Evaluation of the heart sounds in children using a Doppler Phonolyser.

BACKGROUND: Heart auscultation is an easy and inexpensive tool for early diagnosis of congenital heart defects. In this regard, a simple device which can be used easily by physicians for heart murmur detection will be very useful. The current study was conducted to evaluate the validity of a Doppler-based device named "Doppler Phonolyser" for the diagnosis of structural heart diseases in pediatric patients. In this cross-sectional study, 1272 patients under 16 years who were referred between April 2021 and February 2022, to a pediatric cardiology clinic in Mofid Children Hospital, Tehran, Iran, were enrolled. All the patients were examined by a single experienced pediatric cardiologist using a conventional stethoscope at the first step and a Doppler Phonolyser device at the second step. Afterward, the patient underwent trans-thoracic echocardiography, and the echocardiogram results were compared with the conventional stethoscope as well as the Doppler Phonolyser findings. RESULTS: Sensitivity of the Doppler Phonolyser for detecting congenital heart defects was 90.5%. The specificity of the Doppler Phonolyser in detecting heart disease was 68.9% in compared with the specificity of the conventional stethoscope, which was 94.8%. Among the most common congenital heart defects in our study population, the sensitivity of the Doppler Phonolyser was 100% for detection of tetralogy of Fallot (TOF); In contrast, sensitivity of both the conventional stethoscope and the Doppler Phonolyser was relatively low for detecting atrial septal defect. CONCLUSIONS: Doppler Phonolyser could be useful as a diagnostic tool for the detection of congenital heart defects. The main advantages of the Doppler Phonolyser over the conventional stethoscope are no need for operator experience, the ability to distinguish innocent murmurs from the pathologic ones and no effect of environmental sounds on the performance of the device.

Online information-seeking behavior of Iranian web users on Google about Henoch-Schönlein purpura (HSP): an infodemiology study.

BACKGROUNDS: Previous studies have indicated that users' health information-seeking behavior can serve as a reflection of current health issues within a community. This study aimed to investigate the online information-seeking behavior of Iranian web users on Google about Henoch-Schönlein purpura (HSP). METHODS: Google Trends (GTr) was utilized to collect big data from the internet searches conducted by Iranian web users. A focus group discussion was employed to identify users' selected keywords when searching for HSP. Additionally, keywords related to the disease's symptoms were selected based on recent clinical studies. All keywords were queried in GTr from January 1, 2012 to October 30, 2022. The outputs were saved in an Excel format and analyzed using SPSS. RESULTS: The highest and lowest search rates of HSP were recorded in winter and summer, respectively. There was a significant positive correlation between HSP search rates and the terms "joint pain" (P = 0.007), "vomiting" (P = 0.032), "hands and feet swelling" (P = 0.041) and "seizure" (P < 0.001). CONCLUSION: The findings were in accordance with clinical facts about HSP, such as its seasonal pattern and accompanying symptoms. It appears that the information-seeking behavior of Iranian users regarding HSP can provide valuable insights into the outbreak of this disease in Iran.

Prevalence of generalized joint hypermobility in children with anxiety disorders.

BACKGROUND: Concerning the high prevalence of anxiety disorders and joint hypermobility in children and the lack of related studies in this age group, we aimed to assess the association of hypermobility with anxiety disorders in children. METHODS: In this case-control study, 93 children ages 8-15 years with anxiety disorders referring to the Child and Adolescent Psychiatry Clinic of Mofid Children's Hospital, Tehran, Iran, during 2018, were enrolled. The control group consisted of 100 age and sex-matched children without anxiety disorders. Anxiety was evaluated using the Spence Children Anxiety Scale (SCAS). The diagnosis of generalized joint hypermobility was done based on Beighton and Shiari-Javadi criteria. RESULTS: Based on Beighton's diagnostic criteria 52.7% of the children in the case group and 16% of the children in the control group had generalized joint hypermobility. Moreover, based on Shiari-Javadi criteria, 49.5 and 13% of the children in the case and control groups had generalized joint hypermobility, respectively. Moreover, the internal correlation between the two criteria was 0.91 showing almost complete compatibility between the two (P <  0.001). Age was a risk factor that could predict hypermobility in these children. Other variables such as sex, severity, and type of anxiety disorders, and ADHD, were not predictors of hypermobility syndrome. CONCLUSION: The prevalence of hypermobility was three times higher in children with anxiety disorders and only age was a predictor for the possibility to suffer from generalized joint hypermobility in these children.

Correlation between benign joint hypermobility syndrome and primary focal hyperhidrosis in children: a novel concept.

BACKGROUND: Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connective tissue disorders in children in which autonomic nervous system involvement has been reported. This study aimed to evaluate the frequency of primary focal hyperhidrosis in children with BJHS. METHODS: This observational-analytical study was conducted in a case-control setting on children aged 3 to 15 years in 2018 at Mofid Children's Hospital, Tehran, Iran. Benign joint hypermobility syndrome was diagnosed according to the Brighton criteria; then, the patients referred to a dermatologist for evaluation of hyperhidrosis. RESULTS: In total, 130 eligible patients with confirmed BJHS and 160 age- and sex-matched healthy subjects were enrolled in this study. Primary focal hyperhidrosis (PFH) was seen in 56.2 and 16.3% of the cases and controls, respectively, indicating a significant difference (P < 0.05). The severity of hyperhidrosis did not differ between the two groups. CONCLUSION: Although the results of the study showed a significant correlation between BJHS and PFH, more comprehensive studies are needed to confirm these findings.

Monoarthritis as the initial presentation of acute rheumatic fever in Iran: A single-center retrospective cross-sectional study.

Background: In Iran, there is a lack of information and studies on acute rheumatic fever (ARF), a global health issue. The limited understanding of ARF's prevalence and primary clinical symptoms has led to confusion. This research investigates the characteristics of children aged 3-17 years who experience ARF with monoarthritis as their initial symptom. Methods: A retrospective evaluation of medical records of children diagnosed with ARF was conducted. The study aimed to determine the prevalence of monoarthritis as the first manifestation of ARF and its association with age, gender, family history, and cardiac involvement. Categorical variables were analyzed using the chi-square test with a significance level of < 0.05 and a confidence interval of 95%, using SPSS software (Version 23). Results: The study included 62 patients with ARF, comprising 41 (66.1%) boys with an average age of 8.48±3.27 years. Among these patients, 12 exhibited cardiac involvement according to the revised Jones criteria, with 5 clinical carditis and 7 cases of subclinical carditis. Monoarthritis was the initial symptom in seven patients (11.29%); five (71.4%) also had carditis. There was a significant association (p<0.001) between monoarthritis and carditis. Conclusion: The study concludes that monoarthritis may be an early sign of ARF in children and correlates significantly with cardiac involvement. However, more extensive research with more significant participant numbers is necessary to understand ARF in Iran comprehensively. A thorough cardiac examination is also crucial for patients with ARF and monoarthritis.

Case report of streptococcal infection as a potential precipitating factor in cutaneous polyarteritis nodosa in pediatric patients.

Key Clinical Message: This pediatric case report underscores the importance of maintaining a high clinical suspicion for polyarteritis nodosa (PAN) in patients presenting with atypical features, such as migratory arthritis and subcutaneous nodules. Importantly, it highlights the focus on the potential relationship between streptococcal infection and cutaneous PAN. Early recognition and prompt, aggressive treatment is critical, as PAN can be a life-threatening condition if left unmanaged. This case emphasizes the need for a multidisciplinary approach to effectively identify and manage this rare vasculitis disorder in the pediatric population. Abstract: Polyarteritis nodosa (PAN) is a rare and life-threatening vasculitis with diverse clinical presentations, posing a diagnostic challenge. Early recognition and prompt intervention are crucial to prevent organ damage. We present the case of an 8-year-old boy who exhibited atypical symptoms including migratory arthritis, myalgia, digital discoloration and ischemic changes, and subcutaneous nodules. Initial concerns for septic arthritis were ruled out. A comprehensive evaluation revealed elevated inflammatory markers and a confirmatory skin biopsy demonstrating active leukocytoclastic vasculitis, are highly suggestive of a diagnosis of PAN. Notably, elevated ASO titers suggested a possible concurrent streptococcal infection. The aggressive treatment approach with high-dose aspirin, steroids, methotrexate, and tocilizumab is justified given the severity of the patient's symptoms and the nature of the disease process. This case underscores the importance of considering PAN in the differential diagnosis for children presenting with atypical features. Early diagnosis and prompt intervention, including addressing potential infectious triggers, are crucial for optimal outcomes in pediatric PAN.

Correlation between antiphospholipid antibodies and renal involvement in children with Henoch-Schönlein purpura: A cross-sectional study.

Background: Renal involvement is the most damaging long-term complication of Immunoglobulin-A (IgA) vasculitis. In the lack of a definite predictive biomarker for renal involvement, antiphospholipid antibodies (aPL) have been proposed in recent years. Methods: In this prospective cohort of 48 pediatric patients who were admitted with IgA vasculitis from September 2015 to June 2017, two serum samples were taken 12 weeks apart to detect Anti-Phospholipid antibodies. All patients were followed-up for renal involvement for six months. Results: Renal involvement occurred in 14 out of 48 patients with IgA vasculitis (29.16%). APLs were positive in nine out of 14 patients with IgA vasculitis and renal involvement (64.28%), in contrast to only six out of 34 patients with IgA vasculitis without renal involvement (17.64%). The presence of aPL antibodies was statistically associated with renal involvement (P=0.002). Although, the relationship between both sex (P=0.025) and age (P=0.046) with aPL positivity was statistically significant, performing a modified logistic regression test, the odds ratio was significant between the groups with and without renal involvement only in term of age and aPL positivity). Conclusion: The presence of aPL antibodies was statistically associated with renal involvement. We found a significant relationship between the age and aPL positivity. Hence, we need multicenter, more extensive cohort studies to reach a better and more accurate conclusion on the relationship between serum aPLs and renal involvement in IgA vasculitis patients.

Investigating the comparative effect of vitamin D level with the type of complications in Henoch Schönlein purpura and Kawasaki disease.

INTRODUCTION AND OBJECTIVES: Henoch Schönlein purpura (HSP) and Kawasaki disease (KD) are two main inflammatory diseases among childhood vasculitis. Considering the anti-inflammatory effects of 25-hydroxyvitamin D3, we decided to investigate the association of serum 25-hydroxy vitamin D3 level with the type and severity of these conditions. MATERIALS AND METHODS: The present study was performed as a historical cohort of 254 affected children with KD and HSP vasculitis. The required data were extracted, using a researcher-made questionnaire from patients' electronic file, and then they were analyzed after collecting information of the patients. RESULTS: In HSP group, 54% of participants were boys. Similarly, in KD group, boys were more affected than girls. The comparative 25-hydroxyvitamin vitamin D3 level in HSP patients with and without renal involvement (P=0.02), hematuria (P=0.14), and in two groups with and without heart disease, and also with and without coronary artery dilatation in KD patients (P<0.001) were significant. DISCUSSION AND CONCLUSIONS: The findings showed that insufficient level of vitamin D3 were significantly associated with the exacerbation of complications of both diseases, and therefore it seems that vitamin D deficiency can be an effective predictive factor of severity in HSP and KD patients.

Pulmonary involvement in brucellosis: A case report and literature review.

Although pulmonary involvement is rare in brucellosis it should be considered as a causative agent in patients with prolonged fever and arthritis. Also, it should be presented with manifestations resembling systemic juvenile idiopathic arthritis.

Mediastinal Lymphadenopathy in a Child with Kawasaki Disease.

Kawasaki disease is a mysterious childhood vasculitis. It presents with multi-systemic involvement in which the prolonged high-grade fever and mucocutaneous and lymph node manifestations are the prominent features. Sometimes, the disease has an unusual, atypical or incomplete presentation. Herein, we present a child with hilar lymphadenopathy as a manifestation of Kawasaki disease.

Epidemiological and advanced therapeutic approaches to treatment of uveitis in pediatric rheumatic diseases: a systematic review and meta-analysis.

BACKGROUND: Despite the low prevalence of uveitis in pediatric rheumatic diseases, potential problems as well as high disease burden can complicate its management. In this review, we systematically assessed the epidemiological, etiological, and managerial aspects of uveitis in pediatric rheumatic diseases. METHODS: This current study was conducted in accordance with the established methods and the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P). We searched the manuscript databases, including Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane for all eligible studies in line with the considered keywords. We also conducted the statistical analysis using the Stata software. RESULTS: Considering studies focusing on uveitis in Juvenile Idiopathic Arthritis (JIA) yielded a pooled prevalence of 11.8% (95%CI: 11.2 to 12.4%) for uveitis following JIA. In this regard, the prevalence rate of uveitis related to Behçet,s disease and Systemic Lupus Erythematosus (SLE( was estimated to be 15.0 and 0.8%, respectively. The pooled response rate to Adalimumab and Infliximab was estimated to be 68.0% (95%CI: 65.4 to 70.6%), 64.7% (95%CI: 59.8 to 69.3%), respectively. The documents for the systematical assessment of other biological medications (e.g. Tocilizumab, Daclizumab and Rituximab) were inadequate; however, the mean response rate for these drugs was 59, 75 and 80%, respectively. Our meta-analysis showed a pooled response rate of 40.0% (95%CI, 36.0% to 44.2) to Methotrexate. Significant heterogeneity and significant diffusion bias were demonstrated by reviewing studies. CONCLUSIONS: The pooled prevalence of uveitis in pediatric rheumatic diseases widely varied based on the underlying disease requiring more investigations in different subtypes of rheumatic diseases. The biologic medications, especially Adalimumab are the most effective treatments for uveitis in pediatric rheumatic diseases; however, a combination of the safe, available alternatives is preferred to achieve the most desirable treatment response.

Pediatric Granulomatosis With Polyangiitis Mimicking IgA Vasculitis: A Case Report.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. CASE PRESENTATION: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. CONCLUSION: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

Five-day fever: The main presentation of childhood-onset Takayasu arteritis.

In children with a nonspecific constitutional presentation such as prolonged fever, the physician should pay attention to primary vasculitides after ruling out the more common diseases such as infectious diseases, malignancies, and the other rheumatic disorders. The past history of autoimmunity may be a clue for this.

Midfoot Arthritis in Children: Is There Any Relation With Malignancy?

BACKGROUND: Musculoskeletal symptoms are a presenting manifestation in a number of lymphoproliferative disorders including leukemia, especially in children. Among these primary symptoms, midfoot arthritis seems to be an important alarm for malignancy in children. The aim of this study is evaluation association of midfoot arthritis with malignancy in children. METHOD: In this cross-sectional study, all medical records of patients with arthritis were identified and reviewed. All clinical and laboratory data were recorded in the information form and data were analyzed by SPSS 25 software. RESULTS: A total of 557 cases of arthritis were evaluated, of which 18 (3.2%) cases have primary symptoms of midfoot arthritis. Four of 18 patients (22.2%) had B-cell precursor acute lymphoblastic leukemia, that midfoot arthritis was their first manifestation. Also, their laboratory findings confirmed that platelet, lactic acid dehydrogenesis, and uric acid values were significantly higher in these children. Based on statistical evaluation, there was no significant difference between age and sex in these patients. CONCLUSION: According to the findings of the present study, it can be concluded that "midfoot arthritis" may be the first manifestation of leukemia in children even with a near-normal hematologic values.

Recurrent angioedema, Guillain-Barré, and myelitis in a girl with systemic lupus erythematosus and CD59 deficiency syndrome.

BACKGROUND: CD59 deficiency is a congenital mutation disorder in complement pathway which can present with various manifestations. CASE PRESENTATION: Herein, we presented an adolescent 16-years-old girl with recurrent attacks of Guillain-Barre in early childhood and then recurrent attacks of angioedema, paresthesia, and myelitis. Finally, she presented with quadriplegia, malar rash, proteinuria, lymphopenia, and high titer of antinuclear antibody. So, the patient developed systemic lupus erythematosus. Furthermore, we performed whole exome sequencing which revealed homozygote mutations in CD59 for the patient and heterozygote one for her parents. CD flow cytometry showed less than 1 percent expression of CD59 on the surface of the patient's peripheral blood cells confirming the disorder. So, she had CD59 deficiency. The patient's episodes were managed with plasma exchanges, corticosteroids, Cyclophosphamide, and Mycophenolate Mofetil which induced and maintained remission. CONCLUSION: CD59 deficiency can be presented with various clinical features such as neurologic, hematologic, dermatologic, and rheumatologic problems including systemic lupus erythematosus.

Determination of the Relationship Between Kobayashi, Sano, and Egami Criteria and Prevalence of Intravenous Immunoglobulin Resistance and Coronary Artery Aneurysm in Iranian Children with Kawasaki Disease.

INTRODUCTION: Kawasaki disease (KD) is a systemic vasculitis that occurs mostly in children under five years old. Kawasaki affects the middle-size arteries, especially the coronary arteries. Therefore, without adequate treatment, it may cause coronary artery aneurysm in 25% of patients. The purpose of this study was to investigate the relationship between Kobayashi, Sano, and Egami criterions with coronary artery aneurysm in KD patients during the last ten years and to identify risk factors in patients with intravenous immunoglobulin (IVIG)-resistant and coronary artery aneurysms. METHODOLOGY: Medical records of 363 Kawasaki patients referred during 2008-2017 were reviewed. Patients' demographic data and Kobayashi, Sano, and Egami scores of each patient were calculated. Based on echocardiographic findings, cases of coronary artery aneurysm were determined. Sensitivity, specificity, positive and negative predictive value, and the accuracy of each criterion were determined to predicting IVIG resistance and detect coronary artery aneurysm. RESULTS: There was a slight relationship between IVIG-resistance in Kawasaki children and its prediction based on the Kobayashi risk score, but no relationship was found between the Egami and Sano criteria. Sixty-three patients (17.4%) had coronary artery lesions (CALs) on time of diagnosis. There were no statistically significant differences between gender and mean age of children with and without CALs. Also, there was no significant relationship between coronary artery aneurysm in Kawasaki children and its prediction based on the above three risk factors. The area under the ROC-curve of all three risk measures of Kobayashi, Egami, and Sano indicated that all three criteria were not useful in predicting CALs. CONCLUSION: Despite the low accuracy of the three above criteria to predictive of patients with IVIG resistance, it seems that the variables of age, duration of fever, and C-reactive protein (CRP) are more useful than other variables and may be utilized to evaluate patients by establishing a more appropriate cut-off point.

Determination of diagnostic value (validity) leukocyte esterase (urine dipstick strip) in differentiating inflammatory arthritis from bacterial arthritis.

BACKGROUND: The current diagnostic cornerstone for septic arthritis contains gram stains, bacterial culture, and cell count with a differential of aspirated synovial fluid. Recently, a synovial leukocyte esterase (LE) test has been used for diagnosing septic arthritis. Since this test measures the esterase activity of leukocytes, there is always a dilemma for using this test in patients with inflammatory arthritis. METHODS: We collected the synovial fluid specimens as part of the general diagnostic protocol for patients suspected of Juvenile Idiopathic Arthritis (JIA) or Septic Arthritis (SA). Each group included 34 patients. We compared the result of the synovial LE test with the result of the culture of each patient. RESULTS: The mean ages of patients were 64.14 ± 31.27 and 50.88 ± 23.19 months in the JIA group and septic arthritis group, respectively. The LE test results were positive in 30 specimens, trace in 3 and negative in one in the first-time test and were positive in 31 specimens and trace in 3 in the second-time test, while it was negative in all patients with JIA. Hence, the sensitivity of the synovial LE test was 80.8%, the specificity, PPV, and NPV were 78.6, 70.0, 86.8% respectively based on a positive culture. CONCLUSION: The leukocyte esterase strip test can be used as a rapid, bedside method for diagnosing or excluding bacterial infections in different body fluids. The synovial LE test can be used as an accurate test to rapidly rule in or out an acute articular bacterial infection, even in patients with concurrent inflammatory arthritis.

Relationship between ocular involvement and clinical manifestations, laboratory findings, and coronary artery dilatation in Kawasaki disease.

OBJECTIVES: To assess the incidence of ocular manifestations of Kawasaki disease (KD) in children and to evaluate the relationship between ocular manifestations and the other clinical manifestations, laboratory findings, and echocardiographic findings. METHODS: Complete ophthalmologic examination and echocardiography were performed in 36 patients with KD during the acute phase before starting the treatment. Clinical manifestations and laboratory data including white blood cell (WBC) count, neutrophil-to-lymphocyte ratio, platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were obtained from all the patients. All the clinical and laboratory findings were compared between the group with ocular involvement and the one without ocular involvement. RESULTS: The incidence of bilateral non-exudative conjunctivitis was 63.9%. It was significantly higher in patients with skin rashes (P < 0.05). The incidence of uveitis was 36.1%, which consisted primarily of grade 1+ or 2+ acute anterior uveitis. Neutrophil count and CRP levels were significantly higher in the uveitis group than in the group without uveitis (P < 0.05). Coronary artery dilatation showed significant correlation with uveitis (P < 0.05). Uveitis did not show a significant correlation with other clinical manifestations, ESR, ALT level, AST level, and platelet count (P > 0.05). CONCLUSION: In children with Kawasaki disease, uveitis is associated with coronary artery dilatation, higher neutrophil count, and higher CRP level.

Juvenile Clinically Amyopathic Dermatomyositis: A Case Report and Review of Literature.

Juvenile clinically amyopathic dermatomyositis (juvenile CADM) is a rare rheumatologic disease in children defined as the presence of the hallmark cutaneous features of dermatomyositis in absence of muscle involvement. In this article, we report an Iranian 14.5-year-old girl presented to Rheumatology Clinic of Mofid Children's Hospital, Tehran, Iran in Jan 2016 with cutaneous complaints diagnosed with juvenile CADM. Finally, we provide a literature review of previous studies on juvenile CADM.

Systemic lupus erythematosus flare triggered by a mosquito bite: the first case report.

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide, various, and sometimes deceptive clinical and serological manifestations. Environmental factors such as ultraviolet radiation, viral infections, drugs, hormones, and chemicals could trigger SLE flares in genetically predisposed patients. Case report: We presented a 13-year-old girl with the first presentation of systemic lupus erythematosus triggered by a mosquito bite. She presented with a malar rash started after a mosquito bite on her left cheek. She had oral ulcers, photosensitivity, lymphopenia, proteinuria, and positive serologic tests for SLE. Renal biopsy revealed class II lupus nephritis. Conclusion: Environmental factors can trigger the onset of SLE in genetically susceptible cases. Besides microbial agents, UV radiation, hormones, drugs, emotional stresses, immunization, and chemicals are some of the published examples. We presented a case with a mosquito bite as the possible environmental trigger.