Intima media thickness of carotid arteries in familial Mediterranean fever: a systematic review and meta-analysis

Clinical Rheumatology - To perform a systematic review and meta-analysis of studies reporting data on atherosclerosis and inflammatory markers in familial Mediterranean fever (FMF). EMBASE and...     

Lateral cervical approach for salvage total laryngectomy without pharyngeal reinforcement with pedicled or free flaps: our preliminary experience in six patients

European Archives of Oto-Rhino-Laryngology - To analyze the surgical outcomes in terms of postoperative wound complications in a small case series of six patients treated with lateral cervical...     

Deferasirox chelation therapy in patients with transfusion‐dependent MDS: a ‘real‐world’ report from two regional Italian registries: Gruppo Romano Mielodisplasie and Registro Basilicata

Deferasirox (DFX) is an orally administered iron chelator approved for use in patients with transfusion‐dependent iron overload due to myelodysplastic syndromes (MDS). The safety and efficacy of DFX has been explored in clinical trial settings, but there is little data on unselected patients with MDS. The aim of this study was to retrospectively evaluate the safety, compliance, efficacy and effect on haematopoiesis of DFX in a large ‘real‐world’ MDS population. One hundred and eighteen patients with transfusion‐dependent MDS were treated with DFX across 11 centres in Italy. Serum ferritin levels, haematological response, dosing, adverse events and transfusion dependence were recorded at baseline, 3, 6, 12 and 24 months following initiation of treatment. DFX reduced mean serum ferritin levels from 1790 to 1140 ng/mL (P < 0.001), with 7.1% of patients achieving transfusion independence. Significant haematological improvement was seen in erythroid (17.6%), platelet (5.9%) and neutrophil counts (7.1%). Adverse events were reported in 47.5% of patients, including gastrointestinal and renal toxicity. Regression analysis showed that higher starting doses of DFX are associated with transfusion independence at 24 months. DFX is a safe, effective treatment for transfusion‐dependent MDS that can lead to transfusion independence and haematological improvement in a subset of patients.     

Neuropsychiatric clinical manifestations in elderly patients treated with hydroxychloroquine: a review article

Little is known about the development of psychosis during hydroxychloroquine (HCQ) treatment, especially in elderly patients affected by rheumatic diseases, with multiple comorbidities and treatments. To summarize the available evidence on HCQ-induced psychosis in elders, we performed a literature review. Additionally, individual case safety reports sent to the European Pharmacovigilance database (EudraVigilance) with HCQ as suspected drug and related to adverse events belonging to the System Organ Class ‘Psychiatric disorders’ were shown. Over the years, evidence was published about the risk of neuropsychiatric clinical manifestations during HCQ treatment for rheumatic diseases, but few of them were related to elderly patients. These adverse events can include less severe clinical manifestations such as affect lability and nervousness or more severe conditions such as actual psychosis and suicidal tendencies, which frequency are actually unknown. The presence of risk factors in these patients may precipitate HCQ-induced psychosis and their precocious detection could be associated with a risk minimization. Among predisposing risk factors, there are the co-exposure to interacting drugs, alcohol intake, familial history of psychiatric diseases, female gender, and the concomitant use of low-dose glucocorticoids. In some cases it was possible to reverse psychotic behaviour with the antipsychotic treatment or with HCQ suspension.     

Cyclopia: a radiological and anatomical craniofacial post mortem study

Cyclopia is a rare foetal malformation characterized by a single palpebral fissure and a proboscis associated with severe brain malformation. Approximately 1.05 in 100 000 births including stillbirths are identified as cyclopian. Cyclopia is not compatible with life. The authors present an anatomical and histological study of the fronto-orbito-maxillary region carried out after 3-D CT reconstruction in a 21 week-old foetus with cyclopia. Anatomical and histological observations suggest that the integrity of the trigeminal nerves is very important for the normal development of the embryological structures of the face. Fusion of the facial processes in the midline takes place even if central prosencephalic structures are absent. For this reason the face in cyclopia, in both its positive and negative aspects, constitutes a model for the study of the normal development of this region. Copyright 2001 European Association for Cranio-Maxillofacial Surgery.