فهرست مطالب hamide barzegar

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism with an estimated prevalence of 1 in 30,000. Osteoarticular manifestations are a common feature of WD and mainly involve osteopenia, osteoporosis, and arthropathy.

This study aimed to investigate the prevalence of abnormal mineral density in a group of children with WD and evaluate if it is rational to recommend screening in pre-transplantation workups.

This study included all the children with a confirmed diagnosis of WD, followed at Nemazee Hospital affiliated with Shiraz University of Medical Sciences between 2016 and 2018. The researchers also excluded patients with other underlying diseases, abnormalities of calcium, phosphorus, or vitamin D, or those who used other medications leading to osteoporosis. Bone mineral content (BMC)/Bone mineral density (BMD) of the lumbar spine (LS-BMD) was performed for all included patients with DXA scans.

Evaluation of z-scores showed osteopenia in 40% and osteoporosis in 53.33% of the patients. There was no significant association between the z-score values and cirrhosis in WD patients (P=0.559). There was a significant correlation between the value of z-scores with weight (P=0.007) and BMI (P=0.001) in patients with WD.

The results suggest that WD is intrinsically associated with osteoporosis. Also, patients with WD are at risk of osteopenia and osteoporosis, and screening for evaluation of bone mineral density and prophylactic supplementation may be logical, especially for those who are candidates for liver transplant due to the probability of deterioration of osteopathy in the first few months after liver transplantation

Exchange transfusion (ET) is an effective treatment for acute bilirubin encephalopathy and extreme neonatal hyperbilirubinemia (ENH). It can reduce mortality and morbidity. This study aimed to investigate the trends and risk factors of ENH requiring ET in hospitalized neonates in Iran. A retrospective analysis of medical records of neonates who underwent ET due to ENH was conducted from 2011 to 2021, in Shiraz, Iran. Clinical records were used to gather demographic and laboratory data. The quantitative data were expressed as mean±SD, and qualitative data was presented as frequency and percentage. P<0.05 was considered statistically significant. During the study, 377 ETs were performed for 329 patients. The annual rate of ET decreased by 71.2% during the study period. The most common risk factor of ENH was glucose-6-phosphate dehydrogenase (G6PD) deficiency (35%), followed by prematurity (13.06%), ABO hemolytic disease (7.6%), sepsis (6.4%), Rh hemolytic disease (6.08%), and minor blood group incompatibility (3.34%). In 28.52% of the cases, the cause of ENH was not identified. 17 (5.1%) neonates had acute bilirubin encephalopathy, of whom 6 (35.29%) had G6PD deficiency, 6 (35.29%) had ABO incompatibility, and 2 (11.76%) had Rh incompatibility. Although the rate of ET occurrence has decreased, it seems necessary to consider different risk factors and appropriate guidelines for early identification and management of neonates at risk of ENH should be developed. The findings of the study highlighted the important risk factors of ENH in southern Iran, allowing for the development of appropriate prevention strategies.

Inborn errors of metabolism (IEM) are rare genetic disorders that usually cause the disease by blocking a metabolic pathway. Delayed diagnosis is usually due to nonspecific manifestations. In this study, we aim to evaluate the demographic and clinical characteristics of pediatric patients with IEM admitted in Nemazi hospital, a referral center in southern Iran.

All 1-month- to 18-year-old patients who were admitted in the pediatric ward in Nemazi hospital, a referral center in southern Iran, with the diagnosis of IEM were enrolled. Patients with incomplete information were excluded. We collect demographic and clinical data including age, sex, parents’ consanguinity, family history of IEM, presenting symptoms, number of hospitalization, age of onset, and diagnosis of the disease. All the data were entered into SPSS ver.22 and analyzed.

200 patients were enrolled in the study. 53.5% were male and 46.5% female. The most clinical symptoms were lethargy and vomiting. The most prevalent IEM were aminoacidopathies, organic acidemia, and mitochondrial disease. Consanguinity was seen in 57.5% of them, while the family history of metabolic diseases was observed in 16.5%. There was a statistically significant relationship between consanguinity and the type of the disease.

The clinical symptoms of IEM are nonspecific and may be misdiagnosed as other diseases like septicemia. We should always keep IEM in mind to detect the disease earlier and prevent significant morbidities and mortality by appropriate timely treatment. Due to the prevalence of consanguinity marriage in Iran, we should also consider metabolic screening.

 Sepsis is a leading cause of morbidity and mortality in neonates.

 This study aimed to investigate the bacterial profile and antibiotic sensitivity in infants with sepsis.

 This cross-sectional study was conducted on 90 neonates with sepsis admitted to the neonatal intensive care unit of Namazi Hospital, a tertiary-level hospital, in Shiraz, Iran, from 2020 to 2021. Demographic and clinical data including gestational age, previous diseases, maternal infectious, underlying diseases, history and duration of premature rupture of the membrane and delivery, clinical symptoms (fever, reduced breastfeeding, and lethargy), laboratory results (complete blood cell and C-reactive protein), and results of blood and urine cultures were recorded and analyzed.

 Among 90 neonates with sepsis, the average age was 6.7 ± 7.6 days; 54 were male (60.0%), and 36 were female (40.0%). Fifty (55.5%) neonates had early sepsis. Gram-negative organisms were grown in 61% of positive blood cultures; the most common was Klebsiella. Among Gram-positive organisms, Staphylococcus epidermidis was the most common. Most organisms were sensitive to colistin, and most were resistant to cefotaxime. There was a significant relationship between the age of onset of sepsis symptoms and birth weight in infants with positive blood cultures (P = 0.004) (r = 0.3).

 Gram-negative bacteria are the most common causes of sepsis, mainly resistant to aminoglycosides and cefotaxime. Therefore, increasing awareness about the optimal use of antibiotics is necessary to curb the increase in resistance levels.

 Peripherally inserted central catheters (PICCs) are an effective method for medication and nutrition infusion in preterm neonates. The present study aimed to identify the incidence of the most common complications of PICC implantation and evaluate the risk factors of each complication.

 This historical cohort study was conducted on 2500 neonates with birth weights (BWs)≥500 g and gestational age (GA)>24 weeks who had a history of PICC inserted in three NICUs between August 2015 and August 2018. Data were collected by reviewing medical records. Demographic data and indices of catheter placement, duration of catheter placement, and common complications were recorded. Data analysis was done using SPSS-21.

 The median cubital vein had the most PICC placement (43%). The most common complication was tip malposition (48.2%). The incidence rates (95% CI) of the main complications such as malposition, edema/occlusion, and PICC migration were 0.0356 (0.0337-0.0377), 0.0134 (0.0122-0.0147), and 0.0088 (0.0079-0.0099), respectively. PICC insertion position was the strongest predictor of malposition for the cephalic vein. Besides, the incidence of malformation in the cephalic vein was about six times higher than in the median cubital vein. Independent risk factors for non-technical complications included BW (OR=0.59, 95% CI 0.44-0.79), administration of hyperosmolar medications (OR=3.43, 95% CI 2.62-4.51), position (OR=2.43, 95% CI 1.92- 3.08), and duration of catheter presence (OR=1.02, 95% CI 1.01-1.03) (P<0.001).

 The most common complication was malposition related to catheter placement in an emergency. Moreover, BW, administration of hyperosmolar medications, and duration of catheter presence were the most critical risk factors for non-technical complications. Therefore, it is recommended to educate the PICC insertion team to reduce tip malposition and replace long-term catheters.

Patients have the right to make decision about their medical care. Thus, all the available information regarding decision-making should be provided for them. In surgical context, informed consent is essential for therapeutic relationship between patient and surgeon and sustains mutual trust and shared responsibility for decision making. Thus before surgery, the surgeon should describe available treatments, benefits and harms of them to the patients. So the patient can make a correct decision. In this cross-sectional study, 200 patients underwent gynecological surgery in a hospital in Shiraz during 2014 were recruited. The questionnaire which was used in this study had three parts. The validity and reliability of the questionnaires were evaluated using expert panel and SPSS software. In this study, there was no significant association between age, marriage status, job, home place, emergency or elective surgery and the amount of informed consent. There was significant difference between patients with primary school education and patients with higher education. Patients’ participation in decision making was 57%. Patients’ information about their rights in hospital and the amount of reading informed consent form were poor. This survey shows that the amount of informed consent obtained from patients is poor. So, we should consider some changes in content and obtaining informed consent.