[Septic shock following platelet transfusion contaminated with Citrobacter koseri in a child with postchemotherapy febrile neutropenia]. / Choc septique après transfusion d'un concentré plaquettaire contaminé par Citrobacter koseri chez une patiente en aplasie fébrile post-chimiothérapie.

The bacterial transfusion risk is currently the greatest infectious risk of blood transfusion. We report the case of a child with postchemotherapy febrile neutropenia who presented septic shock following platelet transfusion contaminated with Citrobacter koseri. The life-threatening development could have been avoided by strict compliance with good clinical practice. The stability of mortality rates due to adverse effects of bacterial proliferation during platelet transfusions in France since 1994 calls for optimization of all preventive measures throughout the transfusion chain and perfect knowledge of transfusion rules by medical staff and care givers.

Prenatal management of congenital infantile fibrosarcoma: unexpected outcome.

We report the case of a large leg tumor in a fetus at 36 weeks of gestation. The tumor's location, ultrasound characteristics and magnetic resonance imaging (MRI) findings enabled a prenatal diagnosis of congenital fibrosarcoma and allowed us to establish the prognosis. After multidisciplinary discussion, it was decided that the pregnancy should continue to 38 weeks, with surgery or neo-adjuvant chemotherapy scheduled after delivery. Unfortunately, the tumor unexpectedly burst in utero and the newborn died of the consequences of hemorrhagic shock, despite rapid amputation. Histological examination confirmed the nature of the tumor. The prognosis of congenital fibrosarcoma is generally good when there are no metastases. MRI is key to establishing a prenatal diagnosis. However, rupture of the tumor can result in fatal hemorrhaging and the pregnancy should be monitored closely before scheduled delivery.

Cyanosis revealing hepatopulmonary syndrome in a child with dyskeratosis congenita.

We report the case of a 5-year-old child with dyskeratosis congenita who presented cyanosis and dyspnea at exertion. He had severe hypoxemia with elevated alveolar-arterial oxygen gradient in the setting of liver disease. Technetium-99m-labeled macroaggregated albumin scan showed abnormally high uptake in the brain, confirming hepatopulmonary syndrome.

[Sedation using ketamine for pain procedures in Pediatric Oncology.]. / Sédation par kétamine en prévention de la douleur liée aux gestes invasifs en oncologie pédiatrique.

Procedural sedation and analgesia for children is widely practiced. Since 2005 to 2007, we evaluated the safety and efficacy of ketamine to control pain induced by diagnostic procedures in pediatric oncology patients. Eight hundred fifty procedures were carried out in 125 patients aged 2 to 16 years. We associated EMNO (inhaled equimolar mixture of nitrous oxide and oxygen), atropin (oral or rectal), midazolam (oral or rectal) and ketamin (intravenous). An anesthesiologist injected ketamin. Average dose of ketamine was 0.33 to 2 mg/kg depending on number and invasiveness of procedures. This method requires careful monitoring and proper precautions. With these conditions, no complication was observed. All patients were effectively sedated. These results indicate that ketamine - in association with EMNO, atropine and midazolam - is safe and effective in pain management induced by diagnostic procedures in pediatric oncology patients. The sedative regimen of intravenous ketamine has greatly reduced patient, family and practitioners anxiety for diagnostic and therapeutic procedures.

Inherited long QT syndrome revealed by antifungals drug-drug interaction.

A 14-year-old Tahitian girl with acute myeloid leukaemia and a suspected mucormucosis infection was treated with intravenous voriconazole and caspofungin. Because of worsening of fungal infection, voriconazole was switched to posaconazole. During the switch, the patient presented with QT interval prolongation with 'torsades de pointes' and reversible cardiac arrest. Voriconazole plasma level measured 15 h after the last administration was 7 mg/L. Genotyping suggested that the patient was an extensive metabolizer with respect to CYP2C9 and CYP2C19. The association of antifungal agents with pro-arrhythmogenic drugs and other risk factors led to torsades de pointes and the revealing of inherited QT syndrome.

[BK virus infection in a child after an hematopoietic stem cell transplantation]. / Infection à BK Virus après allogreffe de cellules hématopoïétiques. A propos d'un cas.

Polyomavirus hominis 1, better known as BK virus (BKV), infects up to 90% of the general population. Significant clinical manifestations can be seen in immunocompromised patients. We report a case of haemorragic cystitis likely due to BKV in a child after allotransplantation of hematopoietic stem cells. A 10-year old boy with poor-prognosis acute T lymphoblastic leukaemia underwent cord blood allogeneic stem cell transplantation while in his first relapse. Macroscopic haematuria and low back pain occurred by day 95, in the context of acute graft versus host disease and pulmonary aspergillosis. Histopathologic examination showed a cytopathogenetic effect consistent with the diagnosis of BKV infection. Urinary PCR was positive for BKV. Treatment with cidofovir was followed by a marked improvement of urinary symptoms. The current understanding, diagnosis, and treatment of BKV-associated infection is discussed.