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Biomedical subjects

W Proesmans

Publications and source records attributed to W Proesmans.

At least 19 recordsLinked to original sources
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

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↗
PubMed1976

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.

Adolescent↗
PubMed1976

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.

Child↗
PubMed1975

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.

Bucladesine↗
PubMed1975

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).

Autopsy↗