A case of perineal ecthyma gangrenosum.

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.

Gene polymorphisms of adducin GLY460TRP, ACE I/D, AND AGT M235T in pediatric hypertension patients.

BACKGROUND: Hypertension is a major global public health problem that affects both pediatric and adult populations. ACE I/D, AGT M235T, and ADD Gly460Trp polymorphisms are thought to be associated with primary hypertension. In the present study, we examined the frequency of these polymorphisms in a pediatric population with secondary hypertension. MATERIAL AND METHODS: Included in the study were 58 hypertensive and 58 normotensive pediatric patients. ACE I/D and AGT M235T polymorphisms are determined by conventional PCR; ADD Gly460Trp polymorphism was investigated using PCR amplification of genomic DNA. RESULTS: There were significant differences between the control group and pediatric hypertensive group in terms of ACE I/D (P<0.05) and AGT M235T (P<0.05) polymorphisms, but there were no differences in ADD Gly460Trp (P>0.05) polymorphism. CONCLUSIONS: We suggest that RAS gene polymorphisms (ACE-I/D, AGT M235T) are significantly associated with susceptibility to diseases that lead to secondary hypertension.

Bacillus Calmette-Guérin is a preventive factor in mortality of childhood tuberculous meningitis.

OBJECTIVES: Studies have been done that have focused on the efficacy of bacillus Calmette-Guérin (BCG) vaccination in the prevention of cases of childhood tuberculous meningitis (TBM). However the efficacy of the vaccination in the prevention of mortality has not been sufficiently evaluated. This study aimed to determine the main features of TBM cases in childhood and to evaluate the factors related to mortality, proving the protective effect of BCG vaccination in childhood TBM. METHODS: In a retrospective approach, all consecutive cases of TBM in children that occurred between 1997 and 2005, at Dicle University Hospital, were studied. The following data were evaluated: demographic aspects, admission symptoms, radiology and laboratory findings, BCG vaccination status, tuberculin skin test (TST) positivity, and mortality rates. RESULTS: In total, 172 cases of childhood TBM were evaluated (mean age 53.3±55.7 months; 109 boys (63.4%)). The majority of these cases (70.4%) had typical TBM symptoms on admission. BCG vaccination data were available for 152 (88.4%) cases and 29 of them (19.1%) were positive. The TST was performed for 143 patients (83.1%) and 28 (19.6%) were found positive. Hydrocephalus was identified in 118 patients (68.6%) on computed tomography examination. A shunt was placed in 79 cases (45.9%). In total, 24 patients (14.0%) died in the hospital. TST negativity was a significant factor for mortality (p=0.012). BCG positivity was found to be a preventive factor from mortality (p=0.05). CONCLUSIONS: 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 MEFV mutations and their clinical correlations in children with familial Mediterranean fever in southeast Turkey.

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 evaluation of children with prolonged cough accompanied by American College of Chest Physicians guidelines.

INTRODUCTION: In children, coughs lasting longer than 4 weeks are considered to be chronic, with etiological factors varying widely. OBJECTIVE: This study included children with chronic cough who were followed for 1 year. All cases were evaluated with the guidance of the American College of Chest Physicians (ACCP), and etiological factors were analyzed. METHODS: The study included 270 children between the ages of 2 months and 14 years. Their presenting symptoms, physical examination findings and laboratory data were recorded. All patients underwent laboratory tests including direct chest X-ray and spirometric measurements. Several patients required additional advanced examinations such as a sweat test, determination of immunoglobulin levels and bronchoscopy. Patients were reevaluated according to ACCP recommendations, and etiological factors were investigated. RESULTS: The total of 270 patients were included in the study included 43.3% (n=153) females with a mean age of 6.5 ± 2.3 years (7 months to 17 years). After a 1-year follow-up of patients, we determined that the most common etiologic factors were asthma (27%), asthma-like syndrome (15.5%) and gastroesophageal reflux (10%). Other etiological factors included upper airway cough syndrome and protracted bronchitis, respectively. CONCLUSION: 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.

Use of complementary and alternative medicine in children who have no chronic disease.

AIM: In this study, we aimed to evaluate complementary and alternative methods of medicine, determine the frequencies of usage of these methods and investigate the factors which have an impact on their usage. MATERIAL AND METHODS: The study was conducted between October, the 15(th) and November, the 15(th) 2012. Approval was obtained from the local ethics committee (08.10.2012/732). The study form prepared was applied to the mothers of the children who presented to our general outpatient clinic and had no chronic disease. The data were recorded in SPSS for Windows v.16 program. Descriptive statistics, chi-square, Mann-Whitney U and logistic regression analyses were applied. A p value of <0.05 was considered significant. RESULTS: Two hundred and six children were included in the study. Complementary and alternative medicine was used in 83% of the patients. Wearing an evil eye (45%) bead and prayer (35%) to protect from the evil eye were the most commonly used methods. The most common medical conditions which caused to use of complementary and alternative medicine included anemia, diarrhea, constipation and cough. Belief-based application were being used in 73% of the patients and herbal methods were being used in 57%. In 18% of the patients, interventional methods including cutting had been performed. No difference was found between the patients in whom these methods were used and not used in terms of familial income, education levels of the parents, family type and residence (p>0.05). CONCLUSIONS: 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.

Is Chryseobacterium indologenes a shunt-lover bacterium? A case report and review of the literature.

Chryseobacterium indologenes is a non-fermentative Gram-negative bacillus formerly belonging to the Flavobacterium genus. It is widely found in water and soil, also on wet surfaces of the hospital environment. It rarely causes infections and is usually associated with altered immune status or indwelling devices. We present a case of ventriculoperitoneal shunt infection caused by C. indologenes in a premature pediatric patient. A six-month-old male infant with congenital hydrocephalus and ventriculoperitoneal shunt was admitted with complaints of irritability, high fever and projectile vomiting. He was diagnosed as suffering from meningitis based on the clinical symptoms and laboratory findings of cerebrospinal fluid. The ventriculoperitoneal shunt was externalized and cerebrospinal fluid samples were sent for bacterial cultures. The isolated bacterium was identified as C. indologenes by conventional methods and the BD Phoenix™ 100 (Becton Dickinson, MD, USA) fully automated microbiology system. Antimicrobial susceptibility testing was performed by the microdilution method and Kirby-Bauer's disk diffusion method according to the Clinical and Laboratory Standards Institute guidelines. The isolate was found susceptible to ciprofloxacin, levofloxacin, cefoperazone and trimethoprim-sulfamethoxazole, while it was resistant to amikacin, aztreonam, cefepime, ceftazidime, gentamicin, imipenem and ticarcillin-clavulanic acid. The treatment was started with trimethoprim-sulfamethoxazole and cefoperazone-sulbactam The ventriculoperitoneal shunt was then removed. The patient was fully healed after two weeks and discharged. Central nervous system infection is a rare form of C. indologenes infections. The case presented herein may make a useful contribution to the existing literature.

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association.

Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy has increased, and some previously unobserved disease associations are now seen in patients with CF. It is important to follow patients with CF for possible abnormalities that may accompany CF. In this paper, we present two rare cases of CF accompanied by nephrotic syndrome.

The clinical findings and prophylactic treatment in children with factor X deficiency.

Factor X (FX) is the component of both extrinsic and intrinsic coagulation cascade and is the first enzyme of the common pathway which results in thrombus. Congenital FX deficiency (FXD) is an extremely rare coagulation defect. In this study, we aimed to investigate the clinical and laboratory data of the patients diagnosed with FXD. The files of the 15 patients (7 female, 8 male) diagnosed and followed up for FXD within the last 4 years were evaluated retrospectively. The mean age of the patients was 29 months (min-max:1-144 months). The most presenting complaints were easy bruisability (n = 8; 53%) and epistaxis (n = 8; 53%). FX levels were <1% in six patients, 1-5% in four patients, and >5% in five patients. Heparin added-Protrombin Complex was used for prophlaxy (n = 11; 73%). Any treatment-related complication was not observed. Heparin-added PCC can be used safely for effective prophlaxy. We suggest that family history is important when considering prophlaxy and in patients with life-threatening bleeding or with FXD sibling the prophlaxy should be introduced in the early course.

[Papulonecrotic tuberculid: case report]. / Tubercúlide papulonecrótica. Caso clínico.

A 12-year-old girl was seen with widespread millimetric papules which resolved leaving varioliform scars. Tuberculin test was strongly reactive. Underlying pulmonary tuberculosis was diagnosed. The patient responded to antituberculous treatment. Papulonecrotic tuberculid is a skin eruption thought to be due to hypersensitivity to an underlying focus of tuberculosis and is an indicator of good immunologic status.

Tubercúlide papulonecrótica: Caso clínico / Papulonecrotic tuberculid: Case report

Una niña de 12 años fue atendida por presentar pápulas milimétricas diseminadas que involucionaron hacia escaras varioliformes. La prueba de tuberculina fue fuertemente reactiva. Se diagnosticó una tuberculosis pulmonar subyacente y la paciente respondió al tratamiento antituberculoso. La tubercúlide papulonecrótica es una erupción cutánea que se cree debida a hipersensibilidad ante un foco subyacente de tuberculosis y sería un indicador de buen estado inmunitario.

A 12-year-old girl was seen with widespread millimetric papules which resolved leaving varioliform scars. Tuberculin test was strongly reactive. Underlying pulmonary tuberculosis was diagnosed. The patient responded to antituberculous treatment. Papulonecrotic tuberculid is a skin eruption thought to be due to hypersensitivity to an underlying focus of tuberculosis and is an indicator of good immunologic status.

Antioxidant enzyme activities, lipid peroxidation, and total antioxidant status in children with Henoch-Schönlein purpura.

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.

Neutrophil activation, protein oxidation and ceruloplasmin levels in children with Henoch-Schönlein purpura.

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.