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Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzyme system. This disease causes the disordered functioning of phagocytic cells. It is characterized by life-threatening and/or recurrent infections by bacteria and fungi. CGD has both an X-linked recessive (X-CGD) and autosomal recessive (AR-CGD) phenotypes. AR form have four subtypes including defects with one of these NADPH oxidase components (p22, p40, p47 and p67phox)., Objectives: To report the clinical and laboratory characteristics of seven CGD patients based on their genetic characteristics., Methods: Seven boys with CGD were reviewed based on clinical findings and genetic results. Dihydrorhodamine-1,2,3 (DHR) assay was used as a diagnostic test. Genetic analysis was conducted to establish moleculer diagnoses in all patients., Results: The age of diagnosis varied between 1.5 years and 15 years. The most frequent clinical presentation was pneumonia, and two patients had BCG-itis. Four patients had the AR-CGD phenotype, and three patients had the X-CGD phenotype. Severe invasive infections due to Aspergillus, Staphylococcus, and Serratia species were reported. Frequent lung and lymph node involvement was observed during follow-up of the cases., Conclusions: CGD is life-threatening disease that involves deep-seated infection. In our patients, the most commonly affected organs were the lungs and lymph nodes. Phagocytic disorders should be considered in cases of recurrent infectious diseases, invasive fungal diseases, BCG complications that are not self-limiting, unexplained lymphadenitis or osteomyelitis, and chronic inflammatory disorders.

Purpose: Tularemia is an infection caused by Francisella tularensis . Its diagnosis and treatment may be difficult in many cases. The aim of this study was to evaluate treatment modalities for pediatric tularemia patients who do not respond to medical treatment., Methods: A single-center, retrospective study was performed. A total of 19 children with oropharyngeal tularemia were included., Results: Before diagnosis, the duration of symptoms in patients was 32.15±17.8 days. The most common lymph node localization was the cervical chain. All patients received medical treatment (e.g., streptomycin, gentamicin, ciprofloxacin, and doxycycline). Patients who had been given streptomycin, gentamicin, or doxycycline as initial therapy for 10-14 days showed no response to treatment, and recovery was only achieved after administration of oral ciprofloxacin. Response to treatment was delayed in 5 patients who had been given ciprofloxacin as initial therapy. Surgical incision and drainage were performed in 9 patients (47.5%) who were unresponsive to medical treatment and were experiencing abcess formation and suppuration. Five patients (26.3%) underwent total mass excision, and 2 patients (10.5%) underwent fine-needle aspiration to reach a conclusive differential diagnosis and inform treatment., Conclusion: The causes of treatment failure in tularemia include delay in effective treatment and the development of suppurating lymph nodes., Competing Interests: Conflicts of interest: No potential conflict of interest relevant to this article was reported.

HSV 1 encephalitis is the most common cause of sporadic and focal viral encephalitis. Opercular syndrome is characterized by swallowing and speech difficulties which are associated with deterioration of voluntary control of face, pharynx, tongue and chewing muscles. It can be developed in patients with Herpes simplex encephalitis (HSE). Here, a twelve-year-old boy who was diagnosed with HSE and Opercular syndrome, is presented. The patient recovered without sequela as a result of 30 days of intravenous and 10 days of oral acyclovir treatment. It might be important as well, to personalize and elongate the treatment in terms of prognosis., (Copyright © 2016 King Saud Bin Abdulaziz University for Health Sciences. Published by Elsevier Ltd. All rights reserved.)

Staphylococcus aureus is a common cause of musculoskeletal infections. Panton-Valentine leukocidin (PVL) is an exotoxin produced by S. aureus and is an important virulence factor. PVL-positive S. aureus infections have been associated with soft tissue infections and necrotizing pneumonia. In this case report, the clinical course of a 13-year-old boy who was admitted with right hip pain and fever, diagnosed with a gluteal abscess and incidentally discovered multiple cavitary pulmonary lesions, and had a methicillin susceptible and PVL-producing S. aureus (MSSA) extracted from the abscess culture is presented. The related literature is also reviewed.

Brucellosis is a systemic infectious disease that leads to various clinical pictures and is still a significant health problem in Turkey. In this study, 52 pediatric patients diagnosed with brucellosis between January 2008 and December 2013 were examined. Clinical and laboratory findings, response to treatment, prognosis and complications were evaluated. Diagnosis of brucellosis was made based on a clinical picture compatible with the disease, together with standard tube agglutination test (SAT) positivity (1/160 or higer titer) or isolation of Brucella spp. in a sterile body fluid culture. The cases comprised 10 females and 42 males. In 75% of cases, there was a history of consumption of unpasteurized milk or dairy products. The most commonly seen symptoms and findings were fever (75%), arthralgia (54%), fatigue (19%), splenomegaly (44%), hepatomegaly (42%) and arthritis (19%). Atypical presentations were seen in one case of epidydymo-orchitis and three cases of bleeding of the nose and gums. In the laboratory examinations, anemia was determined in 56% of cases, leukopenia in 40% and thrombocytopenia in 27%. In blood cultures taken from 41 patients, Brucella spp. were isolated in 23 (56.1%). All patients recovered, and sequelae were seen only in a patient with osteoarthritis. In conclusion, although brucellosis leads to many different clinical pictures, a very good response to treatment can be obtained. If effective treatment cannot be implemented in time, the disease may become chronic, and complications and relapses may be encountered. Therefore, early diagnosis and treatment is of great importance.

The Bacille Calmette-Guérin (BCG) vaccine is used against tuberculosis. According to the vaccination program in Turkey, DaBT-Hib-IPV (diphtheriapertussis [acellular]-tetanus-haemophilus influenza type b-inactive poliovaccine), PVC13 (pneumoccocal conjugate vaccine) and BCG vaccines are given simultaneously to all 2-months-old infants, using different administration techniques. We report a 2-month-old infant who received an overdose and intramuscular BCG vaccine instead of DaBT-Hib-IPV. The patient was treated with isoniazid for six months and developed no complications during a oneyear follow-up period.

Psoas abscess associated with septic arthritis of the hip is unusual in infants. A 12-month-old infant presented with the complaints of fever, left hip pain and limp. Magnetic resonance imaging revealed left psoas abscess accompanied by septic arthritis of the hip. In this report, we present a case of psoas abscess with hip septic arthritis in an infant, and we describe the clinical and radiological findings and treatment of this case.

We aimed in this study to present the clinical findings in children with iliopsoas abscess (IPA) and to discuss the diagnosis and treatment. The files of five patients, hospitalized between August 2011 and June 2013 and monitored with a diagnosis of IPA, were reviewed retrospectively. Demographic characteristics, symptoms and signs, laboratory examinations, and diagnostic and treatment methods of the cases were evaluated. Two of the cases were females and three were males, and their ages ranged from 10 to 15 years. Before the diagnosis, the duration of symptoms in patients ranged from five days to one year. The primary symptoms included fever and difficulty in walking. One patient presented with septic shock and had a history of trauma as a predisposing factor. All patients except one had a higher erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) value. Psoas abscess was demonstrated by ultrasonography (USG), except in one patient. Four patients underwent percutaneous drainage of the abscess. The isolated microorganisms included Staphylococcus aureus, Mycobacterium tuberculosis, and Enterococcus faecalis. All the cases recovered without sequelae. Diagnosis of IPA in children is difficult, and many physicians are usually consulted before any diagnosis is made. IPA should be considered in the differential diagnosis in patients presenting with complaints of lower back, hip, groin and leg pain and difficulty in walking.

Pertussis is one of the leading causes of death that can be prevented by vaccination. More than 600,000 deaths from pertussis occur annually, with a disproportionate number appearing in unvaccinated infants. Pertussis is particularly troublesome because it does not necessarily present itself in its commonly known classical stages. Therefore, in very young and non-immunized children, the disease may have a fulminant process characterized by severe leukocytosis, neurologic involvement and serious cardiopulmonary failure that can be accompanied by pulmonary hypertension, persistent hypoxia and death. This article describes two infants with fulminant pertussis; they were admitted for acute respiratory failure and severe leukocytosis and ultimately died from multi-organ failure.

Background: In April 2009 a novel strain of human influenza A, identified as H1N1 virus, rapidly spread worldwide, and in early June 2009 the World Health Organization raised the pandemic alert level to phase 6. Herein we present the largest series of children who were hospitalized due to pandemic H1N1 infection in Turkey., Methods: We conducted a retrospective multicentre analysis of case records involving children hospitalized with influenza-like illness, in whom 2009 H1N1 influenza was diagnosed by reverse-transcriptase polymerase chain reaction assay, at 17 different tertiary hospitals., Results: A total of 821 children with 2009 pandemic H1N1 were hospitalized. The majority of admitted children (56.9%) were younger than 5 y of age. Three hundred and seventy-six children (45.8%) had 1 or more pre-existing conditions. Respiratory complications including wheezing, pneumonia, pneumothorax, pneumomediastinum, and hypoxemia were seen in 272 (33.2%) children. Ninety of the patients (11.0%) were admitted or transferred to the paediatric intensive care units (PICU) and 52 (6.3%) received mechanical ventilation. Thirty-five children (4.3%) died. The mortality rate did not differ between age groups. Of the patients who died, 25.7% were healthy before the H1N1 virus infection. However, the death rate was significantly higher in patients with malignancy, chronic neurological disease, immunosuppressive therapy, at least 1 pre-existing condition, and respiratory complications. The most common causes of mortality were pneumonia and sepsis., Conclusions: In Turkey, 2009 H1N1 infection caused high mortality and PICU admission due to severe respiratory illness and complications, especially in children with an underlying condition.