Transfusional iron overload in patients with myelodysplastic syndromes: A 10-year retrospective survey from a French general hospital

We retrospectively assessed the characteristics of 165 MDS patients from our institution having received at least 20 RBC units. In the vast majority of them various comorbidities (range: 1–6 per patient) were registered including mainly cardiovascular disorders. Serum ferritin was over 1000 μg/L in about half of tested individuals. A chelator agent was initiated in 43.6% of patients (mainly low-risk MDS). Transformation in AML occurred in 46 cases (27.8%). Overall, 112 patients died during follow up. The cause of death was documented in 65 cases and included mainly MDS or AML resistance to therapy. There was a context of bacterial or fungal-related sepsis in 35.3% of cases. We noticed a correlation between survival and number of RBC transfusions. Median OS from the 20th RBC unit was significantly prolonged among the chelated subgroup. Consequences of transfusional iron overload and chelation need to be clarified in MDS patients.     

Transcatheter ductus arteriosus stenting in paediatric cardiology: Indications,results and perspectives

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Fistules du dorsum nasal de l’enfant : mise au point à propos d’une série de 6 cas

IntroductionNasal dermal sinus in children (NDSC) is a rare malformation (1/20,000 to 1/40,000). Apart from local infection, they present as median nasal lump or pit on the dorsum and their diagnosis and treatment are often delayed. Consequences of untreated NDSC are: local infection, meningitis, and empyema, due to their frequent intracranial extension.Patients and methodsSix cases of NDSC were retrospectively reviewed (5 boys and one girl), all treated between 2006 and 2012 in our institution (Nancy University Hospital). All patients underwent a brain and facial CT-scan and MR imaging to check any bony lesions, skull base extension by foramen cæcum, course of the sinus and the possible associated brain malformations. Evolution, treatment and follow-up (FU) were conducted by the same multidisciplinary team (neurosurgeon, ENT surgeon, and plastic surgeon). Children were operated on by a conjoined approach (cranial and facial) for removal of the sinus and its intracranial extension.ResultsMean age at diagnosis was 12 months (birth–36 months). Initial presentation consisted of three local infections, one dorsum nasal lump, one CSF leakage, and one asymptomatic child. Five children presented with a skull base extension. There were no associated brain malformations. We observed only one surgical complication (bleeding from the anterior part of the superior sagittal sinus during dissection) leading to blood transfusion. Pathology results confirmed three dermoid cysts, one epidermoid cyst, one cyst with granulation tissue, and negative in one case. Average FU was 30.8 months (4–84 months). Two recurrences (same child) occurred, leading to two re-operations. There were no recurrences or complications at the end of FU.ConclusionNDSC are rare malformations, mostly diagnosed before the age of three years, due to an infectious complication. The aim of the treatment is complete removal to avoid recurrence, and a multidisciplinary strategy is required.