Evaluation of joints in children with haemophilia is important in detecting abnormalities, staging their severity and following the effects of treatment. The aim of this study is to evaluate the correlation of FISH score (Functional Independence Score in Haemophilia) with the scores obtained by MRI and sonography for the diagnosis, evaluation and classification of arthropathy in severe haemophilia. In this cross-sectional study on 25 severe haemophilia patients, FISH, MRI and sonography procedures were performed in the elbow or knee joint. All patients' information, including age, type of haemophilia, affected joint, scores of MRI, sonography and FISH, dose of factor consumed, weight and prophylaxis protocol were collected and analysed. Among the 25 patients (age range of 11-70 years), 22 patients were haemophilia A and three patients were haemophilia B. Affected joints were right knee in 12 patients, left knee in nine and right elbow in four. There was only a statistically significant negative correlation between FISH and MRI Additive (A) scale (rs = -0.537, P = 0.006). Considering cartilage loss domain (related MRI A scale: 13-20), 20 patients (80%) were classified in this group with FISH scores ranged from 17 to 22. On the basis of our results, FISH scores in severe haemophilia patients were negatively correlated with MRI A scale. Also, it seems that a FISH score less than 22 could be considered as loss of cartilage; however, due to the small number of our patients, it needs further assessment in different populations.
Case 1 A 3.5-year-old girl presented with 3 week history of shortness of breath, non-productive cough, fever and right-sided non-pleuritic chest pain. She was taken two courses of oral antibiotics but without any improvement. She was previously fit and well without history of exposure to second hand smoking. Initial investigations showed normal complete blood counts, liver and kidney function tests. Serum electrolytes and urine analysis were also normal. Chest radiography revealed complete opacification of the right hemithorax, contralateral mediastinal shifting consistent with right sided large pleural effusion (Fig. 1). Contrast enhanced chest computed tomography scan (CT scan) confirmed large right pleural effusion with associated mediastinal shift and complete right lung collapse. No abnormality was reported in the left hemithorax or upper abdomen. Right thoracotomy and decortication revealed large un-resectable masses with adherent deposits on the diaphragm and enlarged pericardial lymph nodes. The right middle lobe was obliterated. Multiple biopsies were taken from tumor and lymph nodes. Pleural fluid analysis demonstrated an exudative pattern. Bone marrow aspiration and biopsy were normal.Due to her critical condition with suspicion of primitive neuroec-
Background and Aims A recent Government policy drive is to increase home delivery rates. Data are lacking about whether this strategy is embraced by perinatal healthcare professionals. Our aim was to examine opinions regarding home deliveries held by consultant paediatricians, neonatologists, gynaecologists, obstetricians, GPs and midwives. Methods Cross-sectional survey of UK professionals in East Anglia. Likert scales ranging from 0-10 assessed professionals' general experiences of and enthusiasm for home birthing and support for the Government's plan. Results 52% of professionals responded, including 68% of Paediatricians. Paediatricians and Neonatologists generally reported negative experiences of home delivery and were considerably less enthusiastic regarding home deliveries than any other professionals. Paediatricans generally held a negative outlook on home delivery [median 4, IQ 3-5] and were in opposition to the government's plan [median 3, IQ 2-5], whilst midwives were more enthusiastic about home delivery than any other profession [median 9, IQ 8-10, p<0.0001] and were more likely to support the government plan to increase the rate of home deliveries [median 8.5, IQ 7-10, p<0.0001]. GP's, obstetricians and gynaecologists tended to give more neutral or negative opinions towards home birth [GP (median 5, IQ 3-7.5) ObGyn (median 5, IQ 2-7)] and towards the government's plan [GP (median 5, IQ 2-6) ObGyn (median 5, IQ 2-5)]. Conclusions Negative experiences and opinions of perinatal healthcare professionals regarding home delivery may adversely affect its uptake by women and will need to be addressed if the Government's plan to increase home delivery rates is to succeed.
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