Polyserositis in children

Familial Mediterranean fever is a hereditary disease characterised by recurrent and self-terminated attacks of fever and polyserositis. An earlier study found that adult patients of Familial Mediterranean fever had an abnormally longer QT dispersion and corrected QT dispersion, markers for ventricular arrhythmogenicity. QT dispersion is a simple non-invasive arrhythmogenic marker that can be used to assess homogeneity of cardiac repolarisation; however, it has not been studied in children with Familial Mediterranean fever before...

Clinical data of 39 children with paragonimiasis treated in Chongqing Three Gorges Central Hospital during 2008-2010 were retrospectively analyzed. The cases aged from 3 to 10 years old, with 25 cases of polyserositis (64.1%), 14 cases of cerebral paragonimiasis (35.9%). Among the cases of polyserositis, all showed dyspnea,tachypnea and diminished respiration (100%). Other symptoms or signs included purulent pleurisy, orthopnea, restricted activity, distant heart sounds, purulent pericarditis, abdominal distension, and hepatomegaly...

Familial Mediterranean fever is an autosomal recessive genetic disorder characterized by recurrent febrile polyserositis, especially prevalent in individuals of Mediterranean descent. Familial Mediterranean fever can have nonspecific manifestations that mimic many common acquired disorders such as infections, acute appendicitis, cholecystitis, and arthritis, which can delay diagnosis for many years and subject patients to extensive evaluations and even unnecessary surgery. Untreated familial Mediterranean fever can result in serious complications such as end-stage renal disease and malabsorption secondary to amyloid deposition in the kidneys and digestive tract, male and female infertility, and growth retardation in children...

OBJECTIVE: Familial Mediterranean fever (FMF) is an inherited disease characterized by attacks of febrile polyserositis. In children, attacks of fever alone, or with headache and malaise, may precede other forms of attacks. Our objective was clinical and genetic characterization of FMF and its development in pediatric patients who first presented with attacks of fever alone. METHODS: Clinical characterization and MEFV genotype of all FMF patients < 16 years of age at disease onset and first presenting with attacks of fever alone were analyzed and compared for age, sex, and disease duration with matched FMF patients presenting with serositis at the onset of the disease...

AIM: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterised by recurrent episodes of fever, polyserositis and rash. The aim of this study was to determine the most common mutations and clinical features, and their relationships. METHODS: The medical records of 78 patients were evaluated retrospectively. All of the patients had been diagnosed with FMF according to Tel Hashomer criteria between January 2005 and May 2008 in general paediatric clinics of the School of Medicine at Gaziosmanpasa University...

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent attacks of fever and polyserositis. It is the most frequent periodic fever syndrome. In FMF, sterile peritonitis, pleuritis and arthritis are frequently seen in addition to recurrent febrile attacks. Skin and muscle involvement is less common. Here, we report four patients presented with skin lesions or myalgia. Most striking findings in those patients are the absence of other major criteria for FMF and dominancy of skin lesions or myalgia...

AIM: Familial Mediterranean fever (FMF) is the most frequent periodic syndrome characterised by recurrent attacks of polyserositis. However, recent studies revealed that there might be an ongoing subclinical inflammation between the attacks. As nitric oxide (NO) and adrenomedullin (AM) are both synthesised in the endothelium, and mediates many functions within immune system, we considered them to be an interesting target of investigation in FMF. METHODS: Fifteen children with FMF receiving regular colchicine, ranging in age from 3 to 16 years, were investigated in comparison with 15 healthy age- and sex-matched controls...

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The clinical features of two cases of Hereditary Recurrent Polyserositis HRP (Familial Mediterranean Fever) in related Yemeni children resident in Saudi Arabia are described. One presented with recurrent acute hepatitis, which has not been previously documented. These are the first cases of HRP reported in Arabs originating from the Arabian Peninsula.

9 children with collagenoses were first treated with AHLG "Dessau" in combination with corticosteroids and then with Imurek (azathioprine). The children seemed to be vitally endangered and suffered from panarteritis, dermatomyositis, polyserositis or corticosteroid-resistent systemic juvenile rheumatoid arthritis. As assessed by decrease of inflammation and clinical symptoms the treatment appeared in one case to be very good and in another good; in four other children it was good for a short period; in three children there was no effect at all...

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No abstract text is available yet for this article.

The clinical profile, course and complications of familial Mediterranean fever (recurrent hereditary polyserositis) seen in 88 children over a period of 11 years are presented. Forty eight children (55%) started their illness below the age of 5 years, and the mean age of onset was 4.9 years. Peritonitis occurred in 85% of children, arthritis in 50%, pleuritis in 33% and erysipelas-like lesions in 16%. Two children developed renal amyloidosis, and one third of the children were subjected to unnecessary operative surgery, reflecting the diagnostic difficulties...

A prospective study, with an attempted 24-month-post-treatment follow-up, of children with tuberculosis (TB) treated with short course chemotherapy (SCC) for 6 months was carried out because published experience of SCC in childhood TB was limited. All children in Port Moresby diagnosed as having TB between November, 1984, and November, 1986, entered the trial. Of the 639 children 165 (26%) were younger than 2 years old. Of these, 227 (35%) had extrapulmonary TB (peripheral lymph node, 110; central nervous system, 43; abdominal, 27; miliary, 16; bone and joint, 11; pleural, 11; polyserositis, 9)...

In a six-year study of 46 children with familial Mediterranean fever (recurrent hereditary polyserositis), 20 children (43 per cent) developed cutaneous manifestations. Ten children had 18 episodes of erysipelas-like erythema which proved to be specific for the disease. Twelve children (26 per cent) had 31 episodes of non-specific purpuric rash and six children (13 per cent) had nine episodes of Henoch-Schönlein purpura. Other manifestations included diffuse erythema of the face, and/or trunk, angioneurotic oedema, diffuse erythema of the palms and soles followed by mild desquamation of the skin, Raynaud's phenomenon and a subcutaneous nodule...

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Familial Mediterranean fever is a disorder characterized by recurrent fever and polyserositis. Continuous prophylactic colchicine therapy has been effective in suppressing attacks in affected adults. From 30 children with FMF, 14 were selected for colchicine therapy. Eight children continued prophylactic colchicine therapy for 29 months (mean) and experienced a marked decrease in the frequency of attacks. Six other children did not comply with the treatment regimen. Although no deleterious side effects were noted, the safety of long-term colchicine administration in childhood is unknown...