of which resides in the predictability of the complete reversibility of the nephropathy either spontaneously or after steroid treatment, contrasting with the marked tendency towards relapse of the uveitis. The demonstration of circulating immune complexes in the serum during the acute phase of the illness, as in our patient, further points to the involvement of immune processes in the syndrome, but the origin and pathogenesis remain as yet unknown.

Acute Disease

Recurrent acute renal failure due to nonaccidental poisoning with glafenin in a child.

A 7-year-old boy experienced recurrent attacks of acute renal failure over an 18 month period. Each attack was accompanied by a fluorescent yellow discoloration of his urine. Laboratory data and the kidney biopsy were consistent with acute tubulo-interstitial nephritis. Repeated contacts with the boy in a play setting and thorough toxicologic examination of his urine finally led to the conclusion that the mother systematically poisoned her child with glafenin. The clinical and experimental literature on glafenin-related disease is reviewed. This is the first report of glafenin intoxication in a child. This is also the first case of kidney disease as an expression of child abuse.

Acute Kidney Injury