The economic burden of severe asthma in children: a comprehensive study.

OBJECTIVE: The economic burden of severe asthma (SA) in children is poorly described. We aimed to determine the healthcare costs of SA in children for the French national health insurance (NHI). METHODS: Children (6-18 years of age) with physician-confirmed diagnoses of SA or non-SA (NSA) were identified. Direct and indirect expenditures related to asthma and associated co-morbidities in the previous six months were determined, based on a physician-guided parental questionnaire and confirmed by medical records. The costs for the French NHI were assessed per child over a six month period. RESULTS: Data from 74 children, including 48 with SA (22 requiring omalizumab) and 26 with NSA, were analyzed. The global cost of SA was €3,982 per child over a six-month period, including €3,821 direct costs and €161.9 indirect costs. The global cost was €6,716 (4,220) for those requiring omalizumab vs. €1,669 (3,108) for those who did not (p < 0.01). For children with SA, 65% of direct costs were attributed to medication. Among those on omalizumab, such treatment accounted for 93% of medication costs. The global cost was 10 times higher for children with SA than those with NSA (€3,982 (4,422) vs. €363.2 (352.6), p < 0.01), and 20 times higher for children with SA on omalizumab than those with NSA (p < 0.01). CONCLUSION: The economic burden of SA in children for the French NHI is substantial and mainly driven by the most severe children requiring biologics.

Familial Mediterranean fever in childhood: a single-center experience.

The aim of this study is to present demographic and clinical features, MEFV mutation variations, and treatment response of a large number of pediatric familial Mediterranean fever (FMF) patients from a single tertiary centre. Moreover, we aimed to investigate the current outcome of FMF, namely frequency of amyloidosis in children with FMF. We evaluated 708 FMF patients who were followed up in our clinic and who were under colchicine treatment for at least 6 months. The data were recorded from patient records and also verified by negotiations with patients and parents. The male/female proportion of the cohort was 1.05/1 (n = 362/346). Abdominal pain (89.5%, n = 634) was the most common manifestation of FMF episodes, followed by fever (88.8%, n = 629) and arthritis (40.7%, n = 288). However, arthritis in 23 (8%) of the 288 cases was not self-limited; and they subsequently diagnosed with juvenile idiopathic arthritis in addition to FMF. Homozygote or heterozygote M694V mutation was more frequent in patients with arthritis (63.2%) and chronic arthritis (69.6%) than the whole cohort (53.8%). Erythrocyte sedimentation rate and CRP level were in high levels even during attack-free period in 13.9% (n = 97/697) and 11% (n = 78/670) of the patients, respectively. Proteinuria was found in ten patients (1.4%). Amyloidosis was confirmed by renal biopsy in only two of these cases who were homozygous for M694V and compound heterozygous for M694V/M680I. 47 (6.6%) subjects were considered as colchicine resistant. Homozygote M694V mutation was the most frequent mutation in those resistant cases (63.8%, n = 30), followed by compound heterozygote mutation of M694V/M680I (6.3%, n = 3). Homozygous M694V mutation are still the most frequent mutation and associated with the most severe clinical picture and the worst outcome in Turkish children. M694V genotype seems to be more frequently associated with arthritis as well as with chronic arthritis than other genotypes. Recurrence of FMF episodes as well as amyloidosis could only be managed via strict compliance to colchicine treatment. Frequency of amyloidosis significantly decreased compared to the previous studies. A favorable outcome could be obtained with the anti IL-1 in colchicine-resistant FMF patients.

Comparison of familial Mediterranean fever and juvenile idiopathic arthritis patients according to family origin.

AIM: Familial Mediterranean fever is an inherited condition that is more prevalent in some regions of Turkey. Juvenile idiopathic arthritis is the most common chronic arthritis of childhood. There is lack of studies on the frequency of mentioned conditions across different regions of Turkey. We aimed to compare the Familial Mediterranean fever and juvenile idiopathic arthritis patients according to their family origin. MATERIAL AND METHODS: Patients with diagnosis of familial Mediterranean fever and juvenile idiopathic arthritis followed up at the Division of Pediatric Rheumatology were assessed. Data regarding the family origin of patients were noted from their medical records. Both groups were compared according to their origins. RESULTS: A total of 704 patients with Familial Mediterranean fever, 204 patients with juvenile idiopathic arthritis were enrolled. The main age of the patients at the study time was 12.3±4.4 years and 12.1±4.8 years, respectively. The frequency of familial Mediterranean fever was significantly higher in subjects with the origin of Black Sea and Central Anatolia regions (z score 2.69, and 3.69, respectively). Juvenile idiopathic arthritis was significantly more common in subjects from Marmara and Southeastern Anatolia regions (z score -4.11 and -2.54, respectively). CONCLUSION: The familial Mediterranean fever is more common among subjects from the Black Sea and Central Anatolia regions of Turkey, especially Kastamonu, Sivas and Tokat provinces. Whereas, patients with juvenile idiopathic arthritis more commonly originate from Marmara and Southeastern Anatolia regions of Turkey.

Achromobacter causing a thrombophlebitis and osteomyelitis combination: a rare cause.

Achromobacter xylosoxidans is a Gram-negative, aerobic bacillus, present in normal human flora of the skin and gastrointestinal tract. Infections due to Achromobacter are infrequent and have mostly been reported in immunocompromised patients. Rarely, however, the microorganism can cause soft tissue infections even in healthy subjects with a history of trauma. We report thrombophlebitis complicated with osteomyelitis secondary to Achromobacter in a 15-year-old girl with a history of purulent discharge from the ankle due to local trauma caused by tight fitting shoes.