Vitamin D status at birth in Brussels - Preliminary results

SCOPUS: cp.kinfo:eu-repo/semantics/publishe

Monitorage de la pression fontanellaire chez le nouveau-né et le nourrisson. Evaluation d'une nouvelle technique de mesure, détermination des valeurs normales et utilisation clinique.

The experience with 200 measurements of anterior fontanelle pressure with the Rotterdam Teletransducer in newborns and infants is reported. Statistical analysis of 25 comparative measurements between anterior fontanelle pressure and invasive cerebrospinal fluid pressure showed an excellent correlation (rs = 0.95). Measurements were reproducible and the plot quality allowed visualisation of pulse pressure and pressure waves. Normal values of the anterior fontanelle pressure, pulse pressure amplitude and pressure wave maximal amplitude were established in 15 prematures, 27 term newborns and 10 infants. Anterior fontanelle pressure monitoring was performed in 19 term newborns with post-asphyxial encephalopathy, 18 newborns and infants with hydrocephalus, 8 preterm and term newborns with respiratory distress and 19 patients with subdural haematomas, metabolic diseases, meningitis, subarachnoidal haemorrhage, head trauma, post cardiac arrest encephalopathy and abnormal head growth or bulging fontanelle. Abnormal patterns of anterior fontanelle pressure monitoring were found in moderate or severe neonatal post-asphyxial encephalopathy, evolutive hydrocephalus, subdural haematomas, metabolic diseases with hyperammoniemia and other clinical situations. In contrast, anterior fontanelle pressure monitoring yielded normal values in mild post-asphyxial encephalopathy, arrested hydrocephalus, well functioning ventriculo-peritoneal derivation, and in normal infants with rapid head growth or bulging fontanelle. The Rotterdam Teletransducer provides thus accurate and reproducible values of intracranial pressure. Anterior fontanelle pressure monitoring may be of value in many situations in clinical practice

Neuropathological Findings of a Patient With Pyruvate Dehydrogenase-e1-alpha Deficiency Presenting As a Cerebral Lactic-acidosis

Neuropathological findings are reported of a 6-month-old female child with a ''cerebral'' lactic acidosis. A mutation in the pyruvate dehydrogenase (PDH) E1alpha gene was found. Gross examination of the brain revealed a severe thinning of the cerebral parenchyma marked hydrocephalus sparing the aqueduct and fourth ventricle, agenesis of the corpus callosum and heterotopic noduli of gray matter in subependymal regions. Microscopical examination showed heterotopic inferior olives, absent pyramids and focal neuroglial overgrowth into meninges. In addition some heterotopia of Purkinje cells and dysplasia of the dentate nuclei were observed. There was a marked vascular proliferation with many thin-walled, congestive vessels in the cerebral and cerebellar white matter. and to a lesser extent in the striatum. To our knowledge these cerebellar and vascular abnormalities have not been reported before in patients with ''cerebral'' lactic acidosis.The combination of these neuropathological findings might be characteristic for PDH deficiency and more specifically for its E1alpha subtype. Neuropathological examination could lead to the retrospective diagnosis of PDH E1alpha deficiency in those cases where biochemical investigations were not or incompletely performed. This may have potential implications for genetic counseling

The Belgian and Dutch response to COVID-19 : change and stability in the mayors’ position

How did Belgian and Dutch mayors experience their own role and that of local government during the first few months of the COVID-19 outbreak? This question was addressed by drawing on three analytical perspectives (functional, territorial, and political) on local government systems and using a qualitative case study design. Interestingly, the position and leeway of Belgian and Dutch mayors did not differ that much during the first crisis months. The type of virus and scope of the outbreak necessitated a centralised approach. Consequently, the decisions were made at the national level and mayors focused primarily on implementing these central measures. Moreover, Belgian and Dutch mayors experienced a similar shift in terms of (1) power and authority (respectively to the provincial governor and the national level and to the regional mayor and the national level); (2) tasks (prioritising crisis management over other tasks); and (3) roles (increasing importance of their executive role while lamenting the effects of COVID-19 on their social role vis-à-vis their citizens)

Pyruvate-dehydrogenase Deficiency - Clinical and Biochemical-diagnosis

A female neonate with pyruvate dehydrogenase (PDH) deficiency is presented with clinical, radiologic, biochemical, neuropathologic, and molecular genetic data. She was dysmorphic, with a high forehead, low-set ears, thin upper lip, upturned nose, and rhizomelic limbs. Cranial MRI revealed severe cortical atrophy, ventricular dilatation, and corpus callosum agenesis. Pyruvate and lactate levels were increased in CSF and blood. Urinary organic acid profile was compatible with PDH deficiency. PDH activity was normal in fibroblasts, lymphocytes, and muscle. The PDH E1-alpha gene was sequenced and a single base mutation was found within the regulatory phosphorylation site in exon 10. It is postulated that this mutation causes a cerebral form of PDH deficiency. Tissue-specific expression of the disease could be explained by differential X chromosome inactivation because the PDH E1-alpha gene is located on this chromosome. Dysmorphism with severe cerebral malformations in female patients merits a metabolic evaluation, including determination of lactate and pyruvate levels in CSF

Anterior fontanelle pressure monitoring for the evaluation of asymptomatic infants with increased head growth rate

SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Choosing coalition partners in Belgian local government

Government formation is a crucial phase after elections. This article focusses on coalition formation in Belgian local government after the elections in 2006. Coalition theories have mainly been developed at the national level, but they are also applicable to the local level where there is no shortage of coalitions. This level offers the opportunity to not only test rather classic coalition theories such as minimum size theories or the coalitions' incumbency status. In a federal state like Belgium we can compare the local coalitions with coalitions made at higher levels. The results show that the local political parties prefer to form minimal winning and minimum parties coalitions, but not the smallest coalition that is possible. Coalitions are also more likely to form when they represent the incumbent coalition. Finally, political parties prefer congruent coalitions with the federal and regional government