We report the case of an infant boy born at 39 weeks and 5 days who presented a cleft lip and palate. During the assessment to detect associated malformations, cardiac auscultation revealed a systolic heart murmur. Echocardiography and subsequent thoracic computed tomography angiography identified an aneurysm of the ductus arteriosus. Ductus arteriosus aneurysm, a complicated form of patent ductus arteriosus, may not be as rare as once considered. A clear majority of ductus arteriosus aneurysms are asymptomatic and physiologically resolve after birth. Nevertheless, severe complications can occur, primarily during the postnatal period.

Abdominal aortic aneurysms (AAAs) expand because of aortic wall destruction. Enrichment in Vascular Smooth Muscle Cells (VSMCs) stabilizes expanding AAAs in rats. Mesenchymal Stem Cells (MSCs) can differentiate into VSMCs. We have tested the hypothesis that bone marrow-derived MSCs (BM-MSCs) stabilizes AAAs in a rat model.Rat Fischer 344 BM-MSCs were isolated by plastic adhesion and seeded endovascularly in experimental AAAs using xenograft obtained from guinea pig. Culture medium without cells was used as control group. The main criteria was the variation of the aortic diameter at one week and four weeks. We evaluated the impact of cells seeding on inflammatory response by immunohistochemistry combined with RT-PCR on MMP9 and TIMP1 at one week. We evaluated the healing process by immunohistochemistry at 4 weeks.The endovascular seeding of BM-MSCs decreased AAA diameter expansion more powerfully than VSMCs or culture medium infusion (6.5% ± 9.7, 25.5% ± 17.2 and 53.4% ± 14.4; p = .007, respectively). This result was sustained at 4 weeks. BM-MSCs decreased expression of MMP-9 and infiltration by macrophages (4.7 ± 2.3 vs. 14.6 ± 6.4 mm2 respectively; p = .015), increased Tissue Inhibitor Metallo Proteinase-1 (TIMP-1), compared to culture medium infusion. BM-MSCs induced formation of a neo-aortic tissue rich in SM-alpha active positive cells (22.2 ± 2.7 vs. 115.6 ± 30.4 cells/surface units, p = .007) surrounded by a dense collagen and elastin network covered by luminal endothelial cells.We have shown in this rat model of AAA that BM-MSCs exert a specialized function in arterial regeneration that transcends that of mature mesenchymal cells. Our observation identifies a population of cells easy to isolate and to expand for therapeutic interventions based on catheter-driven cell therapy.

Our previous study found that most Mycoplasma pneumoniae (MP) pneumonia (MPP)patients had elevated serum total immunoglobulin E (IgE) levels.To determine components of MP that can cause an IgE increase in children, and to clarify its specific mechanism.The components of MP cells were isolated by serum IgE from patients with MP pneumonia. These components obtained through the prokaryotic expression were used as allergens to detect the proportion of allergen-specific IgE produced in MPP patients, and the clinical characteristics and related immune parameters of these patients who produced this allergen-specific IgE were also analyzed. In addition, a cell experiment was used to verify the biological effect of these components in vitro.P1-specific IgE was detected in serum of MPP children. An approximately 24-kDa polypeptide of P1 protein was obtained through prokaryotic expression purified by nickel agarose affinity chromatography. Approximately 9.2% of MPP patients produced IgE against this polypeptide of P1 protein, which was more likely to be produced in MPP patients with no history of allergies or family history of allergy-related diseases. P1-specific IgE-positive MPP patients had more severe clinical symptoms, with excessive secretion of interleukin (IL)-4 and IL-5 and overdifferentiation of Th0 cells into Th2 cells. Tests also demonstrated that the P1 protein stimulated excessive secretion of IL-4 and IL-5 in peripheral blood mononuclear cells from the peripheral blood of healthy donors.Mycoplasma pneumoniae is not only an infectious agent but also an allergen for certain individuals. The P1 protein of MP can induce the production of P1-specific IgE.

An 11-year-old girl developed severe varicella with a number of classic complications (pulmonary, neurological, etc.) and bone marrow aplasia. This seems to be the first published of bone marrow aplasia due to varicella.

Chez un petit garcon de 3 ans, il est decouvert a l'examen clinique une masse abdominale de l'hypochondre droit. Certains examens paracliniques ont fait suspecter une tumeur surrenalienne droite, d'autres orientaient vers une origine inframediastinale. Cette imagerie nous a paru interessante car elle rappelle la difficulte a localiser certaines masses paradiaphragmatiques et permet d'envisager les signes radiologiques orientant vers une localisation anatomique precise.

In a three-year-old boy with a palpable mass in the right upper abdominal quadrant, some imaging studies suggested a tumor of the right adrenal gland and others a tumor of the lower part of the mediastinum. This case illustrates the difficulty of localizing neuroblastomas in some patients and provides the opportunity for reviewing signs that point to a given site.

Background. - Cerebral arteriovenous malformations are usually treated by excision or selective embolization. Some untreatable malformations may be presently cured by radiosurgery.Case report. - A 10 year-old girl without previous illness suffered from a sudden quadriventricular hemorrhage due to arteriovenous malformation. Initially the child recovered with symptomatic treatment. Neurosurgery and arterial embolization being impossible. Gamma-Unit treatment was performed. Treatment was first well tolerated but 8 months later, left hemiparesis and language disorders occurred, due to radionecrosis. These complications were dramatically improved by corticosteroids.Conclusion. - Gamma-Unit treatment can be successfully proposed for treating those malformation that are inaccessible to surgery or embolization.

BACKGROUND:Cerebral arteriovenous malformations are usually treated by excision or selective embolization. Some untreatable malformations may be presently cured by radiosurgery.CASE REPORT:A 10-year-old girl without previous illness suffered from a sudden quadriventricular hemorrhage due to arteriovenous malformation. Initially the child recovered with symptomatic treatment. Neurosurgery and arterial embolization being impossible, Gamma-Unit treatment was performed. Treatment was first well tolerated but 8 months later, left hemiparesis and language disorders occurred, due to radionecrosis. These complications were dramatically improved by corticosteroids.CONCLUSION:Gamma-Unit treatment can be successfully proposed for treating those malformations that are inaccessible to surgery or embolization.

Hypokalemia and hyponatremia increase the occurrence of atrial fibrillation. Sinoatrial nodes (SANs) and pulmonary veins (PVs) play a critical role in the pathophysiology of atrial fibrillation.The purpose of this study was to evaluate whether electrolyte disturbances with low concentrations of potassium ([K+]) or sodium ([Na+]) modulate SAN and PV electrical activity and arrhythmogenesis, and to investigate potential underlying mechanisms.Conventional microelectrodes were used to record electrical activity in rabbit SAN and PV tissue preparations before and after perfusion with different low [K+] or [Na+], interacting with the Na+–Ca2+ exchanger inhibitor KB-R7943 (10 μΜ).Low [K+] (3.5, 3, 2.5, and 2 mM) decreased beating rates in PV cardiomyocytes with genesis of delayed afterdepolarizations (DADs), burst firing, and increased diastolic tension. Low [K+] (3.5, 3, 2.5, and 2 mM) also decreased SAN beating rates, with genesis of DADs. Low [Na+] increased PV diastolic tension, DADs, and burst firing, which was attenuated in the co-superfusion with low [K+] (2 mM). In contrast, low [Na+] had little effect on SAN electrical activities. KB-R7943 (10 μΜ) reduced the occurrences of low [K+] (2 mM)– or low [Na+] (110 mM)–induced DAD and burst firing in both PVs and SANs.Low [K+] and low [Na+] differentially modulate SAN and PV electrical properties. Low [K+]– or low [Na+]–induced slowing of SAN beating rate and genesis of PV burst firing may contribute to the high occurrence of atrial fibrillation during hypokalemia or hyponatremia.

Certaines malformations artérioveineuses cérébrales qui ne peuvent pas être traitées par la chirurgie ou par embolisation artérielle sélective peuvent bénéficier de la radiothérapie multifaisceaux focalisée.

L'association diabète insulinodépendant (DID) - pathologie auto-immune a été décrite mais reste mal expliquée. Nous rapportons le cas d'un diabète néonatal vrai associé à une maladle auto-immune.