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Biomedical subjects

R Eeckels

Publications and source records attributed to R Eeckels.

At least 55 records · Page 3Linked to original sources
PubMed1983

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.

Adolescent↗
PubMed1983

Sensorineural hearing loss in sporadic congenital hypothyroidism.

Hearing acuity was assessed in 45 children with sporadic congenital hypothyroidism during adequate long-term treatment. Otoscopy was performed in each and additional tympanometry in some of them. Secretory otitis media was found in 6 and was treated medically or by inserting grommets in the eardrum. In these children, hearing acuity was assessed after the otitis had been cured. Hearing acuity was measured either by conventional monoaural pure-tone audiometry (125-8000 Hz) or by binaural free field testing depending on the child's age (above and below 4 years respectively). Hearing was normal in 36 (80%) children. In the remaining 9, sensorineural hearing loss to some degree was detected affecting the higher frequencies in particular. Perceptive deafness required the use of a hearing aid in 4 children. No relationship could be found between hearing acuity and chronological age or bone age at diagnosis of congenital hypothyroidism or type of hypothyroidism. Sensorineural hearing loss is common in children with congenital hypothyroidism and should be searched for carefully and systematically to avoid difficulties related to speech and language development.

Adolescent↗
PubMed1983

Plasma levels of androgens and 17 alpha-OH-progesterone as an index of the adequacy of treatment in congenital adrenal hyperplasia.

Plasma levels of dehydroepiandrosterone-sulfate (DHEA-S), dehydroepiandrosterone (DHEA), delta 4-androstenedione (delta 4), testosterone and 17 alpha-OH-progesterone (17-OH-P) were studied in 58 samples collected in 18 patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, during long-term ambulatory treatment with hydrocortisone. At each visit the patients were classified as being either in good control (GC) or in poor control (PC), based on well-defined clinical, auxological and biochemical criteria. The results were analyzed in relation to the degree of control and to chronological age (CA), bone age (BA), body surface (BS) and pubertal development. The most clear distinction between the children with GC and those with PC is found for DHEA-S (p less than 0.001 for BA). The majority of the DHEA-S values in the children with GC are closely grouped and significantly below the normal limits for CA, BA, BS and pubertal stage (p less than 0.001). In contrast, the PC children have wide-spread values, most of them being within or above the normal limits. The difference between GC and PC is also significant for testosterone (p less than 0.01) and delta 4 (p less than 0.05), but not for DHEA. Of the five steroids studied, DHEA-S is the most specific, whereas testosterone is the most sensitive and especially useful in girls and in prepubertal boys. delta 4 and 17-OH-P are almost as sensitive as DHEA-S, but they are less specific. DHEA is the less valid criterium.

17-alpha-Hydroxyprogesterone↗
PubMed1982

Plasma androgens in children and adolescents. Part I: control subjects.

In this cross-sectional study, plasma levels of dehydroepiandrosterone sulfate (DHEA-S), dehydroepiandrosterone (DHEA), delta 4-androstenedione (delta 4) and testosterone (T) were measured by RIA in 232 normal subjects of both sexes, aged 2 weeks to 20 years. The results were analyzed in relation to chronological age, body surface and pubertal stage. High levels of plasma androgens were found in newborn infants of both sexes. After 3 months of age, androgen levels were uniformly low and rose with increasing chronological age and body surface. The first significant increase in mean androgen levels was found for DHEA-S. It occurred after 6 years of age in girls and after 8 years in boys. DHEA and T rose in both sexes after 8 years of age. delta 4 increased steadily with chronological age and body surface in both sexes. When androgen levels were related to body surface, a first significant increase was observed above 1.00 m2 for the four androgens, in both boys and girls. Above 1.20 m2 and 12 years of age, girls had higher mean levels of DHEA-S, DHEA and delta 4, but lower mean T levels than boys of the same body surface and chronological age. Before puberty, a positive correlation was found in both sexes between the plasma androgen levels on the one hand, and both chronological age and body surface on the other. Plasma androgen levels markedly increased at stage P2 in both sexes, and further increased with pubertal development. During puberty, girls had higher plasma delta 4, but lower plasma T levels than boys of the same pubertal stage. Plasma DHEA-S and DHEA levels were similar in both sexes. In contrast to the plasma androgens, plasma cortisol levels did not show any change in relation either to chronological age or to body surface or pubertal development. Body surface appears to be as good a discriminating factor as chronological age, at least in young children. It also appears from this study that DHEA-S is a good guide for the clinical evaluation of adrenal maturation and may be very useful in evaluating patients with growth or pubertal disturbances.

Adolescent↗
PubMed1982

Plasma androgens in children and adolescents. Part II. A longitudinal study in patients with hypopituitarism.

In this prospective longitudinal study, plasma androgen levels were determined during 1-7 years in 45 patients aged 5.6-23.8 years with either isolated growth hormone (GH) deficiency or multiple pituitary hormone deficiencies. Dehydroepiandrosterone sulfate, dehydroepiandrosterone, delta 4-androstenedione and testosterone were measured by RIA in 339 blood samples collected during the study period. Mean plasma androgen levels are normal in isolated GH deficiency. Patients with multiple pituitary hormone deficiencies, but normal ACTH reserve, have mean levels lower than normal. Patients with multiple deficiencies including ACTH deficiency have still lower plasma androgen levels. Longitudinal analysis of the data, however, shows that patients with either isolated GH deficiency or multiple pituitary deficiencies without ACTH deficiency constitute a heterogeneous population, with either normal or low to very low plasma androgen levels. Treatment with human GH as such does not have any effect on the adrenal androgen secretion. A dissociation is found in some patients between adrenarche and gonadarche, which indicates that the two events are not controlled by the same mechanisms. Our results support the existence of a specific hypothalamic-pituitary adrenal androgen-stimulating hormone (AASH). ACTH, although not identical to AASH, is essential for normal adrenarche. Induced puberty with estrogens in girls does not influence plasma androgen levels, and pubic and axillary hair growth is not achieved. It is suggested that replacement treatment with dehydroepiandrosterone sulfate should be administered to girls with hypopituitarism and very low plasma androgen levels at the time of the induction of puberty. Finally, it appears from this study that, to interpret the plasma androgens in hypopituitarism, body surface is as good as bone age.

Adolescent↗
PubMed1981

A boy with Leydig cell tumour and precocious puberty: ultrasonography as a diagnostic aid.

A 7 1/2 year-old boy developed pseudoprecocious puberty. The diagnosis of Leydig cell tumour was suggested by clinical and hormonal findings and greatly facilitated by ultrasonographic investigation of the testes. Surgical exploration was in keeping with the diagnosis and the tumour was removed. Orchidectomy was not necessary. It appears that ultrasonography may be very useful in diagnosing a testicular tumour. It facilitates surgical intervention since it permits localization of the lesion which is usually very small and limited to a small part of the testis, as in this case.

Child↗
PubMed1981

The haemolytic uraemic syndrome in childhood: a study of the long-term prognosis.

From January 1970 to June 1976, 45 children with the haemolytic uraemic syndrome were admitted to our department. They all received heparin in addition to supportive therapy. For the last ten patients heparin was given with dipyridamole (Persantin). Three children died in the acute stage of the illness giving an acute fatality rate of 6.6%. A fourth patients immediately needed chronic haemodialysis. In the other 41 patients, kidney function only partially recovered in two; they subsequently developed terminal renal insufficiency after 18 months and four years, respectively. The remaining 39 children have been regularly followed for three to eight years. None has been lost to follow-up. With only one exception, they all have shown a favourable evolution with negative urinalysis, normal blood pressure, and an endogenous creatinine clearance within the normal range for age.

Adolescent↗
PubMed1981

Clinical and biochemical findings before and after portacaval shunt in a girl with type Ib glycogen storage disease.

A girl presented with an important growth retardation, hepatomegaly, fasting hypoglycemia, lactic acidosis, increased serum cholesterol, triglycerides and uric acid, and increased liver glycogen (7.5%). There was no rise in blood glucose after IV galactose or fructose, but glucagon gave a delayed response. Type Ib glycogen storage disease was suggested by the low normal activity of glucose-6-phosphatase (G-6-Pase) which reached 1.8 units/g (normal, 2 to 10 units/g) and the normal activity of other glycogenolytic enzymes, measured in homogenates prepared in H2O (mean +/- S.E. in control subjects: 59% +/- 7; in type Ia GSD: 92% +/- 3). The activity of G-6-Pase measured as described above increased to 3.8 units/g of liver 1 year after PCS and 7.85 units/g of liver after 3 years. At that time, a simultaneous assay of the enzyme in a fresh, previously not frozen liver biopsy, homogenized in 0.25 M sucrose, revealed only about 29% of the activity of the same sample prepared in H2O (mean +/- S.E. in three controls: 95.8% +/- 8.9.

Adolescent↗
PubMed1981

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↗
PubMed1979

A new chromosome anomaly in acute lymphoblastic leukemia (ALL).

A new chromosome anomaly in acute lymphoblastic leukemia (ALL) is reported. Three, possibly four, patients showed an identical karyotype anomaly, characterized by a (4;11)(q13;q22) reciprocal translocation. This anomaly has not so far been found in lymphoproliferative disorders other than ALL. Two of the patients had congenital leukemia, but the anomaly described appears to be more characteristic of ALL than of congenital leukemia, and may help the clinician in establishing the diagnosis of ALL.

Adult↗
PubMed1979

Unilateral galactocoele in a male infant.

An otherwise healthy 1 1/2-year-old boy slowly developed a marked enlargement of his right breast during several months. The lesion was soft, round, fluctuant and translucent. Aspiration revealed the presence of a milk-like fluid. Surgical extirpation was performed. Histologically the cyst showed areas of actively secreting acinar mammary cells.

Amino Acids↗
PubMed1978

Prenatal diagnosis of homozygous familial hypercholesterolaemia. Expression of a genetic receptor disease in utero.

Cultured amniotic-fluid cells from a fetus at risk for homozygous familial hypercholesterolaemia (F.H.) almost completely lacked cell-surface receptors for plasma low-density lipoprotein (L.D.L.), as evidenced by direct measurement of binding, uptake, and degradation of 125I-L.D.L. Functional consequences of L.D.L. binding to the receptor--i.e., suppression of 3-hydroxy-3-methylglutaryl coenzyme A reductase and stimulation of cholesterol esterification--were proportionately reduced when compared with results in cultured amniotic cells from two control fetuses. On the basis of these findings, homozygous F.H. was diagnosed and the pregnancy was terminated at the 20th week. The diagnosis of homozygous F.H. was confirmed by a serum-cholesterol of the aborted fetus of 279 mg/dl, a value 9 times the mean of four control fetuses of similar gestational age. More than 80% of the serum-cholesterol of the affected fetus was contained within L.D.L. Prenatal diagnosis of homozygous F.H. now seems practical; moreover, the finding of a raised serum-L.D.L. in the affected fetus indicates that the L.D.L. receptor is normally functional as early as the 20th week of fetal life.

Adult↗