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   Table of Contents - Current issue
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September-December 2017
Volume 1 | Issue 2
Page Nos. 43-79

Online since Monday, April 23, 2018

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ORIGINAL ARTICLES  

Pattern and clinical profile of thalassemia among pediatric patients attending the Yemeni Society Centers for Thalassemia and Genetic Blood Disorders in Yemen p. 43
Hala A Al-Kherbash, Ahmed Al-Awdi, Nawal S Hasan
DOI:10.4103/sjamf.sjamf_15_17  
Background Thalassemia is a type of inherited anemia. Its management is complex and expensive, and requires a multiple-team approach. Optimal and sufficient clinical care is demanding. Aim The aim of this study was to determine the magnitude, sex and age distribution, clinical profile, complications, investigation, and management of transfusion-dependent β-thalassemia major among Yemeni pediatric patients attending the Yemeni Society Centers for Thalassemia and Genetic Blood Disorders in Yemen as, to our knowledge, no similar previous study has been carried out in Yemen. Patients and methods This was a prospective, cross-sectional, descriptive study carried out during the period from January 2015 to December 2016. A total of 109 Yemeni pediatric transfusion-dependent β-thalassemia major patients who attended the Yemeni Society Centers for Thalassemia and Genetic Blood Disorders in Yemen were included. Detailed assessment of history, clinical examination, investigations, treatment, and follow-up were performed for every patient. The data were collected from the medical records of the patients. Results There was a slight male predominance (53.2%), with a male to female ratio of 1.14 : 1. Approximately 33% of the patients were in the age group 7–10 years, followed by the age group 3–6 years. The mean age of the cases was 7.68±5.8 years. Positive parent consanguinity was present (74.2%) and the majority (64.2%) were first-degree relatives. The best outcome of thalassemia was among those patients 1.7 years old or younger at the time of diagnosis and those patients 2 years old or younger at first blood transfusion. Earlier initiation of chelating therapy yielded better outcomes and reduced the mortality rate with a highly statistically significant difference. The usage of a combination of both oral chelation and subcutaneous iron chelation (iron pump) therapies led to a noticeably better outcome and reduced the mortality rate markedly. A proportional relation was detected between serum ferritin level and mortality among the cases, with a statistically significant difference. The complication rate was high (40.2%). The mortality rate was also high (27.5%). Cardiac dysfunction was the major risk factor of death among our thalassemic patients as about 60% of the deaths were because of cardiac complications, with a highly statistically significant difference. Conclusion Increasing awareness of the morbidity and mortality of transfusion-dependent β-thalassemia major among pediatric Yemeni patients, with an emphasis on the great importance of premarital screening before marriage in Yemen, is mandatory. For better outcomes, routine investigations to detect early complications with proper treatment of the predisposing factors and complications are necessary.
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Treatment of genu valgum in children by percutaneous transverse metaphyseal osteotomy of the distal femur p. 57
Aly M Elgeuoshy, Galal M Mansour, Mohamed M Hegazy, Alhassan M Abdelhamid
DOI:10.4103/sjamf.sjamf_17_17  
Purpose The aim was to measure and correct the angular deformity of 15 patients with genu valgum by a new percutaneous technique. Materials and methods We conducted a prospective study to evaluate the results of using percutaneous supracondylar femoral osteotomy technique with casting as a minimally invasive surgical intervention for the treatment of genu valgum. Results The youngest patient was 7 years and the oldest was 20 years with an average age of 14 years. The range of hospital stay is from 1 or 2 days, but it decreased with the late cases in the study, which can be explained by the improvement in the learning curve of the technique. The least time of follow-up is 6 weeks, but two cases have been followed for 57 weeks with a mean of 24.90 weeks. Conclusion The percutaneous technique provides the privileges of acute correction without the need for dissection, minimizes the formation of ugly scar or keloid, minimizes the risk of infection, and allows for accurate correction. There is no need for plate fixation or a staged operation to remove hardware.On the other side, the percutaneous osteotomy has a learning curve and the compliant patient is crucial for a close assessment of the condition.
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Neurofilaments as a biomarker of axonal loss in multiple sclerosis p. 63
Hoda Mohamed Ali Massoud, Eman Mohamed Saif Eldeen, Sahar Saied Khattab, Ghada Saed Abd Alazim
DOI:10.4103/sjamf.sjamf_14_17  
Background Neurofilaments (NF-H) might be a potential biomarker of CNS tissue damage in MS, but their potential predictive value for the future disease course, disability, disease progression, and treatment response needs to be investigated. Aim of the work The aim of this study is to evaluate the role of serum phosphorylated neurofilament H as a marker in multiple sclerosis and correlate it’s level with clinical expanded disability status scale (EDSS) and magnetic resonance imaging findings. Subjects and methods Study included 40 patients and 20 healthy control, 20 patients with relapsing remitting multiple sclerosis (RRMS) and 20 patients with secondary progressive multiple sclerosis (SPMS). All patients in this study were subjected to full history taking and clinical examination and scoring according to the EDSS scores, routine laboratory investigations, measuring of serum phosphorylated neurofilament-H (PNF-H) levels and neuroradiological assessment. Results Patients with secondary progressive multiple sclerosis were more likely to have a high serum (PNF-H) titer compared to patients with relapsing remitting multiple sclerosis, and these levels were significantly correlated with EDSS scores in both patients groups. Conclusion The increase in (PNF-H) titer during the progressive phase of the disease together with the correlation of (PNF-H) levels with all clinical scales and radiological lesions (e.g. gadolinium-enhanced lesions and black holes) suggests that cumulative axonal loss is responsible for sustained disability and that high (PNF-H) level is a poor prognostic sign.
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Comparison of endoscopic retrograde cholangiopancreatography then laparoscopic cholecystectomy and laparoscopic common bile duct exploration in patients with common bile duct stones p. 69
Hazem A.M Badr, Ashraf A Elsharkawy
DOI:10.4103/sjamf.sjamf_1_18  
Background Choledocholithiasis is concomitant with gallstones in ∼3–10% of patients. In the pre-endoscopic and prelaparoscopic era, the standard treatment for patients suffering from gallstones accompanied with common bile duct stones (CBDS) was open cholecystectomy and common bile duct (CBD) exploration. With the advent of laparoscopic and endoscopic techniques, several alternative treatments, such as laparoscopic cholecystectomy (LC), preoperative or postoperative endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy and laparoscopic common bile duct exploration (LCBDE), have been developed to treat cholelithiasis. The management of stones in the CBD in the laparoscopic era is controversial. The aim of this study was to compare the efficacy, safety, and surgical outcomes of the LCBDE and ERCP with sphincterotomy, in patients with CBDS. Patients and methods The study was performed on 60 patients suffering from CBDS who were admitted and treated in our department in Al-Zahra Hospital and Al-Ameen Hospital, KSA between January 2014 and January 2016.The patients were divided into two groups according to the method of treatment. Group A included 20 patients who were treated by LCBDE whereas group B included 40 patients were treated by ERCP with sphincterotomy. Results This study was carried out on 60 patients with CBD stones. The patients were divided into two groups according to the method of treatment. Group A included 20 patients who were treated by LCBDE, whereas group B included 40 patients who were treated by ERCP with sphincterotomy. Conclusion The optimal management of patients with CBDS should depend on the condition of the patients, and the expertise of the operators. LCBDE is a feasible, safe, and effective procedure that carries low morbidity and mortality and will decrease the need for unnecessary ERCP in the future for suspected or proved choledocholithiasis.
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Comparative study between loupe-assisted and conventional subinguinal varicocelectomy p. 75
Hazem A Megahed, Hatem A Megahed, Salah G Ziada, Amr A Sarhan, MasoudKh El-Syed
DOI:10.4103/sjamf.sjamf_3_18  
Introduction In this prospective randomized study, the outcome and the complication rates of loupe-assisted subinguinal varicocelectomy (LASV) are compared with conventional subinguinal varicocelectomy. Patients and methods Between December 2014 and June 2017, 66 patients aged from 20 to 38 years old were enrolled in this study in Al-Azhar University Hospital, New Damietta, and Al-Zahraa University Hospital, Cairo. The patients were randomly subdivided into two groups: group A included 33 patients who underwent LASV using surgical loupes with three times magnification, group B included 33 patients who underwent conventional open subinguinal varicocelectomy. Postoperative complications along with preoperative and postoperative (3 months) seminal parameters were compared between the two groups. Moreover, the number of identified internal spermatic arteries was also compared between the two groups. Results The hydrocele formation was significantly lesser in group A treated by LASV [none of the patients (0%)] than group B treated by conventional subinguinal varicocelectomy (three patients; 9.09%). The recurrence rate was better in group A (one patient; 3.03%) than group B (two patients; 6.06%), but the result was nonsignificant regarding recurrence rate. Testicular hypertrophy does not occur in both groups. The average number of preserved arteries was more in group A than group B (0.95±0.51 in group A vs. 0.88±0.47 in group B). Semen parameters including semen concentration, sperm motility, and percentage of abnormal forms were improved in both groups, but the improvement was nonsignificantly better in group A than group B, so the improvement was nonsignificantly between the two groups, but it was significant between the preoperative and postoperative results within each group. Conclusion LASV is better than conventional open subinguinal varicocelectomy as loupe allows clear visualization of the testicular artery, lymphatics, and small venous channels, resulting in decrease in the incidence of complications, including hydrocele formation, testicular artery injury, and varicocele recurrence.
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