No abstract

The aim of the study was to determine whether the B-type natriuretic peptide (BNP) plasma level predicted the severity of bronchiolitis without additional pathology in children admitted to an emergency department (ED). A total of 232 children (mean age: 7.7±1.2 months [range: 1-23 months]) presenting to ED with isolated bronchiolitis and 32 age-and gender-matched control subjects were included in the study. BNP levels differed significantly among the control (8.5±1.1 pg/ml) and mild (27.7±2.6 pg/ml), moderate (51.4±0.5 pg/ml), and severe (106.8±4.8 pg/ml) bronchiolitis groups (p<0.001). Clinical severity score (p<0.001; OR: 2.524; 95% CI: 1.826-3.487) and plasma BNP level (p<0.001; OR: 2.231; 95% CI: 1.583-3.242) were independent risk factors for hospitalization. The length of hospital stay was significantly correlated with BNP level (p<0.001; r: 0.698). In conclusion, the plasma BNP level may be a potent biomarker predicting disease severity in ED.

Alparslan C, Kamit-Can F, Anıl AB, Olgaç-Dündar N, Çavuşoğlu D, Göç Z. Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature. Turk J Pediatr 2017; 59: 463-466. Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have been tried, exact treatment strategy is still undetermined. This is the first pediatric case of FIRES from Turkey which was successfully treated with intravenous immunoglobulin (IVIG). A previously healthy 8-year-old boy was referred to our hospital with a pre-diagnosis of status epilepticus and encephalitis. He presented with acute onset of convulsions and unconsciousness following fever and malaise lasting 7 days. On physical examination Glasgow coma scale was 12, his pupils were miotic. He had cafe-au-lait spots on his body. His fundus examination, cerebrospinal fluid findings and cranial magnetic resonance imaging did not reveal any abnormality. Results of comprehensive search for metabolic, toxicological, infectious and autoimmune etiologies were all negative. Generalized slowing was seen on the electroencephalography (EEG) of the patient indicating possible encephalopathy. The patient developed convulsive status epilepticus and was intubated on day 5. His seizures were controlled by continuous infusion of midazolam, thiopental and used for 4 days. Phenytoin, levetiracetam, topiramate were used simultaneously. IVIG was administered as an immunomodulator for refractory seizures on day-9. The patient was extubated on day 11. The diagnosis was made after a comprehensive negative search for central nervous system infection, autoimmune and metabolic diseases. At follow up it was learnt that he had had only two seizures in two years. Status epilepticus did not recur. Clinicians should keep in mind FIRES which is a diagnosis of exclusion especially in refractory status epilepticus. IVIG treatment could have a benefit in these patients.

Background and aims: Familial Mediterranean Fever (FMF) is an autosomal recessive disease most commonly seen in the Middle East and Mediterranean countries. There are few reported cases of FMF with Multiple sclerosis (MS). Here we report a child with FMF who developed MS to remind neurologic involvement of FMF although it is very rare. Methods: Case report A-9.5-year old girl was admitted to our emergency department because of extremity weakness and double vision. She was diagnosed with FMF and was started colchicine therapy 3 years ago. On physical examination vital signs were normal. Neurologic examination revealed ataxia, hyperactive deep tendon reflexes, extensor plantar responses bilaterally. On laboratory examination, the results of full blood count, peripheral smear, blood chemistry, C-reactive protein, erythrocyte sedimentation rate, coagulation tests were normal. Bacteriologic cultures, serological tests for viral infections, agglutination tests for salmonella-brucella were negative. Tests for connective tissue disorders were normal. Cerebrospinal fluid studies revealed high protein level (815 mg/dl (N:15-45 MG/DL)) and oligoclonal bands. Cranial and spinal MR imagings were consisted with MS with demyelinating plaques. She was started on intravenous methylprednisolone (30 mg/kg/d) therapy for five days continued with 1mg/kg/d po for three weeks. Neurologic findings were improved during treatment. She has been following with pediatric neurology and rheumatology departments. Conclusions: Central nervous system involvement in FMF consists of optic neuritis, aseptic meningitis and pseudotumor cerebri. FMF-MS coexistence is controversial but they are both recurrent diseases that show inflammatory reactions. FMF-MS coexistence is very rare and controversial but should be in mind.