Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.

Objectives: This study aimed to extend the literature by analyzing immunoglobulin (Ig) A, IgE, IgG, IgG2, IgG3, and IgM antibody levels in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) patients. Patients and methods: This study retrospectively analyzed the antibody test results of 20 pediatric patients (10 males, 10 females; mean age: 2.5±1.5 years; range, 0.5 to 5.4 years) with and without flare who were initially evaluated for a number of underlying diseases due to periodic fever/infectious symptoms but then diagnosed with PFAPA between January 2015 and December 2020. Antibody levels were determined by chemiluminescence microparticle immunoassay. The results were retrospectively compared with a group of healthy children after the PFAPA diagnosis was confirmed. Results: The chemiluminescence microparticle immunoassay revealed 35%, 65%, 20%, 86.6%, and 55% of PFAPA cases with low serum levels of IgA, IgG, IgG2, IgG3, and IgM respectively, while 56.2% had high IgE levels. Moreover, low serum levels of at least two antibody classes or subclasses were reported in 80% of the PFAPA children. While cases with low IgG serum levels were with the highest incidence rates among the low IgG3 PFAPA patient population, both high IgE and low IgM cases were common in the rest of the patients. Conclusion: Our results suggest an association between PFAPA and low serum antibody levels, particularly of IgG3. Future studies are needed to confirm our conclusion.

Focality in childhood absence epilepsy.

BACKGROUND AND PURPOSE: Childhood absence epilepsy (CAE) has a typical electroencephalography (EEG) pattern of generalized 3 Hz spike and wave discharges (SWD). Focal interictal discharges were also documented in a small number of documents. The aim was to investigate the amplitudes of interictal 3 Hz SWD within the 1st second in drug-naïve CAE patients. In this way, areas with maximal electronegativity at the beginning of clinically generalized discharges will be documented. METHODS: The EEG records of children with drug-naïve CAE were evaluated retrospectively by two child neurologists first for 3 Hz SWD. Then, a machine-learning model evaluated the amplitudes of 3 Hz in the 1st second of SWD. Maximum electronegativity areas were documented and classified as focal, bilateral, and generalized. RESULTS: One hundred and twelve 3 Hz SWD were evaluated in 11 patients. Among discharges within the 1st second, maximum electronegativity areas were documented as focal for 44 (39.2%), bilateral for 8 (7.1%), generalized for 60 (53.5%). Among focal electronegativity areas, mostly right central, left occipital and midline parietal areas were documented in 12 (10.7%), 7 (6.2%), and 6 (5.3%), respectively. Eight (7.1%) of the maximum electronegativity areas were detected bilaterally, of which 7 (6.2%) originated from the frontopolar areas. CONCLUSIONS: Focal maximal electronegativity areas were frequently observed in drug-naïve CAE patients, comprising approximately half of non-generalized discharges. Focal discharges might be misleading in diagnosis. Focal areas within the brain may be responsible for and contribute to absence seizures that appear bilaterally symmetrical and generalized clinically. Although its clinical implication is unknown, this warrants further study.

Co-morbid psychiatric disorders in children with arachnoid cyst.

Arachnoid Cysts (AC) are benign lesions containing cerebrospinal fluid, and although most of them are asymptomatic, they can cause neurological symptoms like headaches, seizures, and neuropsychiatric problems. The aim of this study was to asses and document co-morbid psychiatric disorders in children with AC aged between 6 and 17. Wechsler Abbreviated Scale of Intelligence-Second Edition (WASI-II), a clinical measure used to assess the intelligence quotient (IQ) scores of the patients, Kiddie Schedule for Affective Disorders and Schizophrenia Present and Lifetime Version (K-SADS-PL; semi-structured interview) was used to assess psychiatric disorders among the patients. A total of 12 patients with AC was evaluated with an even distribution of males and females. Half of the patients had a normal IQ score with a mean IQ score of 104.5. Among patients with normal IQ scores, one patient had epilepsy and attention deficit hyperactivity disorder and two patients had epilepsy without any psychiatric disorder. The remaining six patients had moderate intellectual disability with a mean IQ of 48.2. Among them, three out of six had epilepsy and four had accompanying psychiatric disorders. It is therefore apparent that patients with AC have a high rate of co-morbid psychiatric disorders. Our study demonstrates that intellectual disability and psychiatric disorders should be evaluated in children with AC in the clinical settings.

Lessons from Real Life Experience: Importance of In-House Sequencing and Smart Ratio-Based Real-Time PCR Outperform Multiplex Ligation-Dependent Probe Amplification in Prenatal Diagnosis for Spinal Muscular Atrophy: Bench to Bedside Diagnosis.

Spinal muscular atrophy (SMA) is a rare, recessively inherited neurodegenerative disorder caused by the presence of pathogenic variants in the SMN gene. As it is the leading inherited cause of infant mortality, identification of SMN gene pathogenic variant carriers is important for diagnostic purposes with effective genetic counseling. Multiple ligation probe analysis (MLPA), a probe-based method, is considered as the gold standard for SMA carrier analysis. However, MLPA might give false-negative results in cases with variations in the probe-binding regions. Here, we present a case born to consanguineous SMA carrier parents. Prenatal diagnosis with MLPA failed to detect the compound heterozygous mutant state of the proband and she was born unfortunately with SMA phenotype. Further analysis with a real-time polymerase chain reaction kit was able to detect the compound heterozygous state of the patient and was confirmed with targeted next-generation sequencing technology.

Intracranial cysts: incidental or neurodevelopmental?

OBJECTIVES: Intracranial cysts are fluid-filled sacs within the brain. There is a diversity of intracranial cysts with different incidences in addition to the growing awareness about comorbidities and the consequences. The present study aimed to evaluate cystic findings in children who were admitted to the department of pediatric neurology. METHODS: Children who were admitted to the Clinic of Pediatric Neurology and who had an MRI between 2016 and 2021 were evaluated. The MRI examination was performed with the pediatric epilepsy protocol. Children with primary intracranial cysts were enrolled in the study. Demographic and clinical findings were evaluated from the hospital's database and patients' files. RESULTS: Among the 78 patients, 36 (46.2%) were male and 42 (53.8%) were female. The mean age was 7 ± 5.4 years. The most frequent presenting complaint was a seizure (47.4%). Approximately one-quarter (28.2%) had mental and/or motor retardation. Nine (11.5%) of the children had a neuropsychiatric diagnosis. Most of the cysts were located at the midline (41%) with the majority located extra-axial (71.8%) and supratentorial (78.2%). Arachnoid cysts were observed most frequently with a percentage of 64.1%, followed by pineal cysts (15.4%). The history of seizure, epilepsy, presence of mental retardation, and neuropsychiatric problems were evaluated according to the population ratios based on z approximation in which significantly higher rates were observed among cases with intracranial cysts. CONCLUSION: Intracranial cysts should be taken into consideration for comorbid pathologies, especially in the childhood period. Early evaluation in patients with intracranial cysts for developmental delay and neuropsychiatric problems is important.

Cutaneous Allergic reactions to pine processionary caterpillar (Thaumetopoea Pityocampa): a complicated cutaneous reaction in an infant and review of the literature.

BACKGROUND: Thaumetopoea Pityocampa (TP) are frequent in the Mediterranean region especially affecting forest workers in pinewood areas. The common symptoms include swelling, rash or burns like any form of dermatitis. The reactions can be triggered by mechanical, chemical or allergic factors and the `allergic` reaction is caused by sensitization to a hair protein named `thaumetopoein`. This protein triggers the IgE mediated reaction resulting in the mast cell degranulation causing urticaria. Different kinds of allergic reactions like urticaria or anaphylaxis have been reported previously commonly in adults, especially in forest workers while severe reactions without direct contact are rare in pediatric population. CASE: A 28 month old healthy boy was admitted to Near East University Pediatric Allergy and Immunology Outpatient Clinic in March with complaints of pain, hyperemia and swelling on the left hand. His complaints had started the day before his admission just after walking around in their garden which is surrounded by pine trees. On admission, his physical examination revealed serious edema and hyperemia on his left hand limiting his finger movements with a few bullae on the skin. His temperature was 38 C and the other vital parameters were normal. Based on hyperemia, swelling and high acute phase reactants he was hospitalized with the differential diagnosis of soft tissue inflammation and cellulitis. The case was treated with iv antihistamines, systemic steroids and antibiotics. CONCLUSIONS: Pine processionary (PP) is an important irritant and allergen especially in endemic areas like Cyprus which is a Mediterranean Country. It must be kept in mind in case of local or generalized urticaria, dermatitis, bullae and other allergic reactions even if there had been no direct contact with PP. Systemic involvement with fever and elevated acute phase reactants in infancy may necessitate hospitalization and intravenous treatment. Hereby, we reported an infant who presented with fever in addition to severe cutaneous lesions following the exposure to TP without direct contact. This is the first case reported from North Cyprus.

A Near-Global Slowing of Background Activity and Epileptic Discharges in Children With Mild to Moderately Symptomatic COVID-19 Infection: An Electro-Neurophysiological Study.

Background. To assess the functional involvement of the central nervous system (CNS) via quantitative electroencephalography (EEG) analysis in children with mild to moderate COVID-19 infection who were otherwise previously healthy children. Methods. This prospective, case-control study was conducted between June and September 2020. Sleep EEG records of at least 40 min were planned for children who tested positive for COVID-19 using real-time PCR analysis and within 4-6 months post-recovery. All of the EEG analyses in this study were performed on an Ubuntu 20.04.2 LTS Operating System with the developed software using Python 3.7.6. The quantitative analysis of the epileptic discharges within the EEG records was performed using random forest after elimination of the artifacts with a model training accuracy of 98% for each sample data point. The frequency analysis was performed using the Welch method. Results. Among the age and sex-matched groups, the global mean frequency was significantly lower among the COVID-19 patients, with a P-value of 0.004. The spike slow-wave and sharp slow-wave indices were significantly higher in the patients when compared to the controls. The mean frequency values were significantly lower in almost all of the electrodes recording the frontal, central, and occipital areas. For the temporal and parietal areas, those significantly low mean frequencies were limited to the right hemisphere. Conclusion. A near-global involvement of background activity with decreased frequency, in addition to epileptic discharges, was recorded in mild to moderately COVID-19 infected children post-infection.

The pediatric asymptomatic SARS-CoV-2 IgG seropositivity in the Turkish Republic of Northern Cyprus.

BACKGROUND: While children were initially thought to have serious contributions to the coronavirus disease 2019 (COVID-19) transmission, recent studies suggest otherwise. However, the possible effect of asymptomatic pediatric spread still has not yet received enough attention. The aim of our study was to estimate asymptomatic infection rates among children in the Turkish Republic of Northern Cyprus, by using pediatric patients admitted to a university hospital without any COVID-19-associated symptoms. METHODS: Blood samples collected from 80 pediatric patients with no symptoms and history of COVID-19 infection, who were admitted to a university hospital between September 2020 and January 2021, were included in the retrospective study. Isolated serum samples were tested by Dia.Pro SARS-CoV-2 IgG ELISA assays. RESULTS: The patient group included 40 (50%) male and 40 (50%) female patients. The average age of children was 7.6 ± 4.0 years, with min-max ages ranging from 2 to 15 years. Among the 80 patients tested, only one (1.3%) was detected positive by the Dia.Pro IgG ELISA kit. CONCLUSIONS: The asymptomatic seropositivity reported in our study suggests the use of randomly performed serologic tests to monitor SARS-CoV-2 infection among the pediatric population in schools that would contribute to the public health fight against COVID-19.

An investigation to choose the proper therapy technique in the management of autism spectrum disorder.

Aim: Autism spectrum disorder is a class of neurological disorders that affect the development of brain functions. This study aims to evaluate, compare and rank the therapy techniques used in the management of autism spectrum disorder using multicriteria decision-making approaches. Materials & methods: Fuzzy PROMETHEE and fuzzy TOPSIS approaches were used. Fuzzy PROMETHEE utilizes a pair-wise comparison of alternatives under the fuzzy environment while fuzzy TOPSIS utilizes geometric distance from the positive ideal solution under the fuzzy environment for the evaluation of the effectiveness of the alternatives.The techniques selected for evaluation are applied behavioral analysis, cognitive behavioral therapy, speech therapy and pharmacological therapy such as Risperidone and Aripiprazole. Criteria used in this study include efficacy, cost and side effects, and their weights are assigned based on specific patient conditions. Results: The results indicate that applied behavioral analysis, cognitive behavioral therapy and speech therapy are the most preferred techniques, followed by Aripiprazole and Risperidone. Conclusion: More criteria could be considered and the weights could be assigned according to the patient profile.

Lay abstract Autism spectrum disorder is a neurodevelopmental disorder (affecting the development of the brain) that usually presents during childhood. Because autism spectrum disorder has no cure, selecting the best therapy to manage the disorder is important for therapists, parents, health institutions and researchers with an interest in these types of disorders. This study focuses on comparing specific therapy techniques by using multicriteria decision-making methods. The result obtained by a decision-maker is not always the same, as different decision-makers may come up with different solutions depending on things like the cost and how well a therapy works on different aspects of the disorder. The outcome of the study indicates that applied behavioral analysis, cognitive behavioral therapy and speech therapy are preferred treatment alternatives, followed by treatment with Risperidone and Aripiprazole. Further analyses are needed to obtain more accurate patient-specific results that will incorporate specific patient demographics and data, as well as looking at the combination of two or three techniques.

Altered tonsillar toll-like receptor (TLR)-1 and TLR-2 expression levels between periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA), and group A beta-hemolytic streptococcal (GAßHS) recurrent tonsillitis patients.

INTRODUCTION: Tonsillar microenvironment is thought to contribute to innate immune dysregulation responsible for the periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) because of beneficial effects of tonsillectomy on treatment of the syndrome. Accordingly previous studies reported altered lymphocyte frequency, cytokine level and microbial composition in PFAPA tonsils. The aim of our study is to monitor expression levels of pro-inflammatory cell surface Toll-like receptors (TLRs) which have important role in induction of inflammation and maintaining tissue haemostasis. MATERIALS AND METHODS: Seven patients with PFAPA syndrome, and eight patients with group A beta-hemolytic streptococcal (GAßHS) recurrent tonsillitis were included in our study. Tonsillar expression levels of TLR-1, -2, -4, -5, and -6 were monitored by immunohistochemistry (IHC). Expression levels were scored using semi-quantitative analysis method and were statistically analyzed by Two-Way Repeated Measures Analysis of Variance test. RESULTS: IHC analysis demonstrated expression of all TLRs in tonsillar surface epithelium (SE) and lymphoid interior (LI) except for TLR-6 which was not present in the former. There has not been any statistically significant difference in TLR expression levels between PFAPA and GAßHS tonsils, except for TLR-1 and TLR-2 which were higher on LI and lower on SE of PFAPA tonsils, respectively, than that of the GAßHS samples. CONCLUSIONS: Altered TLR expression levels may be involved in PFAPA pathogenesis. Future studies with higher patient number, uninflamed tonsils and cellular markers are required to further enlighten the role of TLRs in the development of syndrome.

Benign epilepsy with centrotemporal spikes: Is there a thalamocortical network dysfunction present?

INTRODUCTION: Benign epilepsy of childhood with centrotemporal spikes (BECTS) is one of the most frequently seen epileptic syndromes in childhood. It is characterized by centrotemporal spikes (CTS) on electroencephalography (EEG) that are typically activated by drowsiness and stage N2 sleep. The location, frequency, and amplitude of the spikes may vary in different EEG records of the same patient, supporting the presence of a global pathology rather than a focal one. Despite the well-known relation between BECTS and stage N2 sleep, the results of sleep studies have been diverse and have mainly focused on sleep cycles. The characteristics of sleep spindles in the interictal periods have not been studied well. METHODS: A retrospective study involving patients with BECTS who were admitted to the Gazi University, Faculty of Medicine, Department of Pediatric Neurology from January 2017 to October 2018 was conducted herein. Patients with BECTS and age-matched controls who had stage N2 sleep records of 10 min were enrolled for spindle amplitude (peak-to-peak difference in spindle voltage), frequency (number of waveforms per second), and duration and density (number of spindle bursts/minute of stage N2 sleep). RESULTS: A total of 30 children with BECTS and 20 age-matched healthy peers were enrolled in the study. There were no significant differences between the age and sex of the patients. Statistically significant lower mean values of the amplitude, and duration and density of the spindle activity were observed in patients with BECTS when compared to the controls (P: 0.034, P: 0.016, and 0.020, respectively). Additionally, the risk of epilepsy was found to increase by 1.9 %, by the decrease of the mean amplitude of the spindles by 1 mV when compared to control group. CONCLUSION: The interictal records of stage N2 sleep differed in the patients with BECTS when compared to the controls. Findings related to the stage N2 sleep of the present study may suggest a network problem involving the thalamus and thalamocortical pathways in patients with BECTS.

STUB1 polyadenylation signal variant AACAAA does not affect polyadenylation but decreases STUB1 translation causing SCAR16.

We present three siblings afflicted with a disease characterized by cerebellar ataxia, cerebellar atrophy, pyramidal tract damage with increased lower limb tendon reflexes, and onset of 31 to 57 years, which is not typical for a known disease. In a region of shared homozygosity in patients, exome sequencing revealed novel homozygous c.*240T > C variant in the 3'UTR of STUB1, the gene responsible for autosomal recessive spinocerebellar ataxia 16 (SCAR16). In other genes, such an alteration of the evolutionarily highly conserved polyadenylation signal from AATAAA to AACAAA is known to highly impair polyadenylation. In contrast, RNA sequencing and quantification revealed that neither polyadenylation nor stability of STUB1 mRNA is affected. In silico analysis predicted that the secondary structure of the mRNA is altered. We propose that this change underlies the extremely low amounts of the encoded protein in patient leukocytes.

Evaluation of Dose Dependent Maternal Exposure to Bisphenol A on Thyroid Functions in Newborns.

Bisphenol A (BPA) is an endocrine-disrupting chemical compound that is mainly used in industrial products as packaging and plastics. It usually transmits to humans via oral route from food-contact material. BPA has demonstrated to be found in body fluids with a higher amount of fetal tissues due to bio-accumulation. Although it has been reported to affect the endocrine system, results on thyroid functions of newborns are conflicting. The aim of the present study is to demonstrate the effect of different levels of BPA in cord blood on the thyroid functions of newborns, according to gender. METHODS: The study population included 88 newborns. The BPA levels, Thyroid stimulating hormone (TSH) and free thyroxine (fT4) levels of cord blood were measured. In addition, SPINA-GT (thyroid' incretory capasity), TSH Index (TSHI), standardized TSHI (sTSHI) were calculated and demographic characteristics of participants were noted. RESULTS: The mean of cord blood BPA was 4.934 ± 2.33 ng/mL. When evaluated according to quantiles of BPA, no association was found between BPA and thyroid hormone levels, as well as, SPINA-GT, TSHI, sTSHI in both genders. CONCLUSION: Although BPA has been shown to contaminate cord blood, no significant effect was detected on thyroid hormones, SPINA-GT, TSHI and sTSHI. Further investigations with larger study populations are warranted.

Tonsillar antimicrobial peptide (AMP) expression profiles of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) patients.

INTRODUCTION: PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most frequent non-infectious cause of high fever observed among the European child population. While its cause is still not yet fully identified, PFAPA patients were previously shown to have altered tonsillar microbiome composition. Our study hypothesized that this is associated with a change in antimicrobial peptide (AMP) expression levels, as in the case of Crohn's disease which is another autoinflammatory disorder. METHODS AND MATERIALS: The tonsil specimens were isolated from seven patients with PFAPA syndrome, and six patients with group A beta-hemolytic streptococcal (GAßHS) recurrent tonsillitis. Tonsillar expression levels of human beta-defensin 1-2, cathelicidin, ribonuclease-7, and liver expressed antimicrobial peptide-1 were monitored by immunohistochemistry (IHC). Expression levels were scored using semi-quantitative analysis method and were statistically analyzed by Two-Way Repeated Measures Analysis of Variance test. RESULTS: Our results showed no significant difference in AMP expression levels between PFAPA and GAßHS patients. Immunolocalization of human beta-defensin 1 was different between the two groups; expressed at higher levels on tonsil surface epithelium (SE) than lymphoid interior (LI) in PFAPA patient group, while this was not evident in GAßHS patients group. CONCLUSIONS: Our results suggest that, PFAPA patients may be associated with altered AMP expression as in other autoinflammatory diseases. Future studies with subjects without any inflammatory condition are required for more precise conclusions.

Magnetic Resonance Imaging and Interictal Electroencephalography Findings in Newly Diagnosed Epileptic Children.

INTRODUCTION: Epilepsy is one of the most frequently diagnosed chronic neurological disorders in children. Diagnosis is often based on seizure history and electroencephalography (EEG) assessment. Magnetic resonance imaging (MRI) is recommended for etiologic workup and intervention requirements. We aimed to detect by MRI if focal structural abnormalities are present in the brain in relation to interictal epileptiform discharges (IED). MATERIAL AND METHODS: The study was designed retrospectively. The data were collected from patients admitted to Near East University, Department of Pediatric Neurology, who were aged between 3 months and 18 years and who were diagnosed with epilepsy. The cases considered in the current study, however, were patients that had an EEG record prior to initiating treatment and an MRI within the first six months following diagnosis. RESULTS: Among 222 patients, 212 (95.5%) had IED, and 92 (41.4%) had abnormal MRI results. The most frequently seen abnormalities detected by MRI were encephalomalacia, hydrocephaly, and atrophy. Among patients who had IED, the ones with multifocal IED were documented to have a statistically significant higher rate of abnormalities in MRI scans. In other patients, IED had no significant correlation with structural lesions detected by MRI. CONCLUSION: IED can be unrelated to MRI findings. Focal IED were not statistically concordant with the structural lesions detected by MRI. However, for the cases with multifocal discharges revealed by interictal EEG, the rate of abnormalities detected using MRI was 68%. Therefore, the likelihood of detecting abnormalities using MRI in patients with multifocal IED does support the necessity of the use of MRI in early diagnosis stages.

Magnetic resonance imaging findings in newly diagnosed epileptic children.

OBJECTIVES: Epilepsy is one of the most common chronic neurologic disorders in childhood and it affects 0.5-1% of children. The purpose of the study was to determine the prevalence and types of structural abnormalities in the epileptic children. METHODS: The study was performed in Near East University and Dr. Suat Gunsel University in North Cyprus. It was conducted at pediatric neurology outpatient clinic of the hospital. The records of 1 to 18 years old epileptic children in whom Magnetic Resonance Imaging (MRI) performed within 6 months after diagnosis were enrolled to the study between the dates of October 2011 and June 2017. RESULTS: Among 220 children; 131 (59.55%) had no abnormality and 89 (45.45%) had at least one abnormality in the MRI. Most commonly documented lesions were generally encephalomalacia, hydrocephaly and brain atrophy with a percent of 5.90 (13 cases), 5.45 (12 cases) and 4.55 (10 cases) respectively. Sixty nine (31.06%) of the patients had one abnormality whereas 20 (9.09%) had two or more lesion. CONCLUSION: Abnormality in MRI examination in newly diagnosed epileptic children was high. These high rates may be due to enrollment of children with new emerging epilepsy on a chronical neurologic disorder. Additionally 20 (9.09%) of patients had a concomitant lesion. Secondary lesions were detected in cases with corpus callosum abnormality, atrophy, encephalomalacia and hydrocephaly. Primarily formed lesions are unknown; further studies are needed to confirm these findings.

A propósito de un caso: enfermedad de Kawasaki incompleta en un niño con hipogammaglobulinemia / A case report: incomplete Kawasaki disease in a hypogammaglobulinemic child

La enfermedad de Kawasaki (EK) es una vasculitis autoinmunitaria sistémica que afecta a los vasos pequeños y medianos. La complicación principal de la enfermedad de Kawasaki es el aneurisma de las arterias coronarias, cuyo riesgo es más alto si se retrasa el diagnóstico y el tratamiento. Si bien hasta la fecha se han presentado casos de EK completa e incompleta en diferentes tipos de enfermedades por inmunodeficiencia, no se ha informado acerca de la evolución clínica de la EK en pacientes con hipogammaglobulinemia (HG). En este artículo, se presenta un caso de diagnóstico de EK incompleta en un niño con HG transitoria de la infancia. También se resumen casos previamente informados de EK e inmunodeficiencia. En el caso de una inmunodeficiencia, las infecciones recurrentes pueden ocultar la EK, lo que retrasa el diagnóstico y aumenta el riesgo de complicaciones. En pacientes inmunodeficientes, debe tenerse en cuenta la posibilidad de EK cuando la fiebre es prolongada.

Kawasaki Disease (KD) is a systemic autoimmune vasculitis that affects small and medium sized vessels. Main complication of Kawasaki Disease is coronary artery aneurism, which has higher risk in case of delayed diagnosis and treatment. Although, complete and incomplete KD cases in different types of immune deficiency diseases have been presented up to date, clinical course of KD in patients with hypogammaglobulinemia (HG) has not been reported. Herein, a case diagnosed as incomplete KD in a child with transient HG of infancy has been reported. Previously reported cases with KD and immunedeficiency have also been summarized. Recurrent infections in case of immunedeficiency may mask KD disease resulting in delay in diagnosis and increased risk of complication. KD should be kept in mind in immunedeficient patients in case of prolonged fever.

A case report: incomplete Kawasaki disease in a hypogammaglobulinémie child. / A propósito de un caso: enfermedad de Kawasaki incompleta en un niño con hipogammaglobulinemia.

Kawasaki Disease (KD) is a systemic autoimmune vasculitis that affects small and medium sized vessels. Main complication of Kawasaki Disease is coronary artery aneurism, which has higher risk in case of delayed diagnosis and treatment. Although, complete and incomplete KD cases in different types of immune deficiency diseases have been presented up to date, clinical course of KD in patients with hypogammaglobulinemia (HG) has not been reported. Herein, a case diagnosed as incomplete KD in a child with transient HG of infancy has been reported. Previously reported cases with KD and immunedeficiency have also been summarized. Recurrent infections in case of immunedeficiency may mask KD disease resulting in delay in diagnosis and increased risk of complication. KD should be kept in mind in immunedeficient patients in case of prolonged fever.

La enfermedad de Kawasaki (EK) es una vasculitis autoinmunitaria sistémica que afecta a los vasos pequeños y medianos. La complicación principal de la enfermedad de Kawasaki es el aneurisma de las arterias coronarias, cuyo riesgo es más alto si se retrasa el diagnóstico y el tratamiento. Si bien hasta la fecha se han presentado casos de EK completa e incompleta en diferentes tipos de enfermedades por inmunodeficiencia, no se ha informado acerca de la evolución clínica de la EK en pacientes con hipogammaglobulinemia (HG). En este artículo, se presenta un caso de diagnóstico de EK incompleta en un niño con HG transitoria de la infancia. También se resumen casos previamente informados de EK e inmunodeficiencia. En el caso de una inmunodeficiencia, las infecciones recurrentes pueden ocultar la EK, lo que retrasa el diagnóstico y aumenta el riesgo de complicaciones. En pacientes inmunodeficientes, debe tenerse en cuenta la posibilidad de EK cuando la fiebre es prolongada.

Prevalence of Thalassemia Trait & Iron Deficiency Anemia during Infancy in 2011-2013 in a Thalassemia Prevalent Region: North Cyprus.

BACKGROUND: Iron Deficiency Anemia (IDA) is an important health problem all around the world especially in developing countries. In the Mediterranean countries another prevelant reason of anemia is Thalassemia. Certain strategies had been established as a government policy to reduce prevalence in North Cyprus, such as pre-marital screening of Thalassemia. The prevalence of thalassemia trait has not been evaluated since then. The aim of this study was to detect the prevalence of IDA, thalassemia trait in infants under regular follow-up and to evaluate the compliance to prophylactic iron supplementation (PIS) and its effect on IDA. METHODS: Healthy children admitted to Department of Pediatrics, Near East University Hospital, in 2011-2013 were included. Data of anthropometric measurements, parental thalassemia trait status, duration of PIS usage, complete blood count, ferritin levels and hemoglobin electrophoresis were collected from hospital database program. Anemic children were grouped as IDA, thalassemia trait, both IDA and thalassemia trait and others. RESULTS: Eighty-nine infants with a mean age 13.52±2.09 mo were included. Compliance with PIS recommendation was 85.3% and, the mean duration of iron usage was 6.44±3.18 mo. IDA and thalassemia trait were found to be 11.2% and 4.5% respectively, while 3.4% of the infants had both IDA and thalassemia trait. CONCLUSION: Prevalence of thalassemia trait was 7.9% demonstrating approximately a 50% decline within 5 decades. This result confirms the success of premarital screening policy in North Cyprus. In addition, prevalence of IDA was relatively low being 14.6% supporting the beneficial effect of PIS on prevention of IDA.

A case of pediatric age anticholinergic intoxication due to accidental Datura stramonium ingestion admitting with visual hallucination.

Datura stramonium (DS) is a hallucinogenic plant that can produce anticholinergic toxicity because of its significant concentrations of toxic alkaloids, such as atropine, hyoscyamine, and scopolamine. DS grows in both rural and urban areas in Turkey. Clinical findings of toxicity are similar to those of atropine toxicity. DS abuse is common among adolescents because of its hallucinatory effects. However, accidental DS poisoning from contaminated food is very rare. Accidental poisonings are commonly seen among children. Children are more prone to the toxic effects of atropine; ingestion of even a small amount can cause serious central nervous system symptoms. Treatment is supportive; antidote treatment is given rarely. An eight-year-old male with accidental DS poisoning who presented to the Pediatric Emergency Department with aggression, agitation, delirium, and visual hallucinations is reported.