The surgeon and the law on patient's rights for minors.

The law of August 22nd, 2002 concerning patients' rights (LPR) gave a new dimension to the relationship between the physician and the patient. According to this law, it is up to the physician to judge if a patient is able to exercise his own rights or if the patient needs assistance from a representative. In the particular case of the patient being a minor, this often leads to a difficult situation because of the absence of validated criteria to evaluate the capacity of judgment of a minor patient. The triangular relationship physician-patient-parents might be hampered when the parents are involved in a divorce. In daily practice, there are many questions concerning the physicians' attitude towards the rights of the minor patient, particularly in cases of medical intervention. By means of case histories, we describe several problematic situations: the right of free choice of the physician, the right of the minor to obtain informational privacy, obtaining consent for a medical intervention. In cases where there is a divorce, the situation is even more difficult. Solutions are provided to act as effectively as possible in the minors' interests and to offer support to the physician. Note: According to article 388 of the Belgian Civil Code a minor is a person, either male or female, who has not attained the age of 18 years.

Camurati-Engelmann disease: contribution of bone scintigraphy to genetic counseling.

Progressive diaphyseal dysplasia (Camurati-Engelmann disease) is a rare hereditary disorder of bone characterized by progressive, bilaterally symmetrical diaphyseal sclerosis of the long bones. Severely affected patients show muscle weakness, waddling gait and severe pain in the extremities. However, clinical and radiological investigations in families with Camurati-Engelmann disease demonstrate a wide variability in expression of the manifestations. Asymptomatic patients were in several instances diagnosed only after radiologic screening of relatives. Although considered an autosomal dominant disorder, families are described where neither clinical nor radiological manifestations can be demonstrated in direct ancestors of patients. Combining roentgenographic examination with bone scintigraphy seems therefore necessary in confirming or ruling out progressive diaphyseal dysplasia in each family member.

Successful use of an intraosseous infusion in an 800 grams preterm infant.

An intravascular access line for the administration of life support drugs and volume expanders may be particularly difficult, especially in very small premature babies. We report on the successful use of an intraosseous accessline in an 800 grams preterm infant for the administration of drugs and fluid. The use and technique of an intraosseous access is an important emergency alternative which may be lifesaving, even in very preterm babies, when other methods fail.

The attitude of Flemish paediatricians regarding informed consent of adolescents.

BACKGROUND: Since 2002 the Belgian law requires an informed consent of the patient before each medical intervention. This provision applies also for adolescent patients on condition that the physician considers the young patient as competent to autonomously participate in the decision making process. AIM: The purpose of this study is to evaluate to what extent Belgian paediatricians from the Flemish part of the country have implemented the legal requirements for informed consent of adolescent patients, in particular when they consult alone. METHODS: In the frame of a larger study regarding the relation between paediatricians and their patients, a questionnaire was sent via regular mail to 570 Flemish paediatricians, evaluating how and how often they obtain an informed consent of the adolescent when consulting a physician unaccompanied. RESULTS: In only 1% of all consultations an adolescent consulted the physician alone and agreed to a medical intervention on his/her own. The information given by the paediatrician did not differ if the adolescent consulted alone or was accompanied by (one of) his/her parents for the following items: purpose and type of treatment (100% vs 100%), duration of treatment (92% vs 94%) and aftercare (89% vs 93%). However, the information differed with regard to alternatives to the treatment (65% vs 76%), degree of urgency (89% vs 95% ), treatment related risks (82% vs 90%) and cost (21% vs 45%). 18.6% of the paediatricians consider age as the single criterion to evaluate the competence of the adolescent to provide an informed consent; other criteria that are considered: experience (92%), insight into and factual understanding of the clinical picture (84%). To fulfil the tasks of providing information and asking for consent, paediatricians rarely had recourse to prior established protocols (14%), they preferred to rely on proper experience and expertise (81%). Fifty percent appealed to the opinion of other health care providers. CONCLUSIONS: Although the Belgian law stipulates regulations that should be complied with by the physician during the decision making process about any medical intervention on adolescents, this legislation has rarely been put into practice, as the adolescents used their right to autonomously consent in barely 1% of all paediatric consultations. For the majority of the respondents other criteria than age were taken into account to consider an adolescent as able to provide informed consent.

Alternative splicing of Bim and Erk-mediated Bim(EL) phosphorylation are dispensable for hematopoietic homeostasis in vivo.

The pro-apoptotic BH3-only protein Bim has a major role in hematopoietic homeostasis, particularly in the lymphocyte compartment, where it strongly affects immune function. The three major Bim isoforms (Bim(EL), Bim(L) and Bim(S)) are generated by alternative splicing. Bim(EL), the most abundant isoform, contains a unique sequence that has been reported to be the target of phosphorylation by several MAP kinases. In particular, Erk1/2 has been shown to interact with Bim(EL) through the DEF2 domain of Bim(EL) and specifically phosphorylate this isoform, thereby targeting it for ubiquitination and proteasomal degradation. To examine the physiological importance of this mechanism of regulation and of the alternative splicing of Bim, we have generated several Bim knock-in mouse strains and analyzed their hematopoietic system. Although mutation in the DEF2 domain reduces Bim(EL) degradation in some circumstances, this mutation did not significantly increase Bim's pro-apoptotic activity in vivo nor impact on the homeostasis of the hematopoietic system. We also show that Bim(EL) and Bim(L) are interchangeable, and that Bim(S) is dispensable for the function of Bim. Hence, we conclude that physiological regulation of Bim relies on mechanisms independent of its alternative splicing or the Erk-dependent phosphorylation of Bim(EL).

TGFbeta-mediated apoptosis of Burkitt's lymphoma BL41 cells is associated with the relocation of mitochondrial BimEL.

In this study, we showed that the transforming growth factor beta (TGFbeta)-mediated apoptosis of Burkitt's lymphoma BL41 cells is dependent on the BH3-only protein Bim. In contrast to what has been observed with other cell types, TGFbeta activation did not promote Bim upregulation in BL41 cells, but instead resulted in Bim release from the mitochondria. Indeed, Bim levels were high in healthy BL41 cells, in which they dimerized with the Bcl-2-like protein Mcl-1 at the mitochondrial surface. In healthy and TGFbeta-activated BL41 cells, unlike in epithelial cells or hepatocytes, Bim did not associate with Bcl-2 or Bcl-xL. TGFbeta activation of BL41 cells triggered the p38-dependent activation of caspase-8, causing the cleavage of Mcl-1 and the transfer of Bim from the mitochondria to the cytoskeleton. In addition to mitochondrial activation, this relocation of Bim may facilitate the complete demise of a cell death that is beyond the commitment point to apoptosis and may represent a hallmark of the TGFbeta-mediated apoptosis of human lymphoma B cells.

The long-term effect of a partial whey hydrolysate formula on the prophylaxis of atopic disease.

At the age of 5 years, the prevalence of atopic manifestations was analysed in 58 formula-fed "at risk" infants because of a history of atopic disease in at least two first degree relatives. Infants were randomly assigned to receive either a partial whey-hydrolysate formula (n: 28) or a regular cow's milk formula (n: 30) during the first 6 months of life; thereafter, feeding was unrestricted. Only non-breastfed infants were included. The groups did not differ in risk factors or in known confounding factors possibly influencing the incidence of manifestations suggestive of atopic disease. At 6 months, the prevalence of cow's milk protein (CMP) sensitivity was significantly decreased in the hydrolysate group (7% versus 43%; P: 0.002). At the age of 12 (21% versus 53%; P: 0.029), 36 (25% versus 57%; P: 0.018) and 60 months (29% versus 60%; P: 0.016) there was still a significant difference in the number of atopic manifestations, if calculated cumulatively. There was no difference between the groups if only the new cases after the age of 6 months were considered. Eczema was less frequent in the whey-hydrolysate group, but only during the 1st year of life, suggesting a decreased prevalence of CMP sensitivity. During the first 6 months, diarrhoea of non-infectious origin occurred in 8/30 infants (27%) of the adapted formula group, and in no infant in the hydrolysate group. "Colic as single manifestation" was considered of "allergic" origin in 1/28 infants in the hydrolysate group, and in 4/30 infants in the adapted formula group.(ABSTRACT TRUNCATED AT 250 WORDS)