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Biomedical subjects

W Proesmans

Publications and source records attributed to W Proesmans.

At least 55 records · Page 3Linked to original sources
PubMed1994

Successful treatment of recurrent thrombotic thrombocytopenic purpura with plasmapheresis and vincristine.

An adolescent girl with severe thrombotic thrombocytopenic purpura (TTP) remained in a critical condition after 3 weeks of combined treatment with antiplatelet drugs, plasma infusions and plasma exchange. The introduction of vincristine resulted in gradual improvement and eventual complete remission which lasted for 2 years. When she relapsed, immediate improvement was observed with the combined treatment of plasmapheresis and vincristine. She has now been in complete remission again for 10 months. It is suggested that plasmapheresis plus vincristine should be used as the initial treatment for children with TTP.

Adolescent↗
PubMed1994

Subcutaneous recombinant erythropoietin in preterminal renal insufficiency.

Recombinant human erythropoietin was given to eight children and adolescents with stable chronic renal failure in the predialysis state. The hormone was administered subcutaneously, twice weekly for 12 weeks, at a starting dose of 50 U/kg per week. The dosage was adapted every 4th week. Target haemoglobin was 10.5-11.5 g/dl, and the target haematocrit 32%-35%. Baseline haemoglobin levels of 8.20 +/- 0.93 g/dl increased to 9.17 +/- 1.10, 10.38 +/- 1.18 and 11.19 +/- 0.84 g/dl (mean +/- SD) after 4, 8 and 12 weeks respectively. Serum ferritin levels decreased progressively despite iron supplementation. No side-effects were observed: creatinine clearances remained stable, blood pressure did not increase and none of the patients displayed either convulsions or thrombotic features. The study shows that subcutaneous recombinant human erythropoietin is both effective and safe in anaemic children and adolescents with chronic renal insufficiency.

Adolescent↗
PubMed1994

Total body mineral mass measured with dual photon absorptiometry in healthy children.

Using dual photon absorptiometry, bone mineral content (BMC) and bone mineral density (BMD) of the total body and the lumbar spine were assessed in 97 healthy, Caucasian children aged 3-14 years. Excellent correlations were found between BMC and BMD on the one hand and age, body height and body weight on the other. No differences were found between boys and girls. There was a strong correlation between lumbar spine measurement as compared to those of the total body. Regression equations for total body and the different parts of the skeleton were calculated with either BMC or BMD as the dependent variable, and age, body height and body weight as independent variables. High variation coefficients were obtained in these multiple regressions, except for the head. For total body BMC and total body BMD, growth charts were constructed using Tanner and Whitehouse data on body height and body height and body weight. Conclusions. The increase in total body mineral content is an important feature of normal growth. Normal data for BMC and BMD in childhood are essential for bone mineralisation abnormalities in paediatric patients.

Absorptiometry, Photon↗
PubMed1994

Childhood haemolytic uraemic syndrome: long-term outcome and prognostic features.

From January 1970 to December 1982, 95 infants and children with the haemolytic uraemic syndrome were admitted to our unit. Six patients died (6.3%) in the acute phase, 4 went into end-stage renal failure (4.2%) within months after the acute episode. The remaining 85 patients (89.5%) survived and recovered. They were followed as outpatients at yearly intervals for 5 years. Arterial hypertension was a major problem in 7. Eighty patients were studied 10 years later: 52 of them (65%) had no sequelae, 21 (26%) had mild defects and 7 (9%) severe sequelae. Clinical and laboratory data at the onset were analysed for prognostic significance. The immediate outcome was significantly worse in patients with either arterial hypertension or central nervous system manifestations on admission. Yet, no single variable studied during the acute phase was predictive of the presence of sequelae after 10 years. Even when all complications i.e. anuria, hypertension and central nervous system involvement, were taken together, there was no difference between patients with and patients without sequelae.

Adolescent↗
PubMed1994

Patients with autosomal nephrogenic diabetes insipidus homozygous for mutations in the aquaporin 2 water-channel gene.

Mutations in the X-chromosomal V2 receptor gene are known to cause nephrogenic diabetes insipidus (NDI). Besides the X-linked form, an autosomal mode of inheritance has been described. Recently, mutations in the autosomal gene coding for water-channel aquaporin 2 (AQP2) of the renal collecting duct were reported in an NDI patient. In the present study, missense mutations and a single nucleotide deletion in the aquaporin 2 gene of three NDI patients from consanguineous matings are described. Expression studies in Xenopus oocytes showed that the missense AQP2 proteins are nonfunctional. These results prove that mutations in the AQP2 gene cause autosomal recessive NDI.

Amino Acid Sequence↗
PubMed1992

Reversible hypophosphatemic rickets following ifosfamide treatment.

A 7-year-old boy developed renal tubular dysfunction and hypophosphatemic rickets following treatment for relapsed embryonal rhabdomyosarcoma. Multi-agent chemotherapy included ifosfamide; the child received a total of 108 g/m2. The complete Fanconi syndrome which ensued, including excessive loss of calcium, resolved spontaneously and progressively 18 months after the last dose of ifosfamide. The patient had no further symptoms of rickets and radiological signs had almost completely normalized. Further follow-up was not possible as, despite further treatment, the child died of progressive disease.

Abdominal Neoplasms↗
PubMed1991

Specific eye fundus lesions in type II membranoproliferative glomerulonephritis.

In three adolescents, suffering from membrano-proliferative glomerulonephritis type II, ophthalmoscopy and fluorescein angiography revealed retinal pigment epithelium lesions, referred to as basal laminar drusen. The patient with the longest renal history had the most pronounced fundus changes. These lesions, earlier described in adult patients, are believed to be specific for this particular form of chronic glomerulonephritis.

Adolescent↗
PubMed1991

Autosomal recessive chronic granulomatous disease associated with 18q-syndrome and end-stage renal failure due to Henoch-Schönlein nephritis.

Chronic granulomatous disease (CGD) is an inherited disorder in which phagocytes, including polymorphonuclear neutrophils, are unable to generate oxygen-derived microbicidal compounds, among them superoxide. Two main types of CGD are known, an X-linked form which is normally associated with the absence of cytochrome b558, a component of the membrane-associated reduced nicotinamide adenine dinucleotide phosphate (NADPH) oxidase which generates superoxide and an autosomal recessive form, in which cytochrome b558 is present, caused by the deficiency of a cytosolic factor required to activate NADPH oxidase. Patients with the X-linked type are highly susceptible to infections; those with the autosomal recessive form may be less severely affected. We report the unusual association of autosomal CGD with 18q--syndrome in a girl who developed terminal renal insufficiency caused by Henoch-Schönlein nephritis, and speculate on the possibility that the gene defect of autosomal recessive CGD may be located on chromosome 18.

Chromosome Aberrations↗
PubMed1991

Heterogeneity of growth of bone in children at the spine, radius and total skeleton.

Bone mineral content (BMC), density (BMD), and size were measured in 202 subjects ranging from 3 to 25 years of age (106 males and 96 females), half of which were children and half young adults. BMC and BMD were measured using single photon absorptiometry at the proximal and distal radius and dual photon absorptiometry at the lumbar spine and the total body. In the pre-pubertal age group (3-9 yrs), no differences were found in BMC nor BMD between males and females at any site. Growth of bone during puberty was characterized by an increase in BMC, bone size and BMD. The percent increase in BMC was greater at the lumbar spine and the total body (+200 to +390 %) than at the radius (+90 to +270 %). The increase in BMC was higher than the increase in BMD (+50 to +90 %). Overall bone growth in the total body was not reflected by changes in BMC of the appendicular skeleton. The increase in BMD was heterogeneous and was higher in the legs than in the arms. In males, the increase in BMC and size during growth was greater than in females resulting in a higher peak bone mass and size in males. The increase in BMD was similar between males and females at the distal radius, the lumbar spine and the total body, but higher at the proximal radius, the arms and the legs in males.(ABSTRACT TRUNCATED AT 250 WORDS)

Absorptiometry, Photon↗
PubMed1990

Recombinant human erythropoietin increases blood pressure, platelet aggregability and platelet free calcium mobilisation in uraemic children: a possible link?

Recombinant human erythropoietin was administered to 10 uraemic children on chronic haemodialysis, all of whom responded by correcting their haemoglobin. In addition, they showed an increase in blood pressure; platelet aggregations, subnormal before therapy, improved during treatment. The intracellular free calcium concentration in platelets after thrombin stimulation also increased significantly during erythropoietin administration. We hypothesize that the effect of erythropoietin on platelet aggregability and on blood pressure may be due to an increase in the intracellular free calcium mobilisation in platelets and possibly in smooth muscle cells respectively.

Adolescent↗