Pulmonary manifestations in a cohort of patients with inborn errors of immunity : an 8-year follow-up study

Background: Inborn errors of immunity (IEIs) are a group of congenital diseases caused by genetic defects in the development and function of the immune system. The involvement of the respiratory tract is one of the most common presentations in IEIs. Methods: Overall, 117 patients with diagnosed IEIs were followed-up within 8 years at the National Research Institute of Tuberculosis and Lung Diseases (NRITLD). Demographic, clinical, and laboratory data were collected in a questionnaire. Pulmonary function test (PFT), chest X-ray (CXR), and high-resolution computed tomography (HRCT) scans were obtained where applicable. Results: Our study population consisted of 48 (41%) patients with predominantly antibody deficiencies (PADs), 39 (32%) patients with congenital defects of phagocytes, 14 (11.9%) patients with combined immunodeficiency (CID), and 16 (14%) patients with Mendelian susceptibility to mycobacterial diseases (MSMD).. Recurrent pneumonia was the most common manifestation, while productive cough appeared to be the most common symptom in almost all diseases. PFT showed an obstructive pattern in patients with PAD, a restrictive pattern in patients with CID, and a mixed pattern in patients with CGD. HRCT findings were consistent with bronchiectasis in most PAD patients, whereas consolidation and mediastinal lesions were more common in the other groups. Conclusions: Pulmonary manifestations vary among different groups of IEIs. The screening for lung complications should be performed regularly to reveal respiratory pathologies in early stages and follow-up on already existing abnormalities. (C) 2022 Codon Publications. Published by Codon Publications.Peer reviewe

Emotional Schema Therapy improves emotion regulation in individuals with a history of child abuse and neglect

Research conducted over the past decade indicates that difficulties in emotion regulation are more prevalent among those who have been abused than in individuals with no such experiences. Emotional Schema Therapy (EST), which is an integration of cognitive and metacognitive models as well as acceptance-based approaches, specifically targets content of beliefs about emotions and dysfunctional coping strategies to deal with difficult emotions. This study provides an initial evaluation of the efficacy of EST in addressing difficulties in six dimensions of emotion regulation in women with a history of child abuse or neglect. The statistical population consisted of all female residents of Isfahan, Iran, aged above 18 years. Five hundred volunteer women responded to a Child Abuse and Neglect questionnaire. Individuals who had higher scores on this questionnaire were invited to participate in the research project. Thirty volunteer participants were randomly assigned into experimental and control groups (n1 = n2 = 15). The intervention was a 15-session group therapy consisting of cognitive, behavioral, experiential, and mindfulness techniques. Participants completed the Difficulties in Emotion Regulation Scale (DERS) 2 weeks before and after the treatment and at 2 months follow-up. The same assessments were conducted with the control group. Data were analyzed by Mann-Whitney U Test, Friedman Test, and Wilcoxon Signed-Rank Test. Results showed that EST significantly improved acceptance of emotional responses, goal-directed behaviors, impulse control, and adaptive emotion regulation strategies in both post-test and follow-up assessments in the experimental group. In contrast, the control group did not have any significant changes on these variables. Significant changes were not detected for lack of emotional awareness and lack of emotional clarity in both groups. These findings indicate that EST is a potential effective treatment to improve aspects of emotion regulation in women with a history of child abuse and neglect

Maximal oxygen consumption, respiratory volume and some related factors in fire-fighting personnel

Background: Firefighters for difficult activities and rescue of damaged people must be in appropriate physical ability. Maximal oxygen capacity is an indicator for diagnosis of physical ability of workers. This study aimed to assess the cardiorespiratory system and its related factors in firefighters. Methods: This study was conducted on 110 firefighters from various stations. An self-administered questionnaire (respiratory disorders questionnaire, Tuxworth-Shahnavaz step test, and pulmonary function test) was used to collection of required data. Average of humidity and temperature was 52% and 17°C, respectively. Background average noise levels were between 55 and 65 dB. Data were analyzed using SPSS software (version 19). Results: The mean age of the study participants was 32 ± 6.2 years. The means of forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and FEV1/FVC were 92% ±9.4%, 87% ±9.2%, and 80% ±6.1%, respectively. The participants' mean VO2-max was 2.79 ± 0.29 L/min or 37.34 ± 4.27 ml/kg body weight per minute. The results revealed that weight has a direct association with vital capacity (VC), FVC, and peak expiratory flow. In addition, height was directly associated with VC, FVC, and VO2-max (P < 0.05). However, there was an inverse and significant association between height and FEV1/FVC (r = −0.23,P< 0.05). Height, weight, body mass index, and waist circumference were directly associated with VO2-max. Conclusions: The findings of this study showed that the amount of maximum oxygen consumption is close with the proposed range of this parameter among firefighters in other studies. Furthermore, the results of the study revealed that individuals had normal amounts of lung volume index. This issue can be attributed to the appropriate usage of respiratory masks

Functional Balance Assessment Scales in Elderly

Objectives Balance impairment is one of the major issues in the elderly that can influence the activities of daily living; thus, treatment of balance impairments is the leading scope of the specialists in this field. The present study identified the valid functional balance assessment scales for clinical purpose. Methods & Materials Electronic searches of PubMed, Embase, CINAHL, Magiran, Iran Medex, and Iran Doc databases were conducted using keyword combinations of &ldquo;assessment or evaluation or result assessment or instrument assessment&rdquo; and &ldquo;balance or equilibrium or posture control or sitting or standing&rdquo; and &ldquo;older adults or resident&rdquo; and &ldquo;fall or fall risk or fall prediction or balance impairment&rdquo;; also manual searches and gray literature were assessed. Inclusion criteria were as follows: stated objective to assess balance, evaluation of validity and reliability properties, standard protocol and evaluation criteria and published in English or Persian. Results A total of 41 balance tests was selected. Among these, BBS, short BBS, TUG, FAB, BST, functional reach test, CTSIB, POMA, and FGA that showed the favored features of this study were selected.&nbsp; Conclusion Selected tests in this study consisted of the items for use in clinical issues and fall prediction

Application of HEART technique in health care system and accuracy of its results

Introduction: Human error is considered as a crucial challenge in occupational settings. Health care system is amongst occupational environments with high rate of human errors. Numerous preceding studies noted that more than 2/3 of medical errors are preventable. Accordingly, different methods are suggested to evaluate human errors, especially in nuclear industries. The aim of this study was to evaluate the application and accuracy of HEART technique in medical health system. Material and Method:&nbsp; This qualitative study was conducted in surgical intensive care units of a hospital in Shiraz city. All nurses recorded errors were categorized regarding the given tasks and then all tasks were ranked based on the number of errors. The probability of nurses&rsquo; tasks error was estimated through AHP-HEART method and the resultant ranking was compared with the recorded errors. Additionally, the prioritization of contributing factors to errors, determined by AHP and AHP-HEART methods, was compared employing Pearson statistical test. Results: Based on the results, there was a concordance in the rate of nurses&rsquo; error determined by HEART method and the recorded errors. However, no significant correlation was between errors contributing factors determined by AHP and AHP-HEART methods. Conclusion: This study suggested that although HEART technique was successful to rank the tasks considering the magnitude of error probability, but the coefficients of error producing conditions should be customized for nurses&rsquo; tasks in order to provide appropriate control measures

Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome

Abstract Background STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. Method Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. Result Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. Conclusion Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies

Evaluation of mobile phone-based tele-monitoring of cystic fibrosis patients during the COVID-19 pandemic: A 3-year experience in Iran

Background: Telemedicine has been used for cystic fibrosis (CF) in a wide range of signs and symptoms even before the COVID 19 pandemic, however, little is known about the health consequences and use of specific health care for cystic CF. This study aimed to evaluate the evolution of clinical trends and data related to mobile based monitoring activities in CF patients at home for 3 years. Methods: This is a semi experimental single group study. Forty five CF patients under 7 years' old who were referred to the Masih Daneshvari Hospital between 2018 and 2021 were selected. A mobile phone_based customized Short Message Service (SMS) application used to monitor patients. Remotely monitored variables included the amount and color of sputum, cough, wheezing, and shortness of breath at rest. SPSS using Chi square and Friedman tests. Results: The condition of patients based on the number and type of cough increased sputum, decreased appetite, fatty stool, fever and dyspnea, headache, noninvasive ventilation, and drug comfortably remained almost unchanged in the study of the 1st, 2nd, and 3rd years, and the studied parameters did not show a significant difference (P > 0.05). Of course, the number of outpatient visits decreased significantly (P value: 0.02). The respiratory rate and arterial oxygen saturation variables were almost the same in three consecutive annual measurements (P values: 0.544 and 0.639, respectively). Conclusion: Telemedicine is a method that is useful in the follow up of chronic diseases such as CF and improves the quality of life and reduces the deterioration of lung function; therefore, there is less need for invasive treatments in the long run, and a fundamental change in referral motivation brings to the hospital

Psychological maladjustment mediates the relation between remembrances of parental rejection in childhood and adults’ fear of intimacy

This study assesses interpersonal acceptance-rejection theory’s (IPARTheory’s) prediction that adults’ (both men’s and women’s) remembrances of parental (both maternal and paternal) rejection in childhood are likely to be associated with adults’ fear of intimacy, as mediated by adults’ psychological maladjustment and relationship anxiety. The study also assesses the prediction that these associations will not vary significantly by gender, ethnicity, language, culture, or other such defining conditions. To test these predictions a sample of 3,483 young adults in 13 nations responded to the mother and father versions of the Adult Parental Acceptance-Rejection Questionnaire (short forms), Adult Personality Assessment Questionnaire (short form), the Interpersonal Relationship Anxiety Questionnaire, the Fear of Intimacy Scale, and the Revised Personal Information Form. Results of multigroup analyses showed that adults’ remembrances of both maternal and paternal rejection in childhood independently predicted men’s and women’s fear of intimacy in all 13 countries. However, remembered maternal rejection was a significantly stronger predictor of adults’ fear of intimacy than was remembered paternal rejection. Results also confirmed the prediction in all 13 countries and across both genders that both maternal and paternal rejection independently predicted adults’ psychological maladjustment and relationship anxiety, which in turn predicted fear of intimacy. In addition, psychological maladjustment partially mediated the relation between remembrances of maternal and paternal rejection, and adults’ fear of intimacy in all 13 countries and both genders