Should aortic stiffness parameters be used in MIS-C patient follow-up?

We evaluated the short- and long-term effects of multisystem inflammatory syndrome in children (MIS-C) on their cardiovascular system. The study population consisted of 38 MIS-C patients and 55 control patients. Standard echocardiographic measurements and aortic stiffness parameters were compared between the two groups at different time points. During the standard echocardiographic examination at the time of diagnosis, mitral valve insufficiency was detected in 42% of the cases, left ventricular systolic dysfunction in 36%, aortic valve insufficiency in 3%, tricuspid valve insufficiency in 13%, and coronary artery dilatation in 31%. The ejection fraction, pulse pressure of the experimental group were significantly lower than the control group (p < 0.01, p = 0.045, respectively). When aortic stiffness parameters were compared, it was seen that the parameters increased in the experimental group and the difference was significant for aortic distensibility. (p = 0.105, p = 0.029 respectively). When comparing the experimental group's results at diagnosis and at the sixth month, there was a decrease in aortic stiffness parameters at the sixth month compared to the time of diagnosis, but the difference wasn't significant (p = 0.514, p = 0.334). However, no statistically significant difference was detected when comparing the aortic distensibility results of the experimental group with the control group at the sixth month (p = 0.667). Our results showed that many pathological echocardiographic findings detected at diagnosis in MIS-C patients returned to normal within six months. Therefore, we believe that the cardiovascular follow-up period of MIS-C cases should be at least six months.

One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study.

Multisystem inflammatory syndrome (MIS-C) in children is a rare complication of SARS-CoV-2 infection. Knowing the course of the affected or unaffected coronary arteries in the patients under follow-up is important in terms of defining the long-term prognosis of the disease and determining the follow-up plan. This is a multicenter and retrospective study. The data were obtained from nine different centers. Between May 2020 and August 2022, 68 of 790 patients had coronary artery involvement. One-year echocardiographic data of 67 of 789 MIS-C patients with coronary artery involvement were analyzed. Existing pathologies of the coronary arteries were grouped as increased echogenicity, dilatation and aneurysm according to Z scores, and their changes over a 1-year period were determined. The data of all three groups are defined as frequency. SPSS Statistics version 22 was used to evaluate the data. In our study, aneurysm was observed in 16.4%, dilatation in 68.7% and increased echogenicity in 13.4% of the patients. All of the patients with involvement in the form of increased echogenicity recovered without sequelae by the end of the first month. No progression to aneurysm was observed in any of the patients with dilatation. No new-onset involvement was observed in patients with previously healthy coronary arteries during the convalescent period. In addition, from the sixth month follow-up period, there was no worsening in the amount of dilatation in any of the patients. At least 94% of the patients who completed the 12th month control period returned to normal.

Anomalous origin of coronary arteries from the pulmonary artery: A single-center experience.

Background: The aim of the study was to evaluate the clinical and diagnostic findings, treatment, and follow-up of cases of anomalous origin of coronary arteries from the pulmonary artery. Methods: Between January 1998 and June 2021, a total of 14 patients (5 males, 9 females; median age: 15 months; range, 3 to 156 months) diagnosed with anomalous origin of coronary arteries from the pulmonary artery were retrospectively analyzed. Demographic and clinical data of the patients, electrocardiographic, echocardiographic, angiographic, surgical, and follow-up findings were evaluated. Results: The most common symptoms were respiratory distress (n=6) and murmur (n=3). With the exception of three cases, all other patients were diagnosed by echocardiography in the first examination. Severe mitral valve insufficiency was detected in four patients and four other patients had moderate mitral insufficiency on echocardiography. Ejection fraction values ranged between 38 and 79%. Eleven patients underwent direct implantation of the coronary artery into the aorta, and three underwent a Takeuchi procedure. Mortality occurred in only one case. After surgery, mitral insufficiency and ejection fraction values improved. Median follow-up was 62 (range, 5 to 170) months and all patients were asymptomatic, except one who required redo surgery. Conclusion: Anomalous origin of the coronary arteries from the pulmonary artery is an uncommon congenital anomaly. Echocardiography is the main diagnostic tool. If all echocardiographic findings of the coronary anomaly are not investigated thoroughly, the diagnosis may be overlooked.

Infective endocarditis in childhood: a single-centre experience of 26 years.

The aim of this study was to present the clinical and microbiological characteristics of patients with infective endocarditis.A retrospective evaluation was made of patients diagnosed with infective endocarditis between 1995 and 2021. The clinical and laboratory characteristics of the patients were recorded together with conditions constituting a risk for the development of endocarditis, treatment, and surgical outcomes.Evaluation was made of 68 patients with a mean age of 7.3 years (3 months-17 years), diagnosed with infective endocarditis. An underlying cause of CHD was determined in 47 (69%) patients and rheumatic valve disease in 3 (4.4%). There was no structural heart disease in 18 (26%) patients of whom 13 of them had other risk factors. A causative organism was found in 41 (60%) cases, and the microorganism most often determined was viridans group streptococcus. No difference was determined between the patients diagnosed before and after 2007 in respect of the frequency of viridans streptococcus (p > 0.05). Septic emboli were seen in 18 (26%) patients, of which 17 required surgical treatments. In 5 of 11 (16.6%) patients with mortality, the agent was S. aureus. Of the total 28 (41%) patients were evaluated as hospital-acquired endocarditis. The most frequently determined agents in this group were staphylococcus epidermidis and S. aureus.Although CHDs continue to be the greatest risk factor for endocarditis, there is an increasing frequency of endocarditis in patients with no structural heart disease. Mortality rates are still high in infective endocarditis, especially in S. aureus endocarditis.

Multisystem inflammatory syndrome in children associated with COVID-19 in 101 cases from Turkey (Turk-MISC study).

AIM: Multisystem inflammatory syndrome in children (MIS-C) may cause shock and even death in children. The aim of this study is to describe the clinical features, laboratory characteristics and outcome of children diagnosed with MIS-C in 25 different hospitals in Turkey. METHODS: The retrospective study was conducted between 8 April and 28 October 2020 in 25 different hospitals from 17 cities. Data were collected from patients' medical records using a standardised form. Clinical and laboratory characteristics and outcomes according to different age groups, gender and body mass index percentiles were compared using multivariate logistic regression analysis. RESULTS: The study comprised 101 patients, median age 7 years (interquartile range (IQR) 4.6-9.3); 51 (50.5%) were boys. Reverse-transcriptase polymerase chain reaction (PCR) assay was positive in 21/100 (21%) patients; 62/83 (74.6%) patients had positive serology for SARS-CoV-2. The predominant complaints were fever (100%), fatigue (n = 90, 89.1%), and gastrointestinal symptoms (n = 81, 80.2%). Serum C-reactive protein (in 101 patients, median 165 mg/L; range 112-228), erythrocyte sedimentation rate (73/84, median 53 mm/s; IQR 30-84) and procalcitonin levels (86/89, median 5 µg/L; IQR 0.58-20.2) were elevated. Thirty-eight patients (37.6%) required admission to intensive care. Kawasaki disease (KD) was diagnosed in 70 (69.3%) patients, 40 of whom had classical KD. Most patients were treated with intravenous immunoglobulin (n = 92, 91%) and glucocorticoids (n = 59, 58.4%). Seven patients (6.9%) died. CONCLUSION: The clinical spectrum of MIS-C is broad, but clinicians should consider MIS-C in the differential diagnosis when persistent fever, fatigue and gastrointestinal symptoms are prominent. Most patients diagnosed with MIS-C were previously healthy. Immunomodulatory treatment and supportive intensive care are important in the management of cases with MIS-C. Glucocorticoids and intravenous immunoglobulins are the most common immunomodulatory treatment options for MIS-C. Prompt diagnosis and prompt treatment are essential for optimal management.

COVID-19 associated multisystemic inflammatory syndrome in 614 children with and without overlap with Kawasaki disease-Turk MIS-C study group.

Multisystemic inflammatory syndrome (MIS-C) diagnosis remains difficult because the clinical features overlap with Kawasaki disease (KD). The study aims to highlight the clinical and laboratory features and outcomes of patients with MISC whose clinical manifestations overlap with or without KD. This study is a retrospective analysis of a case series designed for patients aged 1 month to 18 years in 28 hospitals between November 1, 2020, and June 9, 2021. Patient demographics, complaints, laboratory results, echocardiographic results, system involvement, and outcomes were recorded. A total of 614 patients were enrolled; the median age was 7.4 years (interquartile range (IQR) 3.9-12 years). A total of 277 (45.1%) patients with MIS-C had manifestations that overlapped with KD, including 92 (33.3%) patients with complete KD and 185 (66.7%) with incomplete KD. Lymphocyte and platelet counts were significantly lower in patients with MISC, overlapped with KD (lymphocyte count 1080 vs. 1280 cells × µL, p = 0.028; platelet count 166 vs. 216 cells × 103/µL, p < 0.001). The median serum procalcitonin levels were statistically higher in patients overlapped with KD (3.18 vs. 1.68 µg/L, p = 0.001). Coronary artery dilatation was statistically significant in patients with overlap with KD (13.4% vs. 6.8%, p = 0.007), while myocarditis was significantly more common in patients without overlap with KD features (2.6% vs 7.4%, p = 0.009). The association between clinical and laboratory findings and overlap with KD was investigated. Age > 12 years reduced the risk of overlap with KD by 66% (p < 0.001, 95% CI 0.217-0.550), lethargy increased the risk of overlap with KD by 2.6-fold (p = 0.011, 95% CI 1.244-5.439), and each unit more albumin (g/dl) reduced the risk of overlap with KD by 60% (p < 0.001, 95% CI 0.298-0.559). CONCLUSION: Almost half of the patients with MISC had clinical features that overlapped with KD; in particular, incomplete KD was present. The median age was lower in patients with KD-like features. Lymphocyte and platelet counts were lower, and ferritin and procalcitonin levels were significantly higher in patients with overlap with KD. WHAT IS KNOWN: • In some cases of MIS-C, the clinical symptoms overlap with Kawasaki disease. • Compared to Kawasaki disease, lymphopenia was an independent predictor of MIS-C. WHAT IS NEW: • Half of the patients had clinical features that overlapped with Kawasaki disease. • In patients whose clinical features overlapped with KD, procalcitonin levels were almost 15 times higher than normal. • Lethargy increased the risk of overlap with KD by 2.6-fold in MIS-C patients. • Transient bradycardia was noted in approximately 10% of our patients after initiation of treatment.

Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome Resembling Juvenile Idiopathic Arthritis: A Single-Center Experience from Southern Turkey.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the PRG4 gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. Herein, we report 3 patients with CACP syndrome from 2 unrelated families. All patients are female, born to consanguineous parents, and had camptodactyly since the first years of their lives. Two patients had a prior diagnosis of juvenile idiopathic arthritis. Hip changes were present in 2 patients, and 2 of 3 patients had undergone surgery for camptodactyly. Routine echocardiographic evaluations were normal during the 2-year follow-up. This paper represents the third study including CACP patients from Turkey. Clinically, all 3 patients resembled juvenile idiopathic arthritis cases and received unnecessary medication. There is also an ongoing need for improving awareness of CACP and an effective treatment focusing on the lubrication of the joint space in CACP patients.

Pre-Pulseless Takayasu Arteritis in a Child Represented With Prolonged Fever of Unknown Origin and Successful Management With Concomitant Mycophenolate Mofetil and Infliximab.

Takayasu arteritis (TA) is classified as a large vessel vasculitis of predominantly aorta and its main branches, resulting in fibrosis and stenosis. Only a minority of TA patients are diagnosed in pre-stenosis phase when constitutional symptoms including fever, arthralgia, weight loss, headache, abdominal pain, and elevated acute phase reactants are dominant insidious characteristics. In this article, we present a 12-year-old female patient, who was referred to our department with a one-year history of low-grade fever, fatigue, and myalgia. Physical examination did not reveal pulse and blood pressure discrepancies between any extremities. Acute phase reactants were markedly elevated, and autoantibodies were negative. Magnetic resonance angiography (MRA) findings have confirmed TA diagnosis with prominent vessel wall thickening in the ascendant and abdominal aorta, focal ectasias and a thoracoabdominal fusiform aneurysm. As methotrexate and methylprednisolone treatment during three months was unsuccessful, infliximab was induced. During the next 12 months, patient had clinical improvement, but worsening of MRA findings and new onset of carotidynia forced us to switch methotrexate to mycophenolate mofetil. Six months later, laboratory and radiological remission were achieved. In conclusion, we report a challenge to diagnose pre-pulseless childhood-TA (c-TA) in the state of prolonged fever with no signs of vascular stenosis, systemic hypertension, pulses and blood pressure discrepancies, bruits and claudication. Therefore, we wish to discourse the importance of early diagnosis of TA since, to our knowledge, there are no studies investigating treatment success only in the early phases of c-TA.

Severe Troponin I Elevation and Myocardial Dysfunction in a Child with Scorpion Sting.

Background Scorpion sting is a common medical problem and major cause of mortality and morbidity in many countries. The symptoms of envenomation are more severe in the pediatric population. The clinical manifestations of scorpion envenomation appear by activation of sympathetic and parasympathetic nervous system. Envenomation can cause multiorgan dysfunction and death. Case Presentation and Findings In this case report, we present a pediatric patient from Adana, Southeast Anatolia, with severe scorpion envenomation with Leiurus abdullahbayrami which is an especially dangerous species for children. Severe systolic dysfunction was detected by echocardiography. Troponin I levels elevated significantly and rapidly responded to three doses of antivenom therapy. Conclusion Early echocardiographic examination is an important procedure for patients with scorpion envenomation. Troponin I is a significant parameter to monitor the degree of cardiac injury. Antivenom therapy is the only recommended specific therapy for children with severe scorpion envenomation due to Leiurus abdullahbayrami . Abnormal echocardiography findings may require repeated doses of scorpion antivenom.

Prevalence of resistant occipital lobe epilepsy associated with celiac disease in children.

PURPOSE: Celiac disease (CD) is a chronic, inflammatory autoimmune disorder caused by intolerance to ingested gluten. Increased frequency of CD has been reported in occipital lobe epilepsy. The aim of the present study is to investigate the frequency of CD among children followed up due to epilepsy and diagnosed with epileptic activity in the occipital lobe in at least one electroencephalography (EEG) test. METHODS: For this research, 90 pediatric epilepsy patients with epileptic activity in the occipital lobe were enrolled in the study group, while the control group comprised of 100 healthy children. In addition to the EEG examination, tissue transglutaminase (tTG) antibody was determined on duodenal biopsy. RESULTS: None of the healthy children in the control group was positive in terms of the tTG antibody test used to scan CD. In the group with epileptic activity in the occipital lobe, two patients out of 90 were tTG antibody positive. The seroprevalence was 1/45 (2.22 %) in this group. These two patients were diagnosed with CD based on the endoscopic duodenal biopsy. In these patients, the seizures were uncontrollable through monotherapy. CONCLUSIONS: Our results showed that the prevalence of CD is observed to be higher than the normal population among the patients with occipital lobe epilepsy. This type of seizure disorder seems to be more resistant to monotherapy, compared with other types of occipital epilepsy. Therefore, screening for CD is recommended in children with resistant epileptic activity in the occipital lobe.