Tumor- and treatment-related side effects after multimodal therapy of childhood intracranial germ cell tumors

Multimodality treatment approaches have dramatically improved the outcome of patients with intracranial germ cell tumors and are resulting in an increasing number of long-term survivors. The aim of the present study was to evaluate prospectively the development of side effects in children, adolescents and young adults after treatment for intracranial germ cell tumors. Nine patients with a median age of 14 y at diagnosis and a median follow-up of 7.25 y underwent a detailed long-term evaluation including physical and neuro-ophthalmologic examinations, routine laboratory and endocrine stimulation tests, neuropsychometric testing, audiometry and spirometry at repeated intervals. Endocrine deficiencies requiring hormone replacement therapy occurred in all patients. Neuro-ophthalmologic side effects were observed in 8 of the 9 patients, urinary electrolyte wasting in 4 of the 9, alopecia in 3 of the 9 and high-frequency hearing loss in 2 of the 9. Neuropsychologic examinations revealed pathologic results in all five tested patients. Conclusion: The present study indicates that former intracranial germ cell tumor patients suffer from remarkable long-term side effects, and that some of these late effects can develop or worsen months or years after cessation of oncologic therapy. Since life quality is an important parameter of cancer survival, careful follow-up of long-term survivors is mandatory, aimed at counteracting side effects as early as possible and therefore at minimizing long-term morbidity, which may considerably compromise quality of life.     

Hematopoietic stem cell transplantation for CD40 ligand deficiency: Results from an EBMT/ESID-IEWP-SCETIDE-PIDTC study

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Composite restorative resins: Composition versus wall-to-wall polymerization contraction

Defects in the prenatal development of the brainstem can result in cranial nerve deficiencies. As the development of tooth germ is dependent on n. trigeminus, which originates from the brainstem, the hypothesis underlying this study was that anomalies of the brainstem would lead to an increased prevalence of tooth agenesis. Twenty-three patients (13 F and 10 M, age range 6-37 years) were studied, all with myelomeningocele and brainstem anomalies (Chiari II). They were examined retrospectively from the data in journals and dental radiographs and compared to available data on the prevalence of tooth agenesis in the Swedish population. Our hypothesis was rejected, since there was insignificant difference in the frequency of agenesis in our material (8.7%) compared with that of the Swedish population (7.4%).     

Dysoria,a new dimension of pathology

Summary The starting point of our research was the recognition that such pronounced brain-toxins as lead, manganese and thallium affect the central nervous system without being present there and that they operate through mediators. Other poisons which permeate easily the brain are also dependent on mediators to produce lasting brain injury. Representatives of this group are ethyl alcohol and tellurium.Amongst the mediators most prominent are the toxic deficiencies of thiamine, of riboflavin, of vitamin E and of the very important Geiger's liver factors. All these deficiencies result in dyskrasia, in the terminology of Rokitansky's humoral pathology. The dyskrasia which interfere with the energy metabolism have morphological correlates while other non interferring ones, as LSD, marihuana, and the opium alkaloids do not. This cognizance instigated a series of investigations of the dyskrasic dysergosis (dys = faulty and ergon = work, i.e. energy) which produce structural changes only when chronic. In contrast chronic hypoxia, hypoxemia and cardiopathic oligemia have no neuropathology because of the brain's adaptational mechanisms against chronic circulatory disorders.Another basic difference is that while the brunt of the damage in hypoxic hypoxemic and cardiopathic dysergosis is borne by the neuron, the prime target of the dyskrasic dysergosis is the blood-tissue barriers, the dysfunction of which results in dysoria (Schürmann and McMahon, 1933). The dysory plays a decisive role in such a vast number of chronic degenerative diseases of the brain and of the extracerebral organs, that it amounts to a new dimension of pathology.This investigation was supported by a Research Grant NB-02275 from the National Institute of Neurological Diseases and Blindness, National Institutes of Health, Bethesda, Maryland.     

Conduite à tenir pratique pour l’exploration d’une malabsorption,d’une maldigestion,et d’une entéropathie exsudative

Malabsorption syndrome can be suspected in situations ranging from isolated deficiencies to the classic association between abundant chronic diarrhea and weight loss. Pitfalls in diagnosis come from the multiplicity of pathophysiological mechanisms. Malabsorption can happen in the gut lumen (maldigestion), the intestinal wall, or involve transporters beyond the enterocyte. The challenge is in the diagnostic process, which requires a thorough questioning and physical examination, conducting laboratory tests, endoscopy and imaging studies to confirm malabsorption and find its etiology so as to provide the best treatment.     

Traitement psychomoteur de la carence en milieu institutionnel. Soutenir le développement du jeune enfant dans le cadre d’une pouponnière de l’Aide Sociale à L’Enfance

When a young child (from 0 to 3 years old) is taken away from his family due to sanitary, emotional, or educational deficiencies, the Children's Social Aid generally places him in a nursery. It is in this antechamber that he will wait until justice and social services find an “educational solution” more favorable to his development. In this context of community, it is not uncommon for the child to freeze, an anxious observer of an environment in perpetual movement, thus putting development and acquisitions on hold. The care of the psychomotricist care then has two successive objectives: firstly, to support the emergence of self-confidence, and secondly, to allow physical experiences likely to remobilize the child in the pursuit of his development.