A neglected cause for chronic spontaneous urticaria in children: Helicobacter pylori.

BACKGROUND: The aetiology of chronic urticaria is usually considered idiopathic. There is a paucity of research both on the prevalence of Helicobacter pylori infection in the aetiology of chronic spontaneous urticaria (CU) in children and also on which patients H. pylori should be investigated. METHODS: All paediatric and adult patients who presented to the allergy outpatient clinic due to CU between January 2011 and July 2012 were included in this prospective, randomised study. Stool samples from all patients were examined for the H. pylori antigen. Paediatric and adult patients who had a positive stool test for the H. pylori antigen were reassessed following eradication therapy. RESULTS: Thirty-two children with CU and 35 adults with CU were enrolled in the study. Ten of the 32 (31.2%) children and 18 of the 35 (51.4%) adults were H. pylori positive (p=0.09). All children with positive-H. pylori were older than eight years of age. There was a significant positive correlation between age and the frequency of H. pylori infection (p<0.001; r=0.61). The presence of H. pylori was not significantly associated with the presence of GI (gastrointestinal) symptoms (p>0.05). Following H. pylori eradication, urticarial symptoms recovered in 15 of the adults (83.3%) and 10 of the paediatric (100%) patients (p=0.172). CONCLUSION: In the current study we found that H. pylori is common among children with CU, particularly after eight years of age. We suggest that CU patients with an unknown aetiology should be routinely screened for H. pylori even if they do not present with GI symptoms and that those with H. pylori-positive results may receive treatment.

The role of mean platelet volume as an inflammatory marker in children with chronic spontaneous urticaria.

BACKGROUND: Previous studies have shown that platelets are involved in the inflammatory process. Mean platelet volume (MPV) has been frequently used as an inflammatory marker in various diseases associated with inflammation. The role of MPV in children with chronic spontaneous urticaria (CU), however, has not yet been evaluated. In this study we compared MPV levels between children with and without CU. METHODS: Children with CU and age-matched healthy children were enrolled in the study. Complete blood count and C-reactive protein (CRP) levels were assessed in children with CU whilst MPV levels were compared between children with and without CU. RESULTS: Forty children with CU (19 males; mean age: 8.0 ± 3.8 year; range: 3-15 years) and 40 healthy children (17 males; mean age: 6.9 ± 3.0 year; range: 2-14 year) were enrolled on the prospective, case-control study. MPV (fL) levels were significantly lower in children with CU when compared to healthy children (7.42 ± 0.77 and 7.89 ± 0.65, respectively; p=0.004). Both mean platelet number and median CRP levels were significantly higher in children with CU when compared to healthy children (p=0.008, p=0.014, respectively). CONCLUSION: To our knowledge, this study is the first to evaluate the role of MPV as an inflammatory marker in children with CU. A decline in MPV may be considered as an indicator of inflammation in children with CU.

Level of serum neutrophil gelatinase-associated lipocalin in childhood asthma.

BACKGROUND: The role of neutrophil gelatinase-associated lipocalin (NGAL) in childhood asthma remains unknown. This study aimed to measure the serum levels of NGAL in children with asthma and to investigate the correlation between NGAL and transforming growth factor beta 1 (TGF-ß1), a good indicator of airway remodeling in children with asthma. METHODS: This prospective, cross-sectional study was conducted on 75 children. Serum NGAL and TGF-ß1 concentrations were measured by the ELISA method. Complete blood count, high sensitive C reactive protein (hsCRP), eosinophil cationic protein (ECP), and total serum IgE were investigated in the study population. Atopy in the asthma group was investigated using a skin prick test and specific IgE measurements. RESULTS: Forty-three asthmatic children and 32 healthy children were enrolled in the study. Total eosinophil numbers, white blood cell count, total serum IgE levels and ECP levels were significantly higher in the asthma group than in the control group (p<0.05). Similarly, serum TGF-ß1 levels were significantly higher in children with asthma (p=0.012). The difference in NGAL levels between the groups was insignificant (p=0.268). NGAL levels did not show a significant correlation with total IgE, ECP, eosinophil numbers and TGF-ß1 levels (p>0.05). CONCLUSION: As a conclusion, while elevated TGF-ß1 levels in children with asthma might be regarded as an indicator of airway remodeling, we did not find a similar prediction strength for NGAL. Further studies are required to better identify the role of NGAL in childhood asthma and to determine its potential use as a clinical marker.

Elevated level of serum osteopontin in school-age children with asthma.

BACKGROUND: The role of osteopontin (OPN) has not been elucidated in childhood asthma. OBJECTIVE: Our purpose was to investigate whether OPN levels change due to allergic inflammation in pre-school and school-age children. METHODS: In this prospective, cross-sectional study, 42 healthy children and a total of 51 children with asthma were recruited. OPN levels and its association with clinical and laboratory parameters were investigated in the study population. The asthma group were divided into two groups with respect to age, ≤ 5-years (n = 23) and >5-years (n = 28), and labelled Asthma Group 1 and Asthma Group 2, respectively. OPN levels were compared between subgroups. RESULTS: Serum OPN levels were significantly higher in the asthma group when compared to the control group (p = 0.004). OPN levels were similar in Asthma Group 1 and control groups, whereas it was found to be higher in Asthma Group 2 (p>0.025, p = 0.001, respectively). In the >5-years age asthmatic group, OPN levels of the patients with allergic rhinitis (n = 15) were higher than those of the patients (n=13) without allergic rhinitis (p = 0.021). CONCLUSION: The study underscores the relationship between childhood asthma and OPN as the first study in the literature. In this study we found that OPN, which plays a role in Th2 mediated inflammation, may also play a role in childhood asthma. The fact that OPN levels do not increase in preschool-age children with asthma might be due to the transient wheezing in this group.

Multiloculated hydrocephalus of intrauterine-onset: a case report of an unexpected MTHFR A1298C positive test result.

Loculated hydrocephalus is a condition in which discrete fluid-filled compartments form in association with the ventricular system of the brain. Multiloculated hydrocephalus is a subgroup of this entity involving more than one segment of the ventricular system. Abnormal descent of the cerebellar components can cause multiloculated hydrocephalus due to various pathogenesis. However, studies report no more than 10% of correlation between cerebellar herniation and hydrocephalus. We report an infant with MTHFR A1298C homozygosity, who had hydrocephalus of intrauterine-onset. Alterations in the folate metabolism might lead to congenital hydrocephalus and there is growing data on the prothrombotic effects of MTHFR polymorphisms. To the best of our knowledge, there has been no reported case of MTHFR A1298C homozygosity and intrauterine-onset multiloculated hydrocephalus as a co-existence in the literature.

Syndrome of inappropriate antidiuretic hormone secretion refractory to treatment in a newborn with alobar holoprosencephaly.

Alobar holoprosencephaly (AHP) is a serious malformation of the central nervous system characterized by complete failure of cleavage of the prosencephalon resulting in fusion of the cerebral hemispheres and a massive single ventricular midline fluid collection. Secretion of inappropriate antidiuretic hormone syndrome (SIADH) is a disorder of fluid-electrolyte balance caused by the inability to suppress the secretion of antidiuretic hormone (ADH) resulting in the development of hyponatremia. Coexistence of both of these entities has not been described in the literature. We report a newborn infant with AHP who had resistant SIADH refractory to treatment, to draw attention to the coexistence of these entities and to contribute to the literature with the management of this challenging condition.

Characteristic MR Imaging Findings of the Neonatal Brain in RASopathies.

BACKGROUND AND PURPOSE: Neuroimaging features in neonates with RASopathies are rarely reported, and to date, there are no neuroimaging studies conducted in this population. Our aim was to investigate the occurrence of supratentorial and posterior fossa abnormalities on brain MRIs of neonates with a RASopathy. MATERIALS AND METHODS: An observational case-control study of neonates with a confirmed RASopathy was conducted. The presence of an intraventricular and/or parenchymal hemorrhage and punctate white matter lesions and assessments of the splenium of the corpus callosum, gyrification of the cortical gray matter, and enlargement of the extracerebral space were noted. The vermis height, transverse cerebellar diameter, cranial base angle, tentorial angle, and infratentorial angle were measured. RESULTS: We reviewed 48 brain MR studies performed at 3 academic centers in 3 countries between 2009 and 2017. Sixteen of these infants had a genetically confirmed RASopathy (group 1), and 32 healthy infants were enrolled as the control group (group 2). An increased rate of white matter lesions, extracerebral space enlargement, simplification of the cortical gyrification, and white matter abnormalities were seen in group 1 (P < .001, for each). The vermis height of patients was significantly lower, and tentorial and infratentorial angles were significantly higher in group 1 (P = .01, P < .001, and P = .001, respectively). CONCLUSIONS: Neonates with a RASopathy had characteristic structural and acquired abnormalities in the cortical gray matter, white matter, corpus callosum, cerebellum, and posterior fossa. This study provides novel neuroimaging findings on supratentorial and posterior fossa abnormalities in neonates with a RASopathy.