Platelet role in the prediction of MIS-C severity.

Introduction: Multisystem inflammatory syndrome in children (MIS-C) has been reported as one of the cytokine storm syndromes associated with COVID-19. Despite the several proposed diagnostic criteria, MIS-C remains a diagnostic and clinical challenge. Recent studies have demonstrated that platelets (PLTs) play a crucial role in COVID-19 infection and its prognosis. This study aimed to investigate the clinical importance of PLT count and PLT indices in predicting MIS-C severity in children. Patients and methods: We conducted a retrospective single-center study at our university hospital. A total of 43 patients diagnosed with MIS-C during a 2-year period (from October 2020 to October 2022) were included in the study. MIS-C severity was evaluated according to the composite severity score. Results: Half of the patients were treated in the pediatric intensive care unit. No single clinical sign was associated with a severe condition, except for shock (p = 0.041). All the routine biomarkers, such as complete blood count (CBC) and C-reactive protein (CRP), used for MIS-C diagnosis were significant in predicting MIS-C severity. Single PLT parameters, such as mean PLT volume, plateletcrit, or PLT distribution width, did not differ between the severity groups. However, we found that a combination of PLT count and the previously mentioned PLT indices had the potential to predict MIS-C severity. Conclusions: Our study emphasizes the importance of PLT in MIS-C pathogenesis and severity. It revealed that together with routine biomarkers (e.g., CBC and CRP), it could highly improve the prediction of MIS-C severity.

Atrial and Ventricular Structural and Functional Alterations in Obese Children.

Background and objectives: Childhood obesity has reached epidemic levels in the world. Obesity in children is defined as a body mass index (BMI) equal to or above the 95th percentile for age and sex. The aim of this study was to determine early changes in cardiac structure and function in obese children by comparing them with their nonobese peers, using echocardiography methods. Materials and methods: The study enrolled 35 obese and 37 age-matched nonobese children. Standardized 2-dimensional (2D), pulsed wave tissue Doppler, and 2D speckle tracking echocardiography were performed. The z-score BMI and lipid metabolism were assessed in all children. Results: Obese children (aged 13.51 ± 2.15 years; 20 boys; BMI z-score of 0.88 ± 0.63) were characterized by enlarged ventricular and atrial volumes, a thicker left ventricular posterior wall, and increased left ventricular mass. Decreased LV and RV systolic and diastolic function was found in obese children. Atrial peak negative (contraction) strain (-2.05% ± 2.17% vs. -4.87% ± 2.97%, p < 0.001), LV and RV global longitudinal strain (-13.3% ± 2.88% vs. -16.87% ± 3.39%; -12.51% ± 10.09% vs. -21.51% ± 7.42%, p < 0.001), and LV global circumferential strain (-17.0 ± 2.7% vs. -19.5 ± 2.9%, p < 0.001) were reduced in obese children. LV torsion (17.94° ± 2.07° vs. 12.45° ± 3.94°, p < 0.001) and normalized torsion (2.49 ± 0.4°/cm vs. 1.86 ± 0.61°/cm, p = 0.001) were greater in obese than nonobese children. A significant inverse correlation was found between LV and RV global longitudinal strain and BMI (r = -0.526, p < 0.01; r = -0.434, p < 0.01) and total cholesterol (r = -0.417, p < 0.01). Multivariate analysis revealed that the BMI z-score was independently related to LV and RV global longitudinal strain as well as LV circumferential and radial strain. Conclusion: 2D speckle tracking echocardiography is beneficial in the early detection of regional LV systolic and diastolic dysfunctions, with preserved ejection fraction as well as additional RV and atrial involvement, in obese children. Obesity may negatively influence atrial and ventricular function, as measured by 2D speckle tracking echocardiography. Obese children, though they are apparently healthy, may have subclinical myocardial dysfunction.

Unusual case of chronic recurrent multifocal osteomyelitis.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare auto-inflammatory bone disorder that primarily affects young girls, with a mean age of 10 years at onset. Generally, it is a self-limited disease. However, recent data indicate that more than 50% of patients have a chronic persistent disease and about 20% a recurring course of this condition. Also, there are more cases reported with associated auto-inflammatory and autoimmune diseases. In this case report, we present a rare case of sporadic CRMO in which the patient eventually developed C-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibodies)-associated renal vasculitis and hyperparathyroidism. CASE PRESENTATION: A 14 year old female patient was brought to the emergency department with a sudden onset of left leg pain and oedema. After physical evaluation and initial investigation, she was diagnosed with femoral and pelvic deep vein thrombosis. While searching for possible thrombosis causes, osteomyelitis of the left leg was identified. Additional CT and MRI scans hinted at the CRMO diagnosis. Due to the multifocal lesions of CRMO, endocrinological evaluation of calcium metabolism was done. The results showed signs of hyperparathyroidism with severe hypocalcaemia. Moreover, when kidney damage occurred and progressed, a kidney biopsy was performed, revealing a C-ANCA associated renal vasculitis. Treatment was started with cyclophosphamide and prednisolone according to the renal vasculitis management protocol. Severe metabolic disturbances and hyperparathyroidism were treated with alfacalcidol, calcium and magnesium supplements. Secondary glomerulonephritis (GN) associated hypertension was treated with ACE (angiotenzine converting enzyme) inhibitors. Anticoagulants were prescribed for deep vein thrombosis. After 1.5 years of treatment, the patient is free of complaints. All microelement and parathormone levels are within normal range. Kidney function is now normal. To date, there are no clinical or diagnostic signs of deep vein thrombosis. CONCLUSIONS: This case report presents a complex immunodysregulatory disorder with both auto-inflammatory and autoimmune processes. We hypothesize that the long lasting active inflammation of CRMO may induce an autoimmune response and result in concomitant diseases like C-ANCA-associated vasculitis in our patient. Any potential specific pathogenic relationships between these two rare pathologies may need to be further studied. Furthermore, there is a lack of specific biomarkers for CRMO and more studies are necessary to identify CRMO's characteristic patterns and how to best monitor disease progression.

Early development of endocrine and metabolic consequences after treatment of central nervous system tumors in children.

BACKGROUND AND OBJECTIVE: Survival after childhood cancer has dramatically improved during last few decades, implying the need for evaluation and correction of late consequences of the disease and its treatment. The aim of this study was to characterize endocrine and metabolic late effects after treatment of brain tumors in children. MATERIALS AND METHODS: Late complications were analyzed in 51 children treated for brain tumors at the Hospital of Lithuanian University of Health Sciences during 2000-2011. Data on late endocrine and metabolic effects were collected from medical records. Most frequently patients suffered from low-grade glioma (n=17, 33.3%) and medulloblastoma (n=13, 25.5%). The majority (n=42, 82.4%) of the patients underwent surgery; 29 (56.9%) received radiotherapy (RT); 26 (51.0%), chemotherapy; and 17 (33.4%), combined treatment. RESULTS: The median follow-up was 21 months (range 0.25-10.6 years). Most common endocrine consequence was low serum insulin-like growth factor (IGF-I) levels (58.3%), found on average in 30.7 months after cancer treatment. Short stature was observed in 34.6% (mean time to development, 47.7 months), and hypothyroidism in 40.7% of patients (mean time to development, 63.6 months). Low bone mineral density was found in 50.0% of the cases after 44.5 months and overweight in 30.0% after 49.9 months of follow-up. CONCLUSIONS: Survivors of brain tumors suffer from numerous endocrine and metabolic consequences, majority of them developing within the first 5 years after brain tumor therapy. An active follow-up aiming for early diagnosis and therapy is essential for improvement of quality of life in these patients.

Rare refractory Kawasaki disease in an adolescent boy with cardiac and diffuse coronary artery involvement.

Kawasaki disease is an acute multisystemic vasculitis occurring predominantly in infants and young children and rarely in adolescents and adults. At elderly age, Kawasaki disease may remain unrecognized with a subsequent delay in appropriate therapy and an increased risk of coronary artery aneurysms. We report a case of intravenous immunoglobulin- and aspirin-resistant Kawasaki disease and severe cardiovascular damage in an adolescent boy. The article discusses major issues associated with the management of refractory Kawasaki disease.

Heart rate variability and atria function in children at late follow-up evaluation after atrioventricular node slow-pathway radiofrequency ablation.

This study was designed to assess the changes in the conductive system, autonomic dysfunction, and global and regional function of the atria and ventricles in children late after slow-pathway radiofrequency ablation (RFA). The study enrolled 22 children, who has successfully undergone RFA 2 to 5 years previously (RFA group) and 20 healthy children (control group). Electrophysiologic study was performed for the RFA group. Holter monitoring and echocardiography were performed for all the children. At a late follow-up assessment, the RFA children were free of paroxysms, whereas 8 of the 22 children (36%) reported transient palpitations. Both mean and maximal heart rates (HR) were significantly increased, whereas indices of HR variability (% of successive normal sinus RR intervals exceeding 50 ms [pNN50], root mean square of the successive normal sinus RR interval difference [rMSSD], high-frequency component [HFC]) were significantly decreased in the RFA group compared with preablation and control data. Left atrial (LA) and right atrial (RA) volumes were significantly higher, and atria deformation indices were significantly lower in the RFA group. Correlations were found between the mean HR and the volumes of LA (r = 0.477; p < 0.001) and RA (r = 0.512; p < 0.001). A negative correlation between the maximal LA volume and the longitudinal strain rate (SR) during relaxation (r = -0.476; p = 0.03) and a positive correlation between the minimal LA volume and both longitudinal SR (r = 0.361; p = 0.03) and strain (ε) (r = 0.375; p = 0.024) during contraction were shown. These data suggest a possible link between atrial dysfunction and the hyperadrenergic state after RFA.

Changes in electrophysiologic properties of the conductive system of the heart in children with atrioventricular nodal reentrant tachycardia after 2-8 years following radiofrequency catheter ablation of the slow pathway.

UNLABELLED: Radiofrequency ablation of the slow pathway is an effective method of treatment in children with atrioventricular nodal reentrant tachycardia. The aim of our study was to evaluate anterograde conduction properties in children before and after radiofrequency ablation of the slow pathway and to determine the efficacy and safety of this method. MATERIAL AND METHODS: Noninvasive transesophageal electrophysiological examination was performed in 30 patients at the follow-up period (mean duration, 3.24 years) after radiofrequency ablation of the slow pathway. RESULTS: The slow pathway function was observed in 13 patients one day after ablation, in 26 patients during the follow-up period, and in 28 patients after administration of atropine sulfate. Atrioventricular node conduction was significantly decreased the following day after ablation and at the follow-up versus the preablation (165.2 [30.2] bmp and 146.3 [28.5] bpm versus 190.9 [31.4] bpm; P<0.001). The atrioventricular node effective refractory period prolonged significantly the following day after ablation and at the follow-up versus the preablation (319.3 [55.3] ms and 351.0 [82.1] ms versus 248.3 [36.6] ms; P<0.001). Effective refractory period of the fast pathway prolonged significantly as compared with the preablation (from 408.0 [60.4] ms to 481.2 [132.9] ms; P=0.005). The prolongation of effective refractory period of the slow pathway was more significant than effective refractory period of the fast pathway at the follow-up (P<0.001). Two late recurrences occurred; one patient had atrial tachycardia. CONCLUSION: Children with atrioventricular nodal reentrant tachycardia can be effectively and safety cured by ablative therapy. The end-point during slow pathway ablation should be the abolition of tachycardia with preservation of dual atrioventricular nodal physiology.

Atrial tachyarrhythmias in infants.

This report describes clinical features and tactics of treatment of atrial tachyarrhythmias in infancy. Electrophysiologic study was performed in all 20 infants (2 weeks - 12 months old) in order to determine the mechanism of arrhythmia and to predict the clinical efficacy of management. Reciprocating mechanism was revealed in 12 infants. Atrial flutter was diagnosed for 10 infants among whom only 2 patients were older than two months. Six infants were found to have flutter conduction with a rate of 1:1. Four infants had congestive heart failure, 3 of them with a structurally normal heart. Half of the infants with atrial flutter needed long-term antiarrhythmic therapy. Electrotherapy for termination of atrial flutter was effective in all of them. Automatic atrial tachycardia in eight infants presented no major problems unless it became incessant and resistant to pharmacological treatment. The average tachycardia rate reached 171+/-7 beats/min. Atrial reciprocating tachycardia usually affects patients with diseased myocardium.

Idiopathic ventricular tachycardia in children: curative therapy with radiofrequency ablation.

UNLABELLED: Idiopathic ventricular tachycardia is a rare condition, and there is a lack of clear guidelines for the necessity and indications for prophylactic antiarrhythmic or curative treatment. The aim of this study was to review the clinical picture of idiopathic ventricular tachycardia and evaluate the efficacy and safety of radiofrequency ablation therapy in children. MATERIAL AND METHODS: The subjects of this study were 16 children with idiopathic ventricular tachycardia. The mean age at onset of idiopathic ventricular tachycardia was 12 years. All patients underwent electrophysiological examination. Nonfluoroscopic mapping technology (Carto) was used in one case. Radiofrequency ablation was performed in all children (mean duration of follow-up was 46 months). RESULTS: Six children with idiopathic ventricular tachycardia were free of symptoms. Palpitation was the only complain in four patients, and six patients presented with symptoms of circulatory disorder (the tendency of the higher rate of ventricular tachycardia and more premature contractions and episodes of ventricular tachycardia in one day were noticed in five of them). All children after radiofrequency ablation were alive, and only one complication (complete right bundle branch block) occurred. Success at last follow-up included five children with left and six with right idiopathic ventricular tachycardia. CONCLUSIONS: Catheter ablation seems a promising therapeutic option with the outlook possible of the idiopathic ventricular tachycardia in children. It is safe enough and should be considered as the therapy of choice even in children without of symptoms if they wish to live active social and physical life.

[Henoch-Schönlein purpura - one of the most common types of systemic vasculitis in childhood]. / Henoch-Schönlein purpura - dazniausias vaiku sisteminis vaskulitas.

UNLABELLED: The aim of our study was to estimate etiology, most common clinical findings, the course and peculiarities of treatment in children to whom Henoch-Schönlein purpura was diagnosed during 1996-2002 at Clinic of Children's Diseases of Kaunas University of Medicine Hospital. METHODS: The Henoch-Schönlein purpura diagnosis was based on the association of non-trombocytopenic purpura, arthritis and abdominal pain in 45 children, mostly at preschool age. Routine laboratory blood tests usually were normal. Serum level of imunoglobulin A (Ig A), complement 3, 4 (C(3), C(4)), antineutrophil cytoplasm antibodies were measured for some patients. Urinary analyses were performed for all patients in order to assess nephritis. RESULTS: In 1/3 (33.3%) of children Henoch-Schönlein purpura was preceded by an upper respiratory tract infection. The most common clinical signs were: non-trombocytopenic purpura -100%, subcutaneous edema - 53%, arthritis of large joints - 64%, gastrointestinal symptoms (pain, diarrhea with bleeding) - 37.5%. The incidence of renal involvement was 13.3%, but usually not at the onset of the disease. Relapses were often, and they had tendency to repeat in 1-2 week periods. Late relapses were absent. Treatment of Henoch-Schönlein purpura was symptomic. Nonsteroidal anti-inflammatory drugs which are helpful in joint pain, were prescribed for 33.3% patients. Steroids which reduce abdominal pain, melena, massive hemorrhage, nephritic symptoms, were given for 28.8% patients. CONCLUSIONS: Non-trombocytopenic purpura, arthritis and colic abdominal pain, classic triad of Henoch-Schönlein purpura that occurs in early stage of the disease, were most common. Renal involvement had tendency occur later. Nonsteroidal anti-inflammatory drugs were prescribed for patients with arthritis; prednisone reduced gastrointestinal, nephritic symptoms. Relapses were often.