[Influence of age, growth potential and functionality in the management of bone tumours in children : example of the rotationplasty]. / Cas clinique. Influence de l'âge, du potentiel de croissance et de la fonctionnalité dans la prise en charge des tumeurs osseuses de l'enfant : exemple de la plastie de rotation.

Malignant bone tumours in children are rare diseases whose survival rate has progressively improved in recent years thanks to advances in pharmacology, surgery and radiotherapy. We present the particular case of a very young child with Ewing's sarcoma of the thigh. We discuss the influence of this young age on short and long term care. Limb skeletal immaturity and the residual growth potential need to be integrated to define the safest oncological and functional strategy. For this purpose, we summarize the different possible reconstructive options. We describe the Van Nes rotationplasty that was proposed in our patient's case and we will detail the issues in terms of functional well-being and self-image.

Les tumeurs malignes osseuses de l'enfant sont des pathologies rares dont la survie s'est progressivement améliorée au cours des dernières années grâce aux progrès médicamenteux, chirurgicaux et radiothérapeutiques. Nous présentons le cas particulier d'une très jeune enfant atteinte d'un sarcome d'Ewing de la cuisse. Nous discutons de l'influence de ce jeune âge sur la prise en charge à court et à long termes. L'immaturité squelettique et le potentiel de croissance résiduelle des membres doivent être intégrés pour définir l'attitude la plus sûre sur le plan oncologique et la plus fonctionnelle. à cette fin, nous résumons les différentes options reconstructives possibles. Nous décrivons la plastie de rotation de Van Nes qui a été proposée à notre patiente et nous détaillerons les enjeux relatifs au bien-être fonctionnel et à l'image de soi.

[Prostaglandins and the immune response at the intestinal submucosal level. A potential site for interference with the repeated use of paracetamol and ibuprofen at a young age?]. / Les prostaglandines et l'immunité du chorion sous-muqueux intestinal. Questions à propos de l'usage répété du paracétamol et de l'ibuprofène en bas âge.

Immune deviations have been shown to exponentially increase in young children. As a consequence, research investigating possible environmental reasons for this increase is considered a public health priority. An improved understanding of the immunity of the intestinal submucosal lamina propria has demonstrated the importance of prostaglandins (PGE2s) on its local development with general immune consequences further on. PGE2s appear at this intestinal submucosal level from the metabolism of arachidonic acid mediated by type-2 cyclooxygenases (COX2s) situated in the membranes of many immune cells. The potential risk of repeated inhibition of PGE2 synthesis at a young age has been demonstrated in experiments with animals systemically exposed to a non-steroidal anti-inflammatory drug (NSAID). The repeatedly exposed animal cannot develop tolerance to food antigens and exhibits autoimmune deviations. Acetaminophen (paracetamol) and ibuprofen are analgesic and antipyretic medications given to children either alone or in combination, most often without medical prescription. Recently, it has been demonstrated that paracetamol, like ibuprofen, also carries, besides its central action, a non-selective inhibitory action on peripheral COXs. However, this inhibitory action only relates to physiological concentrations of arachidonic acid and explains the difference in their respective anti-inflammatory effects. Since recently published data have repeatedly reported an increase of immune deviations associated with paracetamol exposure at a young age, it appears important to better understand the possible negative impact of excessive and repetitive inhibitions of the physiological synthesis of prostaglandins by COX2s in childhood during which all immune mechanisms are built up at the intestinal submucosal level. Therefore, a well-designed prospective strategy for pharmacovigilance of these COX inhibitors repeatedly given during childhood is urgently needed.

Neonatal acute myeloid leukemia in an infant whose mother was exposed to diethylstilboestrol in utero.

We report on an acute myeloid leukemia in a neonate whose mother was exposed to diethylstilboestrol in utero. The newborn presented with leukemia cutis, hemorrhagic skin lesions, hyperleucocytosis and disseminated intravascular coagulation. A bone marrow examination confirmed the diagnosis of acute monocytic leukemia with a t(11;19) MLL-ELL fusion transcript. Chemotherapy was initiated but the child developed a bilateral pulmonary infection that led to fatal respiratory distress. This case shows acute myeloid leukemia and the third pediatric leukemia reported after maternal diethylstilboestrol exposure.

[A particular hereditary anemia in a two-month-old infant: elliptocytosis]. / Une forme particulière d'anémie constitutionnelle chez un nourrisson de deux mois: l'elliptocytose.

We report the case of a 2.5-month-old infant with severe anaemia discovered fortuitously during an acute febrile illness. The patient was admitted because of a septic arthritis of the knee. Initial biology showed a 3.5 g/dl haemoglobin concentration. The anaemia was microcytic and hypochromic, with obvious haemolysis and reticulocytosis. Standard analysis was not contributive. Further investigations allowed the diagnosis of elliptocytosis. The patient was treated by antibiotics, orthopaedic measures and iterative transfusions. Now, 18 months from the initial episode, she is in good health. With this history, we discuss the clinical process facing severe anaemia during infancy and review the particularities of such uncommon congenital anaemia. Elliptocytosis is a haemolytic anaemia caused by congenital anomalies of the erythrocyte membrane. Diagnosis requires morphological studies of the red blood cells on peripheral blood smear. The disease is often overlooked by membrane protein electrophoresis. The condition is heterogeneous concerning clinical, biochemical and genetic aspects. Most of the cases are linked to mutations of the alpha-spectrin gene, in autoassociation regions. Search of spectrin and protein 4.1 genes mutations can confirm the diagnosis but is not routinely performed.

Clonal monosomy 7 and 5q--in a child with myelodysplastic syndrome.

The authors report the case of a 5-year-old boy referred for thrombocytopenia and neutropenia. Bone marrow examination showed a myelodysplasia with clonal monosomy 7. The acceleration of the disease was marked by the appearance of an additional cytogenetic abnormality, i.e., the deletion of the long arm of chromosome 5 in the clonal cells. RAS gene mutation was not detected. Chemotherapy was started to achieve complete remission before a bone marrow transplantation. This treatment was complicated by a prolonged aplasia and the patient died of systemic mycotic infection.

[Perinatal thrombocytopenia of maternal origin]. / Les thrombopénies périnatales d'origine maternelle.

Maternal causes of perinatal thrombocytopenia include neonatal alloimmune thrombocytopenia, autoimmune disorders, intrauterine infections and hypertensive diseases. The diagnosis of these pathologies is difficult because they can occur in sick neonates, but also in healthy babies without a history suggesting illness. The treatment has to be quickly established in order to decrease eventual hemorrhagic complications. These latter can be amplified by several clinical circumstances and especially by the platelet immaturity of this time of life. The risk of recurrence for the next pregnancies has to be determined and can lead to diagnostic or therapeutic measures during the antenatal period.

Arteriomesenteric syndrome as a cause of duodenal obstruction in children with cerebral palsy.

Two cases of gastroduodenal outlet obstruction caused by arteriomesenteric compression in children who have cerebral palsy are reported. Clinical symptoms of gastrointestinal obstruction include recurrent postprandial nausea and vomiting, upper abdominal distension, and pain. In such patients, multiple predisposing factors can contribute to the development of arteriomesenteric compression, including marked weight loss, supine position, and severe scoliosis. Upper gastrointestinal x-rays using barium contrast allow diagnostic confirmation. In our experience, this cause of acute gastroduodenal outlet obstruction may usually resolve after conservative treatment using a jejunal feeding tube passed beyond the compression, left lateral positioning, and renutrition.