Search

Juvenile Behcet’s disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behcet’s disease in a tertiary center. The retrospective study included patients who were diagnosed Behcet’s disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded. Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone. In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death.

Aim: The aim of this study is to evaluate the demographic and clinic findings in immunoglobulin A-associated vasculitis (IgAV) patients with scrotal involvement and also to determine predictive factors for assessing the development of scrotal involvement. Materiel and Methods: The medical records of 181 boys who were diagnosed with IgAV in the Pediatric Rheumatology Clinic of our center between September 2015-January 2021 were evaluated retrospectively. Results: A total number of 181 boys with IgAV included in the study. Twenty-seven (14.9%) of the 181 boys with IgAV had scrotal involvement. Among the scrotal-involved patients, 160 boys (88.4%) had scrotal swelling, 120 (66.3%) had erythema and 145 (80.1%) had scrotal pain or tenderness. Scrotal involvement was the first symptom of IgAV in one patient. CRP and WBC were significantly higher in the scrotal-involved group compared to the non-involved group (p=0.018, p=0.04, respectively). There were no significant differences in clinical findings and organ involvements between two groups. On ultrasonography, bilateral scrotal soft tissue thickening was observed in all patients. In 130 of the patients (71.8%), the epididymis was swollen with increased vascularity. The size, echogenicity and vascularity of the testicles were within normal limits. Eighty-seven of the patients (48%) were given steroids, 22 of them (11.7%) nonsteroidal anti-inflammatory drug. Conclusion: Scrotal involvement in boys with IgAV is not rare, it should be considered in the differential diagnosis in patients with scrotal pain, swelling and erythema. In addition, inflammatory markers may be higher in patients with scrotal involvement.

Behcet's disease is an inflammatory vasculitic disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular findings, and multiple organ involvement. Mucocutaneous findings are the most common symptoms. The most used diagnostic criteria are International Criteria for BD (ICBD), International Study Group (ISG) criteria and pediatric Behcet’s disease criteria (PEDBD). Although diagnostic criteria have been defined, the diagnosis is still difficult due to clinical findings developed in pediatric patients. The aim of this study was to evaluate the clinical findings, phenotype characteristics, sensitivity and specificity of diagnostic criteria, and the course of pediatric Behcet's disease (BD). We evaluated retrospectively the files of 67 (29 M/38 F) patients diagnosed with BD according to expert opinion. The patients were reclassified according to ISG, ICBD, and PEDBD criteria. The control group consisted of a total of 69 patients with BD-mimicking diseases or presenting at least one major BD sign followed at the same center. Sensitivity and specificity were evaluated for the criteria. The mean age of diagnosis was 13.38 ± 3.2 years. There were oral aphthous ulcers in 98.5%, genital ulcers in 68.7%, skin lesions in 31.3%, vascular involvement in 17.9%, neurological involvement in 11.9%, positive pathergy test in 33.8%, and positive HLA-B51 in 57.1% of patients. The sensitivity of ICBD, ISG and PEDBD criteria was 88.1, 43.3, and 37.3%, respectively. The specificity of ICBD, ISG, and PEDBD criteria was 100%. Diagnosis of BD in childhood is still difficult. ICBD criteria have the highest sensitivity among the diagnostic criteria. These criteria can also be used in childhood.

Background Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). Objective To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. Methods Fifteen key points selected regarding the diagnosis and management of FMF were assessed by 14 paediatric rheumatologists with a three-round modified Delphi panel. Results Consensus was reached on the following aspects: genetic analysis should be ordered to all patients when clinical findings support FMF, but its result is not decisive alone. In the absence of clinical features, colchicine should be commenced when two pathogenic alleles and family history of amyloidosis are present. Serum amyloid A testing at each visit is recommended in patients resistant to colchicine, with subclinical inflammation and family history of amyloidosis. Consensus was reached on both the definition of colchicine resistance and starting biologic in resistant cases. Cost, efficiency, ease of use, treatment adherence, accessibility and emergence of adverse events are the factors affecting the choice of biologic agents. In patients without any attack and evidence of subclinical inflammation within the last 6 months following initiation of biologics, treatment dose intervals can be prolonged. Conclusion A consensus was achieved regarding the routine diagnosis and screening and treatment of FMF patients. The definition of colchicine resistance was made and a protocol was created for prolongation of treatment intervals of biologic agents. We anticipate that the results of the study reveal real-life data on the approach to patients in clinical practice. Supplementary Information The online version contains supplementary material available at 10.1007/s00296-020-04776-1.

OBJECTIVE: Although the initial reports of coronavirus disease (COVID-19) cases in children described that children were largely protected from severe manifestations, clusters of pediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection began to be reported in the latter half of April 2020. A novel syndrome called "multisystem inflammatory syndrome in children" (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease (KD), toxic shock syndrome (TSS), and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (MAS). Our objective was to develop a protocol for the evaluation, treatment, and follow-up of patients with MIS-C. METHODS: The protocol was developed by a multidisciplinary team. We convened a multidisciplinary working group with representation from the departments of pediatric critical care, cardiology, rheumatology, surgery, gastroenterology, hematology, immunology, infectious disease, and neurology. Our protocol and recommendations were based on the literature and our experiences with multisystem inflammatory syndrome in children. After an agreement was reached and the protocol was implemented, revisions were made on the basis of expert feedback. CONCLUSION: Children may experience acute cardiac decompensation or other organ system failure due to this severe inflammatory condition. Therefore, patients with severe symptoms of MIS-C should be managed in a pediatric intensive care setting, as rapid clinical deterioration may occur. Therapeutic approaches for MIS-C should be tailored depending on the patients' phenotypes. Plasmapheresis may be useful as a standard treatment to control hypercytokinemia in cases of MIS-C with severe symptoms. Long-term follow-up of patients with cardiac involvement is required to identify any sequelae of MIS-C.

OBJECTIVES: The aims of this study were to evaluate the role of biological agents in the treatment of severe multisystem inflammatory syndrome in children (MIS-C) and to assess the current application, outcomes, and adverse effects in patients who are followed up in a pediatric intensive care unit (PICU). PATIENTS AND METHODS: This observational, descriptive, medical records review study was performed on patients with MIS-C admitted to the PICU between September 1 and November 1, 2020. Through medical records review, we confirmed that patients were positive for current or recent SARS-CoV-2 infection or for COVID-19 exposure history within the 4 weeks before the onset of symptoms. RESULTS: A total of 33 patients with severe MIS-C were included (21 male) with a median age of 9 years. The most common signs and symptoms during disease course were fever (100%) and abdominal pain (75.5%). Clinical features of 63.6% patients were consistent with Kawasaki disease/Kawasaki disease shock syndrome, and 36.4% were consistent with secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Myocardial dysfunction and/or coronary artery abnormalities were detected in 18 patients during the PICU stay. Intravenous immunoglobulin and corticosteroids were given to 33 patients. Anakinra was administered to 23 patients (69.6%). There was a significant increase in lymphocyte and platelet counts and a significant decrease in ferritin, B-type natriuretic peptide, and troponin levels at the end of the first week of treatment in patients who were given biological therapy. Two patients were switched to tocilizumab because of an insufficient response to anakinra. The mortality rate of MIS-C patients admitted in PICU was 6.0%. CONCLUSIONS: Management of systemic inflammation and shock is important to decrease mortality and the development of persistent cardiac dysfunction in MIS-C. The aggressive treatment approach, including biological agents, may be required in patients with severe symptoms and cardiac dysfunction.

Objective: Sinus vein thrombosis often presents to the emergency department with headache and is a rare disease among all strokes. Epidemiological data on sinus vein thrombosis are insufficient. Our aim in this study is to examine the findings of patients with sinus vein thrombosis who applied to our emergency department. Material and Method: 267 patients with suspected sinus vein thrombosis in patients who came to our emergency department between 2013-2018 were included in the study. Patients were divided into 2 groups as present and without sinus vein thrombosis. Women were divided into 3 subgroups as not pregnant, pregnant and postpartum period. Demographic data, comorbid diseases, hemogram parameters and radiological data of the patients were recorded in a form. The relationship of the data with sinus vein thrombosis was examined. Results: Sinus vein thrombosis was detected in 19.48% of the patients. 76.3% of the patients were women. 65.4% of them were between the ages of 18-40. The most common complaint was headache with 51.9%. And most of the patients’ neurological examinations were normal. We found that hemoglobin

AIMS: Recently, multisystem inflammatory syndrome in children (MIS-C) has been recognized in association with coronavirus disease 2019 as a cytokine storm syndrome. MIS-C presents with symptoms similar to Kawasaki disease and macrophage activation syndrome (MAS). We aimed to better understand this cytokine storm syndrome by comparing the initial laboratory findings of MIS-C and MAS. METHODS: Patients who were diagnosed with MAS due to systemic juvenile idiopathic arthritis in our clinic between March 2002 and November 2020 and with MIS-C between 20 September and 20 October 2020 were enrolled into the study. The medical files of all patients were reviewed retrospectively. RESULTS: A total of 13 MAS (9 boys, 4 girls) and 26 MIS-C (16 boys,10 girls) patients were included in the study. Hemoglobin, absolute neutrophil and lymphocyte counts, C-reactive protein (CRP), ferritin, fibrinogen and lactate dehydrogenase (LDH) levels showed significant differences between the two groups (P

Objectives: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that FMF patients with early disease onset have more severe disease. The aim of this study was to describe the demographic, clinical and genetic features of FMF patients who had disease onset during the neonatal period. Methods: Medical records of all patients diagnosed as FMF and had been seen in the outpatient clinic of Paediatric Rheumatology department between January 2013 and January 2014 were retrospectively evaluated. Patients with disease onset during the first month of life were included to the study. Results: Among 317 patients; 19 (12 males) were included to the study. Approximately 60% of the patients had family history of FMF. Homozygous p.M694V mutation was detected in 42% of the cases. Thirteen patients present with attacks of fever and remaining had attacks in the form of restlessness, resembling infantile colic starting in the neonatal period. Majority of these patients developed classical abdominal attacks between the ages of 1 and 2.5 years. The diagnosis of FMF was significantly delayed; the median age at onset of therapy was 3.5 years (range 7 months-17 years). Conclusion: Patients with FMF could have complaints even in the neonatal period. Homozygous p.M694V mutation is a prominent mutation in this group of patients. In order to prevent diagnostic delay physicians dealing with these type of patients should be more vigilant.

Familial Mediterranean fever (FMF) is characterized by recurrent, self-limited attacks of fever with serositis involving the peritoneum, pleura and joints. Fatigue is a common problem in many pediatric rheumatic diseases; however, has not been evaluated systematically in FMF patients. Accordingly, the aim of this study was to evaluate fatigue and its possible allied factors in patients with FMF.Patients with FMF, aged between 10 and 21 years, were assessed by completed validated fatigue questionnaire (Checklist Individual Strength-20). As a control group, patients with chronic rheumatic diseases and healthy children without any chronic disease were included.The study group comprised 111 patients with FMF, 54 with other chronic rheumatic diseases and 79 healthy subjects. While the CIS-20 total score and subscale scores (including subjective experience of fatigue) were similar between patients with FMF and those with other chronic rheumatic diseases (p .05); both groups had significantly higher scores when compared with healthy subjects (p .05). FMF patients with musculoskeletal complaints had significantly higher scores of subjective experiences of fatigue when compared to those without those complaints.Fatigue is a common but unrecognized complaint in patients with FMF. Familial Mediterranean fever seems to be a chronic disease with inter attack ongoing complaints.

Introduction Chronic obstructive pulmonary disease (COPD) leads to physical activity limitation and a significant reduction in quality of life. This study aimed to investigate the correlation between The COPD Assessment Test (CAT) score and peak expiratory flow (PEF), and the factors effecting hospital admission rates of patients with COPD. Methods This observational study was conducted prospectively. CAT score was calculated and PEF measured at the time of emergency department admission. Descriptive statistics were expressed as number ( n ), percentage (%), and mean ± standard deviation. Chi-Square and correlation tests were used for statistical analyses. A p value of less than 0.05 was considered statistically significant. Results Of 123 patients included in the study, 85 (69.1%) were male and 38 (30.9%) were female. Pulse pressure, pH, blood urea nitrogen, oxygen saturation measured by pulse oximetry (SpO 2 ), PO 2 , PCO 2, and SpO 2 values on arterial blood gas analysis, and PEF value were significantly correlated to CAT score ( p Conclusion High CAT score and low PEF value can be used to make the decision of hospitalization from emergency department in acute exacerbations of COPD.

Background Familial Mediterranean fever (FMF) is the most common hereditary monogenic autoinflammatory disease caused by mutations in the MEFV gene. It is controversial whether E148Q alteration is an insignificant variant or a disease-causing mutation. The aim of this study was to evaluate the clinical features and disease severity of FMF patients carrying E148Q mutation. Methods Files of FMF patients were retrospectively evaluated. Patients with at least one E148Q mutation were included to the study. The clinical characteristics and disease severity of the patients who were carrying only E148Q mutation were compared with the patients who were compound heterozygous for E148Q and homozygous for M694V mutation. Results The study group comprised 33 patients who were homozygous or heterozygous for E148Q; 34 with compound heterozygous E148Q mutations and 86 patients who had homozygous M694V mutation. Patients who had only E148Q mutation were found to have the oldest mean age of disease onset and lowest mean disease severity score. Attack frequency and colchicine doses were lower in patients with only E148Q mutation as compared with the other two groups. The frequency of clinical findings such as fever, abdominal pain, arthralgia, and arthritis among the three groups was similar. Conclusion Familial Mediterranean fever patients with only E148Q mutation are presenting with late-onset and milder disease course despite having similar clinical findings as compared with patients who had other mutations. Finally, we imply that E148Q is a mutation and colchicine treatment should be given.

Introduction: Necrotizing fasciitis is a serious infection of the subcutaneous tissue and fascia. Despite being rare, it may have a high mortality rate because it usually leads to rapidly progressing fascial necrosis. The purpose of this study was to report a case of early stage necrotizing fasciitis that developed after cryotherapy for a penile condyloma, 19 days before the index emergency department admission. Case Report: A 32-year-old man without any history of systemic disorder was admitted to our emergency department for groin swelling and burning sensation that developed 12 hours before his hospital admission. Edema and hyperemia starting from the left inguinal region and extending to the penis and scrotum were noted on physical examination. While initially being considered a case of early stage necrotizing fasciitis and tested for it, the patient rapidly developed a bulla and entered intermediate stage. We hospitalized the patient with an initial diagnosis of necrotizing fasciitis and initiated dual antibiotic therapy. Furthermore, we debrided the wound and excised the necrotic tissue debris. We discharged the patient on the 12th day. Conclusion: Clinical suspicion should be high for necrotizing fasciitis in emergency. Initiating the most appropriate medical and surgical therapy as soon as the diagnosis is made is the key for preventing mortality and morbidity

BackgroundMedicine is a profession that carries certain risks. One risky area of practice is the emergency department. Emergency physicians diagnose and treat a high volume of patients, and are also responsible for preparing reports for forensic cases. In this study, we aim to investigate emergency physicians' legal-administrative problems and reveal their level of understanding on forensic cases.MethodsAn electronic questionnaire form was prepared after the approval of an ethical committee. This form was sent to the residents, specialists and academicians of emergency medicine by e-mail. The physicians were asked to fill out the form online. All the gathered data was analyzed. Descriptive statistics were presented as frequency percentages with mean and standard deviation. Chi-square tests were used to compare the groups. Correlation between number of complaint cases and age, sex, career, institution, and duration of service in emergency department were investigated. pResults294 physicians participated in the questionnaire. According to the questionnaire, 170 of the physicians were reported to the patient communication units due to medical malpractice. Mean number of compliant reports was 3.20±3.5. 29 of the physicians received administrative penalties. 42 of the physicians were judged in the court for medical malpractice. 1 physician was fined 5000 Turkish Liras as a result of these judgments.ConclusionWe found that the number of complaint reports is negatively correlated with duration of service in emergency medicine and age. There was a significant difference between number of complaint reports and career (p

This study aims to analyze and evaluate the clinic and demographic features of immunocompetent children that have been diagnosed with cytomegalovirus (CMV) infection. The data of children diagnosed with CMV infection between January 2005 and December 2010 and their follow-ups for 2 years were retrospectively evaluated. Ninety-eight patients were included, and the median age at admission was 5.6 months (5 days–36 months). 54.1 % was male. The diagnosis of CMV infection was performed by measurement of serum anti-CMV specific Ig M and IgG titers and PCR method in blood and/or urine. In 3.06 % of the patients, congenital infection was detected, whereas possible congenital infection was observed in 36.7 % of the patients. Furthermore, 44 patients (44.8 %) were detected to have perinatal infection while postnatal infection was spotted in 15.3 % of the patients. The common presenting manifestations were prolonged jaundice, diarrhea, vomiting, abdominal distension, skin eruption, and seizure. And the most common physical examination findings were hepatosplenomegaly, microcephaly, jaundice, and petechia. The mainstream laboratory results were elevated transaminases (50 %), anemia (30.6 %), leukocytosis (27.5 %), and thrombocytopenia (18.3 %). There were intracranial calcification in 5.1 % and eye findings in 5.1 %. On follow-up of patients, complete improvement (59.1 %), neuromotor developmental delay (11.2 %), epilepsy (10.2 %), hearing loss (3.06 %), hemolytic anemia (2.04 %), and growth retardation (1.02 %) were detected. Conclusion: CMV infection is a significant disease both in congenital and perinatal period. It must be considered that diagnosed patients need to be monitored for a long time with special attention to their neurodevelopmental follow-ups.

Background Crimean-Congo hemorrhagic fever (CCHF) virus causes a severe disease in humans with a mortality up to 30%. In Turkey there has been an increase in the number of cases during years since 2002. Humans of all ages living in endemic areas,especially those who are working as shepherds and toddlers, have high risk of acquiring CCHF. Objectives The epidemiological, clinical, and laboratory characteristics of the children, who were diagnosed as Crimean-Congo hemorrhagic fever (CCHF) were described. Study design The children infected with CCHF virus between April 2008 and October 2009, and hospitalised in Ankara Diskapi Children's and Research Hospital were included. Results Laboratory diagnosis was set by detection of CCHF IgM antibodies and/or genetic detection of CCHF virus. Thirtyone cases included to the study, and all were from the northeastern Anatolia and the southern parts of Black sea region. The mean age was 9.45 ± 4.9 years, the proportion of females was 38.7%. The majority (87%) of the cases had the history of tick bite. There was no fatal case. All the patients had the history of fever. Malaise,tonsillopharyngitis, nausea-vomiting, headache, diarrhea, myalgia and rash were the most common symptoms. The mean AST and ALT levels on the admission were 116 (range 25–389) and 61 (range 8–180) U/L respectively. The mean platelet count on admission was 125,000/mm3, and the lowest was 23,000/mm3. The mean of the lowest white blood cell count was 2353/mm3 and the mean of the highest lactate dehydrogenase was 861 IU/L. Conclusions The clinical course of CCHF among children seems to be milder than in adults. Tonsillopharyngitis is a common symptom among children with CCHF.

PubMedID: 21859378 Background: In April 2009 a novel strain of human influenza A, identified as H1N1 virus, rapidly spread worldwide, and in early June 2009 the World Health Organization raised the pandemic alert level to phase 6. Herein we present the largest series of children who were hospitalized due to pandemic H1N1 infection in Turkey. Methods: We conducted a retrospective multicentre analysis of case records involving children hospitalized with influenza-like illness, in whom 2009 H1N1 influenza was diagnosed by reverse-transcriptase polymerase chain reaction assay, at 17 different tertiary hospitals. Results: A total of 821 children with 2009 pandemic H1N1 were hospitalized. The majority of admitted children (56.9%) were younger than 5 y of age. Three hundred and seventy-six children (45.8%) had 1 or more pre-existing conditions. Respiratory complications including wheezing, pneumonia, pneumothorax, pneumomediastinum, and hypoxemia were seen in 272 (33.2%) children. Ninety of the patients (11.0%) were admitted or transferred to the paediatric intensive care units (PICU) and 52 (6.3%) received mechanical ventilation. Thirty-five children (4.3%) died. The mortality rate did not differ between age groups. Of the patients who died, 25.7% were healthy before the H1N1 virus infection. However, the death rate was significantly higher in patients with malignancy, chronic neurological disease, immunosuppressive therapy, at least 1 pre-existing condition, and respiratory complications. The most common causes of mortality were pneumonia and sepsis. Conclusions: In Turkey, 2009 H1N1 infection caused high mortality and PICU admission due to severe respiratory illness and complications, especially in children with an underlying condition. © 2011 Informa Healthcare. Ankara Universitesi Medical School, University of Michigan 7Pediatric 5Pediatric Faculty of Medicine, Assiut University From the 1Department of Pediatrics,Ankara University Medical School,Ankara,Turkey,2Department of Pediatric Infection Diseases,Dr.Sami Ulus Maternity and Children Training and Research Hospital,Ankara,Turkey, 3Department of Pediatrics, Republic of Turkey Ministry of Health Ankara Dıs¸kapı Children’s and Research Hospital, Ankara,Turkey, 4Department of Pediatrics, Ondokuz Mayıs University Medical School, Samsun,Turkey, 5Pediatric Infectious Diseases Unit,Dr.Behçet Uz Children’s Hospital,Izmir,Turkey,6Division of Pediatric Infectious Diseases,S¸is¸li Etfal Training and Research Hospital, Istanbul,Turkey, 7Pediatric Infectious Diseases Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine,Ankara,Turkey,8Division of Pediatrics,Adana Numune Research and Training Hospital,Adana, Turkey,9Department of Pediatrics,Gazi University Medical School,Ankara,Turkey,10Department of Pediatrics,Selçuk University Meram Medical Faculty, Konya,Turkey, 11Department of Pediatrics and Pediatric Infectious Diseases, Uludag^ University Medical School, Bursa,Turkey, 12Clinics of Pediatrics,Tepecik Training and Research Hospital, Izmir,Turkey, 13Division of Pediatric Infectious Diseases, Department of Children’s Health and Diseases, Çukurova University Faculty of Medicine,Adana,Turkey,14Department of Pediatrics,Eskis¸ehir Osmangazi University Faculty of Medicine,Eskis¸ehir, Turkey, 15Pediatric Infectious Diseases Unit, Department of Pediatrics, Süleyman Demirel University Faculty of Medicine, Isparta,Turkey, 16Department of Pediatrics, Marmara University Medical School, Istanbul,Turkey, 17Department of Pediatrics,Mersin University Medical School,Mersin,Turkey,and 18Department of Biostatistics,Ankara University Medical School, Ankara, Turkey