Tehran University Medical Journal
Year:2020 | Volume:78 | Issue:2|Papers Count:10

Neurodegeneration with brain iron accumulation (NBIA) is a rare set of inherited neurodegenerative disorders with abnormal accumulation of iron in basal ganglia. It is a clinically and genetically heterogeneous disorder that is characterized by movement disorders, dystonia, dysarthria, Parkinsonism, intellectual disability, and spasticity. The age at onset varies from childhood to adulthood and the rate of progression is different among affected individuals. Although there is no information about the exact prevalence of NBIA in the world-wide, it is estimated less than 1/1, 000, 000 in population. NBIAs are inherited in autosomal recessive, autosomal dominant or X-linked fashions. Until now more than 10 genes have been identified for this group of disorders. Among these, only two genes encode proteins that directly involved in iron metabolism. Therefore, how iron contributes to the pathogenesis of NBIA remains unknown. The remaining NBIA-causing genes participate in lipid metabolism, lysosomal functions or autophagy process, and the roles of some of them remain unknown. NBIA is categorized based on the genetic cause of the disease. PKAN, PLAN, MPAN, and BPAN are the most common forms of the disease result from mutations in the PANK2, PLA2G6, C19orf12, and WDR45 genes, respectively. The diagnosis of NBIA is usually based on clinical features and a specific pattern of brain MRI which results from the abnormal accumulation of iron. For example, the pattern of “ eye of the tiger” is observed in the brain MRI of PKAN cases. Since, clinical evaluations and neuroimaging have failed in the diagnosis of the disease in some NBIA cases, genetic testing will be helpful. Development of whole-exome sequencing (WES) has facilitated the identification of disease-causing genes but it seems some of NBIA-genes have remained unknown, yet. Identification of novel genes and molecular pathways will enable a deeper understanding of the underlying molecular bases and our knowledge about the pathogenesis of the disease. There is currently no comprehensive study about the NBIA in Iran, however, the latest discovered NBIA gene, GTPBP2, has been identified in an Iranian family.

Background: Hepatic encephalopathy is defined as a neuropsychiatric brain dysfunction in acute or chronic liver failure. Infection and inflammation have crucial role in its pathophysiology. The purpose of our study was to demonstrate the relationship between toll-like receptor 4 (TLR4) expression and the encephalopathy induced with endotoxin in biliary cirrhotic rats. Methods: The present study was conducted experimentally on male adult Wistar albino rats from May to January 2018 at the Pharmacology Department of Medical Faculty, Tehran University of Medical Sciences, Tehran, Iran. The animals were divided into two groups, cirrhotic vs sham-operated. Cirrhosis was induced by surgical ligation of the bile duct in male Wistar rats but in sham group the bile duct was not ligated during abdominal surgery (control). The animals in each group were divided to two subgroups that half of them were given intraperitoneally saline or low dose of endotoxin (0. 1 mg/kg) on day 29. Then animal behavior study was done on the clinical sign of hepatic encephalopathy. Also, the histopathology of brain cortex and the expression of brain cortex toll-like receptor four protein were evaluated. Results: Our results showed that endotoxin treatment decreased the patient's level of alertness and produced hepatic encephalopathy signs in cirrhotic rats and control groups. Cirrhosis increases toll-like receptor four expression in brain and acute endotoxin treatment increases toll-like receptor four expression in this group still more. It means that acute endotoxin treatment-induced clinical signs of acute encephalopathy in sham and cirrhotic rats and significant toll-like receptor four overexpression in cirrhotic animals. The histopathological assessment did not show a significant difference between the groups and did not show any changes after induction of cirrhosis and/or acute endotoxin treatment. Conclusion: Cirrhosis and acute low dose endotoxin injection in cirrhotic rats induced hepatic encephalopathy signs that parallels with significant increased toll-like receptor 4 protein expression in brain cortex. Since the severity of the signs of encephalopathy was the same as intact animals, based on the definitions in pharmacology, in the cells of cirrhotic groups endotoxin tolerance has developed.

Background: Human papillomavirus (HPV) is a large group of DNA viruses that cause skin and mucosal warts. Zinc is used in the treatment of skin diseases. Zinc has been used in the treatment of various skin and systemic diseases. Warts are benign proliferation of the skin and mucosa. The prevalence of skin warts is higher in children and its peak is in adolescence and then decreases with age. Some species of HPV can cause malignancies. The effective role of zinc in the treatment of warts has recently been discussed. This study aimed to evaluate the serum zinc levels in patients with cutaneous warts compared to healthy controls. Methods: This case-control study was performed on patients, aged 18 to 60 years old, referred to the Dermatology Clinic of Bo’ Ali Sina and Razi Hospitals, Mazandaran Province in, Iran, from April to March 2016. Serum zinc level and severity of disease were assessed in case and control groups. Data were analyzed by SPSS software, version 22 (SPSS Inc., Chicago, IL, USA). Results: A total of 94 subjects (47 in the case and control group) entered the study. The mean age of the case group was 26. 40± 9. 33 years and in the control group 28. 32± 7. 35 years. The gender status was 42 (44. 7%) male and 52 (56. 3%) female. Single and married were 63. 8% and 36. 2%, respectively. The mean zinc level in patients with cutaneous wart was 82 and the control group was 85. 65. The mean number of warts was 5. 09± 6. 33. The most frequent site of lesions were on the hands and foot with 48. 93% and 40. 42%, respectively, and the face (3. 2%) had the lowest rate. Almost half of the patients were affected by the disease for 12 to 18 months. There was no significant relationship between age, sex, and severity of disease with serum zinc level (P>0. 05). Serum zinc level was significantly associated with the duration of warts involvement (P=0. 043). Conclusion: Serum zinc levels were lower in patients with cutaneous warts than in healthy controls, but this difference was not statistically significant. Serum zinc level and duration of warts involvement were related. The duration of warts and serum levels were inversely correlated.

Background: Premature infants undergo a lot of stressors during treatment procedures in the neonatal intensive care units which causes significant physiological changes in these neonates. Multi-sensory stimulation is a broad category of interventions designed to improve the evolutionary and physiological outcomes of premature infants hospitalized in the neonatal intensive care unit to minimize stress in this environment. So, the study aimed to evaluate the effect of multi-sensory stimulation on physiological parameters in preterm infants. Methods: This double-blind clinical trial conducted in the neonatal intensive care unit of Shahid Motahari Hospital in Jahrom from April to December 2016. In this study, 80 preterm infants with a gestational age of 34 to 36 weeks were selected by nonprobability sampling method and were randomly divided into two groups of multisensory stimulation and control. Neonates in the intervention group received a multisensory stimulation program for 60 minute. The multi-sensory stimulation program was included a combination of auditory, tactile, motor and visual stimulation. The preterm infants in the control group received only usual care. The data collecting tool was a questionnaire and checklist for physiological parameters of preterm infants. Results: The results showed that the two groups were homogeneous in terms of fetal age, birth weight, the height of birth, first and fifth minute Apgar score of birth. The results showed that there was no significant difference between the mean of physiological indexes in the two groups before the intervention. Statistical tests showed that there was a decreasing trend in the average of all physiological indices during the intervention (first and second half during the intervention) (P<0. 001), However, these changes were not significant in the control group (P<0. 05). Also, analysis of variance (ANOVA) with repeated measures showed that there was a significant difference between changes in physiological variables between the two groups at different stages of evaluation (P<0. 001). Conclusion: Multi-sensory stimulation leads to a decrease in heart rate and respiratory rate and the stability of blood pressure in preterm infants.

Background: Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin's lymphoma (NHL), which is the most common type of lymphoma. NHL comprises a group of clinically and biologically diverse diseases, which range from indolent to aggressive clinical courses. Despite treatment advances in the last three decades with the use of combination immunotherapy, a significant fraction of patients relapses or are refractory to these treatments. Actually, there is no standard method for detection of recurrence. The purpose of this study was to evaluate the level of this enzyme in the patients with recurrent DLBCL compared to healthy controls. Methods: In this randomized double-blind, placebo-controlled trial, the serum level of pyruvate dehydrogenase (PDH) enzyme in total of 26 patients with DLBCL recurrence in compare with 26 healthy individuals in the Sayed Al-Shohada Hospital, Isfahan University of Medical Sciences in Iran from September 2016 to September 2018, were assessed. The clinical data including age criteria, the Eastern Cooperative Oncology Group (ECOG) score rating, total bilirubin, alanine aminotransferase (ALT), aspartate aminotransferase (AST), prothrombin time and international normalized ratio (PT/INR), CT-scan, serum creatinine, platelet count, the absolute number of neutrophils, and the interval until the last treatment were gathered. After obtaining informed consent, blood samples were taken. and the PDH enzyme was measured in case and control groups. Results: Fifty-three percent of patients were male and the mean age of participants in case and control groups was 37. 2± 17. 3 and 34. 8± 8. 9, respectively. Subsequently, the PDH levels were measured according to the enzyme protocol. The levels of enzyme in patients with relapse were significantly lower than normal ones (P=0. 0003). The PDH serum level was also evaluated by age and sex, which did not show any significant differences (P=0. 86). Conclusion: In patients with relapsing B-cell lymphoma, the pyruvate dehydrogenase (PDH) enzyme serum levels were significantly lower than healthy subjects, but this difference was not related to age and gender. In the case of further studies and comparisons beyond this study, this enzyme could be a good candidate, used as an alternative diagnosis tool, in patients with recurrent lymphoma.

Background: Acute myeloid leukemia (AML) is characterized by the proliferation of myeloid precursors and abnormal differentiation of hematopoietic stem cells, which results in the accumulation of immature cells in the bone marrow (BM). The accumulation of these cells in the bone marrow causes molecular and cellular changes in the microenvironment of the bone marrow. The adiponectin hormone originates from adipose tissue of the bone marrow, which in addition to effective functions in cellular metabolism, suppresses cancer through various mechanisms, including inhibition of metastasis, angiogenesis, and proliferation. In the bone marrow sample, patients with acute myeloid leukemia are associated with different subtypes of the disease. Methods: In this basic-fundamental research, a total of 40 BM samples from de novo AML patients and 15 BM samples from healthy volunteers as the healthy group referred to the Stem Cell Transplantation Laboratory and Cell Therapy of Taleghani Hospital and with assisting the Research Center, Shahid Beheshti University of Medical Sciences, Tehran, from March 2015 to February 2017, were entered into the study. Then used the Real-time polymerase chain reaction (RT-PCR) method for diagnosis level of adiponectin gene expression in BM samples patients and the healthy group. Results: The results of the present study showed that the level of adiponectin gene expression in the BM sample of patients was significantly decreased in comparison with the healthy group (P=0. 002). While, there was no significant difference (P<0. 05) in adiponectin gene expression in AML subtypes myeloblastic, promyelocytic, and myelomonocytic/monocytic. Conclusion: The results of this study indicate that there was a decrease in adiponectin gene expression in the bone marrow of acute myeloid leukemia patients compared to healthy controls. This decrease in adiponectin expression may be due to myeloid hyperplasia and a decrease in bone marrow adipocytes. In fact, The nutritional, metabolic, and mechanical stresses associated with myeloid cells accumulation cause alterations in bone marrow microenvironment structure and destruction of bone marrow adipose tissue. Therefore, reduced adiponectin gene expression in AML patients is one of the key indicators of bone marrow microenvironmental changes in AML patients.

Background: Cubital tunnel syndrome, a neuropathy of ulnar nerve, is the second most common entrapping syndrome in the upper limb surpassing carpal tunnel syndrome. This syndrome arises from a high pressure decompressing the nerve for a long time. Patients with cubital tunnel syndrome show numbness in the ring and small fingers. The aim of this study was to evaluate the etiology of cubital tunnel syndrome in patients who underwent surgery for treatment of the syndrome. Methods: In this retrospective study, medical records of 100 patients who underwent surgery for treatment of cubital tunnel syndrome at Department of Neurosurgery, Poursina Hospital, Rasht, Iran from March 2008 to March 2017, were checked. The etiology of cubital tunnel syndrome including inflammatory, tumoral, vascular, orthopedic causes and anomalies in nerve site and information about age, gender, job, and weight were collected and analyzed. Results: In this study, the mean± SD of age was 33. 8± 11. 5 years. Females and males constituted 21% and 79% of the cases, respectively. Idiopathic and orthopedic etiologies were seen in 84% and 13% of the operated cases, respectively. Vascular, tumoral, and inflammatory causes were found in 1% of the cases. Regarding location of nerve involvement, 57% of the cases had the ulnar nerve entrapment between MCL (medial collateral ligament) and Osborne ligaments; and in 22% of the cases, the cubital tunnel was involved. Arcade of Struthers and medial epicondyle and medial intermuscular septum were involved in 8%, 8%, and 5% of the patients, respectively. Forty-five percent of the cases performed a repetitive manual task by hand and 1. 9% of cases were vibrator workers. Moreover, 12. 4% of cases had a history of cubitus valgus or cubitus varus. Finally, 20. 9% and 18% of the cases suffered from obesity (BMI>30) and diabetes mellitus, respectively. Furthermore, one patient had a history of head injury with contractions of upper limb flexion and one patient had arthritis of medial epicondylitis. Conclusion: The evidence from the present study indicates that the idiopathic cubital tunnel syndrome constituted the bulk of involvement on ulnar nerve in the patients. Job, obesity, and diabetes were the most important accompanying factors with cubital tunnel syndrome.

Background: The supratrochlear foramen (STF) is an important and relatively common anatomic variation in the lower end of the humerus in humans. In recent years it has become clear that STF should be emphasized because anatomical knowledge of STF is useful for anatomists, anthropologists, orthopedic surgeons, and radiologists. The anatomical structure of the humerus may play an important role in the intramedullary fixation thereby stressing the need of prior anatomical knowledge and preoperative planning in the presence of variations like STF in the distal end of the humerus. This study focuses on STF in the distal of the humerus bone and as well as the intertubercular sulcus (ITS) in the Iranian population. Methods: This study was performed on 57 adult human humeral bones regardless of their gender at Arak University of Medical Sciences in Iran that it was done from October 2014 to March 2015. A total of 57 dried humerus (27 right side and 30 left side) were examined to determine the presence of supratrochlear foramen and septum. They were free from any pathological changes and fractures. The STF was analyzed for morphology and morphometric correlation. Results: STF was found in only four cases (6. 8%) of the humerus bones, two cases (3. 4%) on the left and two cases (3. 4%) on the right. The shape of the STF was oval, round and triangular. The mean STF transverse diameter in the right bones was 2. 60± 2. 68 mm and in the left bones 0. 57± 0. 31 mm and the mean vertical STF diameter in the right and left bones were 1. 75± 1. 76 mm and 0. 55± 0. 07 mm, respectively. Conclusion: There was no significant difference in the shape of the STF and its number in the right and left bones. Twenty-seven bones (47%) out of 53 bones without supratrochlear foramen (STF) had septum.

Background: Long after the implementation of the “ Health System Transformation Project” , no comprehensive assessment of patient and nurse satisfaction rate has been carried out in Iran based on available databases. Thus, this review study was designed and performed to answer this question: “ How is the evaluation of the Health System Transformation Project in nurse and patient satisfaction dimensions. ” Methods: A systematic review of related studies based on preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines was performed via keywords such as “ Health System Transformation Project” , “ Transformation Project” , “ health” , “ Iran” , “ challenges” , “ physician” , “ nurse” , “ patient” , and “ satisfaction” to search Iranian (Magiran and Scientific Information Database, SID) and international databases (PubMed, ISI web of knowledge, Scopus, Google Scholar, and ProQuest) with OR and AND operators from March 2014 to April 2019. Also the quality of studies was assessed using STROB checklist (special for cross-sectional studies). Then required information (for example type of studies, sample size, mean age of participants and satisfaction score) were gathered from studies. Results: From 43 articles that were gathered from primary stages, 18 articles were selected after passing different screening levels. On average, the level of patient satisfaction with the Health System Transformation Project was acceptable. Ghazvin province was seems more successful in satisfaction of patients against of other provinces. However, various positive and negative reports were found about the satisfaction of nursing groups. Generally, it seems that Tehran province (at the center of Iran) was more successful in performing the Health System Transformation Project (from the aspect of satisfaction of different groups). Conclusion: Based on obtained results, patient’ s satisfaction was appropriate in most of studies and also in nursing group was under average level.

Background: Resistant hypertension is defined when the blood pressure remains elevated above the therapeutic target levels despite the use of three antihypertensive agents including calcium channel blocker (CCB), renin-angiotensin system (RAS) blocker and a diuretic. These drugs should be used at maximally tolerated doses and white coat effect and non-adherence should also be excluded. In this article, by describing a case of resistant hypertension, we discuss a diagnostic and therapeutic approach to resistant hypertension. Case Presentation: A 59 years old man, known case of diabetic nephropathy with a serum creatinine level of 1. 2 mg/dL and proteinuria of about 3300 mg in 24 hours referred to Hypertension Clinic of Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, for evaluation of resistant hypertension. The patient was under treatment by losar-H, diltiazem, and furosemide, which are not appropriate combinations of antihypertensive agents. The ambulatory blood pressure monitoring (ABPM) of the patient revealed daytime and nighttime hypertension and also the nondipping status of blood pressure. Dihydropyridine CCB was prescribed in combination with RAS blocker (Exforge 50 mg/ 160 mg) and the diuretic was changed to thiazidelike agents. In the next step, spironolactone and carvedilol were added for better control of blood pressure. The patient referred to the sleep clinic for polysomnography and evaluation of obstructive sleep apnea. Following three months prescription of these medications, ABPM revealed relatively appropriate control of blood pressure, so the average 24-hour blood pressure decreased from 157/91 mm Hg to 136/83 mm Hg. Conclusion: The complications and mortality of hypertension would be increased dramatically in patients with resistant hypertension. So, an appropriate diagnostic and therapeutic approaches should be considered for these patients. Choosing appropriate diuretic agents would be important in the management of resistant hypertension.