TEHRAN UNIVERSITY MEDICAL JOURNAL (TUMJ)
Year:2021 | Volume:79 | Issue:2|Papers Count:11

Primary Immune Deficiencies are a group of heterogeneous disorders that involve the innate or acquired immune system, or a combination of them. The underlying disorder may be related to decreased levels or function, or a complete lack of one or more components of the immune system in general. These diseases can occur with a prevalence of about 1 in 10000 live births. According to the fourth update on the Iranian national registry of Primary Immune Deficiency in October 2018, the total number of registered PIDs in Iran are 3056 patients. However, it is supposed to be more prevalent and it seems increasing awareness shall reveal many new cases, especially in societies with prevalent consanguineous marriages like Iran. These disorders predispose patients to recurrent infections, autoimmunity and malignancy and can cause a huge burden on health care systems. This group of diseases has a wide range of symptoms, which quick recognition and timely treatment of them, can greatly reduce the complications of the disease. These symptoms may include recurrent or severe infections, failure to thrive, autoimmune disorders, as well as articular-skeletal manifestations. A variety of skeletal manifestations are seen in patients with primary immunodeficiency, among which septic arthritis caused by pyogenic bacteria or mycoplasma arthritis is the most common joint-bone manifestation. Joint and skeletal involvement is less commonly seen as a sign of primary immune defects. This issue is importance in reducing the cost of diseases and improving the patients’ quality of life. Our review attempted to introduce the most common manifestations of bone and joint in patients with primary immunodeficiency and available treatments for these manifestations. Because of the wide range of symptoms in these patients, it is recommended to observe the rare and suspicious manifestations in the patients with any atypical bone and joint presentations such as: recurrent septic arthritis, infection with unusual germs, immunodeficiency in their relatives, and any history of well-known red flags of PIDs. The Rheumatologist should consider these manifestations and think about the possibility of deficiency disorder.

Background: Neurosurgery practice conflicts with many challenges during the COVID-19 pandemic; Including the lack of beds in intensive care units, as well as the use of some methods such as drills and trans-nasal and trans-oral approaches that produce aerosols or are directly in contact with patient discharge. Due to these challenges, developing a clinical guideline to help neurosurgeons and medical staff in decision making and improving patients and medical staff safety during the COVID-19 pandemic is the purpose of this study. Methods: First, all of the relevant clinical guidelines to neurosurgical practice during the COVID-19 pandemic were extracted from the data centers. Finally, five clinical guidelines were selected. The questions and the items were designed according to these guidelines. The answers to each of the questions were extracted from these guidelines. The complementary evidence was extracted by searching in the data centers again. Finally, the answers were edited and the edited answers were considered as the recommendations. These recommendations were sent to 4 experts in the Neurosurgery field and 2 experts in the infectious diseases field. The appraisers evaluated the recommendations according to the AGREE-REX instrument. This instrument has 9 items and 3 domains including clinical applicability, values and preferences, and implement ability. Recommendations with above 80% agreement were considered as the final recommendations. Results: The final recommendations were presented as “ Clinical guideline of neurosurgical practice during the COVID-19 pandemic” in the results section. Conclusion: This clinical guideline was developed by using similar guidelines and available evidence. Proper usage of personal protective equipment, reduction of unnecessary contacts between medical staff and patients, use of Telemedicine for follow-up, proper air conditioning, screening patients for COVID-19, reduction of elective surgery, use of less invasive methods, management of aerosol production and reduction of trans-oral and trans-nasal approaches are the most important recommendations of this clinical guideline.

Background: Systemic lupus erythematosus is a systemic autoimmune disease that affects almost all organs of the body, and viral infections are involved in its development and progression. The present study aimed to evaluate the serological status of some viral infections in patients with systemic lupus erythematosus and a healthy population. Methods: This descriptive study conducted from May 2017 to April 2018 at Shiraz HIV/AIDS Research Center, Shiraz University of Medical Sciences, Shiraz, Iran on 70 patients with systemic lupus erythematosus and 70 healthy individuals who had no autoimmune diseases and were matched with the patient group for age and sex. All patients had active records and were routinely visited in rheumatology clinic of Hafez hospital, affiliated with Shiraz University of Medical Sciences. The evidence of active disease was assessed by the physicians of this practice according to the American College of Rheumatology criteria. Peripheral blood samples were collected in tubes containing EDTA and centrifuged at 3000 rpm for 5 min. The plasma of study participants was evaluated for HBsAg, HCVAb, HIVAb, EBV-VCA-IgG, and CMVIgG using a commercially available ELISA kit. Results: The seropositivity of CMV-IgG and EBV-VCA-IgG in the systemic lupus erythematosus group was 70 (100%) and 65 (92. 9%), and in healthy individuals was 68 (97. 1%) and 57 (81. 4%), respectively. The prevalence of EBV-VCA-IgG in the systemic lupus erythematosus group was significantly higher than healthy ones (P=0. 043). The optical density (OD) of CMV-IgG and EBV-VCA-IgG in patients with systemic lupus erythematosus was significantly higher than in healthy individuals (P<0. 0001). All patients with systemic lupus erythematosus were negative for HBsAg and HIVAb, but HCVAb was detected in 1 (1. 4%) patient. Conclusion: Considering the higher frequency of EBV-VCA-IgG and the higher titer of antibodies against CMV and EBV in patient groups compared to healthy individuals group, it seems that periodical assessment of viral load in patients with systemic lupus erythematosus will be beneficial to prescribe medication by physicians if it is needed.

Background: Early diagnosis of patients in the early stages of Alzheimer's, known as mild cognitive impairment, is of great importance in the treatment of this disease. If a patient can be diagnosed at this stage, it is possible to treat or delay Alzheimer's disease. Resting-state functional magnetic resonance imaging (fMRI) is very common in the process of diagnosing Alzheimer's disease. In this study, we intend to separate subjects with mild cognitive impairment from healthy control based on fMRI data using brain functional connectivity and graph theory. Methods: In this article, which was done from April to November 2020 in Tehran, after pre-processing the fMRI data, 116 brain regions were extracted using an Automated Anatomical Labeling atlas. Then, the functional connectivity matrix between the time signals of 116 brain regions was calculated using Pearson correlation and mutual information methods. Using functional connectivity calculations, the brain graph network was formed, followed by thresholding of the brain connectivity network to keep significant and strong edges while eliminating weaker edges that were likely noise. Finally, 11 global features were extracted from the graph network and after performing statistical analyses and selecting optimal features; the classification of 14 healthy individuals and 11 patients with mild cognitive impairment was performed using a support vector machine classifier. Results: Calculations were showed that the mutual information algorithm as a functional connectivity method and five global features of the graph network, including average strength, eccentricity, local efficiency, coefficient clustering and transitivity, using the support vector machine classifier achieved the best performance with the accuracy, sensitivity and specificity of 84, 86 and 93 percent, respectively. Conclusion: Combining the features of brain graph and functional connectivity by the mutual information method with a machine learning approach, based on fMRI imaging analysis, is very effective in diagnosing mild cognitive impairment in the early stages of Alzheimer’ s which consequently allows treating or delaying this disease.

Background: Diabetic foot infection is the most common complication of diabetic foot ulcer (DFU) leading to amputation. The treatment requires special attention to disease conditions, proper diagnosis, appropriate sampling for cultures, careful selection of antibiotics, rapid determination of the patient’ s need for surgical intervention, and wound care. Clearly a systematic approach or, if possible, an evidence-based approach by using clinical guidelines about diabetic foot infections will lead to better outcomes. In this regard, this article aims to adopt the IWGDF clinical practice guideline on DFU infection. Methods: In this study, the adoption of the international evidence-based clinical guidelines on the DFU infection was carried out using the ADAPTE methodology, which involved three stages of setup, adoption, and finalization. AGREE II tool was used to evaluate the quality of the selected clinical guideline. Results: The results of this adopted clinical guideline according to the national facilities and access to equipment are described with 26 recommendations along with related reasons for the diagnosis and treatment of diabetic foot ulcer infections. The recommendation categorized into six different categories including Classification/ Diagnosis, Osteomyelitis, Assessing severity, Microbiological considerations, Surgical treatment, Antimicrobial therapy. Conclusion: The mentioned recommendations in the diagnosis and treatment of diabetic foot ulcer infections extracted based on the methodology of systematic review studies. Actually the opinion of experts have been prepared and adjusted according to the limitations, equipment and facilities in the country. But in general, there are some challenges in diabetic foot infections should be considered as well. So monitoring the treatment of infection, optimal duration of antibiotic treatment, optimal treatment of infection in low-income countries, time and type of imaging studies, selection of medical or surgical treatments and hospitalization conditions are some of these challenges. However the definition and practical clinical application for the concept of bacterial bioburden of the wound for wound healing, evaluating the cost-effectiveness of new technologies in improving treatment and appropriate interpretation of microbiological (genotypic) microbiological tests are important too.

Background: The advent of Severe Acute Respiratory Syndrome Coronavirus 2 (socalled SARS-CoV-2) causing Coronavirus Disease 2019 (so-called COVID-19) occurred in Dec. 2019 in Wuhan, China. Having an inconceivable worldwide contagion, the outbreak was labeled a pandemic by the WHO. Dental services and related professions (including dentists, dental assistants, dental hygienists and the personnel of cleansing, remedial, triage, dental laboratories, radiographic laboratories and other related paraclinicals), facing galore aerosol and droplets, are in the topmost risk groups exposed to the queer virus. This study was fulfilled to round up evidencebased data to break a link at any part of the virus transmission chain in dental services and related professions. Methods: Relevant online databases, as PubMed, MEDLINE, Embase, Scopus, Google Scholar and TripDatabase were searched meticulously and evaluated for relevant published original research papers. Subsequently, to fulfill the investigation, ADA, CDC and WHO websites were reviewed to gain relevant guidelines and protocols. Consequently, 476 resources were included considering the canonical inclusion criteria. For the sake of quality assessment of the resources, an authentic checklist was exploited to score the resources from 1 to 15, wherein the admissible score was 10. After deliberation of resources, 366 of them were excluded and finally, 110 resources were selected and overhauled to attain a comprehensive perception on the subject of the investigation. Results: Transmission of SARS-CoV-2 includes direct transmission (via droplet and aerosol inhalation) and indirect transmission (via surface and instrument contamination), which can amply occur in dental services and related professions. Therefore, an all-inclusive evidence-based miscellany was rallied on several exigent topics, containing genuine esteem in reputable scientific authorities, to present a consummate report for the dental clinicians and related practitioners, working in the course of the running pandemic. Conclusion: Contemplating the ongoing crisis, undertaking a set of miscellany elected guidelines and protocols, is indispensable in this vital interval of history to bridle the current pandemic, which has been abridged via this systematic perusal.

Background: Ewing's sarcoma/Primitive neuroectodermal tumor (PNET) is a group of tumors with small round cells that originate from nerve stem cells. They are generally more common in children and often occur in the soft or bony tissues of the limbs, trunk, head, and neck. Ewing's sarcoma is a rare disease in the kidney and its tumor thrombosis into Inferior Vena Cava (IVC) is assumed as a very rare condition. Case Presentation: The patient was a 14-year-old boy who underwent an MRI of the thoracic and lumbar vertebrae due to paresthesia of the lower limbs, which showed the presence of the mass in the vertebrae of T3, T4, T5, and concurrently right kidney. The pathology report confirmed the diagnosis of Ewing’ s sarcoma, PNET. The patient underwent T4 laminectomy and bone mass resection and then received 4 courses of chemotherapy with VAC + IE. In the next stage of treatment, the patient had abdominal MRI and MR Venography (MRV) that demonstrated a mass in the middle of the upper right kidney with a 10 cm length tumor thrombosis into IVC with extension to the suprahepatic area and involvement of several aortocaval lymph nodes. In January 2020, the patient was operated in Imam Khomeini Hospital Complex, Tehran by a midline incision, at first, the kidney artery and vein were controlled, and without thrombectomy, the IVC was controlled from above and below the liver, then the tumor thrombosis was removed. The right kidney underwent a radical nephrectomy. Three days later, the patient was discharged in good general condition and referred for chemotherapy. Conclusion: Because Ewing's sarcoma is a rare condition in the kidney, this case is considered a very rare case due to its tumor thrombosis. This case study showed that despite the advanced stage of the disease, early diagnosis and treatment of patients with Ewing's sarcoma, along with adjuvant treatments can play an important role in the survival of these patients.

Background: Purulent cervical lymphadenitis presents with different manifestations such as fever and Erythema of the skin at the site of infection. The appropriate treatment for purulent lymphadenitis are antibiotics. If there is no response to antibiotic treatment, other causes should be considered, including uncommon infectious causes (for example Mycobacterium infections or fungal infections), malignancy and Kawasaki diseases, etc. This study aimed to introduce a child with suspected purulent cervical lymphadenitis, who was initially presented with purulent lymphadenitis and was treated with broad-spectrum antibiotics. Due to the lack of response to treatment, further laboratory and clinical examinations were performed for him, then he was diagnosed with Kawasaki disease. Case Presentation: A 10-year-old boy with fever and severe cervical lymphadenitis was referred to Tehran Children's Medical Center Hospital in May 2018. He had leukocytosis in his lab test and the acute reactant phase was high. He was diagnosed with purulent lymphadenitis and he was treated with broad-spectrum antibiotics. But no therapeutic response was observed, the patient's fever continued and erythrocyte sedimentation rate (ESR) increased in the laboratory tests. The patient's knee developed arthralgia, and Inflammation of the neck spread to the chest wall. Therefore echocardiography was performed. The patient's left anterior descending artery (LAD) had ectasia in echocardiography (LAD>2/5 SD). The patient was diagnosed with atypical Kawasaki disease, therefore the appropriate treatment was started for him and a dramatic clinical response was seen. His fever stopped and the patient's cervical lymphadenitis had completely improved. In the follow-up, the patient's left anterior descending artery (LAD) ectasia was resolved. Conclusion: Kawasaki disease is one of the causes of cervical lymphadenitis in children. If a patient with suspected purulent lymphadenitis is treated with appropriate antibiotics but no adequate response is seen, Kawasaki disease should be considered and the patient must be re-evaluated for Kawasaki disease.

Background: Chest pain is the most common reason for patients to be referred to the emergency department of hospitals. This study was performed to compare the GRACE and TIMI scores in predicting important cardiovascular events in patients. Methods: This descriptive-analytical study was performed on 862 cardiac patients who were referred to the emergency department of Taleghani Hospital in Urmia in the period of April 1, 2016 to the end of September 2016 by census method. A checklist containing demographic information, medical history and risk factors was used to collect data. After scoring patients, we examined and followed up both groups over the next 30 days and recorded any cardiovascular events such as sudden death, AMI or immediate revascularization. The data were tested using SPSS16 and descriptive statistics tests. T-test and ROC curve were analyzed. Results: The results showed that in general, out of 862 patients who were studied (50. 3%), 433 were female and (49. 7%) 429 were male. Most of the patients were under 65 years old 627 (72. 7%) and the age range was (17-91). The highest initial diagnosis of nonSTEMI patients was UA with 811 (94. 9%) cases. On the other hand, the highest risk factors of patients were HTN 449(52%), CAD 314 (36. 425) and DM 22 (25. 55). The highest blood pressure was between (100-120) with 328 cases and serum creatinine between (0. 6 to 1. 3) with 770 cases. MACE after 30 days in GRACE system 17 people (32. 69%) were in high risk group and in TIMI 3 people (37. 5%) were in high risk group. In MACE evaluation, the specificity of GRACE system (cutoff point=30) was 89. 27 vs. 52. 24, the specificity of TIMI system (cutoff point=7. 3) and the sensitivity of GRACE 93 (cutoff point=10) versus TIMI 85. 71 (cutoff point=8. 3) is. Conclusion: The results showed that GRACE was more sensitive and characteristic than TIMI.

Background: Health equity is “ having fair access to healthcare, utilizing it according to actual needs, paying for it based on financial capacity and finally, having an acceptable level of health” . Health equity is an underlying principle of the universal declaration of human rights. Equitable distribution of hospital beds increases people’ s access to healthcare services and as a result, improves their health status. This study aimed to examine the equity in the geographic distribution of hospital beds in Tehran city, Iran. Methods: The data for this descriptive and cross-sectional study were obtained from the Ministry of Health and the Iranian statistics center in April 2019. All hospitals in Tehran city were included in this study. Lorenz curve and the Gini coefficient were used to measure the equity in the geographic distribution of hospital beds. Excel software was used for data analysis. Results: Tehran city had a population of 8, 693, 706, and 142 hospitals with 24, 535 beds in 2016. There was 1. 6 hospitals per 100, 000 people and 2. 8 hospital beds per 1000 people in this city. Nearly half of the hospitals were private (49%) and the remaining were public or semi-public. About 77% and 23% of hospitals were general and specialized respectively. Almost half of the hospitals are more than 40 years old. The average number of beds in hospitals was 173. The Gini coefficient was 0. 619 for hospital bed distribution among Tehran districts. Districts 6, 12 and 3 have had the highest hospital beds per 1000 people. Districts 6 had 23% of the total hospitals and 24% of the hospital beds. Conclusion: The geographic distribution of hospital beds in Tehran city is not equitable. Hospital services should be accessible based on actual need rather than on the ability to pay. Achieving health equity is a prerequisite of universal health coverage. Hence, healthcare policymakers should reduce or eliminate the existing disparities and inequalities in access to hospital beds.