The Prevalence and types of congenital anomalies in new born

Objective: The primary objective; determin the prevalence and types of congenital anomalies among newborns admitted in Tishreen Hospital. The secondary objective : determin the relationship between  the congenital anomalies and ,sex of the baby, age of mother, consanguinity of parents, family story. Methods: The study sample included  neonates with congenital malformations who were admitted to the Tishreen Hospital during 2018- 2019.The following  is done for  each child who has seen  congenital malformation : Echocardiography ,echoencephalogram ,abdominal ultrasound, and another investigation according to examination assets .. Results : Congenital abnormalities were observed  in 60 out of 1871 child, giving a prevalence of 3,2%.Among the various types of congenital abnormalities , the highest percentage was observed in the ones related to digestive system malformations(43,3), followed by nervous and heart anomalies (21,6%), the gender of fetus affected the prevalence of congenital abnormalities , males comprised 65% of anomalies ,females comprised 33,3% births . the highest congenital abnormality is observed among babies delivered by mother age between (30-45)years . from 60 cases with anomalies ,35(58,3%) cases were from familial marriage هدف  الدراسة: -الهدف اساسي : - تحديد نسبة انتشار التشوهات الخلقية الملاحظة لدى  حديثي الولادة المقبولين في شعبة أمراض الوليد في مشفى تشرين الجامعي . دراسة نوع التشوهات الخلقية الملاحظة لدى حديثي الولادة المقبولين في شعبة أمراض الوليد. - الهدف الثانوي: - دراسة علاقة هذه التشوهات مع كل من: جنس  الوليد، ترتيب الحمل ، عمر الأم ، وجود قرابة بين الوالدين ، وجود قصة عائلية . طريقة البحث والمرضى: شملت  الدراسة  سجلات الولدان المصابين بتشوهات خلقية ، الذين تم قبولهم في شعبة الوليد والخديج في مشفى تشرين الجامعي في اللاذقية بين عامي 2018-2019 . _ أجري إيكو قلب ، إيكو بطن ، إيكو عبر اليافوخ الأمامي لدى كل تشوه خلقي، كما أجريت استقصاءات أخرى حسب المعطيات السريرية والتوجه السببي . النتائج: تم قبول 1871 وليداً في شعبة أمراض الخديج والوليد في مشفى تشرين الجامعي في اللاذقية خلال فترة الدراسة الممتدة بين عامي 2018-2019, إذ بلغ عدد الولدان المصابين بالتشوهات الخلقية 60 وليداً (39ذكراً، 21أنثى ) مما شكل نسبة انتشار3،2% ، و توصلت هذه الدراسة إلى أن نسبة تشوهات الجهاز الهضمي هي الأعلى إذ بلغت ( 43،3% ) ، تلتها تشوهات  الجهاز العصبي وتشوهات الجهاز القلبي الوعائي ، إذ بلغتا(6،21% ) ، وبالنظر إلى جنس الوليد فإن( 39) من الذكور سجلت لديهم تشوهات خلقية أي بنسية(65%)، مقابل بينما عانت21 من الإناث منها ،أي بنسبة (35%) ، وتوصلت هذه الدراسة إلى أن نسبة التشوهات الخلقية تزداد عندما تنتمي الأمهات إلى المجموعة العمرية(30-45) سنة .كما سجلت 35 حالة تشوه خلقي من عائلاتٍ بدرجات قرابة مختلفة أي بنسبة 58,3%

Suppression of mitoxantrone cardiotoxicity in multiple sclerosis patients by dexrazoxane

Objective To explore the potential of dexrazoxane to suppress subclinical cardiotoxicity in MS patients receiving mitoxantrone. Methods An open-label study was performed to evaluate possible subclinical cardiotoxicity in multiple sclerosis patients treated quarterly with mitoxantrone (48mg/m 2 cumulative), with and without concomitant dexrazoxane, using blinded serial radionucleide ventriculography. Results No patient experienced symptoms of heart failure. Patients receiving dexrazoxane, which is cardioprotective for anthracyclines, exhibited a significantly lesser decline in left ventricular ejection fraction (mean change, −3.80% vs −8.55%, p < 0.001). Interpretation These results support a cardioprotective effect of dexrazoxane in mitoxantrone treated multiple sclerosis patients. Ann Neurol 2006;59:206–209Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/49281/1/20747_ftp.pd

Assessment of potential cardiotoxic side effects of mitoxantrone in patients with multiple sclerosis

Previous studies showed that mitoxantrone can reduce disability progression in patients with multiple sclerosis (MS). There is, however, concern that it may cause irreversible cardiomyopathy with reduced left ventricular (LV) ejection fraction (EF) and congestive heart failure. The aim of this prospective study was to investigate cardiac side effects of mitoxantrone by repetitive cardiac monitoring in MS patients. The treatment protocol called for ten courses of a combined mitoxantrone (10 mg/m(2) body surface) and methylprednisolone therapy. Before each course, a transthoracic echocardiogram was performed to determine the LV end-diastolic diameter, the end-systolic diameter and the fractional shortening; the LV-EF was calculated. Seventy-three patients participated (32 males; age 48 +/- 12 years, range 20-75 years; 25 with primary progressive, 47 with secondary progressive and 1 with relapsing-remitting MS) who received at least four courses of mitoxantrone. Three of the 73 patients were excluded during the study (2 patients discontinued therapy; 1 patient with a previous history of ischemic heart disease developed atrial fibrillation after the second course of mitoxantrone). The mean cumulative dose of mitoxantrone was 114.0 +/- 33.8 mg. The mean follow-up time was 23.4 months (range 10-57 months). So far, there has been no significant change in any of the determined parameters (end-diastolic diameter, end-systolic diameter, fractional shortening, EF) over time during all follow-up investigations. Mitoxantrone did not cause signs of congestive heart failure in any of the patients. Further cardiac monitoring is, however, needed to determine the safety of mitoxantrone after longer follow-up times and at higher cumulative doses. Copyright (C) 2005 S. Karger AG, Basel

Influence of Pretransplant Restrictive Lung Disease on Allogeneic Hematopoietic Cell Transplantation Outcomes

We conducted a 15-year retrospective cohort study to determine the prevalence of restrictive lung disease prior to allogeneic hematopoietic cell transplant (HCT), and to assess whether this was a risk factor for poor outcomes. 2545 patients were eligible for the analysis. Restrictive lung disease was defined as a total lung capacity (TLC) <80% of predicted normal. Chest x-rays and /or computed tomography scans were reviewed for all restricted patients to determine whether lung parenchymal abnormalities were unlikely or highly likely to cause restriction. Multivariate Cox-proportional hazard and sensitivity analyses were performed to assess the relationship between restriction and early respiratory failure and nonrelapse mortality. Restrictive lung disease was present in 194 subjects (7.6%) prior to transplantation. Among these cases, radiographically apparent abnormalities were unlikely to be the cause of the restriction in 149 (77%) subjects. In unadjusted and adjusted analyses, the presence of pulmonary restriction was significantly associated with a 2-fold increase in risk for early respiratory failure and nonrelapse mortality, suggesting that these outcomes occurring in the absence of radiographically apparent abnormalities may be related to respiratory muscle weakness. These findings suggest that pulmonary restriction should be considered as a risk factor for poor outcomes after transplant

Switching Multiple Sclerosis Patients with Breakthrough Disease to Second-Line Therapy

BACKGROUND: Multiple sclerosis (MS) patients with breakthrough disease on immunomodulatory drugs are frequently offered to switch to natalizumab or immunosuppressants. The effect of natalizumab monotherapy in patients with breakthrough disease is unknown. METHODS: This is an open-label retrospective cohort study of 993 patients seen at least four times at the University of California San Francisco MS Center, 95 had breakthrough disease on first-line therapy (60 patients switched to natalizumab, 22 to immunosuppressants and 13 declined the switch [non-switchers]). We used Poisson regression adjusted for potential confounders to compare the relapse rate within and across groups before and after the switch. RESULTS: In the within-group analyses, the relapse rate decreased by 70% (95% CI 50,82%; p<0.001) in switchers to natalizumab and by 77% (95% CI 59,87%; p<0.001) in switchers to immunosuppressants; relapse rate in non-switchers did not decrease (6%, p =  0.87). Relative to the reduction among non-switchers, the relapse rate was reduced by 68% among natalizumab switchers (95% CI 19,87%; p = 0.017) and by 76% among the immunosuppressant switchers (95% CI 36,91%; p = 0.004). CONCLUSIONS: Switching to natalizumab or immunosuppressants in patients with breakthrough disease is effective in reducing clinical activity of relapsing MS. The magnitude of the effect and the risk-benefit ratio should be evaluated in randomized clinical trials and prospective cohort studies

Management of acute promyelocytic leukemia: Recommendations from an expert panel on behalf of the European LeukemiaNet

The introduction of all-trans retinoic acid (ATRA) and, more recently, arsenic trioxide (ATO) into the therapy of acute promyelocytic leukemia (APL) has revolutionized the management and outcome of this disease. Several treatment strategies using these agents, usually in combination with chemotherapy, but also without or with minimal use of cytotoxic agents, have provided excellent therapeutic results. Cure of APL patients, however, is also dependent on peculiar aspects related to the management and supportive measures that are crucial to counteract life-threatening complications associated with the disease biology and molecularly targeted treatment. The European LeukemiaNet recently appointed an international panel of experts to develop evidence- and expert opinion-based guidelines on the diagnosis and management of APL. Together with providing current indications on genetic diagnosis, modern risk-adapted front-line therapy and salvage treatment, the review contains specific recommendations for the ide

Principle of impartiality and judge\'s iniciative in criminal proceedings

A presente dissertação visa a profunda análise sobre os poderes instrutórios do juiz em sua atuação no processo, a qual deve ser sempre imparcial. O princípio da imparcialidade do juiz e a sua possibilidade de agir de ofício são importantes características listadas pela doutrina para indicar se o sistema penal adotado é mais tendente ao sistema acusatório ou inquisitório. Por isso, a primeira análise desse trabalho recai sobre o princípio da imparcialidade, os institutos que têm como fim a sua proteção e a sua previsão em todo o ordenamento jurídico brasileiro. Em seguida, estudam-se os poderes instrutórios do juiz, com uma sucinta análise da teoria geral da prova no processo penal e as previsões de poderes instrutórios estabelecidas na legislação brasileira. Posteriormente, debruça-se sobre o instituto do juiz das garantias, o qual foi recentemente incluído no Código de Processo Penal pela lei nº 13.964/2019 e a sua influência no princípio da imparcialidade e nos poderes instrutórios. Por fim, é feita a análise dos poderes instrutórios do juiz no processo civil, em cotejo com o sistema probatório no processo civil, traçando-se um paralelo entre o princípio da imparcialidade e os poderes instrutórios no processo penal e no processo civil.This dissertation seeks to analyse in depth the judge\'s initiative during judicial proceedings, which must always be impartial. The principle of impartiality and the possibility of a judge perform without being provoked are both two important aspects highlighted by legal scholars in order to determine whether the criminal proceedings fits better to the adversarial system or to the inquisitory system. Thus, this work\'s first analysis is about the principle of impartiality, the legal mechanisms designed for its protection, as well as the referrences the Brazilian legal system make to it. Further, the judge\'s initiative to request evidence approached, in the light of a brief analysis on the general theory of evidence in criminal proceedings and on the existent rules about judge\'s initiative according to the Brazilian legal system. Moreover, this work examine the pre-trial judge\'s concept, recently introduced on the Criminal Proceedings Code by the law 13.964/2019, and its influence on the principle of impartiality, as well as on the judge\'s initiative extent. Finally, it offers an analysis about the judge\'s initiative on civil procedure, in the light of general theory of evidence in civil proceedings, establishing a parallel between the principle of impartiality and the judge\'s initiative on criminal proceedings and civil proceedings