Objectives: To evaluate the responsiveness of children with juvenile idiopathic arthritis (JIA) to hepatitis B vaccination and to determine the most useful vaccination schedule. Methods: 39 children with JIA were enrolled in the study; all were in remission and negative to serological testing for hepatitis B surface antigen (HbsAg). The control group consisted of 41 healthy children. There were two different vaccination schedules: group I was vaccinated at 0, 1, and 3 months; group II was vaccinated at 0, 1, and 6 months. Positive responsiveness to the vaccine was defined as an anti-hepatitis B antibody titre above 10 mIU/ml. Results: All the children except one with systemic JIA developed an antibody response. None of the JIA patients experienced a flare up or clinical deterioration related to the vaccination. The antibody levels in children with JIA were significantly lower than in the healthy controls. Comparison of the antibody levels between the two vaccination schedules showed no statistical difference in the controls; in the JIA subjects the group II schedule resulted in a trend to a greater response than the group I schedule (p,0.07). Vaccine responsiveness was not influenced by either methotrexate or prednisolone treatment. Conclusions: Children with JIA had an adequate response to hepatitis B vaccination and the response was not affected by immunosuppressive treatment. A vaccination schedule at 0, 1, and 6 months seems to be preferable to 0, 1, and 3 months.

Nicolau syndrome (livedoid dermatitis) is a very rare complication of intramuscular injections and manifests as excruciating pain immediately after injection. We describe a 3-year-old boy with diagnosis of Nicolau syndrome after intramuscular benzathine penicillin injection to the midanterior part of the left thigh. He was treated with hyberbaric oxygen and pentoxyphilline in addition to supportive treatment and recovered with no sequelae.

An acute complaint of abdominal pain was usually attributed to a self-limited disease. However, the percentage of surgical etiology is not negligible.

Continious renal replacement therapy (CRRT) is a well recognizied treatment of choice in acute renal failure, however CRRT became a preferred treatment of metabolic emergencies with high leucine and ammonia levels like Maple syrup urine disease (MSUD). MSUD is a rare metabolic disorder caused by deficiency in the activity of the branched-chain a-ketoacid dehydrogenase complex. The toxic accumulation of branched chain amino acids during acute metabolic decompensation is associated with the appearance of permanent neurological symptoms. Four patients were admitted to our pediatric intensive care department with complains of poor feeding, vomitting, irratibility and coma. Physical examination of the neonates were similar having stupor, hypotonia and depressed newborn reflexes. The leucine levels were between 930-4400 μmol/L. The diagnosis of MSUD was confirmed in all four. They were treated successfully with high flow CRRT having the rates were between 4120 ml/h/1.73m2 and 9830 ml/h/1.73m2. Early treatment is essential to prevent neurotoxicity and death. CRRT is a choice of treatment in metabolic crisis of MSUD. Herein, we report the successful treatment of acute metabolic decompensation of MSUD with CRRT in 4 neonates.

This study was designed to determine the relationship of dimensions, wall thickness and function of the left ventricle with diabetes duration, fasting blood glucose, lipid profile, beta-OH-butyrate, free fatty acids (FFA) and carnitine levels in children and adolescents with type 1 diabetes mellitus (DM1) who had no cardiovascular complications. Thirty-five patients with DM1 (18 F/17 M, mean age: 12.0 years) and age matched control children (n = 24) were enrolled in the study. Patients with DM1 were subdivided into Group I (mean DM1 duration 3.5 years, n = 14), and Group II (mean DM1 duration 8.2 years, n = 21). Dimensions, wall thickness and systolic functions of the left ventricle were normal in all patients with DM1. Diastolic functions were normal in Group I. In Group II, peak A wave velocity (AVEL) (p = 0.004), velocity-time integral of A wave (AVTI) (p = 0.007) and isovolumetric relaxation time corrected by heart rate (cIVRT) (p = 0.048) were high, and peak E wave velocity (EVEL) and velocity-time integral of E wave (EVTI) were normal. E/A (p < 0.0001) and EVTI/AVTI (p = 0.001) were low in this group. In Group I, systolic and diastolic blood pressure, HDL-cholesterol and FFA values were normal; total cholesterol (p = 0.047), LDL-cholesterol (p = 0.017), beta-OH-butyrate (p = 0.003), and acetyl carnitine (p = 0.006) levels were high. In Group II, diastolic blood pressure (p = 0.008), total cholesterol (p < 0.0001) and LDL-cholesterol (p < 0.0001) were increased; and total carnitine (p = 0.019), free carnitine (p = 0.002) and HDL-cholesterol (p = 0.039) were decreased. Correlations were detected between total carnitine and AVEL and HR; free carnitine and AVEL, E/A and HR; HbA1c and EVTI/AVTI and cIVRT; LDL-cholesterol and E/A, EVTI/AVTI ratios and cIVRT; HDL-cholesterol and AVEL; FFA and LVDD, IVSD, LVPWD, LVmass and CO; metabolic parameters and DM1 duration and echocardiographic findings such as AVEL, EVEL, EVTI, VmaxAV and CO. In conclusion, left ventricular dimensions, wall thickness and systolic functions were normal in children and adolescents with DM1 who had no obvious cardiovascular complications. Left ventricular diastolic functions were abnormal in patients of Group II. Left ventricular diastolic function abnormalities were associated with glycemic control, free and total carnitine, and LDL- and HDL-cholesterol levels.

Background and objective: Severe sepsis and septic shock are life-threatening organ dysfunctions and causes of death in critically ill patients. The therapeutic goal of the management of sepsis is restoring balance to the immune system and fluid balance. Continuous renal replacement therapy (CRRT) is recommended in septic patients, and it may improve outcomes in patients with severe sepsis or septic shock. Therapeutic plasma exchange (TPE) is another extracorporeal procedure that can improve organ function by decreasing inflammatory and anti-fibrinolytic mediators and correcting haemostasis by replenishing anticoagulant proteins. However, research about sepsis and CRRT and TPE in children has been insufficient and incomplete. Therefore, we investigated the reliability and efficacy of extracorporeal therapies in paediatric patients with severe sepsis or septic shock. Materials and methods: We performed a multicentre retrospective study using data from all patients aged <18 years who were admitted to two paediatric intensive care units. Demographic data and reason for hospitalization were recorded. In addition, vital signs, haemogram parameters, and biochemistry results were recorded at 0 h and after 24 h of CRRT. Patients were compared according to whether they underwent CRRT or TPE; mortality between the two treatment groups was also compared. Results: Between January 2014 and April 2019, 168 septic patients were enrolled in the present study. Of them, 47 (27.9%) patients underwent CRRT and 24 underwent TPE. In patients with severe sepsis, the requirement for CRRT was statistically associated with mortality (p < 0.001). In contrast, the requirement for TPE was not associated with mortality (p = 0.124). Conclusion: Our findings revealed that the requirement for CRRT in patients with severe sepsis is predictive of increased mortality. CRRT and TPE can be useful techniques in critically ill children with severe sepsis. However, our results did not show a decrease of mortality with CRRT and TPE.

Bacillus cereus can cause serious, life-threatening, systemic infections in immunocompromised patients. The ability of microorganism to form biofilm on biomedical devices can be responsible for catheter-related bloodstream infections. Other manifestations of severe disease are meningitis, endocarditis, osteomyelitis, and surgical and traumatic wound infections. The most common feature in true bacteremia caused by Bacillus is the presence of an intravascular catheter. Herein, we report a case of catheter-related bacteremia caused by B. cereus in a patient with propionic acidemia.

Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1:1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture. (Türk Ped Arş 2014; 49: 247-9)