Syndrome In Children Research Articles

Metabolic syndrome in children and adolescents

Background: Metabolic syndrome is a serious condition associated with multiple diseases. The prevalence of metabolic syndrome is increasing caused by an increase in obesity rates among children and adolescents.Current Concepts: Owing to the various definitions used, there is lack of consensus regarding the diagnostic criteria for metabolic syndrome in children and adolescents. Although the exact pathogenesis remains unclear, insulin resistance and subsequent inflammation are key mechanistic contributors. Metabolic syndrome is characterized by overweight and obesity accompanied by abnormalities in glucose metabolism, dyslipidemia, and hypertension and is associated with other conditions such as fatty liver, polycystic ovary syndrome, and a pro-inflammatory state. Prevention and management of metabolic syndrome can be achieved through lifestyle modifications, behavioral interventions, pharmacotherapy, and surgical interventions.Discussion and Conclusion: Childhood and adolescent obesity serves as a risk factor for metabolic syndrome during later life. Early identification and intervention are important to prevent the progression of metabolic syndrome. Clinicians must identify at-risk children and adolescents and provide guidance for the prevention and management of metabolic syndrome.

Modern concepts about cyclic vomiting syndrome in children

Despite the relatively low prevalence in the world and in Russia, in particular, of such a pathology as cyclic vomiting syndrome, the relevance of the problem is due to the lack of research and sufficient information about the etiology, pathogenesis, and most importantly about methods of treatment and prevention of the disease. This syndrome is typical for children aged 3 to 7 years and is manifested by repeated stereotypical episodes of vomiting, alternating with periods of complete well-being. Cyclic vomiting syndrome worsens the child’s quality of life and seriously affects their further development and socialization. The article provides an overview of scientific research on cyclic vomiting syndrome in children.

Patients with Steroid Sensitive Nephrotic Syndrome: A Case Series

Nephrotic syndrome (NS) is one of the most common kidney diseases found in childhood. The prevalence of nephrotic syndrome worldwide is approximately 16 cases per 100,000 children with an incidence of two to seven per 100,000 children. Nephrotic syndrome may affect adults and children of both genders and any race. Nephrotic syndrome in children can be classified into three groups; secondary, congenital, infantile, and idiopathic. In first case, a 3-year-old male patient was presented with complaints of fever, cough for 6 days and swelling all over the body, decreased urine output, oral intake for 2 days, edema of face, body and tiredness for 3 days. Laboratory investigations showed elevated ESR, ferritin and lipid profile. Urine routine examination showed elevated urine albumin levels (+++). USG abdomen and pelvis conveyed right basal pleural effusion. In second case, a 4-year-old male patient was presented with complaints of facial puffiness, fever, breathing difficulty, abdominal distension and pain but no edema. He had history of right inguinal hernia and atrial septal defect. Cholesterol level was found to be elevated. Urine routine examination showed elevated urine albumin levels (+++). USG abdomen and pelvis showed mild ascites and right pleural effusion. Presenting third case, a 12-year-old female patient was presented with complaints of edema and decreased urine output. Patient had history of recurrent episodes of edema and decreased urine output for 11 months. Laboratory investigations showed elevated total cholesterol, declined LFT test and RFT test was found to be normal. Urine routine examination showed elevated urine albumin levels (+++), pus cells, RBC, granular cast and bacteria present. USG abdomen and pelvis indicate acute glomerulo nephritis and acute to moderate ascites. Here case series with 3 cases showed the sequence of nephrotic syndrome which is treated with corticosteroid with its side effects and cumulative dose of steroid in children. Keywords: Nephrotic syndrome, Hypocholesteremia, Hypoproteinuria, Hyponatremia, pediatric

Epidemiological Characteristics of Shiga Toxin-Producing Escherichia coli Responsible for Infections in the Polish Pediatric Population.

Shiga toxin-producing Escherichia coli (STEC) are zoonotic pathogens causing hemorrhagic colitis and hemolytic uremic syndrome (HUS) in children and the elderly. Stool samples were collected from 180 children hospitalized in five pediatric centers in Poland in 2018-2022. Direct stx1/stx2 gene detection by PCR in feces and E. coli isolates was performed. Antibiotic susceptibility was tested according to EUCAST v.12. Randomly selected isolates were serotyped with O157 antiserum and genotyped by pulsed-field gel electrophoresis (PFGE). A total of 44 E. coli isolates were confirmed as STEC by PCR. Among them, 84.4% were positive for stx2, and equally 6,8% for only stx1 and both stx1 and stx2 genes. The stx1 gene was also found in one Citrobacter freundii isolate. E. coli serotype O157 was present in 97.6% of the isolates. STEC infections most often occurred between June-October with a peak in July and August (51%). The highest, 77.8% of STEC isolates were found in the 1-5 years old group. No extended-spectrum β-lactamases (ESBL) were found. Resistance only to amoxicillin/clavulanic acid (24.4%), piperacillin/tazobactam (3%), cefotaxime (6%), gentamicin (6%), ciprofloxacin (3%), azithromycin (3%), trimethoprim/sulfamethoxazole (24,2%) was detected. PFGE analysis showed 18 PFGE types with no clonal distribution. Eight isolates with A, B, and C PFGE types showed genetic relatedness in the type with no detection of transmission way of distribution. STEC strains pose a serious threat to human health, therefore demographic and epidemiological characteristics are crucial for their surveillance.

The Association Between Early Initiation of Systemic Corticosteroids and the Hospital Length of Stay in Patients With Multisystem Inflammatory Syndrome in Children.

Patients with multisystem inflammatory syndrome in children (MIS-C) often require hospital admission. Treatment of MIS-C has included intravenous immunoglobulin, systemic corticosteroids, and/or immunomodulators. There is no standardized approach to when steroids should be initiated during treatment. The study objective was to determine whether early initiation of steroids in patients with MIS-C is associated with the duration of hospital length of stay (LOS). This is a single-center retrospective cohort study of patients younger than 21 years who were hospitalized with MIS-C between March 2020 and September 2021 and received steroids. Cases were obtained from an institutional MIS-C log. Patients with culture proven sepsis and/or those who received intravenous immunoglobulin or steroids within the previous 30 days were excluded. We used a multivariable linear regression model, controlling for potential confounders, to assess the association between early steroids and LOS. A total of 56 patients hospitalized with MIS-C were identified; 38 received systemic corticosteroids and were included in the study. The mean time from admission to steroid administration was 9.8 hours (SD = 7.7) in the early group and 44.6 hours (SD = 14.2) in the late group. There was a statistically significant difference in baseline characteristics of patients receiving early versus late steroids in initial C-reactive peptide, procalcitonin, brain natriuretic peptide, and cardiac dysfunction. After controlling for confounders, initiating steroids within 24 hours of admission for MIS-C was associated with a decreased hospital LOS: in patients treated with early steroids, LOS was 58.3 hours less (95% confidence interval, -100.0 to -16.6; P = 0.007) than in those who received late steroids. Among patients with MIS-C, initiating systemic corticosteroids within 24 hours of admission was associated with decreased hospital LOS.

Multisystemic inflammatory syndrome in children and the BNT162b2 vaccine: a nationwide cohort study.

This research demonstrates that vaccinating children and adolescents against COVID-19 has reduced the risk of MIS-C during the study period. • Most of the published literature regarding vaccine effectiveness is based on case-control studies, which are limited due to small sample sizes and the inability to fully estimate the risk of MIS-C among vaccinated and unvaccinated children and adolescents. • The known underestimation of COVID-19 diagnosis among children and adolescents is challenging, as they often have few to no symptoms. • Significant risk difference was found in favor of the vaccinated group, even after including extreme assumptions regarding the underdiagnosed COVID-19 rate. • During this nationwide study period, it was found that vaccinating children and adolescents reduced the risk of MIS-C and its complications.

Association between Mothers' Working Hours and Metabolic Syndrome in Children and Adolescents: Data from the Korea National Health and Nutrition Examination Survey, 2016-2020.

Prevention and management of metabolic syndrome (MetS) during childhood are crucial. Recently, obesity among children and adolescents has increased with an increase in mothers' working hours. The present study was conducted to determine the relationship between mothers' working hours and MetS in their children. Data from the 2016-2020 National Health and Nutrition Examination Survey were used, and 2,598 children and adolescents aged 10-18 years were included. Logistic regression analysis was conducted to confirm the association between MetS and mothers' working hours for each risk factor. Linear regression analysis was conducted to confirm the association between mothers' working hours and the number of risk factors for MetS. Abdominal obesity in children was higher when the mothers' working hours were 53 hours or more (odds ratio [OR], 2.267; 95% confidence interval [CI], 1.21-4.25). In the trend analysis, the OR of children's abdominal obesity increased significantly as mothers' working hours increased (P-value <0.05). Additionally, sex-stratified analysis revealed a significant trend between maternal work hours and the presence of MetS in female children (P=0.016). The adjusted OR of the presence of MetS in female children with mothers working 53 hours or more weekly was 6.065 (95% CI, 1.954-18.822). Mothers' working hours were highly correlated with the risk of abdominal obesity in their children. The OR of the presence of MetS significantly increased in female children with mothers having longer working hours compared with those with stay-at-home mothers.

Multisystem inflammatory syndrome in children: A review.

To comprehensively review the literature on multisystem inflammatory syndrome in children (MIS-C). Narrative review of relevant studies published between April 2020 and January 2024. MIS-C is a SARS-CoV-2-related hyperinflammatory syndrome developing 2-6 weeks after COVID-19 in genetically susceptible individuals. Persisting fever, mucocutaneous manifestations, GI and cardiac involvement, together with lymphopenia and elevated inflammatory and cardiac markers are the main clinical features. It is believed to recognise some pathogenetic and clinical overlap with Kawasaki disease. New case definitions have been proposed after an assessment of the diagnostic performance of existing criteria; epidemiological criterion is however progressively losing its usefulness as the pandemic turns into an endemic and in the areas with the highest rates of COVID-19 vaccination. Current guidelines recommend both intravenous immunoglobulin and glucocorticoids in the first-line immunomodulatory treatment, mainly based on comparative retrospective cohorts; the actual role of biologics remains to be adequately established. Strict follow-up is mandatory, especially for those with severe cardiac involvement, as longitudinal studies evaluate the long-term evolution of cardiac damage. In this paper, we review the epidemiological, pathogenetic, clinical and prognostic features of MIS-C, and outline the main questions which still remain unanswered after more than 3 years of research.

Atypical Dermatological Presentation of Multisystem Inflammatory Syndrome in a Child with Polyarticular JIA: A Case Report

Multisystem inflammatory syndrome in children (MIS-C) is an inflammatory process that occurs after infection with SARS-CoV-2 virus. In this syndrome children usually present with fever, rash, abdominal pain, diarrhea or vomiting, hypotension and shock. Children may need urgent care if heart, blood vessels or other vital organs are involved. Poly-articular Juvenile Idiopathic arthritis is a subset of JIA defined by presence of arthritis of five or more joints within 6 months of disease onset along with other criteria of JIA. In this case report we report mucocutaneous manifestation of MIS-C in a child with poly-articular rheumatoid factor (RF) negative JIA case. BANGLADESH J CHILD HEALTH 2022; VOL 46 (2) : 86-88

Capgras syndrome in children and adolescents: A systematic review

ObjectivesTo improve understanding of Capgras syndrome (CS) in the pediatric population, this study investigates its clinical features and discerns similarities and differences compared to CS in adults. MethodsWe conducted a descriptive systematic review of case reports following PRISMA guidelines, including cases of pediatric patients with CS. Patient demographics, medical and psychiatric history, imposter identity, underlying diagnosis, clinical manifestation, treatments, and outcomes were extracted and analyzed. ResultsWe included 37 articles comprising 38 cases. The median age of patients was 15, with 23 (60.5%) being male. The most prevalent underlying diagnoses were schizophrenia spectrum and other psychotic disorders (47.3%). Imposter identity involved parents in 32 cases (84.2%). Associated symptoms included persecutory delusions (63.1%), auditory hallucinations (42.1%), aggression (31.5%), and depression (21.0%). ConclusionThere is a significant gap in our understanding of CS, particularly in pediatric patients. This is the first systematic review of CS in pediatric patients, encompassing all cases found in English literature since 1923.

Rapunzel syndrome in children: a retrospective review of ten cases combined with literature review in a tertiary referral center.

Rapunzel syndrome is an uncommon condition in children, and its clinical features remain unclear. This study presents the largest single-center series of pediatric cases to date, with the objective of documenting the clinical characteristics and treatment approaches for children with Rapunzel syndrome. A retrospective study was conducted in children with Rapunzel syndrome from 2019 to 2023. We recorded age, gender, symptoms, locations of bezoar, complications, and treatment options. Ten patients with Rapunzel syndrome were included. The median age was 9.1years, with all of whom were female. The most common clinical symptoms were upper abdominal mass (90%), abdominal pain (80%), and nausea and vomiting (50%). Complications occurred in six cases (60%), including small bowel obstruction (20%), severe gastric dilatation (10%), intestinal perforation (10%), choledochodilation (10%), acute pancreatitis with cholecystitis (10%). Preoperative ultrasonography suggested low-echoic foreign bodies continuing to the jejunum or ileocecal region in five cases (50%). Preoperative gastroscopy attempted in four cases (40%) to remove the foreign bodies, all of which failed. All patients underwent surgical treatment, with nine cases undergoing gastric incision foreign body removal, and one case undergoing gastric incision foreign body removal combined with intestinal perforation repair. All patients recovered well. No recurrence was observed during follow-up. The accuracy of ultrasound diagnosis in identifying Rapunzel syndrome is high; however, it may lead to misdiagnosis if not complemented with the patient's medical history. Endoscopic presents a heightened treatment risk and a reduced success rate. The condition commonly presents with severe complications, thus making laparotomy a safe and effective option for intervention.

Xiaoer-Feire-Qing granules alleviate pyretic pulmonary syndrome induced by Streptococcus pneumoniae in young rats by affecting the lungs and intestines: An in vivo study based on network pharmacology

Ethnopharmacological relevanceThe traditional Chinese medicine (TCM) Xiaoer-Feire-Qing granules (XEFRQ) has been used to treat pyretic pulmonary syndrome (PPS) in children for many years. The function of the lungs is considered to be closely related to the large intestine in TCM. Purpose: We aimed to investigate the effects of XEFRQ on PPS and the underlying mechanisms via network pharmacology and animal experiments. MethodsThe TCMSP platform was used to identify the ingredients and potential targets of XEFRQ. The GeneCards, OMIM, and TTD databases were used to predict PPS-associated targets. Cytoscape 3.9.1 was employed to construct the protein–protein interaction network, and target prediction was performed by GO and KEGG analyses. For the animal experiment, a PPS model was constructed by three cycles of nasal drip of Streptococcus pneumoniae (STP; 0.5 mL/kg). The animals were randomly divided into the following four groups according to their weight (n = 10 rats per group): the blank group, the model group, the XEFRQ-L (16.3 g/kg) group, and the XEFRQ-H (56.6 g/kg) group. Rats in the blank group and the model group were given 0.5% CMC-Na by gavage. The general conditions of the rats were observed, and their food-intake, body weight, and body temperature were recorded for 14 days. After the intervention of 14 days, serum was collected to detect inflammatory cytokines (TNF-α, IL-1β, and PGE2) and neurotransmitters (5-HT, SP, and VIP). H&E staining was used to observe the pathological morphology of lung and colon tissue. AQP3 expression was detected by Western blot. In addition, the gut microbiota in cecal content samples were analyzed by 16S rDNA high-throughput sequencing. ResultsOur network analysis revealed that XEFRQ may alleviate PPS injury by affecting the levels of inflammatory cytokines and neurotransmitters and mitigating STP-induced PPS.In vivo validation experiments revealed that XEFRQ improved STP-induced PPS and reduced the expression of inflammatory cytokines and neurotransmitters. Notably, XEFRQ significantly decreased the protein expression levels of AQP3, which was associated with dry stool. Our gut microbiota analysis revealed that the relative abundance of [Eubacterium]_ruminantium_group, Colidextribacter, Romboutsia, and Oscillibacter was decreased, which means XEFRQ exerts therapeutic effects against PPS associated with these bacteria. ConclusionOur results demonstrate that XEFRQ alleviates PPS by affecting the lungs and intestines, further guiding its clinical application.

Cardiovascular involvement in children with COVID-19 temporally related multisystem inflammatory syndrome (MIS-C): can cardiac magnetic resonance arrive to the heart of the problem?

BackgroundMultisystem inflammatory syndrome in children (MIS-C) shows a significant overlap of symptoms with other hyper-inflammatory diseases such as Kawasaki disease (KD), but the real difference of the two conditions is still matter of debate. Coronary artery lesions (CAL) are the most relevant complication in KD. Nonetheless, CAL, myocarditis, pericarditis, arrhythmia are the main cardiovascular complications in MIS-C. A close clinical assessment is mandatory, both at the diagnosis and during the follow-up, by ECG and echocardiography. Cardiac magnetic resonance (MRI) adds important data to ultrasound findings. However, cardiac MRI studies in MIS-C are limited to a small number of cohorts.MethodsWe enrolled 20 children (age:1–16 years; 11 F; 9 M) with cardiac involvement secondary to MIS-C, all evaluated by cardiac MRI.Results8 children showed pathological cardiac MRI: 2 showed pericardial effusion; 2 showed myocardial oedema; 1 showed aortic insufficiency; 3 showed delayed enhancement (one for acute myocarditis with oedema; 2 for myocardial fibrosis). Delayed enhancement was reduced significantly 5.6-9 months after the first MRI evaluation. 25% of patients with pathological MRI had CAL associated with valvular insufficiency of 2 valves. 17% of patients with normal MRI had CAL, associated with valvular insufficiency of 1 valve in 1 patient. The correlations between haematological, clinical, cardiologic parameters, treatment, did not reach the statistical significance. 4 patients were treated with anakinra. Among those, 2 patients showed a normal cardiac MRI. Cardiac lesions resolved in all the patients during the follow-up. Some patients with pathological cardiac MRI could not underwent a control with MRI, for the low compliance. However, echocardiography and ECG, documented the resolution of the pathological data in these cases.ConclusionsA higher risk of CAL was documented in patients with an association of other cardiac lesions. Cardiac MRI is difficult to perform routinely; however, it is useful for evaluating the acute myocardial damage and the outcome of patients with MIS-C.

Correlation between benign joint hypermobility syndrome and headache in children and adolescents

BackgroundBenign Joint Hypermobility Syndrome (BJHS) is a most common hereditary connective tissue disorders in children and adolescents. This study aimed to investigate the prevalence and subtypes of headache in children with BJHS.MethodsThis observational-analytical study was conducted in a case-control setting on school children aged 7 to 16 years in 2021–2023 in Isfahan, Iran. Students were examined for BJHS using Beighton criteria by a pediatric rheumatologist. Headache disorder was diagnosed according to the Child Headache-Attributed Restriction, Disability, and Social Handicap and Impaired Participation (HARDSHIP) questionnaires for child and adolescent and International Classification of Headache Disorders (ICHD-III).ResultsA total of 4,832 student (mean age 10.3 ± 3.1 years), 798 patients with BJHS and 912 healthy children were evaluated. The probability of headache in children aged 7–11 with hypermobility was 3.7 times lower than in children aged 12–16 with hypermobility (P = 0.001). The occurrence of headache in children with BJHS was more than the control group (P = 0.001), and the probability of headache in children with BJHS was 3.7 times higher than in healthy children (P = 0.001). Migraine was the most common headache type reported of total cases. The probability of migraine in children with BJHS was 4.5 times higher than healthy children ( P = 0.001).ConclusionThis study showed a significant correlation between BJHS and headache (especially migraine) in children and adolescents.

Post-traumatic carpal tunnel syndrome in children

BackgroundGiven limited information about acute carpal tunnel syndrome (CTS) in children, the purpose of this study is to describe the presentation and treatment results of CTS after traumatic wrist or forearm injury in a pediatric population. MethodsIn this retrospective single-center cohort study, all children with post-traumatic CTS were identified. Demographic and clinical presentation, treatment, and outcomes data were collected from the electronic medical record. Descriptive statistics were calculated for variables of interest, and Χ2 and independent sample t-tests were used for subgroup comparisons. Results18 patients (16 male, average age 12.8 ± 3.6 years) with post-traumatic CTS were identified. 13 presented acutely (<3 days) versus 4 subacutely (1–6 weeks) and one delayed presentation (>6 weeks) after injury. Of associated traumas, 83% (15/18) involved distal radius fractures, of which 40% (6/15) had an associated ulna fracture. The most common symptoms were numbness (78%), paresthesias (33%), and increasing pain (28%). Three patients had clinical concerns for concomitant compartment syndrome. 6 patients were initially monitored (2 acute, 3 subacute, 1 delayed). Ultimately, 16/18 underwent operative release (13/13 acute, 2/4 subacute, 1/1 delayed). At follow-up, 81% of the surgically treated patients had complete symptomatic relief. There was no statistically significant difference in treatment outcomes between acute versus subacute or delayed presentation (OR: 6.67, p = .214). Of patients with acute CTS, delayed surgery resulted in prolonged recovery (28.0 vs. 2.4 weeks, p < .001). ConclusionsPost-traumatic CTS more often presents acutely than as a delayed complication of distal radius fractures in children. The type of injury and clinical presentation can vary, but patients most often present with median neuropathy symptoms like numbness. Acute CTS should be treated urgently with carpal tunnel release. While patients with delayed presentation can trial observation, the patient will likely warrant surgery for complete symptom relief. Operative treatment for both acute and subacute cases generally results in positive outcomes. Key Concepts(1)Post-traumatic CTS may present acutely as a complication of distal radius fractures in children with symptoms of numbness, paresthesias, and increasing pain.(2)Acute CTS should be treated with urgent carpal tunnel release to avoid prolonged recovery.(3)Children with delayed presentation of CTS can be safely monitored, but the patient often needs surgery to experience complete symptom relief.(4)Operative treatment for post-traumatic CTS in children generally results in positive outcomes. Level of EvidenceIV Case Series

Diagnostic markers of acute encephalitis syndrome and COVID-associated multisystem inflammatory syndrome in children from Southern India.

Acute encephalitis syndrome (AES) in children poses a significant public health challenge in India. This study aims to explore the utility of host inflammatory mediators and neurofilament (NfL) levels in distinguishing etiologies, assessing disease severity, and predicting outcomes in AES. We assessed 12 mediators in serum (n = 58) and 11 in cerebrospinal fluid (CSF) (n = 42) from 62 children with AES due to scrub typhus, viral etiologies, and COVID-associated multisystem inflammatory syndrome (MIS-C) in Southern India. Additionally, NfL levels in serum (n = 20) and CSF (n = 18) were examined. Clinical data, including Glasgow coma scale (GCS) and Liverpool outcome scores, were recorded. Examining serum and CSF markers in the three AES etiology groups revealed notable distinctions, with scrub typhus differing significantly from viral and MIS-C causes. Viral causes had elevated serum CCL11 and CCL2 compared with scrub typhus, while MIS-C cases showed higher HGF levels than scrub typhus. However, CSF analysis showed a distinct pattern with the scrub typhus group exhibiting elevated levels of IL-1RA, IL-1β, and TNF compared with MIS-C, and lower CCL2 levels compared with the viral group. Modeling the characteristic features, we identified that age ≥3 years with serum CCL11 < 180 pg/mL effectively distinguished scrub typhus from other AES causes. Elevated serum CCL11, HGF, and IL-6:IL-10 ratio were associated with poor outcomes (p = 0.038, 0.005, 0.02). Positive CSF and serum NfL correlation, and negative GCS and serum NfL correlation were observed. Median NfL levels were higher in children with abnormal admission GCS and poor outcomes. Measuring immune mediators and brain injury markers in AES provides valuable diagnostic insights, with the potential to facilitate rapid diagnosis and prognosis. The correlation between CSF and serum NfL, along with distinctive serum cytokine profiles across various etiologies, indicates the adequacy of blood samples alone for assessment and monitoring. The association of elevated levels of CCL11, HGF, and an increased IL-6:IL-10 ratio with adverse outcomes suggests promising avenues for therapeutic exploration, warranting further investigation.

Multisystem Inflammatory Syndrome in Children (MIS-C) in a Lowincome Country: What Treatment Should be Adopted in Case of a Lack of Immunoglobulin?

In multisystem inflammatory syndrome (MIS-C), children typically present high-grade fever, gastrointestinal symptoms, Kawasaki-like symptoms, and even a toxic shock-like syndrome days to weeks after recovering from SARS-CoV-2 infection. It is important to raise awareness of this condition in order to have early diagnosis and immediate treatment of patients. We have, herein, reported 44 cases of MIS-C with various risk factors and symptoms. Furthermore, we have emphasized the efficacy of experience in treating children with MIS-C with high-dose corticosteroids as an alternative to immunoglobulin in low-income countries. We conducted a targeted survey of MIS-C from early May 2020 to October 2022 on 44 children and adolescents with characteristics of multisystem inflammatory syndrome admitted to the pediatric department of the university hospital center in Oujda, Morocco, to which patients diagnosed with MIS-C were referred. The case definition included six criteria: serious illness leading to hospitalization, age under 18 years, fever of at least 24 hours, laboratory evidence of inflammation, multi-organ involvement, biological inflammatory syndrome, and evidence of coronavirus infection based on polymerase chain reaction, antibody testing or exposure to people with COVID-19 in the past month. The criteria used to diagnose myocarditis were impaired left ventricular function, central mitral leak, and elevation of BNP or pro-BNP. Coronary involvement was assessed by the z-score and the criteria for its presence was a z-score equal to or greater than 2.5. Our study included 44 children and adolescents with MIS-C in our hospital, with male predominance (79%) and a median age of six years. Cardiovascular involvement was present in 91%, mucocutaneous in 78%, gastrointestinal in 70%, hematologic in 84%, and respiratory in 2% of patients. Coronary abnormalities (z-score ≥ 2.5) were documented in 21 cases (48%). Glucocorticoids were frequently used in comparison to immunoglobulin, which were uncommonly available and expensive. The therapeutic protocol that was adopted was high doses of short-term prednisone (Cortancyl) at 4mg/kg/day for 4 days. Favorable outcome was noted in all patients over a 2-year period.

Serum proteomics reveals hemophagocytic lymphohistiocytosis-like phenotype in a subset of patients with multisystem inflammatory syndrome in children

Children with Multisystem Inflammatory Syndrome in Children (MIS-C) can present with thrombocytopenia, which is a key feature of hemophagocytic lymphohistiocytosis (HLH). We hypothesized that thrombocytopenic MIS-C patients have more features of HLH. Clinical characteristics and routine laboratory parameters were collected from 228 MIS-C patients, of whom 85 (37%) were thrombocytopenic. Thrombocytopenic patients had increased ferritin levels; reduced leukocyte subsets; and elevated levels of ASAT and ALAT. Soluble IL-2RA was higher in thrombocytopenic children than in non-thrombocytopenic children. T-cell activation, TNF-alpha and IFN-gamma signaling markers were inversely correlated with thrombocyte levels, consistent with a more pronounced cytokine storm syndrome. Thrombocytopenia was not associated with severity of MIS-C and no pathogenic variants were identified in HLH-related genes. This suggests that thrombocytopenia in MIS-C is not a feature of a more severe disease phenotype, but the consequence of a distinct hyperinflammatory immunopathological process in a subset of children.

Proteomic mapping identifies serum marker signatures associated with MIS-C specific hyperinflammation and cardiovascular manifestation

Multisystem inflammatory syndrome in children (MIS-C) shares several clinical and immunological features with Kawasaki Disease (KD) and pediatric hyperinflammation, but the immuno-phenotypic overlap among these clinical mimics is still incompletely understood. Here we analyzed serum samples from treatment-naïve patients with MIS-C (n = 31) and KD (n = 11), pediatric hyperinflammation (n = 13) and healthy controls (HC, n = 10) by proximity extension assay (PEA) to profile 184 blood biomarkers. Collectively, immunophenotypic overlap between MIS-C and hyperinflammation exceeds overlap with KD. Overexpression of IL-17 A in MIS-C and KD could best separate these conditions from hyperinflammatory conditions, while those were hallmarked by overabundance of adenosin deaminase and IL-18. Depletion in serum TNF-related subfamily member 9 (TNFRSF9) and apoptosis inducing ligand (TRAIL) linked with cardiovascular manifestations and myocarditis in MIS-C. Altogether, our analysis highlights important differences in molecular marker signatures also across different MIS-C and KD cohorts and suggests several previously unidentified molecular associations in context of cardiovascular inflammation.

Hemolytic uremic syndrome in children: A case report

Hemolytic uremic syndrome in children: A case report