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Introduction: Alkaptonuria is an autosomal extremely rare recessive metabolic disorder with incidence reported to occur as 1:100 000-1:250 000 live births worldwide. This rare metabolic disorder is characterized by the accumulation of homogentisic acid due to a deficiency in homogentisic acid 1,2 dioxygenase. Homogentisic acid subsequently oxidizes and accumulates in the connective tissue. The knee is the most significant peripheral joint to be affected by the disorder. The authors present the first case of ochronotic arthropathy in Syria. Case presentation: A 46-year-old male presented with bilateral pain in the knees. the pain was affecting his day-to-day activities, and not responding to conservative management. Anteroposterior standing radiographs demonstrated extensive degenerative disease. Intraoperatively, the diagnosis was done after noticing that the quadriceps tendon and the articular cartilage of the femur, tibia, and patella were blackened during cemented total knee replacement of the knee. Conclusion: Ochronotic arthropathy should be kept in mind in middle age patients with severe osteoarthritis to not be surprised by the rare alkaptonuria diagnosis if arthroplasty was indicated.
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BACKGROUND AND AIMS: Basic life support (BLS) training rates vary widely worldwide, and there is a general scarcity of surveys that assess students' knowledge and awareness of BLS in middle eastern nations. This study aims to evaluate medical students' knowledge and awareness towards basic life support. METHODS: A cross-sectional study, using an online web-based questionnaire, assessing BLS awareness and knowledge, was conducted from 3 to 30 November 2021. The study included 2114 medical students from Syria, Iraq, and Jordan. The questionnaire consisted of three sections: demographic baseline characteristics, knowledge about BLS, and ability to apply basic life support. A binominal logistic regression was done between the total score and other demographic characteristics to determine if we could predict the research sample's appropriate knowledge of BLS level. RESULTS: There was a moderate knowledge of BLS and cardiopulmonary resuscitation (CPR) skills among participating students with an average score of 19.67 (0-34). Seventy-eight of the participants (1656) stated that they have not attended a basic life support course. There was a significant difference between the participants from different countries, where the mean score in Syria, Jordan, and Iraq was 18.3, 24.3, and 18.8, respectively (p < 0.05). Considering the level of knowledge, 18.3%, 72.8%, and 8.9% of the participants had a high, intermediate, and low level, respectively. Furthermore, students who took a BLS course had a higher level of knowledge than those who did not, with an odds ratio of 5.168 (p value < 0.0001). CONCLUSION: The overall knowledge of medical students' basic life support knowledge is insufficient and need to be greatly improved. According to this study, previous participation in basic life support training had a positive effect on knowledge level. As a result, universities must establish basic life support programs as quickly as possible.
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INTRODUCTION: Sphincter of Oddi dysfunction is a rare disease caused by sphincter of Oddi functional or mechanical abnormality. Misdiagnosis of familial Mediterranean fever is very high due to overlapping symptoms with many diseases. Our case is the first case report in the medical literature which describes the misdiagnosis of Sphincter of Oddi dysfunction as familial Mediterranean fever. CASE PRESENTATION: A 46-year-old woman presented with recurrent episodes of abdominal pain and arthralgia. The patient had familial Mediterranean fever for ten years which was diagnosed clinically without performing genetic tests. Analysis of the mutation in the MEFV gene was performed and was negative. Thereby, the diagnosis of familial Mediterranean fever was eliminated and colchisine was discontinued. Afterward, laboratory and radiological tests were performed, and the diagnosis of sphincter of Oddi disfunction was confirmed. The patient underwent biliary sphincterotomy and take sulpiride daily. DISCUSSION: The most common diseases were misdiagnosed with familial Mediterranean fever are appendicitis, acute rheumatic fever, gastrointestinal diseases and inflammatory arthritis. Endoscopic retrograde cholangiopancreatography with Manometry of the Sphincter of Oddi is the gold-standard test. CONCLUSION: Sphincter of Oddi dysfunction may interfere with many other disorders and should be considered as a differential diagnosis for any recurrent abdominal pain. Misdiagnosis of familial Mediterranean fever is common in endemic countries due to the reliance on clinical symptoms without analysis of the mutations in the MEFV genes particularly, before 1997.
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Background: Ewing's sarcoma (ES) can affect any bone, but its occurrence in the scapula is extremely rare. Only 15 studies investigating this condition exist in the medical literature. Materials and methods: A literature search was conducted in PubMed and Scopus, and studies on ES of scapula published in the English medical literature were retrieved. A total of 15 studies were found and were included in our study. Results: ES prevalence was highest in Asia. Moreover, ES was predominant in males (60%), with a male-to-female ratio of 3:2. ES in 53.3% and 46.6% of the cases were found in the right and left scapula, respectively. The main presentation of patients with ES of scapula was swelling, which was observed in 73.33% of the cases. Of the included studies, 46.6% used plain radiography as the primary investigation method, and 60% used computed tomography for staging and metastasis detection. For definitive diagnosis, 86.6% of the studies used immunohistochemistry markers. Adjuvant chemotherapy was considered in most studies (80%). Neoadjuvant chemotherapy was given in 6 out of 10 cases who underwent surgical treatment. Complications included malignant pleural effusion, respiratory failure, and movement restriction. Conclusion: The scapula is an extremely rare site for ES. Local invasion was found in 63.64% of the cases, whereas pre-metastases were found in 35.71% of the cases. Magnetic resonance imaging was considered to be the best radiological method used to diagnose ES of scapula. Adjuvant chemotherapy, neoadjuvant chemotherapy, and surgery were the main treatments for ES.
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INTRODUCTION: One of the relatively rare hemostatic disorders is coagulation factors' deficiency, where a single factor or multiple factors can be deficient. All hereditary coagulation factors' deficiencies are autosomal recessive, so they can manifest in both genders, but Hemophilia A and B are X-linked disorders. Therefore, females can rarely be affected. This paper reports the first case of simultaneous coagulation factors' deficiencies of FVIII and FXI in a female. CASE PRESENTATION: A 17-year-old female came to the office due to prolonged epistaxis, with a history of severe menstrual bleeding and frequent episodes of epistaxis. In her familial history, a brother complained of epistaxis episodes. Bleeding time and prothrombin time were normal but activated partial thromboplastin time was increased. Von Willebrand disease was excluded, and she was diagnosed with hemophilia A and C. DISCUSSION: Females can be affected with X-linked disorders such as hemophilia A and B in some rare cases: a carrier mother and affected father, skewed X chromosome inactivation, Turner syndrome, inhibiting antibodies (acquired hemophilia), or a sporadic mutation on the most activated X chromosome. On the other hand, Hemophilia C is an autosomal recessive disease. Treatment of such cases is a challenge, and the recombinant coagulation factors are the treat-of-choice. CONCLUSION: Although Von Willebrand disease is the most common hereditary bleeding disorder in females, other rare diseases could be suspected such as Hemophilia. X-linked Hemophilia should be kept in mind as a differential diagnosis in any female patient suffering from hemorrhage.
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BACKGROUND: Group A Streptococcus is a very common pathogen which infects a large scale of people around the world causing many symptoms such as scarlet fever, sinusitis, and pneumonia. Most strains of group A and many other strains of group C and G Streptococcus bacteria secreted antigen called Streptolysin O. Anti-streptolysin O (ASO) is an antibody produced against streptolysin O that rises after 1 week of infection by streptococcus bacteria, which helps in diagnosing this type of infectious diseases. We conducted a Cross-Sectional study to determine the Upper Limit of Normal (ULN) for healthy adult in Aleppo, Syria. MATERIALS AND METHODS: A sero-epidemiological cross-sectional study was conducted from September to October in 2019. ASO titers were determined on 267 healthy patients' companions who visited Aleppo University Hospital. Geometric mean titer and Upper Limit of Normal of ASO were calculated according to sex, age and residency. Upper Limit of Normal were defined as the 80th percentile. RESULTS: Out of 267 participants; 126 (45.7%) were males and 150 (54.3) were females. The Upper Limit of Normal for total participants was 210.8 IU/ml. There was no significant difference (P-value > 0.05) among males (204.6 IU/ml) and females (225.8 IU/ml). In contrast to sex, there was a significant difference (P-value < 0.05) according to age groups, where the highest Upper Limit of Normal was in the 30-39 age group (256.0 IU/ml). CONCLUSION: ASO test is a common, easy, cheap method, so getting enough data about it is very important in the developing countries (such as Syria). In Aleppo, Syria we found that the ULN was higher than ULN from other studies. There was no significant difference according to sex and residency. On the other hand, there was significant difference according to age groups.
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INTRODUCTION: Headache disorders are among the most common 10 causes of disability worldwide according to the global burden of disease survey 2010. Headache is also wildly common among universities students when compared with other populations. The purpose of this study is to assess headache prevalence among Aleppo University medical, dental and pharmaceutical undergraduate students. METHODS: A questionnaire-based cross-sectional study was conducted among medical, dental and pharmaceutical students at Aleppo University, Syria. We determined the type of headache according to the International Classification of Headache Disorder-III. The total number of participants was 2068. A χ2 test was used to evaluate the association between the categorical outcomes. P<0.05 was considered significant. RESULTS: Out of 2068 participants, 1604 (77.6%) were medical students, 205 (9.9%) were dental students and 259 (12.5%) were pharmaceutical students. The effect on daily activities was higher in chronic tension headache (96.7%) and migraine without aura (94.6%) than migraine with aura (91.3) and episodic tension headache (85.1%). Out of 1191 who had a headache, only 188 (15.9%) had a medical consultation. CONCLUSIONS: There was no a statistically significant difference in prevalence of tension headache and migraine according to faculties. There was a statistically significant difference in patients with migraine according to academic year, living with family and smoking. The effect on daily activities was higher in chronic tension-type headache and migraine without aura. There is a significant lack of medical consultation among students and most of them took over the counter analgesics depending on personal choice.
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INTRODUCTION: Cholecystocutaneous fistula is an extremely rare complication of gallstones. The majority of the fistulae localize into the right upper quadrant of the abdomen. Diagnosis is not easy because the symptoms are nonspecific. External biliary fistula is more common in females between the 5th-7th decades because in this age group the frequency of cholecystitis is increased. CASE PRESENTATION: A 65-year-old man presented with a swelling in the right hypochondrium. He had not complained of any serious symptoms. Laboratory investigations were normal. Both abdominal ultrasound and Multislice computed tomography (MSCT) showed that the gallbladder contained stones, in addition to a cystic mass in the abdominal wall. The previous findings suggested that there was an abscess. So, the patient underwent laparoscopic procedure, and we found a fistula between the gallbladder and the abdominal wall causing the abscess. The greater omentum was adherent to the inflamed gallbladder. So that we transformed into open procedure to perform cholecystectomy and resect the fistula. DISCUSSION: Biliary fistulae have two types, external and internal. The most common type is internal. External biliary fistulae emerge like a complication of cholecystolithiasis in general. Spontaneous cholecystocutaneous fistulae occur due to acute inflammation caused by cholecystitis or chronic gallstones disease. The most common clinical manifestation is an abscess in the abdominal wall that discharges bile to the outside. CONCLUSION: Computed tomography is considered the gold standard in diagnosing a spontaneous cholecystocutaneous fistula, and it should be considered as a differential diagnosis for any abscess that is evident in the abdominal wall.
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BACKGROUND: Seminoma is the most common subtype of testicular cancer and occurs most commonly in patients aged 30-49 years, but decreases to a very low level in men in their 60s or older. CASE PRESENTATION: A 90-year-old Syrian man with a 6-year history of an increase in size of his right scrotum, presented to the urological clinic and, on clinical examination, the findings suggested testicular tumor. After orchiectomy and histology results based on microscopic and immunohistochemical examinations, a pure seminoma was diagnosed, so we describe in this case report the second-oldest patient with classical seminoma in the medical literature. CONCLUSION: This case report has been written to focus on the probability of any type of testicular tumor occurring at any age or decade; urologists should consider seminoma as a differential diagnosis with any testicular swelling even in elderly patients.
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Seminoma , Neoplasias Testiculares , Adulto , Anciano , Anciano de 80 o más Años , Diagnóstico Diferencial , Humanos , Masculino , Persona de Mediana Edad , Orquiectomía , Escroto , Seminoma/diagnóstico por imagen , Seminoma/cirugía , Neoplasias Testiculares/diagnóstico por imagen , Neoplasias Testiculares/cirugíaRESUMEN
"Hydatid cyst" which also known as cystic Echinococcosis is a parasitic infestation caused by the larval stage of Echinococcus granulosus. The liver and lungs are the most sites to occur. Incidence in muscles is exceptionally rare. We report a case of a 36-year-old female presented with an uncomfortable mass in the upper medial of her right thigh without any presence of other symptoms. She lived in a rural area in Manbij, which is an endemic area of hydatid cysts in Syria. She was a shepherdess; therefore she had direct contact with sheep and dogs. Ultrasound examination showed a cyst located between adductor longus muscle and gracilis muscle closed to the deep femoral artery. The patient was treated with pharmaceutical therapy for a week before cystectomy, which was done under general anesthesia. The cyst was dissected between the fibers of adductor longus muscle from the lateral side and fibers of the gracilis muscle from the medial side. The cyst with all its layers was resected. Musculoskeletal Echinococcosis is a rare disease, because of intramuscular growth of cysts is restricted by muscle's contractility, the muscles are undesirable habitat for Echinococcus granulosus and because of the hepatic barrier role. Many cysts are revealed by complications such as nerve compressions, infections simulating an acute abscess or a malignant tumor. Hydatid cyst present as mass of soft tissue, particularly in endemic areas, as a result of contaminated water. MRI considered the best technique in the diagnosis.
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INTRODUCTION: Trichobezoar is a rare cause of bowel obstruction. In general, Trichobezoars are composed of hair and usually found incidentally in patients undergoing upper gastrointestinal endoscopy or imaging. Patients diagnosed with Trichobezoar may have psychiatric disorders. DISCUSSION: Trichobezoars cause nonspecific symptoms like asymptomatic abdominal mass, vomiting, nausea, and anorexia. Diagnosis of Trichobezoar is made by endoscopic examination and radiological methods. Therapeutic options for trichobeazoar are chemical dissolution, endoscopic removal or surgery. CASE PRESENTATION: Here we present a case of an 18-year-old girl who has a unique type of Trichobezoars caused by ingestion of hair and bristle clothes brush for 14 years. She presented to the surgical clinic complaining of vomiting, anorexia and epigastric mass. Abdominal computed tomography scanner showed nonattached intragastric mass which was consistent with trichobezoar. The patient was managed by surgical removal of the intragastric mass. CONCLUSION: Trichobezoar is caused by chronic ingestion of hair; it is commonly seen in young females who may have psychological disorders, such as trichophagia and trichotillomania. Common symptoms are abdominal pain, nausea, vomiting, and weight loss. Surgical intervention is performed for the majority of the patients.