Evaluation of cardiac functions in children with familial Mediterranean fever.

OBJECTIVES: Familial Mediterranean fever is an autosomal recessive autoinflammatory inherited disease. We aimed to evaluate cardiac involvement in children with familial Mediterranean fever during the attack-free period. MATERIAL AND METHODS: The prospective study included 75 familial Mediterranean fever patients during the attack-free period and 50 healthy children. Cardiac evaluation was performed using electrocardiography, 24-hour ambulatory Holter monitoring, and conventional and tissue Doppler echocardiography. Aortic stiffness indices were calculated. RESULTS: There were no differences between the groups in age, height, sex, body mass index, and arterial blood pressure parameters (p > 0.05). QT and corrected QT dispersion parameters were similar in both groups (p > 0.05). The E wave velocity and the E/A ratio of the mitral and tricuspid valves decreased, and the A wave velocity of the tricuspid and mitral valve increased in familial Mediterranean fever by the Doppler echocardiography (p < 0.05). The myocardial contraction velocities (Sd), early relaxation velocity (Ed), and Ed/late relaxation velocity (Ad) of both ventricles were decreased in familial Mediterranean fever group, whereas the Ad of both ventricles and the interventricular septum was increased in familial Mediterranean fever group. Aortic strain and distensibility were decreased, and pressure strain elastic modules (Ep), pressure strain normalised (Ep*) by diastolic pressure, and aortic stiffness ß index were increased in familial Mediterranean fever patients (p < 0.05). When time domain heart rate variability parameters were evaluated, SDNN-i, RMSSD, and PNN50 significantly decreased in familial Mediterranean fever patients (p < 0.05), whereas SDNN and SDANN were similar in both groups (p > 0.05). CONCLUSION: Our findings showed that cardiac involvement could exist in familial Mediterranean fever patients, even during nonattack periods.

Coronary artery bypass grafting in adolescent with myocardial infarction complicating undiagnosed Kawasaki disease.

Although Kawasaki disease is often self-limiting, significant cardiovascular sequelae may occur in the acute or late stage. The most common late complication is persistent coronary artery aneurysm, which can lead to myocardial ischaemia and even myocardial infarction. We report a case of coronary artery bypass grafting in a 16-year-old boy with a history of undiagnosed Kawasaki disease. Increased awareness of Kawasaki disease, especially among children between the ages of 6 months and 5 years, can increase early treatment and prevent serious complications that may occur in the future.

Successful treatment of acute myocardial injury of Duchenne muscular dystrophy with steroids: a case report.

BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked muscular disease which is caused by the absence of dystrophin. Troponin elevation with acute chest pain may indicate acute myocardial injury in these patients. We report a case of DMD that presented with ACP and troponin elevation, who was diagnosed with acute myocardial injury, and successfully treated with corticosteroids. CASE PRESENTATION: A 9-year-old with DMD was admitted to the emergency department with the complaint of acute chest pain. His electrocardiogram (ECG) revealed inferior ST elevation and serum troponin T was elevated. The transthoracic echocardiography (TTE) demonstrated inferolateral and anterolateral hypokinesia with depressed left ventricular function. An ECG-gated coronary computed tomography angiography ruled out acute coronary syndrome. Cardiac magnetic resonance imaging revealed mid-wall to sub-epicardial late gadolinium enhancement at the basal to the mid inferior lateral wall of the left ventricle and corresponding hyperintensity on T2-weighted imaging, consistent with acute myocarditis. A diagnosis of acute myocardial injury associated with DMD was made. He was treated with anticongestive therapy and 2 mg/kg/day of oral methylprednisolone. Chest pain resolved the next day, and ST-segment elevation returned to normal on the third day. Troponin T decreased in the sixth hour of oral methylprednisolone treatment. TTE on the fifth day revealed improved left ventricular function. CONCLUSION: Despite advances in contemporary cardiopulmonary therapies, cardiomyopathy remains the leading cause of death in patients with DMD. Acute chest pain attacks with elevated troponin in patients with DMD without coronary artery disease may indicate acute myocardial injury. Recognition and appropriate treatment of acute myocardial injury episodes in DMD patients may delay the development of cardiomyopathy.

Electrocardiographic Findings in Children With Growth Hormone Deficiency.

Introduction It has been shown that cardiac functions begin to deteriorate in growth hormone (GH) deficiency even in childhood. However, little is known about how GH deficiency affects arrhythmogenesis. The aim of this study was to evaluate the parameters of P wave dispersion (Pd), QT dispersion (QTd), corrected QT (QTc) dispersion (QTcd), T wave peak-to-end (Tp-e) interval, Tp-e/QT ratio, and Tp-e/QTc ratio in children with GH deficiency. This study also aimed to evaluate the relationship of these parameters with insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3). Method In the study, records of children diagnosed with GH deficiency in Adana City Training and Research Hospital Pediatric Endocrine Outpatient Clinic between September 2021 and December 2022 were retrospectively reviewed. The control group consisted of children in the same age group who applied to the Emergency Outpatient Clinic with a complaint of chest pain and no pathological finding was detected. The electrocardiograms (ECGs) of all patients were retrospectively evaluated. Results There were a total of 82 children in the study, 41 of whom were diagnosed with GH deficiency and 41 in the healthy control group. The age and male/female ratio of children with GH deficiency were similar to those in the control group (p>0.05). There were 27 (66%) children with complete GH deficiency and 14 (34%) children with partial GH deficiency. P wave dispersion was similar in both GH-deficient children and control group children. It was also similar in children with complete and partial GH deficiency (p>0.05). QT and QTc dispersions were found to be increased in children with GH deficiency, although not statistically significant, compared to the control group (p>0.05). Tp-e interval, Tp-e/QTmax (longest QT interval), and Tp-e/QTcmax (longest QTc interval) ratios were increased in children with GH deficiency compared to the control group (p=0.001, p=0.003, and p=0.001, respectively). QT and QTc dispersion, Tp-e interval, Tp-e/QTmax, and Tp-e/QTcmax ratios were found to be increased in children with complete GH deficiency compared to children with partial GH deficiency, but the difference was not significant (p>0.05). No correlation was found between these ECG parameters and IGF-1, IGFBP-3, and peak GH levels after stimulation tests (p>0.05). Conclusion We found in our study that the Tp-e interval was longer and Tp-e/QT and Tp-e/QTc ratios were increased in children with GH deficiency. These results suggest that the risk of ventricular arrhythmias in children with GH deficiency may start to increase from childhood. However, further prospective studies are needed to confirm our results.

Pericardial rupture of mediastinal mature cystic teratoma: An unusual cause of pediatric acute chest pain.

A previously healthy 12-year-old girl presented to the emergency department with severe chest pain and dyspnea that woke her from sleep. She had short-term syncope just before the emergency admission. On physical examination, respiratory rate was 26 breaths per minute while resting, and blood pressure was 92/56 mmHg. Other physical examination findings were insignificant. She had no past medical history, and her family history was unremarkable. Laboratory test results showed elevated C-reactive protein (27 mg/L; reference range: <5 mg/L) and white blood cell count (13.7 K/µL; reference range: 4-12 K/µL). Other laboratory test results were within normal limits, including troponin T value (3 ng/L; reference range: 3-14 ng/L). An electrocardiogram showed 1 mm ST-segment elevation in bipolar (D1 and D2) limb leads, and augmented vector foot leads, and echocardiography revealed a complicated pericardial effusion and a suspicious mass adjacent to the left ventricle.

The Incidence and Clinical Characteristics of Infective Endocarditis in Children: A Five-Year, Single-Centre Retrospective Evaluation.

INTRODUCTION: Infective endocarditis (IE) is a rare disease with high mortality and morbidity. In recent years, an increase in the frequency of infective endocarditis has been observed due to the increase in the survival of cases with congenital heart diseases (CHDs) and the use of central catheters. In addition to revealing the incidence of IE in our clinic, this study aimed to evaluate the demographic characteristics, predisposing factors, clinical, laboratory, microbiological, and echocardiographic findings, and the complications of follow-up of our patients diagnosed with IE in light of the literature. MATERIAL AND METHODS: Thirteen patients with IE who were hospitalized in Pediatric Cardiology Clinic, Pamukkale University Medical Faculty Hospital between January 2016 and August 2021 were retrospectively reviewed. The patients included in the study were evaluated in terms of demographic characteristics, predisposing factors, clinical, laboratory and microbiological findings, echocardiography data, surgical intervention needs, and complications. The incidence of IE in our clinic was defined as the rate of IE among patients admitted to the hospital and reported as the number of patients with IE per 100,000 hospital admissions. RESULTS: The median age of these 13 patients was 11 (7-14) years, and the male/female ratio was 6/7. The five-year IE incidence in Pediatric Cardiology Clinic, Pamukkale University Medical Faculty Hospital, was found to be 2.5 in approximately 100,000 hospital admissions. A predisposing factor was detected in all patients. Six patients (46%) had CHDs, and four patients (31%) had acquired heart disease. The other three (23%) patients were receiving immunosuppressive therapy, and these patients had long-term port catheters. Eight (62%) patients had positive blood cultures. Streptococcus viridans, Streptococcus gordonii, Staphylococcus aureus, coagulase-negativeStaphylococcus, Brucella spp, and Candida albicans were isolated in the blood cultures of these patients. IE-related complications developed in seven (54%) patients. Five (38%) had heart failure, three patients (23%) had thromboembolic events, two (15%) had glomerulonephritis, and one (8%) had thrombophlebitis. Four patients were referred to early surgery. Two patients with recurrent IE attacks were referred to surgery after their treatment was completed. CONCLUSION: The incidence of IE has shown an increase recently with increased rates of survival attributable to corrective surgeries performed for congenital heart diseases, increased prosthetic materials used in cardiac surgeries, and increased use of permanent catheters. In our study, the incidence of IE was found to be 2.5 in 100,000 hospital admissions. Our results have shown that rheumatic heart diseases, besides CHDs, are still an important risk factor for Turkey. Due to the low number of cases in IE studies in the pediatric population, there is a need for further studies to be conducted in large series in this field.

Severe acute kidney injury induced by crescentic glomerulonephritis in a child with infective endocarditis.

BACKGROUND: Kidney involvement related to infective endocarditis (IE) may present with different clinical findings. The most common histopathological finding of renal involvement is a combination of proliferative and exudative glomerulonephritis. However, severe acute kidney injury (AKI) induced by crescentic glomerulonephritis (CGN) is extremely rare in children with IE. To date, only 4 pediatric cases with IE-induced CGN had been reported. We present a 14-year old girl with IE-induced CGN. CASE: A 14-year old girl with fever, macroscopic hematuria, oliguria, and acute kidney injury (AKI) was admitted to our clinic. The medical history revealed that the patient had undergone several cardiac interventions due to truncus arteriosus type 1, and she recovered from IE-induced glomerulonephritis following antibiotherapy six months ago. During admission, the patient was diagnosed with IE according to one major (positive imaging finding) and three minor (fever, predisposing cardiac disease, and immunological criterion) criteria. Immediate antibiotic treatment was initiated. A kidney biopsy was performed, which showed crescentic glomerulonephritis (CGN with crescents, > 50%). Daily pulse steroid (3 days), monthly pulse cyclophosphamide (6 doses), and oral steroid (2 mg/kg/day) therapy were initiated with gradual dose tapering. The patient underwent 12 hemodialysis sessions until the 38 < sup > th < /sup > day of the treatment. She was discharged on the 45th day of treatment with normal kidney function tests and negative acute phase reactants. Treatment was maintained with mycophenolate mofetil (MMF) after a 6-month course of cyclophosphamide. MMF was discontinued in the 12th month. At the 18thmonth follow-up visit the patient had mild proteinuria, and was on ramipril therapy. CONCLUSIONS: The occurrence of CGN should be considered in children with predisposing cardiac disease, who develop hematuria, proteinuria, and severe AKI. Although antibiotic therapy alone is often sufficient in this immune complex GN induced by infection, early initiation of additional immunosuppressive therapy in the presence of CGN may be beneficial for long term preservation of kidney functions.

The effectiveness and safety of ibuprofen and acetylsalicylic acid in acute rheumatic fever.

BACKGROUND: Acetylsalicylic acid (ASA) is a non-steroidal anti-inflammatory drug used in the treatment of acute rheumatic fever (ARF) and it can cause serious adverse effects. This study aimed to evaluate the clinical efficacy and side effects of ibuprofen in the treatment of ARF compared to the classic treatment, ASA. METHODS: Children who were hospitalized for the treatment of ARF with isolated arthritis and mild carditis between October 2015 and October 2018 and who received non-steroidal anti-inflammatory therapy were evaluated in the study. We compared the demographic data, clinical findings, efficacy, and side effects of the treatments of the children, who were divided into ASA and ibuprofen groups. RESULTS: Of the 38 patients included in the study, 21 were treated with ASA (ASA group), and 17 were given ibuprofen (IBU group). There was no difference between the groups regarding the length of hospital stay, total treatment time, time to resolution of clinical symptoms, and normalization of acute-phase reactants. Liver enzymes increased during treatment in 13 patients (62%) in the ASA group and three patients (18%) in the IBU group (P = 0.009). In the ASA group, 69% of patients with increased liver enzymes were under 11 years of age during treatment. Clinical side effects were observed in three patients in the ASA group while none were observed in the IBU group. CONCLUSION: The results of this study suggest that ibuprofen can be a safe alternative in the treatment of ARF, especially in young children. Although ibuprofen can be a safe and effective alternative to ASA, studies including larger series are needed on this subject.

Coexistence of Rhabdomyolysis, Myocarditis and Arrhythmia after Spider Bite: A Case Report.

BACKGROUND: Rhabdomyolysis after spider bite has been reported in a small number of patients, and myocarditis in even fewer. However, arrhythmia associated with latrodectism in children has not been described in the literature to date. CASE SUMMARY: A girl presented approximately 4.5 h after being bitten on the left ankle by a black spider. Two unifocal premature ventricular contractions (PVCs) were observed on the electrocardiogram. In laboratory tests, creatine kinase was elevated. On day 2, levels of troponin, pro-brain and natriuretic peptide were elevated. Electrocardiogram revealed inverted and biphasic T waves. Echocardiography revealed mild left ventricular dilation, mitral and aortic valve regurgitation. Holter electrocardiogram showed PVCs. Her laboratory and echocardiography findings completely normalized after discharge, and no arrhythmia was observed on the Holter electrocardiogram during outpatient follow-up. CONCLUSION: Although spider bites are uncommon, they can cause serious systemic effects. These patients should be evaluated for arrhythmia, rhabdomyolysis and myocarditis.

Rarely, spider bites can cause serious systemic effects, severe morbidity and death. In a small number of patients, spider envenomation causes rhabdomyolysis and myocarditis. In the present case, the elevated troponin and pro-brain natriuretic peptide levels and electrocardiogram/echocardiography findings were consistent with myocarditis, and an increase in creatinine kinase level indicated rhabdomyolysis. In addition, the electrocardiogram and Holter electrocardiogram revealed unifocal premature ventricular contraction. To our knowledge, arrhythmia due to Latrodectus spider bite has not been described in children to date. In addition, this case demonstrates the coexistence of two serious systemic effects, rhabdomyolysis and myocarditis, with full recovery after appropriate treatment.

Histopathological and Clinical Analysis of Skin Rashes in Children With Multisystem Inflammatory Syndrome Associated With COVID-19.

INTRODUCTION: A new entity, which occurs a few weeks after SARS-CoV-2 infection and resembling incomplete Kawasaki disease or toxic shock syndrome, has been defined and named multisystem inflammatory syndrome (MIS-C) associated with COVID-19 in children. The aim of our study was to describe histopathological characteristics of skin lesions of MIS-C patients to reveal whether there is a relationship between histopathological features and clinical manifestations. MATERIALS AND METHODS: Seventeen who had skin involvement of 57 patients who were diagnosed with MIS-C between December 2020 and February 2021 were included in this prospective study. Demographic information, laboratory findings, and patients' managements were recorded. Skin biopsies were taken simultaneously of each patient. Formalin-fixed, paraffin-embedded skin samples were examined microscopically. RESULTS: The rate of skin rash was 30% in patients with MIS-C and was predominantly the maculopapular type. The anatomical distribution of the rash was evaluated as localized in 10 and generalized in 7 patients. In patients with myocarditis, C-reactive protein and fibrinogen were found to be significantly higher, and lymphocyte and albumin values were found to be low. Herpes-like inclusions were found in the microscopic examination of 2 patients with a history of zona zoster in themselves or in their mother. There was a significant difference between keratinocyte necrosis and some clinical parameters. DISCUSSION: Localized skin lesions appear to be associated with a more severe inflammatory.

Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study.

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.

Pentraxin 3 levels and correlation with disease severity in patients with acute rheumatic fever.

OBJECTIVES: This study aims to investigate serum pentraxin 3 (PTX3) levels during acute episode of acute rheumatic fever (ARF) and their relationship with disease severity. PATIENTS AND METHODS: The prospective study was conducted between January 2015 and December 2018 and included 52 ARF patients (22 girls, 30 boys, mean age 10.7±2.1 years; range, 5 to 16 years) experiencing an acute episode and 22 healthy children (13 girls, 9 boys, mean age 10.3±3.8 years; range, 5 to 16 years). ARF patients were classified into three groups based on the clinical course: isolated arthritis (n=17), mild carditis (n=19), and moderate/severe carditis (n=16). Blood samples were collected from all patients before treatment and from the healthy children in the control group to measure PTX3 levels. PTX3 was measured using sandwich enzyme-linked immunosorbent assay method. RESULTS: Plasma PTX3 levels were significantly higher in ARF group compared to the control group (4.7±5.2 and 1.2±1.7 ng/mL, p<0.001). Subgroup analysis of serum PTX3 levels in ARF patients with isolated arthritis, mild carditis, and moderate/severe carditis (3.2±3.1 ng/mL, 4.3±5 ng/mL, and 6.7±6.6 ng/mL, respectively) showed that serum PTX3 was significantly higher in the moderate/severe carditis group compared to the other groups (p<0.05). Analysis of echocardiographic data showed that serum PTX3 was positively correlated with left ventricular end-diastolic diameter, left atrial diameters, and mitral A velocity and negatively correlated with E/A ratio (p<0.05; r=0.231, 0.402, 0.562, -0.586, respectively). CONCLUSION: High PTX3 level during an acute episode of ARF may help predict the clinical course and the severity of accompanying carditis. However, prospective studies with larger sample sizes are needed.

Incidence and clinical characteristics of acute rheumatic fever in Turkey: Results of a nationwide multicentre study.

AIM: To evaluate the incidence and clinical features of acute rheumatic fever (ARF) in Turkey, following the revised Jones criteria in 2015. METHODS: This multicentre study was designed by the Acquired Heart Diseases Working Group of the Turkish Pediatric Cardiology and Pediatric Cardiac Surgery Association in 2016. The data during the first attack of 1103 ARF patients were collected from the paediatric cardiologists between 1 January 2016 and 31 December 2016. RESULTS: Turkey National Institute of Statistics records of 2016 were used for the determination of ARF incidence with regard to various cities and regions separately. The estimated incidence rate of ARF was 8.84/100 000 in Turkey. The ARF incidence varied considerably among different regions. The highest incidence was found in the Eastern Anatolia Region as 14.4/100 000, and the lowest incidence was found in the Black Sea Region as 3.3/100 000 (P < 0.05). Clinical carditis was the most common finding. The incidence of clinical carditis, subclinical carditis, polyarthritis, aseptic monoarthritis, polyarthralgia and Sydenham's Chorea was 53.5%, 29.1%, 52.8%, 10.3%, 18.6% and 7.9%, respectively. The incidences of clinical carditis, subclinical carditis, polyarthritis and polyarthralgia were found to be significantly different among different regions (P < 0.05). CONCLUSION: The findings of this nationwide screening of ARF suggest that Turkey should be included in the moderate-risk group.

Myocarditis and intracardiac thrombus due to Henoch-Schönlein purpura: case report and literature review.

Cardiac involvement is very rare in patients with Henoch-Schönlein purpura (HSP). In this case study, we present an 8-year-old girl presenting with HSP-induced myocarditis and thrombus in the right atrium and HSP nephritis. To date, 15 cases of HSP-related cardiac involvement have been reported in the PubMed/MEDLINE, Scopus, and Google Scholar databases. These cases, together with our case, are included in this review. We excluded those patients with other rheumatologic diseases (acute rheumatic fever, acute post-streptococcal glomerulonephritis, Kawasaki disease) accompanied by HSP. Three were children and 13 were adults and all were male except our case. This review revealed tachyarrhythmia, chest pain, dyspnea, murmur, and heart failure as the major signs. Cardiac tests, electrocardiogram (ECG), and imaging methods (echocardiography in all patients, cardiac magnetic resonance imaging (MRI) in three, cardiac biopsy in one, and post-mortem necropsy in three) showed that the cardiac involvements were pericardial effusion, intra-atrial thrombus, myocarditis, coronary artery changes, myocardial ischemia, infarction and necrosis, subendocardial hemorrhage, and left ventricular dilatation. Kidney involvement was not observed in three patients. As the treatment, high-dose prednisolone and cyclophosphamide, oral corticosteroid, azathioprine, nadroparin calcium, ACE inhibitors, calcium antagonists, beta-blockers, and diuretics were used. Eleven patients (all three children and eight of the adults) had a complete cardiac recovery. Cardiac involvement in adults was more likely to be fatal. Death (three patients), ischemia, and infarct have been reported only in adults. We suggested that early and aggressive treatment can be life-saving. MRI examination is effective at identifying cardiac involvement.

Violence against health employees in a child health and diseases clinic: A tertiary-level hospital example.

AIM: Violence in health is an important public health problem that threatens community peace. In our study, it was aimed to examine the state of exposure to violence among employees in our clinic in the last one year and our employees' opinions and attitudes about violence. MATERIAL AND METHODS: The study was performed cross-sectionally. In this study the Violent Incident Form developed by Arnetz (1998) was used as a data collection tool. RESULTS: A total of 182 healthcare workers were included in the study. Of the participants, 14 (7.7%) were faculty members, 37 (20.3%) were physicians, 24 (13.2%) were interns, 70 (38.5%) were nurses, 10 (5.5%) were medical secretaries, and 27 (14.8%) were ancillary health personnel. The female/male ratio was 143/39. Seventy-nine (43.4%) of the employees had been exposed to violence at least once in the last year. Of those experiencing violence, 57 (72%) were female, 28 (35%) were nurses, 21 (27%) were residents, 16 (20%) were interns, five (6.5%) were faculty members, five (6.5%) were allied health personnel, and four (5%) were medical secretaries. Physicians were exposed to violence with a higher rate (p<0.05). Exposure to violence was observed most frequently in the pediatric emergency department. Of all the violent incidents, 58% occurred during night shifts and 46% occurred during examination/treatment/physical care. The perpetrator was a patient in only one incident, the other perpetrators were patients' relatives, and 63% of the perpetrators were men. All violent incidents involved verbal violence, seven (8.8%) incidents contained elements of physical violence, such as spitting, pushing, kicking, biting, restraining or using an object. Only 29 (36.7%) of the 79 staff reported violence, and 50 (63.3%) did not take any action after the violence. CONCLUSION: Violence in health is a common and serious problem even in tertiary hospitals. In our study, only one-third of the affected staff reported violence after exposure to violence. The risk of violence should be reduced in order to ensure a safe work environment, which will be possible by raising awareness of healthcare workers and effective implementation of violence prevention programs.

Deteriorated Systolic Blood Pressure Recovery and Heart Rate Recovery After Graded Exercise in Children With Familial Mediterranean Fever.

OBJECTIVES: This study aims to investigate if cardiac involvement may occur in children with familial Mediterranean fever (FMF) without cardiovascular symptoms by using heart rate recovery (HRR) and systolic blood pressure recovery (SBPR) parameters. PATIENTS AND METHODS: A total of 50 FMF patients (26 males, 24 females; mean age 151±33.4 month; range 60 to 216 month) and 30 healthy controls (18 males, 12 females; mean age 143±43.9 month; range 84 to 228 month) were included in the study. All patients were evaluated by echocardiography. All patients underwent a maximal graded exercise stress test. HRR and SBPR parameters were calculated. RESULTS: There was a significant decrease in HRR1 value in FMF group (p=0.03). SBPR1 and SPBR2 values were higher in FMF group compared to control group (0.96±0.12 vs 0.88±0.12 and 0.95±0.09 vs 0.91±0.11, respectively); and the high SBPR1 value was statistically significant (p=0.02). FMF presence had a negative correlation with HRR1 (r= -0.26, p=0.03) and a positive correlation with SBPR1 (r=0.29, p=0.02). There was a negative correlation of M694V homozygous mutation with HRR1 and HRR2 values (r= -0.43, p=0.004, r=-0.42, p=0.005). CONCLUSION: Cardiac involvement may occur in FMF patients without cardiovascular symptoms. Impaired SBPR and decreased HRR response may indicate increased cardiovascular risk in these patients despite normal exercise stress test results.

Fatal Pulmonary Embolism Due to Inherited Thrombophilia Factors in a Child With Wolfram Syndrome.

Wolfram syndrome-1 is a rare and severe autosomal recessive neurodegenerative disease characterized by diabetes mellitus (DM), optic atrophy, diabetes insipidus, and deafness. Poorly controlled type 1 DM increases the risk for thrombosis. However, coexistence of DM and hereditary thrombosis factors is rarely observed. Here we present the case of a 13.5-year-old, nonfollowed girl newly diagnosed with poorly controlled Wolfram syndrome on the basis of the results of clinical and laboratory examinations. On the eighth day after diabetic ketoacidosis treatment, pulmonary embolism developed in the subject. Thrombus identified in the right atrium using echocardiography was treated by emergency thrombectomy. Homozygous mutation in the methylenetetrahydrofolate reductase gene C677T, heterozygous factor-V Leiden mutation, and active protein C resistance were identified in the patient. The patient was lost because of a recurring episode of pulmonary embolism on the 86th day of hospitalization. We present this case to highlight the need for investigating hereditary thrombosis risk factors in diabetic patients in whom thromboembolism develops.

[Four Hydatid Cysts in One Family: Is Family Screening Necessary?]. / Ayni Aileden Dört Kist Hidatik Olgusu: Aile Taramasi Gerekli mi?

Hydatid cyst is a parasitic infection mostly caused by Echinococcus granulosus. As transmission occurs from infected dogs, it is endemic in animal husbandry regions. Here four patients within the same family are presented. The first patient is a 10 year-old girl admitted with nausea, vomiting, and fever. On her physical examination, there were decreased respiratory sounds in the right lung, rales, and hepatomegaly. In the radiological examination, cysts were seen in both her lung and liver. After the confirmation of the diagnosis with a serological examination, surgical resection was performed, and albendazole treatment was given. On family screening, cysts were detected in the liver and spleen in her asymptomatic 6-year-old brother; in the lung, liver, spleen, and right kidney in her 33-year-old mother who had repeating abdominal pain; and in the liver and left kidney in her 33-year-old asymptomatic father. Hydatid cyst infection was serologically confirmed in all patients, and they were given albendazole and were surgically treated. In this case report, four patients in the same family and diagnosed as having hydatid cysts were presented. It was emphasized that once a hydatid cyst was diagnosed, family screening became important, in endemic regions in particular.

Evaluation of oxidative stress and erythrocyte properties in children with henoch-shoenlein purpura.

OBJECTIVE: Pathogenesis of Henoch-Schönlein purpura (HSP) is not clearly defined. The present study was conducted to investigate the alterations in erythrocyte deformability and oxidative stress in HSP and to examine the possible relationship between erythrocyte deformability and organ involvement in this disease. METHODS: Plasma malondialdehyde (MDA) levels, total antioxidant status (TAS), erythrocyte deformability and aggregation were measured in 21 children with HSP at the disease onset and during the remission period in comparison with healthy subjects. FINDINGS: HSP patients at the active stage had significantly higher MDA and lower TAS levels (P<0.05). Erythrocyte deformability was decreased at the active-stage and increased again at the remission period of HSP (P<0.05). Erythrocyte deformability was significantly decreased at four different shear stresses in patients with gastrointestinal system or renal involvement; and decreased at six different shear stresses in patients with gastrointestinal system, and renal involvement compared to the patients without organ involvement (P<0.05). No significant difference was observed in aggregation parameters (P>0.05). CONCLUSION: The present findings emphasize the association between impaired erythrocyte deformability and organ involvement in HSP.