Risk Factors and Clinical Determinants in Bronchiolitis of Infancy.

OBJECTIVES: The aims of this study was to demonstrate the viral pathogens, to evaluate the clinical prognosis, risk factors for recurrence, severity of acute viral bronchiolitis episodes among pediatric patients. MATERIALS AND METHODS: Our study included 101 children aged between 2 months and 2 years diagnosed with clinical bronchiolitis between September 2011 and April 2012. The demographics and clinical, laboratory, and radiological results of the patients were recorded. Nasopharyngeal swab samples were collected and analyzed through polymerase chain reaction (PCR) method. The patients were followed up for at least one year for new episodes, existence of wheezing, frequency of pulmonary infections, and progression of asthma. RESULTS: In half of the patients, determinants were indicated through the PCR method, with the most frequent being respiratory syncytial virus (44%). The frequency of bronchiolitis was higher in prematures (p<0.005). There was a relationship between crowded family structure and the existence of wheezing (p=0.003), increased recurrence (p=0.014), and need for inhaler treatment (p=0.014). The frequency was higher in patients living in urban cities (p<0.001), in houses with heating stoves (p=0.001), and in houses with smokers (p=0.001). Patients living in houses with heating stoves had more severe episodes (p=0.018). Recurrent wheezing and the need for regular inhaler usage were positively correlated with high API scores (p=0.008 and p=0.002, respectively). CONCLUSION: Prematurity, exposure to smoking, living in a crowded house with heating stoves, and an urban life are the risk factors for frequent bronchiolitis. The API can be used to predict the recurrence of bronchiolitis.

Postinfectious bronchiolitis obliterans masked by misdiagnosis as asthma.

OBJECTIVES: Asthma and postinfectious bronchiolitis obliterans (PIBO) have similar clinical findings, and PIBO may be misdiagnosed with asthma. This study aimed to determine the clinical features of PIBO in children and the causes of delay in its diagnosis. METHODS: We retrospectively evaluated all patients diagnosed with PIBO in four pediatric pulmonology centers between 2007 and 2018. In total, 64 PIBO patients were retrospectively reviewed. We compared the clinical and laboratory differences between PIBO patients who had initially been misdiagnosed with asthma and correctly diagnosed with PIBO. RESULTS: Of the 64 patients, 22 (34.4%) had initially been misdiagnosed with asthma. Adenovirus was the most common infectious agent in children. The age upon diagnosis was older, and the symptom duration was significantly longer in patients misdiagnosed with asthma (P < .05). There were no statistical differences in terms of sex, history of prematurity, duration of hospitalization, treatment, history of oxygen or mechanical ventilation support, pulmonary function test (PFT) results and asthma-predisposing findings between the two groups (P > .05). CONCLUSIONS: Patients with PIBO who had initially been misdiagnosed with asthma were correctly diagnosed at older ages and had longer symptom duration. Asthma may mask PIBO diagnosis by the similarity of symptoms and the clinical response to inhaled ß2-agonist or steroid treatment. PFTs may not help clinicians because of the age of children. The delay in the diagnosis of PIBO is probably attributable to the fact that some clinicians fail to include PIBO in the differential diagnosis when there is no clinical response to asthma medication.

Visual Hallucinations Induced by Clarithromycin in a Child: A Case Report and Literature Review.

OBJECTIVE: Our aim was to present a child with visual hallucinations possibly associated with oral clarithromycin administration. CASE REPORT: A 4-year-old child was admitted to our hospital with an onset of visual hallucinations after taking the second dose of clarithromycin by mouth. The symptoms gradually disappeared in a week once the clarithromycin therapy had been discontinued. She was observed for a month without any symptoms or further treatment. She was suspected of having Hoigne syndrome (also called as antibiomania) induced by clarithromycin syndrome. CONCLUSION: This report highlights neuropsychological adverse effects due to therapeutic doses of clarithromycin therapy as a possible adverse effect in children.

Ocular Findings in Children With 22q11.2 Deletion Syndrome.

PURPOSE: To identify the ocular features of children diagnosed as having 22q11.2 deletion syndrome in a Turkish population, which is the most common microdeletion syndrome with a wide range of facial and ocular abnormalities. METHODS: Sixteen children aged between 4 months and 18 years with a microdeletion in chromosome 22q11.2 underwent a detailed ophthalmological examination including uncorrected and best corrected visual acuity testing, stereoscopic vision examination, biomicroscopic and indirect fundus examination, and ocular motility testing. RESULTS: All patients had at least one ocular abnormality. The major abnormalities were eyelid abnormalities (eye hooding, narrow palpebral fissure, telecanthus, hypertelorism, sparse and thin eyebrows and eyelashes, blepharitis, and distichiasis), posterior embryotoxon, and tortuous retinal vessels in at least half of the patients. Other ophthalmological disorders were refractive errors, iris remnants, and strabismus. CONCLUSIONS: The chromosome 22q11.2 deletion syndrome is associated with a wide range of ocular disorders, which necessitates a comprehensive eye examination for appropriate treatment and follow-up. Ocular findings sometimes can provide a clue to the diagnosis of 22q11.2 deletion. [J Pediatr Ophthalmol Strabismus. 2016;53(4):218-222].

Would mean platelet volume/platelet count ratio be used as a novel formula to predict 22q11.2 deletion syndrome?

BACKGROUND: The diagnosis of 22q11.2 deletion syndrome depends on a time-consuming and expensive method, fluorescence in situ hybridisation (FISH). OBJECTIVES: We aimed to determine new parameters which can aid for in the diagnosis of 22q11.2 deletion syndrome. METHODS: Twenty two patients with 22q11.2 or 10p13 deletion were evaluated retrospectively. RESULTS: Facial-dysmorphism and mental-motor retardation were detected in 100% of patients. Mean platelet (PLT) counts were lower (224,980 versus 354,000, p = 0.001), mean PLT volume (MPV) (9.95 versus 7.07, p = 0.002), and MPV/PLTx105 ratios (5.36 versus 2.08, p < 0.001) were higher in patients with 22q11.2 deletion compared with the control group. Area under the receiver-operator characteristic (ROC) curve was 0.864, sensitivity was 84.6%, specificity was 90.9%, positive predictive value (PPV) was 91.7%, and negative predictive value (NPV) was 83.3% when MPV was 8.6. Area under ROC curve was 0.864, sensitivity was 76.9%, specificity was 90.1%, PPV was 90.1%, and NPV was 76.3% when PLT was 265,500. Area under ROC curve was 0.906, sensitivity was 84.6%, specificity was 100%, PPV was 100%, and NPV was 84.6% when MPV/PLTx105 was 3.3. Expression of PLT surface markers which were not in the GPIb-V-IX receptor complex (CD61, CD41a) increased as the surface area increased, but markers which were in a complex (CD42a, CD42b) did not change. CONCLUSIONS: High MPV/PLT value can be a good predictor for the diagnosis of 22q11.2 deletion syndrome. We suggest that in patients with facial dysmorphism and retardation in neurodevelopmental milestones and if MPV≥8.6fl, MPV/PLTx105 ratio≥3.3 and PLT count ≤265,500/mm3, the patients should be tested by FISH analysis to confirm the 22q11.2 deletion. If there are no macrothrombocytes, the 10p13 deletion should be tested in suspected cases.

Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation.

BACKGROUND AND AIMS: The appropriate treatment of pandemic H1N1 influenza which was first identified in April 2009 in Mexico is insufficient especially for immunocompromised patients. We aimed to evaluate the features and prognostic factors of the children with H1N1, especially immunocompromised ones, and whether intravenous immunoglobulin G (IVIG) replacement could aid for a better outcome. METHODS: Twenty-one hospitalized children with laboratory-confirmed H1N1 were evaluated retrospectively. Data were extracted from files and electronic medical records. RESULTS: The median age was 37 (1-216) months; 62% of them were under 5 years of age and 71.4% had one or more underlying disorders. Main symptoms were high fever, cough, fatigue and vomiting. Lower respiratory tract manifestations were seen in 66.6% of children. Mortality rate was 4.7%. The patient who died had the lowest lymphocyte (100/mm(3) ), thrombocyte (21 000/mm(3) ) and highest blood urea nitrogen (87 mg/dL) levels. Fifty-eight percent of evaluated patients had one of the primary immunodeficiency disorders. Surprisingly, none of the six patients with primary immunodeficiency who are on regular IVIG replacement needed intensive care unit and died. Although median durations of cough, fever and hospitalization were lower, they did not change statistically according to get IVIG replacement regularly (P = 0.47, 0.97, 0.09, respectively). CONCLUSION: Our study is important while it is the first one that shows the course of primary immunodeficient children with H1N1 infection who were on regular IVIG replacement. A trial of high-dose IVIG may be a useful adjunctive therapy in severe H1N1 influenza, particularly in the immunocompromised patients.

Infectious diseases, autoimmunity and midline defect in a patient with a novel bi-allelic mutation in IL12RB1 gene.

Clinical disease caused by weakly pathogenic mycobacterial species, which is known as Mendelian susceptibility to mycobacterial disease (MSMD), is a rare entity. IFN-γ and IL-17 production are defective due to insufficient response to IL-2 and IL-23 in IL-12Rß1 deficiency; so this also causes tendency to intracellular microorganisms and candidal diseases. Here, we present a patient who suffers IL-12Rß1 deficiency caused by a novel bi-allelic mutation with recurrent salmonellosis, mycobacterial, fungal infections and remained asymptomatic during 13 months of follow-up after hIFN-γ treatment. In addition she had hemolytic anemia and midline defects like cleft lip and palate which have not been reported in a patient with MSMD in the literature prior to this case report. In conclusion, diagnosis of MSMD should be kept in mind in patients with recurrent salmonellosis, mycobacterial and fungal infections especially in countries with a high consanguinity rate.

Unilateral nevoid telangiectesia: report of two pediatric cases.

Unilateral nevoid telangiectasia (UNT) is characterized by the dermatomal distribution of telangiectasia, which are distributed unilaterally and frequently found in C3-C4 or the trigeminal dermatomal areas. It may be congenital or acquired and has a 2:1 female: male ratio. Several theories where developed in order to explain its etiopathogenesis. The most widely accepted is the one which establishes its probable association with an increase in estrogen levels. Its incidence is unknown, although it's probably subdiagnosed and more frequent than previously recognized. We present two pediatric cases of UNT followed at our instution during the past year.

Spontaneous pneumomediastinum as a complication in human bocavirus infection.

The most common causes of spontaneous pneumomediastinum (SPM) in children are asthma attack and respiratory tract infection. Here, we describe a case of SPM in a human bocavirus-infected 2-year-old boy with bronchiolitis.

Systemic atopy and immunoglobulin deficiency in Turkish patients with vernal keratoconjunctivitis.

PURPOSE: To determine the prevalence of systemic atopy and immunoglobulin (Ig) deficiencies in vernal keratoconjunctivitis (VKC). METHODS: Sixty-seven VKC subjects (79.1% boys) with a mean age of 11.3 ± 4.3 years were included. Serum Ig levels and specific IgE levels were measured using the nephelometric method and reversed enzyme immunoassay with sandwich ELISA technique, respectively. The patients underwent epidermal skin tests with commercial extracts. RESULTS: Family history of atopy and associated systemic allergies were detected in 32.8 and 40.3% of the subjects, respectively. Blood eosinophilia, elevated total, and specific IgE and positive skin tests were detected in 33.8, 42.2, 50, and 35% of the subjects, respectively. Out of 62 subjects, low levels of IgA, IgG, IgM, and IgG3 were detected in 12.9, 8, 6.5, and 1.6% of the patients, respectively. CONCLUSION: IgE-mediated mechanisms are involved in approximately 40% of VKC patients. A new finding was the higher incidence of Ig deficiency.

Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy.

We aimed to evaluate the role of the CD19 complex in the pathogenesis of transient hypogammaglobulinemia of infancy (THI) and to better characterize the subsets of memory B cells. The study population consisted of 22 male and 14 female patients with a mean age at presentation of 20 ± 9.9 months. The CD19 complex and B cell subsets were evaluated by flow cytometry. While the CD19 median fluorescence index (MFI) in patients with THI was significantly lower than controls (122.9 ± 66.7 in patients; 184.2 ± 39 in controls, p < 0.01), expression of CD21 and CD81 was increased (94.4 ± 3, 96.8 ± 2.5 % in patients; 91 ± 3.9; 94.7 ± 3.5 % in controls, p < 0.01 vs. p < 0.05, respectively). The expressions of switched memory B cells and IgM memory B cells were found to be reduced in THI. Considering that the CD19 complex regulates the events following antigen stimulation, the change in CD19 complex detected in THI may be related to insufficiency of antibody production.

Haloperidol: a possible medication for the treatment of exacerbation of intractable psychogenic sneezing.

Sneezing is one of the physiological defense mechanisms that develops generally due to nasal irritation. But intractable sneezing episodes are uncommon and generally detected among the adolescents. It is difficult to distinguish physiologic sneezing from psychogenic sneezing. Herein, we report a 12-year-old girl who was complaint with intractable sneezing. She was diagnosed as intractable psychogenic sneezing and haloperidol treatment was started. All symptoms had resolved completely within 2 weeks. As a result, haloperidol can be considered as a different treatment modality for intractable psychogenic sneezing.

Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: 'similarities and differences'.

Transient hypogammaglobulinemia of infancy (THI) is characterized by recurrent infections and one or more reduced serum immunoglobulin (Ig) levels. Usually, this clinical picture resolves spontaneously by 3 yr of age. However, hypogammaglobulinemia persists until adolescence in some patients. In recent years, those patients have been classified as undefined/unclassified hypogammaglobulinemia (UCH). We aimed to evaluate the clinical and immunologic features of patients with THI and UCH considering age of recovery and to assess relationships between hypogammaglobulinemia, infections, and allergic manifestations. We reviewed the medical records of children followed with a diagnosis of hypogammaglobulinemia from 2001 to 2007. Patients with decreased levels (<2 s.d.) of one or more major Ig isotypes (IgG, IgA, IgM) with normal antibody responses and lymphocyte subpopulations were included (n = 374). Those patients whose Igs normalized during the follow-up period were classified as THI and the others as UCH. The THI group consisted of 71 patients (27 females, 44 males) with a mean recovery age of 68.87 +/- 36.5 months. About 95% of patients with THI recovered before 10 yr of age. The UCH group consisted of 303 patients (105 females, 198 males) with a mean age at diagnosis of 52 +/- 42 months. The most common presenting manifestations in the THI and UCH groups were upper respiratory tract infections (URTIs), lower respiratory tract infections, and asthma (42%, 50%, and 52% in the THI group vs. 39%, 53%, and 55% in the UCH group, respectively). In the THI group, the prevalence of atopic disease was related to age and found to be increased markedly after 44 months. In all patients, the prevalence of asthma was independently and positively associated with family history of atopy and age, whereas it was negatively associated with recurrent URTIs. Patients with THI and UCH have similar clinical and immunologic features. The normalization of Igs may be delayed in a majority of the patients with hypogammaglobulinemia. This observation may be a contribution to the classical definition and diagnostic criteria for THI.

Late-onset adenosine deaminase deficiency presenting with Heck's disease.

Focal epithelial hyperplasia, also known as Heck's disease, is a rare but distinctive entity of viral etiology with characteristic clinical and histopathological features. It is a benign, asymptomatic disease of the oral mucosa caused by human papilloma viruses (HPV). Previous studies postulated an association between these lesions and immunodeficiency. Genetic deficiency of adenosine deaminase (ADA) results in varying degrees of immunodeficiency, including neonatal onset severe combined immunodeficiency (ADA-SCID), and milder, later onset immunodeficiency. We report a 12-year-old girl with the late onset-ADA deficiency presenting with Heck's disease. Our case report should draw attention to the possibility of immunodeficiency in patients with HPV-induced focal epithelial hyperplasia.

Shigella antibiotic resistance in central Turkey: comparison of the years 1987-1994 and 1995-2002.

BACKGROUND: The epidemiology and antibiotic susceptibility of Shigella species changes over time. Updated susceptibility knowledge is necessary for appropriate empirical antibiotic treatment. METHODS: In this study the clinical characteristics and the antibiotic susceptibility test results of Shigella species isolated from patients admitted to Hacettepe University Ihsan Dogramac Children's Hospital Diarrheal Diseases Training and Treatment Unit between 1995-2002 (n = 218) were evaluated. The results were compared with the results of 1987-1994 from the same center (n = 618). RESULTS: The predominant species was S. sonnei in both periods with increasing prevalence in the later period (64% and 71.5% respectively, P < 0.05). Although the prevalence of bloody diarrhea among Shigella cases did not change between periods, the prevalence of mild to moderate dehydration (11% and 20.6%) increased. Approximately 40% of cases were prescribed antibiotics on the suspicion of shigellosis. When the two periods were compared, Shigella resistance to trimethoprim/sulfamethoxazole increased from 39% to 70% (P < 0.0001), whereas resistance to ampicillin decreased from 41% to 23% (P < 0.0001). However, the resistance of S. flexneri to ampicillin was found to be quite high (72.9%) between 1995-2002. No Shigella species resistant to ciprofloxacin was detected. Multi-drug resistance was present in 52 (24%) of the cases, of which 10 were S. sonnei (6.3%) and 42 S. flexneri (26.4%) (P < 0.0001). CONCLUSION: The need to treat all Shigella cases in areas where S. sonnei is predominant should be reevaluated, as should the appropriate empirical antibiotic.