Havva Evrengül

Could pentraxin-3 be a new marker for subclinical inflammation in familial Mediterranean fever?

Pentraxin-3 (PTX-3) is a long pentraxin that is structurally related to the short pentraxins as C-reactive protein (CRP). It is known to play an important role in innate immunity and inflammatory regulation. CRP and serum amyloid A (SAA) are sensitive and reliable markers of inflammation in FMF attack as well as chronic and subclinical inflammation during attack-free period. To date, there is no information about PTX-3 in FMF inflammation.

Deteriorated Systolic Blood Pressure Recovery and Heart Rate Recovery After Graded Exercise in Children With Familial Mediterranean Fever

Objectives This study aims to investigate if cardiac involvement may occur in children with familial Mediterranean fever (FMF) without cardiovascular symptoms by using heart rate recovery (HRR) and systolic blood pressure recovery (SBPR) parameters. Patients and methods A total of 50 FMF patients (26 males, 24 females; mean age 151±33.4 month; range 60 to 216 month) and 30 healthy controls (18 males, 12 females; mean age 143±43.9 month; range 84 to 228 month) were included in the study. All patients were evaluated by echocardiography. All patients underwent a maximal graded exercise stress test. HRR and SBPR parameters were calculated. Results There was a significant decrease in HRR1 value in FMF group (p=0.03). SBPR1 and SPBR2 values were higher in FMF group compared to control group (0.96±0.12 vs 0.88±0.12 and 0.95±0.09 vs 0.91±0.11, respectively); and the high SBPR1 value was statistically significant (p=0.02). FMF presence had a negative correlation with HRR1 (r= -0.26, p=0.03) and a positive correlation with SBPR1 (r=0.29, p=0.02). There was a negative correlation of M694V homozygous mutation with HRR1 and HRR2 values (r= -0.43, p=0.004, r=-0.42, p=0.005). Conclusion Cardiac involvement may occur in FMF patients without cardiovascular symptoms. Impaired SBPR and decreased HRR response may indicate increased cardiovascular risk in these patients despite normal exercise stress test results.

Neonatal Bartter syndrome with cholelithiasis and hydrocephalus: Rare association

Neonatal Bartter syndrome (NBS) is a rare autosomal recessive renal tubular disorder. This disease is characterized by hypokalemia, hypochloremia, and metabolic alkalosis that is often associated with failure to thrive and recurrent episodes of dehydration. The combination of BS and cholelithiasis in an infant is very rare. Herein, we report a premature male infant with NBS who developed cholelithiasis and hydrocephalus on clinical follow up. We recommend that periodic routine hepatobiliary ultrasonograpic screening for cholelithiasis should be performed in patients with NBS.

Quality of life in children with familial Mediterranean fever

Method A prospective cross-sectional study was conducted between September 2013 and September 2014. A total of 70 consecutive children with FMF who were diagnosed according to the Tel-Hashomer and Yalcinkaya criteria during the attack free period and 70 healthy children who were matched in terms of age and sex were enrolled. The Pediatric Quality of Life Inventory 4.0 (PedsQLTM 4.0), Child Depression Inventory (CDI) and Screen for Child Anxiety and Related Disorders (SCARED) were used for the psychosocial assessment.

Eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome) without respiratory symptoms in a boy

Eosinophilic granulomatosis with polyangiitis or Churg-Strauss Syndrome (CSS) is rare in children. It is characterized by eosinophilia, extravascular necrotizing granuloma, and eosinophilic infiltration of multiple organs particularly lungs, but may also involve the gastrointestinal tract, heart, and the kidneys.

Intracardiac thrombus in a child with behçet’s disease

Behcet’s disease (BD) is recognized as a systemic vasculitis involving both arteries and veins of any size and characterized by recurrent oral aphthous ulcers, genital ulcers, and uveitis and skin lesions. The mean age of onset is in the fourth decade.