Biomedical subjects
W Proesmans
Publications and source records attributed to W Proesmans.
Rickets due to dietary calcium deficiency.
Three children, aged 15-18 months were referred because of clinical, radiological and biochemical manifestations of rickets. Serum 25-hydroxycholecalciferol (25-OH-D3) values were within the normal range but 1,25-dihydroxychole-calciferol (1,25-diOH-D3) levels were markedly elevated. All signs and symptoms could be explained by dietary calcium deficiency. They all received the commercial Soya-drink - not adapted for infants - as their main source of nutrition for at least 6 months. Soya-drink has an extremely low calcium content. It should be remembered that defective calcium intake causes severe bone lesions and rickets in children in spite of adequate vitamin D supply.
Idiopathic infantile arterial calcification with cardiac, renal and central nervous system involvement.
Idiopathic infantile arterial calcification was diagnosed in a 2 week old infant with failure to thrive associated with neurological, renal and cardiac signs. Therapy with diphosphonate resulted in a complete resolution of vascular calcifications. At the age of 2 years the child is doing well but requires medical treatment for arterial hypertension.
Cutaneous telangiectasia, sparse hair and membranoproliferative glomerulonephritis. A new case of a newly recognized entity.
A boy with sparse red hair, absent eyebrows and eyelashes, cutaneous telangiectasia, poorly developed subcutaneous fat and normocomplementaemic membranoproliferative glomerulonephritis is described. Additional findings were an old-looking, peculiar face, mild developmental delay, calcified choroid plexus and renal arteriolosclerosis. It is believed that this is a new case of a newly recognized entity.
Growth from birth to adulthood in a patient with the neonatal form of Bartter syndrome.
Growth from birth to the age of 19 years was studied in a patient with the neonatal form of Bartter syndrome. The initial modes of therapy (extra fluid, potassium supplements and triamterene) resulted in satisfactory but not optimal growth. Treatment with spironolactone together with potassium led to impressive catch-up growth. When the patient reached the age of 9 years, indomethacin therapy was started, which resulted in a second growth acceleration and was also accompanied by a significant reduction of both polyuria and hypercalciuria. Puberty developed normally, menarche occurred at 12 years 4 months and a normal adult height of 162 cm was reached at the age of 14 years. Treatment with prostaglandin synthetase inhibitors seems to be the best therapy for children with the neonatal form of Bartter syndrome.
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.
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.
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.
Prenatal diagnosis of Bartter syndrome.
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Progressive tubulointerstitial renal disease in infancy with associated hepatic abnormalities.
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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.
The hemolytic uremic syndrome of childhood and its variants.
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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.
[Idiopathic hypercalciuria in children].
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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.
Oligomeganephronic renal hypoplasia in two siblings.
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Prostacyclin production by whole blood from children: impairment in the hemolytic uremic syndrome and excessive formation in chronic renal failure.
The capacity of leukocytes to produce prostacyclin (PGI2) from endogenous and from platelet-derived endoperoxides was tested in whole blood. During the acute phase of the hemolytic uremic syndrome (H.U.S.), the PGI2-production was lower than the controls, whereas the blood from children with chronic renal failure produced higher amounts. Production of PGI2 by blood from children 3/12 to 6 years after the acute phase of H.U.S. was normal, as was the case with blood from their parents. Furthermore, in two H.U.S.-patients studied serially, the decreased PGI2-production capacity normalized 2 1/2 months after the acute phase.
Sonographic evaluation of the normal ureteral submucosal tunnel in infancy and childhood.
Sonography was used to evaluate in vivo the length of the submucosal segment of the intravesical ureter in 35 normal infants, children and adults. Age varied between 3 and 30 years. Sonographic measurements were plotted against, age, height and weight. Analysis of the statistical data shows an almost linear growth profile in this age group. The dimensions obtained in vivo by ultrasound technique are comparable to the published dimensions obtained in vivo by endoscopic calibration or in vitro on fresh specimens. Although promising, ultrasound of the vesico-ureteral junction is because of technical limitations not yet applicable in neonates and infants in which measurement of the submucosal tunnel could be of clinical importance.