Polyserositis in children

BACKGROUND: An infection with SARS-CoV-2 can trigger a systemic disorder by pathological autoimmune processes. A certain type of this dysregulation is known as Multisystemic inflammatory syndrome in children (MIS-C). However, similar symptoms may occur and have been described as Multisystemic inflammatory syndrome after SARS-CoV-2 Vaccination (MIS-V) following vaccination against SARS-CoV-2. We report the case of a 12-year-old boy who was identified with MIS-C symptoms without previous SARS-CoV-2 infection after receiving two doses of the Pfizer-BioNTech COVID-19 vaccine approximately one month prior to the onset of symptoms...

BACKGROUND: Familial Mediterranean fever is a hereditary autoinflammatory disease affecting mainly Arabs, Turks, Armenians, and Jews with genotype-phenotype heterogeneity, presenting as recurrent episodes of fever along with polyserositis and rash. To date, more than 370 mutations in the MEFV gene have been recognized to cause the disease. METHODS: We conducted a retrospective cohort study involving 124 patients in Hebron, Palestine, diagnosed with FMF at the Al-Ahli, and Palestinian Red Crescent Society (PRCS) Hospitals...

BACKGROUND AND AIM: Familial Mediterranean fever (FMF) is an autosomal recessive disorder. Typical clinical manifestations are self-limiting attacks of recurrent fever, abdominal pain, arthralgia, and chest pain due to aseptic polyserositis. Renal involvement is common in FMF patients. Shear wave elastography (SWE) is a noninvasive method that provides the measurement of tissue stiffness. In this study, we aimed to show that SWE can be used as an adjunctive method for evaluating renal involvement in children with FMF...

(1) Background: Familial Mediterranean Fever (FMF) is the prototypal autoinflammatory disease, characterized by recurrent bursts of neutrophilic inflammation. (2) Methods: In this study we look at the most recent literature on this condition and integrate it with novel information on treatment resistance and compliance. (3) Results: The canonical clinical presentation of FMF is in children with self-limited episodes of fever and polyserositis, associated with severe long-term complications, such as renal amyloidosis...

BACKGROUND AND AIM: MIS-C is characterized by intense immune activation and increased production of cytokines. The aim of our study was to analyse the changes of cellular and humoral immune responses in children with MIS-C, depending on the severity of the disease. METHODS: To conduct the study, the results of clinical, hematological and immunological parameters in children with severe and extremely severe MIS-C were compared. A total of 50 patients participated in the study, which were divided into 3 groups, of which: 20 children with extremely severe MIS-C treated in the ICU (MIS-C ICU "+"); 15 children with severe MIS-C, but without the need for hospitalization in the ICU (MIS-C ICU "-"); 15 children who had COVID-19 and absence MIS-C (MIS-C "-") made up the control group...

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) associated with Severe Acute Respiratory Coronavirus 2 (SARS-CoV-2) usually develops 1-1.5 months after mild or asymptomatic COVID-19 in countries with high incidence. MIS-C has a polymorphism of clinical manifestations, which include prolonged fever, polymorphic rash, non-purulent conjunctivitis, pneumonia complicated by distress syndrome, myocarditis, coronary artery disease, toxic shock syndrome, limb edema, polyserositis, severe abdominal syndrome with diarrhea and others...

Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent attacks of fever and serositis. Diagnosis is made according to clinical findings and supported by genetic analysis. The most commonly used adult diagnostic criteria are the Tel-Hashomer criteria. Pediatric criteria for FMF diagnosis were described in 2009, but their reliability should be supported by additional reports. In this study, we aimed to compare the pediatric criteria and the Tel-Hashomer and 2019 Eurofever/PRINTO classification criteria using our FMF cohort...

Children with multisystem inflammatory syndrome (MIS-C) tend to develop a clinical condition of fluid overload due both to contractile cardiac pump deficit and to endotheliitis with subsequent capillary leak syndrome. In this context, the ability of point-of-care ultrasound (PoCUS) to simultaneously explore multiple systems and detect polyserositis could promote adequate therapeutic management of fluid balance. We describe the PoCUS findings in a case-series of MIS-C patients admitted to the Emergency Department...

Since December 2019, Covid-19 has become a challenge for doctors around the world, including pediatricians. In most infected children, the disease manifests itself in a mild or is char- acterized by a subclinical course. At the same time, in some cases, a severe clinical picture of the so-called late Covid disease may develop, in the form of a multisystem syndrome and other complications. In 2020-2021 at the Academic Pediatric Clinic named after G. Zhvania of Tbilisi State Medical University, we observed 60 children with post-Covid complications and late Covid syn- drome...

Blau syndrome (BS) is a rare, chronic autoinflammatory disease with onset before age 4 and mainly characterised by granulomatous arthritis, recurrent uveitis, and skin rash. Sporadic (also known as early-onset sarcoidosis) or familial BS is caused by gain-of-function mutations in the NOD2 gene, which encodes for a multi-task protein that plays a crucial role in the innate immune defense. We report on three Mexican patients clinically diagnosed with BS who exhibited a likely pathogenic variant in NOD2 as revealed by whole-exome sequencing (WES) and Sanger sequencing: two variants (c...

BACKGROUND: Familial Mediterranean fever (FMF) is the most common auto-inflammatory disease and is characterized by self-limiting episodes of fever and polyserositis. The aim of this study was to determine the atopic clinical findings associated with the MEFV gene. METHODS: A retrospective chart review was conducted of pediatric patients who had received a diagnosis of familial Mediterranean fever between August 2015 and November 2018. RESULTS: A total of 454 patients with familial Mediterranean fever were evaluated...

OBJECTIVE: To study the clinical features, etiology, treatment, and prognosis of neonatal chylous effusions. METHODS: A retrospective analysis was performed for the clinical data of 21 neonates with chylous effusions. RESULTS: Among these 21 neonates, 13 had chylothorax, 2 had chyloperitoneum, 2 had chylopericardium, and 4 had chylous polyserositis. Five neonates with congenital chylous effusions were enrolled as the congenital group, which mostly occurred in the fetal period, and symptoms were observed shortly after birth...

INTRODUCTION: Familial Mediterranean fever (FMF), the most frequent autoinflammatory disease, is caused by mutations in the MEFV gene. It is characterized by recurrent febrile attacks of polyserositis. Liver abnormalities may develop during its course, but they remain poorly defined. OBJECTIVE: To describe liver involvement in FMF patients. METHODS: A systematic search was conducted through PubMed/Medline and Embase from 1946 to January 2020...

Familial Mediterranean fever is a chronic inflammatory disease characterized by periodic and self-limited episodes of fever and aseptic polyserositis. Although colchicine treatment has altered the course of the disease, it is believed that subclinical inflammation is still present, leading to endothelial dysfunction and atherosclerosis in the course of time. In this review, following the published recommendations, we queried online databases such as MEDLINE Pubmed, Scopus, and Web of science for peer-reviewed studies and reviews written in English language, using the following keywords: familial Mediterranean fever, children, endothelial dysfunction, atherosclerosis, cardiovascular disease...

BACKGROUND: Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent episodes of fever and polyserositis and by the onset of reactive amyloid-associated amyloidosis. Amyloidosis due to familial Mediterranean fever can lead to end-stage renal disease, culminating in kidney transplantation for some patients. In this study, we report the clinical outcome of two brothers with familial Mediterranean fever who were the inadvertent donor and recipient, respectively, of a kidney...

Objective: To analyze the clinical, pathological and radiological characteristics of paragonimiasis in children for accurate diagnosis and therapy. Methods: A total of 31 patients with paragonimiasis treated from 2002 to 2016 were selected, including 17 cases from migrant areas and 14 cases from Wenzhou area. Results: In migrant children group, the serum IgE was significantly higher than that in Wenzhou area group [(2 379±944) IU/mL∶(1 552±1 121) IU/mL, t =-2.23, P <0.05], and the duration of therapy was remarkable longer [(13...

Systemic juvenile idiopathic arthritis (sJIA) is characterized by a large range of extra-articular manifestations in addition to joint inflammation. The child with sJIA usually presents with a spiking fever pattern, evanescent rash, and arthralgias/arthritis. Other features often present are lymphadenopathy, hepatosplenomegaly, and polyserositis. The systemic manifestations are frequently more prominent and severe than the arthritis. Laboratory studies reflect systemic inflammation with significant anemia, and elevation of acute phase reactants, platelets, and white blood cell count...

BACKGROUND: Familial Mediterranean fever (FMF) is an inherited disease characterized by recurrent bouts of fever and polyserositis and caused by MEditerranean FeVer gene (MEFV) mutations. Given the febrile characteristics of the disease one would expect higher frequency of febrile seizure in this group of pediatric patients. OBJECTIVES: To evaluate the frequency of febrile seizure and related factors in patients with FMF. METHODS: The children with the diagnosis of FMF were enrolled in the study...

PURPOSE: In this study, we present clinical data from 16,000 familial Mediterranean fever patients. We also discuss the clinical manifestation of a subset of these patients and their potential symptom associations with other disorders. METHODS: Familial Mediterranean fever patients were confirmed using Tel-Hashomer criteria and were tested for the 12 most common mutations using the familial Mediterranean fever StripAssay. A total of 100 samples were selected, and their MEFV gene exons and intron junctions were completely sequenced...

BACKGROUND: Eosinophilia in children is commonly caused by or parasitic, allergic and immunologic problems. OBJECTIVES: To study clinical and laboratory profile of patients with eosinophilia and to identify possible causes. METHOD: A prospective and descriptive study was conducted from January 2009- December 2011. All the cases of eosinophilia with symptoms and signs of single or multiple organ were included. The known cause of eosinophilia like allergy, asthma and drugs were excluded...