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Biomedical subjects

O Oetliker

Publications and source records attributed to O Oetliker.

At least 19 recordsLinked to original sources

Familial hypercholesterolaemia with severe cardiac involvement in a boy: successful management and mid-term follow-up.

A 13-year-old boy with double heterozygosity for familial hypercholesterolaemia with a 90% left coronary artery main stem stenosis is reported. The patient's cholesterol levels were effectively controlled with weekly sessions of selective low-density lipoprotein cholesterol removal through immunoadsorption by use of an extracorporeal system. Left main coronary artery stenosis was successfully treated with percutaneous transluminal balloon dilation. At 30 months after the intervention and still under treatment with weekly sessions of low-density lipoprotein apheresis the patient is free of cardiac symptoms. He shows normal exercise capacity and normal myocardial perfusion. It is concluded that aggressive management is justified in such patients and may result in a near-normal quality of life.

Angiography↗

[Enuresis in childhood: what should one know? What should one do?].

Bladder control is a developmental process which is determined by somatic, individual psychological and psychosocial factors. It depends on the maturation of bladder capacity and of adequate neuromuscular coordination, on the quantity of urine and on appropriate recognition of bladder expansion. The latter especially is clearly correlated to healthy individuation and psychosocial integration of the child. Statistically, 80-90% of children have successfully developed bladder control at the age of 4-6 years. In the remaining 10 or 20% who do not attain dryness during daytime or at night, wetting is often felt to be disturbing state, sometimes more by the parents than by the children. The state is called enuresis. The physician is expected to master the problem of night or daytime enuresis diagnostically as well as therapeutically. Although in most cases enuresis represents a retardation of normal development, it is important not to miss the rare, but, if present, important disturbing factors. Disorders can occur at all above mentioned levels, either in isolation or combined. Although proposing the simplest diagnostic measures, the present paper represents an optimal workup during which none of the rare somatic or complicated psychological disturbances, which would need specialized attention, should be missed. After this workup the therapeutic pathways are clear. On the one hand, relevant somatic and/or psychological disorders must be treated specifically (often in collaboration with the specialist). On the other side, there is the large number of enuretics who are, by all criteria, normal children. In these it is possible to accelerate the developmental process by performing an elaborate micturition protocol which has a good chance of success, provided, however, there is optimal cooperation between physician, parents and child.

Adolescent↗

[Growth in children with non-dialysis dependent chronic kidney insufficiency].

Chronic renal failure leads to growth retardation in every second patient with the congenital and in every third with the acquired form. The pathophysiologic events of chronic renal failure are discussed, together with their possible direct or indirect impact on growth. Documentation of such events has important therapeutic implications. In recent years dietary measures have assumed paramount importance in the treatment of growth retardation in chronic renal failure. Treatment of individually assessed pathophysiologic conditions constitutes essential support for the dietary measures.

Anemia↗

The effect of temporary hypoxia on prostaglandin synthesis in mouse brain cell cultures during development.

Cultures of dissociated brain cells of new born mice represent a model for the study of brain development. One and two weeks old, they correspond in regard to oligodendrocyte differentiation to about the developmental stage of a human newborn and a six months old infant respectively. Such cultures were used to establish the developmental prostaglandin pattern and to study early and late recovery of prostaglandin synthesis from temporary hypoxia. Basal and bradykinin stimulated prostaglandin release were examined. Most prominently in stimulated release, the developmental prostaglandin pattern at one week showed a prevalence of PGE2 (33 +/- 4%) over PGD2 (12 +/- 5%), which in two weeks old cultures changes to an opposite distribution (PGE2 10 +/- 4%; PGD2 25 +/- 6%). This change goes parallel with the number and differentiation of oligodendrocytes. During the first day post hypoxia, imposed at the end of one week, the production of 6 oxo PGF1 alpha, PGE2, PGD2 and TXB2 was significantly decreased in two study series and increased compared to control in another. Since the arachidonic acid uptake was the same in all three series, this differential observation indicates differential early recovery. 8 days post hypoxia (late recovery), PG release was not different from control, indicating complete recovery at that time. During early recovery from hypoxia on 14 days old cultures, basal PG release was not significantly different from control, however bradykinin stimulated release was significantly inhibited in all three series. This may indicate that mainly regulatory influences on PG release in older cultures are compromised by hypoxia.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Twenty four hour intermittent, ambulatory blood pressure monitoring.

Blood pressure and heart rate were measured every 30 minutes during the day and every hour during the night in 43 children (20 girls and 23 boys, aged 10 to 16) with a portable automated monitor. The apparatus was better accepted in girls than in boys, and the failure rate was lower during the day. The overall failure rate was 22%, which corresponds with comparable studies in adults. During the night blood pressure and heart rate fell by 10% and 14% of the daytime values, respectively. Mean (SD) blood pressure was significantly higher in boys than in girls (126/72 (17/8) v 109/64 (9/5) mm Hg) and measurements correlated positively with age, body weight, and height of the subjects. Heart rate was not significantly influenced by gender or age. A positive correlation between heart rate and blood pressure was found when expressed as standard normal deviations or hourly variations. In children intermittent monitoring of ambulatory blood pressure and heart rate is a suitable method for measuring individual diurnal patterns.

Adolescent↗

Urinary calculi in association with glomerular immaturity.

The syndrome of glomerular immaturity associated with renal tubular acidosis and nephrocalcinosis or urolithiasis is illustrated by a case in which the diagnosis was made before the histological confirmation. Cases of urolithiasis in infants may be associated with this syndrome and attention must be paid to this rare but severe condition.

Acidosis, Renal Tubular↗

Extensive glomerular immaturity associated with renal tubular acidosis, nephrogenic diabetes insipidus and nephrocalcinosis.

Neonatal renal failure and glomerular immaturity have been described in 1983. The present paper describes 3 cases with the same histological features. However, follow-up of the patients during 14, 18 and 4 years, respectively, showed the following: The histologic immaturity may be followed by maturation. Thus, late maturation may be a term more suitable to designate this constellation. Renal failure may not necessarily ensue, at least not during early infancy. In the 3 cases presented in this report, the histological finding is associated with renal tubular acidosis, renal diabetes insipidus and nephrocalcinosis. The association of transient glomerular immaturity (or late glomerular maturation) and renal tubular acidosis may enhance the development of nephrocalcinosis.

Acidosis, Renal Tubular↗

[Influence of molsidomine, SIN-1 and nitroglycerin on the metabolism of prostaglandins by human fibroblasts].

In human skin fibroblasts, exposed during 24 h to molsidomine and SIN-1, the functional status of prostaglandin synthesis is altered in so far, that the bradykinin stimulation of thromboxane is inhibited whereas the production of other PG's is not different from control. Exposure to GTN induces no such inhibition of thromboxane. During the first 5 minutes of exposure, both SIN-1 and GTN stimulate the synthesis of 6-oxo-PGF1-alpha and of PGE2 but not of TXB2. Whereas SIN-1 shows a cumulative stimulatory effect with bradykinin, GTN lacks this effect. According to the findings of this human cell model, we conclude that the sydnonimines, like GTN, stimulate, at least under the present conditions, the formation of vasodilatory prostaglandins and, in addition, that they inhibit irreversibly the formation of proaggregatory thromboxane.

Bradykinin↗

Effects of dipyridamole on prostaglandin synthesis in human fibroblasts.

The effect of varied doses of dipyridamole (Persantin) on the arachidonic acid metabolites prostaglandin (PG)I2, PGE2 and thromboxane (TX)A2 has been studied in human skin fibroblasts cultured in vitro. A 24-h exposure to the drug induced a dose-dependent increase of all the examined prostaglandins (PG's). Bradykinin stimulation of cells that had been preexposed to the drug induced a continuous increase in PG-production as compared to controls, despite the absence of the drug. In addition, an acute 5-min exposure to dipyridamole produced an increased thromboxane production, but no consistent changes of PGI2 or PGE2, and it inhibited significantly and dose-dependently the comcomitant bradykinin stimulated production of all the examined PG's. The implications of a combined dipyridamole-acetylsalicylic acid therapy is discussed.

6-Ketoprostaglandin F1 alpha↗

Extra- and intracellular potassium concentration and prostaglandin production of skin fibroblasts grown from patients with Bartter's syndrome.

In studies on human skin fibroblasts originating from three patients with Bartter's syndrome and in corresponding age and sex matched controls, the bradykinin stimulated release of PGE2, PGI2, PGF2 alpha and of arachidonic acid was examined. The studies were aimed at demonstrating the possible changes of prostaglandin production under the influence of changing extracellular potassium concentrations (0-12 mmol K/l) in the two study groups. Earlier studies were confirmed and extended by one more pair of fibroblast cultures, showing a decreased bradykinin stimulated PGE2 production in fibroblasts from patients with Bartter's syndrome as compared to control. The difference in bradykinin stimulated PGE2 production was significant, irrespective of the extracellular potassium concentrations, to which the cultures were exposed. The bradykinin stimulated PGE2 and PGF2 alpha-production by control fibroblasts was directly proportional to extracellular potassium concentrations, whereas the PG-production of Bartter's syndrome fibroblasts remained uninfluenced by extracellular potassium. Extra- and intracellular potassium concentrations were directly proportional and there was no difference in this relationship between controls and Bartter's syndrome. The direct proportionality between bradykinin stimulated PGE2 production and potassium concentrations in control fibroblasts is, despite the apparent contradiction, in accordance with findings in the literature. The lack of a comparable proportionality in fibroblasts from patients with Bartter's syndrome is interpreted to correspond to an insensitivity to changes of potassium concentrations and thus to an insensitivity to one of the modulators of AA metabolism.

6-Ketoprostaglandin F1 alpha↗

Plasma exchange: an important part of the therapeutic procedure in a small child with autoimmune hemolytic anemia.

The case history of a child with autoimmune hemolytic anemia with complement activating warm antibodies of the pdl type is presented. This child also had giant cell hepatitis. Although plasma exchange is not curative in such a patient, the removal of antibodies and bilirubin by means of plasma exchange has alleviated the patient's condition on two occasions (at age 19 and 33 months respectively), and has permitted the institution of effective immunosuppression. A technique for plasma exchange is described, which by use of plasma filters and the usual hemodialysis equipment allows to safely perform the procedure in any pediatric hemodialysis unit. Its use in this patient stresses the technical practicability even in very young children. It is suggested that plasma exchange, if indicated, should be considered more often as part of a therapeutic concept.

Anemia, Hemolytic, Autoimmune↗

[Genetic heterogeneity and pathology of congenital nephrotic syndrome and its significance in prenatal diagnosis].

Familial occurrence of congenital nephrotic syndrome (CN) has been most extensively studied in Finland where it is known to be inherited as an autosomal recessive disease. In this Finnish type of congenital nephrotic syndrome (CNF) prenatal diagnosis by alphafetoprotein (AFP) determination in the amniotic fluid has been reported to be conclusive. Within the last six years we observed 10 cases of CN occurring in 5 families. In all patients renal histology has been studied. Although each family presented a different type of CN, the appearance within the same sibship was always identical.

Adult↗

Outcome of pediatric patients with end-stage renal disease in Switzerland 1973-1982.

The annual incidence of end-stage renal disease (ESRD: 1.32 patients aged 1/2-16 years per million population or 6.5 per million children of same age) and the causes have remained remarkably constant over the last 15 years in Switzerland. 58 patients (69%) were treated by dialysis and/or renal transplantation. The overall survival rate was 77% at 5 years and 65% at 10 years. 26 untreated patients (31%) died of uremia. They had not been treated despite existing facilities for the following reasons: Young age (7 of the 8 patients being less than 2 years old), psychosocial reasons (6), severe mental retardation (6) and associated problems (6). It is estimated that even in the future 20% of all pediatric patients with ESRD will be considered unsuitable for therapy by dialysis and renal transplantation.

Adolescent↗