The aim of this study was to determine the Mediterranean fever (MEFV) gene mutations and their clinical correlations in children with familial Mediterranean fever (FMF) in southeast Turkey. Clinical and laboratory characteristics of 147 (65 males, 82 females) consecutive children with FMF having a positive MEFV gene mutation were prospectively investigated. Patients with negative MEFV gene mutations or atypical FMF presentations and those from other regions of the country were excluded. Clinical manifestations and disease severity scores were recorded. The six most frequent MEFV mutations including M694V, V726A, R726H, P369S, E148Q and P369S were investigated by a reverse hybridization test method. The median age of study group was 9.0 years, median age at diagnosis was 7.8 years, median age at disease onset was 5.0 years, and median follow-up duration was 4.0 years. A positive family history of FMF and parent-to-offspring transmission was found in 58.5 and 42.2 % of families, respectively. The frequencies of independent alleles, with decreasing order, were E148Q (30.7 %), M694V (26.0 %), R761H (13.5 %), V726A (13.0 %), P369S (10.5 %) and M680I (6.3 %) in FMF patients. The M694V subgroup had higher mean disease severity score and longer attack duration compared with E148Q and other mutations subgroups (p < 0.05). Two patients with amyloidosis had the M694V homozygote genotype. In conclusion contrast to other regions and many other ethnicities of the world, the most frequent MEFV gene mutation was E148Q in southeast Turkey. The M694V mutation frequency was lower, and disease severity was relatively mild in FMF children of this region.

The use of a standardized clinical approach such as the ACCP increases the possibility for fast and accurate diagnosis during the treatment of children with chronic cough, and the use of these guidelines should be required.

Complementary and alternative methods of medicine are applied substantially frequently in children. Physicians should have information about these methods in terms of their effects and side effects and warn families and especially mothers about potential risks.

The aim of this study was to assess the role of oxidative stress in the pathogenesis of Henoch-Schönlein purpura (HSP) vasculitis. The activities of catalase (CAT), arylesterase (ARYL), and paraoxonase (PON) as antioxidant enzymes and serum malondialdehyde (MDA) level as an indicator of lipid peroxidation, together with total antioxidant status (TAS), were measured in 29 children with HSP (mean age 9.3 +/- 2.7 years), both at the onset of the disease and at the remission period and in matched controls. Active-stage HSP had significantly higher MDA level (15.5 +/- 7.3 vs 7.8 +/- 3.9 nmol/l, respectively, P < 0.001) and lower TAS (524 +/- 122 vs 699 +/- 122 mumol Trolox Equiv/l, P < 0.001), PON (97 +/- 47 vs 136 +/- 95 U/l, P = 0.042), ARYL (158 +/- 39 vs 212 +/- 52 U/l, P < 0.001), and CAT (50 +/- 27 vs 69 +/- 20 U/l, P = 0.002) activities compared with the control subjects. Although CAT (P > 0.05) and PON (P > 0.05) activities were found to be similar between active and remission stages of HSP, the active stage of the disease had significantly lower ARYL (P = 0.011) and TAS (P = 0.006) and higher MDA (P < 0.001) values compared with remission period. Significant positive correlations were found between CAT and MDA (r = 0.433, P = 0.019) and between CAT and C-reactive protein (r = 0.386, P = 0.035) in the active stage of HSP. No significant differences were detected in oxidant/antioxidant parameters between patients with or without renal, gastrointestinal, or joint involvement (P > 0.05). Increased oxidative stress and lipid peroxidation may play important roles in the pathogenesis of HSP vasculitis. Antioxidant therapeutic interventions in long-lasting vasculitis and risk of atherosclerosis secondary to increased oxidant stress remain to be investigated.

Ecthyma gangrenosum is a skin lesion associated with Pseudomonas aeruginosa. A previously healthy one-year-old boy who had been diagnosed with varicella 10 days ago was admitted to our hospital with complaints of diarrhea, green ear discharge and new lesions in the diaper area. Intravenous meropenem and amikacin had been previously initiated. Physical examination revealed greenish, well bordered necrotic ulcers on both gluteal areas and the perianal area. Pseudomonas aeruginosa grew in wound culture. A colostomy was opened due to recalcitrant diarrhea and ulcers. The patient remitted in one month and was discharged. T lymphocyte subgroups and immunoglobulins were found to be normal on immunologic evaluation. We presented this case to draw attention to the fact that ecthyma gangrenosum due to Pseudomonas aeruginosa may occur in the diaper area and that sepsis due to Pseudomonas should be investigated. (Turk Pediatri Ars 2016; 51: 46-8)

BCG vaccination is effective in the prevention of TBM-associated mortality in childhood. TST negativity may be a sign of a poor prognosis in TBM cases.

The aim of this study was to investigate the role of neutrophil activation, protein oxidation and ceruloplasmin (CLP) in the pathogenesis of Henoch-Schönlein purpura (HSP), which has not been investigated previously. Serum activities of myeloperoxidase (MPO) and arylesterase (ARYL) and levels of free thiol groups, CLP and total oxidant status (TOS) were measured in 29 children with HSP at the onset of the disease and during remission in comparison with 30 healthy subjects. Patients at active stage had significantly higher MPO activity (391+/-277 vs. 155+/-154 U/l, P<0.001), higher CLP (832+/-120 vs. 682+/-114 mg/dl, P<0.001) and TOS values (20.7+/-11.8 vs. 7.5+/-2.8 micromol H2O2/l, P<0.001) than the controls, respectively. Patients had significantly lower ARYL activity (158x10(3)+/-39x10(3) vs. 187x10(3)+/-46x10(3) U/l, P<0.001) and lower free thiol levels (234+/-48 vs. 279+/-26 micromol/l, P<0.001) than the controls, respectively. Significantly positive correlations were found between TOS and MPO (r=0.437, P=0.018) and TOS and CLP (r=0.409, P=0.028) at disease onset, whereas a negative correlation was found between MPO and thiol (r=-0.597, P=0.001) during remission. In conclusion, protein oxidation and neutrophil activation may play important roles in the pathogenesis of HSP. Further research is required to understand the potential linkage between oxidant stress and complications and to develop therapeutic strategies in HSP.

Complete and early resection of bronchiectasis provides a successful outcome. Duration of symptoms and timely intervention have a major impact on the management and prognosis.