A case of Bartonellosis mimicking systemic lupus erythematosus.

BACKGROUND AND AIM: Cat-scratch disease (CSD) is a systemic bacterial infection caused by Bartonella henselae. The disease is typically characterized by regional lymphadenopathy developing after scratches from domestic or feral cats. Rarely, systemic involvement may be observed. The co-occurrence with glomerulonephritis and positive antinuclear antibody (ANA) tests have been reported before. In these cases, the disease can be misdiagnosed as systemic lupus erythematosus. Ocular involvement occurs in 5%-10% of the cases with CSD, and neuroretinitis is among the common manifestations. Administration of corticosteroids (CSs) in addition to antibiotics has been shown to improve prognosis in neuroretinitis cases. However, the optimal dose and duration, remain ill-defined. CASE REPORT: In this article, we present an 11-year-old girl with CSD and neuroretinitis with a positive ANA test and hematuria, who benefited from high-dose methyl-prednisolone and antibiotics. CONCLUSION: Further research is warranted in order to determine the dose and duration of CSs in the treatment of Bartonella neuroretinitis.

CAMLG-CDG: a novel congenital disorder of glycosylation linked to defective membrane trafficking.

The transmembrane domain recognition complex (TRC) pathway is required for the insertion of C-terminal tail-anchored (TA) proteins into the lipid bilayer of specific intracellular organelles such as the endoplasmic reticulum (ER) membrane. In order to facilitate correct insertion, the recognition complex (consisting of BAG6, GET4 and UBL4A) must first bind to TA proteins and then to GET3 (TRC40, ASNA1), which chaperones the protein to the ER membrane. Subsequently, GET1 (WRB) and CAML form a receptor that enables integration of the TA protein within the lipid bilayer. We report an individual with the homozygous c.633 + 4A>G splice variant in CAMLG, encoding CAML. This variant leads to aberrant splicing and lack of functional protein in patient-derived fibroblasts. The patient displays a predominantly neurological phenotype with psychomotor disability, hypotonia, epilepsy and structural brain abnormalities. Biochemically, a combined O-linked and type II N-linked glycosylation defect was found. Mislocalization of syntaxin-5 in patient fibroblasts and in siCAMLG deleted Hela cells confirms this as a consistent cellular marker of TRC dysfunction. Interestingly, the level of the v-SNARE Bet1L is also drastically reduced in both of these models, indicating a fundamental role of the TRC complex in the assembly of Golgi SNARE complexes. It also points towards a possible mechanism behind the hyposialylation of N and O-glycans. This is the first reported patient with pathogenic variants in CAMLG. CAMLG-CDG is the third disorder, after GET4 and GET3 deficiencies, caused by pathogenic variants in a member of the TRC pathway, further expanding this novel group of disorders.

Coats plus syndrome: a rare cause of severe gastrointestinal tract bleeding in children - a case report.

BACKGROUND: Coats plus syndrome, cerebroretinal microangiopathy with calcifications and cysts, is a rare disease with autosomal recessive pattern occurring due to a mutation in CTC1, encoding conserved telomere maintenance component 1, gene. Besides retinal involvement, abnormalities in brain and osteopenia, serious life-threatening bleeding in gastrointestinal tract and portal hypertension can be observed. CASE PRESENTATION: A 6-year-old girl with Coats plus syndrome presented to the pediatric emergency department with vomiting blood and blood in stool. An upper and lower gastrointestinal endoscopy revealed esophageal varices and vascular telangiectasia in the pyloric antrum, duodenum, and colon. She received palliative care and the bleeding was stopped after receiving intravenous octreotide. She then was followed in the pediatric gastroenterology, neurology, and ophthalmology clinics. She was later hospitalized and admitted to the intensive care unit as she continued to have intermittent gastrointestinal system bleeding. She eventually died due to severe gastrointestinal system bleeding. CONCLUSIONS: Coats plus syndrome can lead to life-threatening gastrointestinal bleeding and portal hypertension. As Coats plus syndrome is quite rare, there is little published data on this syndrome. This report presents a case of Coats plus syndrome as a rare cause of gastrointestinal bleeding and portal hypertension.

A Case of Smith-Lemli-Opitz Syndrome Diagnosed with Hypertrophic Pyloric Stenosis.

Smith-Lemli-Opitz syndrome (SLOS) is caused by a deficiency in the enzyme 7-dehydrocholesterol reductase (DHCR7) that results in an abnormality in cholesterol metabolism. SLOS is inherited as an autosomal recessive genetic disorder. In this case, we describe a 34-day-old patient with postnatal progressive projectile vomiting, diagnosed with hypertrophic pyloric stenosis, who was suspected to have SLOS during treatment clinical and biochemical profile. A 34-day-old patient with progressively worsening vomiting and abdominal distention, diagnosed as hypertrophic pyloric stenosis, was operated by pediatric surgery department. After operation, the patient required pediatric intensive care unit admission due to respiratory distress, anemia, hypoalbuminemia, and generalized edema. Physical examination of our patient revealed dysmorphic facial features, finger anomalies, sacral dimple, and ambiguous genitalia, with chromosomal determination as XY. Molecular genetic testing was performed, and mutations in the DHCR7 gene of homozygous c.1342G>A/p.Glu448Lys (rs80338864) were detected. Infants with progressive projectile vomiting, feeding problems, and multiple anomalies with dysmorphic facial anomalies may be suspected to have SLOS and their families should be advised to have genetic testing and genetic counseling.

Midterm Neuromotor Development Results of Preterm Babies less than 34 Weeks Gestational Age.

OBJECTIVES: This study aimed to evaluate the neuromotor development of premature babies and to determine the risk factors affecting neuromotor development in the middle time (3 years). METHODS: All babies with ≤34 weeks gestational age and born between 2011-2014 and hospitalized in our neonatal clinic were included in this study. Prenatal, perinatal and postnatal features of the babies were recorded. Consent was obtained from the families who had an outpatient follow-up and agreed to participate in this study. Neurological examination and Denver II Developmental Screening Test (DDST-II) were applied to babies and their results were recorded. Factors affecting neurodevelopment were evaluated. RESULTS: Complete data for 96 of the study infant were obtained. Fifty (52.1%) of the cases were female. The mean birth weight was 1542±518 grams. The mean corrected age was 20.9±10.7 months at the time of the examination. It was found cerebral palsy in 11 babies (11.5%) with the neurological examination and developmental retardation in 15 babies (15.6%) with DDST-II. Low birth weight, a gestational period of 25-26 weeks, Apgar score at 5th minute <7 were found to be the main risk factors for cerebral palsy and abnormal DDST-II result (p<0.05). In babies with abnormal neurological examination, the frequency of bronchopulmonary dysplasia, sepsis and intraventricular hemorrhage were found to be high (p<0.05), and in babies with abnormal DDST-II results the frequency of respiratory distress syndrome, bronchopulmonary dysplasia and sepsis were found to be high (p<0.05). CONCLUSION: In our study, abnormal neurological examination rate was found 11.5% in preterm infants with gestational age ≤34 weeks, and the rate of abnormal DDST-II was found 15.6%. The main factors affecting neuromotor development were gestational week, birth weight and 5th minute Apgar score. The frequency of bronchopulmonary dysplasia, sepsis and intraventricular hemorrhage in babies with abnormal neurological examination, and the frequency of respiratory distress, bronchopulmonary dysplasia and sepsis were found to be high in babies with abnormal DDST-II.

Neuromotor Development Evaluation of Preterm Babies Less than 34 Weeks of Gestation with Bayley III at 18-24 Months.

OBJECTIVES: To assess and evaluate the risk factors affecting the neuromotor development of preterm babies at corrected age 18 to 24 months. METHODS: Preterm babies ≤ 34 weeks of gestational age (GA) who were born in our hospital between 2011 and 2014 were prospectively included in the study. Prenatal, perinatal, and postnatal features of the babies were recorded. Bayley Scales of Infants and Toddler Development, Third Edition (Bayley-III), was applied at corrected age 18 to 24 months. RESULTS: All data of 96 babies were obtained during the study, mean birth weight was 1542 ± 518 g, and mean corrected age was 20.9 ± 4.7 months. Cerebral palsy was found in 11 babies (11.5%). According to Bayley III scores, 13.5% cognitive delay, 19.8% language delay, and 33.3% motor delay rations were detected. A positive correlation was found between GA and motor composite scores (p = 0.011). The mean motor composite score was lower in babies with the Apgar score less than 7 at 1st and 5th minutes (p = 0.007 and p = 0.003) and applied resuscitation in the delivery room (p = 0.033). The mean language composite score was found to be higher in babies with antenatal steroid administration (p = 0.003). A negative correlation was found between the motor composite score and the oxygen treatment time and mechanical ventilation support time (p = 0.001 and p = 0.007). CONCLUSION: In preterm babies less than 34 weeks, the birth weight, GA, Apgar score, oxygen treatment time, mechanical ventilation support time, and resuscitation in a delivery room were determined to affect the Bayley III motor score. Language development was found better in babies with antenatal steroid administration.

Retrospective analysis of children with myelin oligodendrocyte glycoprotein antibody-related disorders.

BACKGROUND: Knowledge has been expanding on myelin oligodendrocyte glycoprotein (MOG) antibody-associated central nervous system disorders. We delineate the clinical and paraclinical findings and outcome of our pediatric patients with MOG antibody seropositive disease. METHODS: We retrospectively analyzed the clinical presentation, cerebrospinal fluid findings, magnetic resonance imaging (MRI) studies, course and outcome of children seropositive for anti-MOG IgG. RESULTS: Total 20 children with neurological symptoms and serum anti-MOG IgG were identified from six centers in Turkey. Median age at onset was 9 years (mean 8.8 ±â€¯5.0 years, range: 1.5-16.5 years). Final diagnoses were acute disseminated encephalomyelitis (ADEM) (n = 5), ADEM + optic neuritis (n = 4), neuromyelitis optica spectrum disorder (NMOSD) (n = 3), myelitis (n = 2), relapsing optic neuritis (n = 2), multiphasic DEM (n = 3), and unclassified relapsing demyelinating disease (n = 1). Seven/20 (35%) children experienced a single episode while 13/20 (65%) had a least one relapse during follow-up. On MRI, subcortical white matter, brainstem, and corpus callosum were preferentially involved regions. Full recovery was observed in 15/20 (75%) children. CONCLUSION: MOG autoimmunity in children has a wide clinical spectrum, tendency to relapse, and a favourable outcome compared with other relapsing demyelinating diseases.

Guillain-Barré syndrome in children: subtypes and outcome.

OBJECTIVE: This study reviews the clinical features, subtypes, and outcomes of childhood Guillain-Barré syndrome (GBS). METHODS: Fifty-four children who attended a tertiary care training and research hospital in Turkey were enrolled in the study. RESULTS: The mean age was 6.5 ± 4.2 years and 32 patients (59.5%) were male. The most common subtype of GBS was acute inflammatory demyelinating polyneuropathy (AIDP), which was seen in 27 patients (50%). Having antecedent history, especially upper respiratory tract infection was significantly more common in AIDP (P = 0.028). Sensorial symptoms were significantly more frequent in axonal type GBS (P = 0.001). When we compare the demyelinating and axonal forms, all of the groups had favorable outcome. CONCLUSION: The diagnosis of pediatric GBS can be delayed because of its variable presentation. Early admission to hospital and early treatment are important for decreasing the need for respiratory support and improving the outcome.

Microsomal triglyceride transfer protein gene mutations in Turkish children: A novel mutation and clinical follow up.

Abetalipoproteinemia (ABL; OMIM 200100) is a rare autosomal recessive disease that affects the absorption of dietary fats and fat soluble vitamins. Here, we describe the clinical and genetic characteristics of three patients with ABL. Two patients (patients 1 and 2) who were carriers of the c.398-399delAA mutation (previously known mutation) had developmental delay and hepatic steatosis developed at the age of five in patient 1. Patient 3 was the carrier of a novel mutation (g.10886-10902delAAGgtaagtttgtgttg in intron 3 and c.506A>T exon 5) in microsomal triglyceride transfer protein (MTP) gene and had hepatic steatosis.

Outcomes of Botulinum Toxin Type A Injection Followed by Rehabilitation in Cases of Cerebral Palsy With Upper Extremity Involvement.

We evaluated the efficiency of botulinum toxin type A injection followed by a rehabilitation program including individual therapy, group therapy, and occupational therapy in cases of cerebral palsy with upper extremity involvement. A total of 29 injections were performed on 25 patients, and the patients were placed on rehabilitation program. At 3-month and 6-month assessments, there was a significant improvement in lateral grip strength, 9 Hole Peg test, Upper Limb Physician's Rating Scale and pediatric functional independence measure total scores. There were significant decreases in active range of motion in elbow extension, supination, and wrist extension, and Modified Ashworth Scale in elbow flexion, elbow pronation, and wrist flexion at 6-week, 3-month, and 6-month assessments. Combination of group therapy with traditional therapy methods after injection is effective in cases of cerebral palsy with upper extremity involvement.

The evaluation of risk factors for recurrent hospitalizations resulting from wheezing attacks in preschool children.

BACKGROUND: We aimed to evaluate the risk factors in preschool children admitted to inpatient services with a diagnosis of recurrent attacks of wheezing. METHOD: The medical files of 44 preschool children with 2 or more recurrent hospitalizations resulting from wheezing between November 2011 and January 2012 were retrospectively investigated. RESULTS: There were 28 males (64 %) and 16 females. The median age was 14 months (2.0-50). The median numbers of previous wheezing attacks and hospitalizations were 4 (2-10) and 2 (2-8), respectively. Fourteen patients (32 %) had been treated for gastroesophageal reflux (GER). The previous and recent hospital evaluations were investigated. Bronchopulmonary dysplasia and anemia were significantly more common in patients with 3 or more hospitalizations for wheezing than in those with 2 hospitalizations (p = 0.010 and p < 0.001, respectively). A review of the cases with 3 or more hospitalizations revealed that a history of GER and anemia were significant risk factors. CONCLUSION: Anemia and GER are risk factors for recurrent hospitalizations resulting from wheezing and should be treated. If the history and physical examination suggest asthma, inhaler therapy treatment should be administered, with other investigations planned for patients who do not respond to treatment as expected.

A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system caused by the measles virus (MV). Its prevalence remains high in resource poor countries and is likely to increase in the Northern Europe as vaccination rates decrease. Clinical knowledge of this devastating condition, however, is limited. We therefore conducted this multinational survey summarizing experience obtained from more than 500 patients treated by 24 physicians in seven countries. SSPE should be considered in all patients presenting with otherwise unexplained acquired neurological symptoms. In most patients, the diagnosis will be established by the combination of typical clinical symptoms (characteristic repetitive myoclonic jerks), a strong intrathecal synthesis of antibodies to MV and typical electroencephalogram findings (Radermecker complexes). Whereas the therapeutic use of different antiviral (amantadine, ribavirin) and immunomodulatory drugs (isoprinosine, interferons) and of immunoglobulins has been reported repeatedly, optimum application regimen of these drugs has not been established. This is partly due to the absence of common diagnostic and clinical standards focusing on neurological and psychosocial aspects. Carbamazepine, levetiracetam, and clobazam are the drugs most frequently used to control myoclonic jerks. We have established a consensus on essential laboratory and clinical parameters that should facilitate collaborative studies. Those are urgently needed to improve outcome.

Colorectal Cancer due to Constitutional Mismatch Repair Deficiency Mimicking Neurofibromatosis I.

Colorectal carcinoma (CRC) is an extremely rare tumor of childhood that can be associated with cancer predisposition syndromes. A patient with CRC related to constitutional mismatch repair deficiency (CMMRD) syndrome with features of neurofibromatosis type 1 (NF-1) is presented here. A 13-year-old boy was admitted for a 4-month history of diarrhea and rectal bleeding. The patient had extensive café au lait spots, freckling, and Lisch nodules. He fulfilled the NF-1 diagnostic criteria. Colonoscopy showed numerous polyps and a colorectal mass lesion, of which a biopsy revealed adenocarcinoma, an uncommon pathology associated with NF-1. High microsatellite instability and homozygous mutation of PMS2 gene in tumor tissue and blood lymphocytes, respectively, confirmed the diagnosis of CMMRD. Unfortunately, because family history related to CMMRD was negative, the parents denied the diagnosis and refused the therapy, and the patient was lost to follow-up. CMMRD is a rare cancer predisposition syndrome with phenotypical features resembling NF-1. The disease may be suspected in the setting of NF-1 features and CRC, high-grade brain tumors, or hematologic malignancies. Lack of family history related to CMMRD may be a major obstacle to convincing parents of the presence of an inherited disease in their progeny.

Pediatric syncope: is detailed medical history the key point for differential diagnosis?

Syncope is a transient loss of consciousness as a result of global cerebral hypoperfusion. It is generally benign but may be a sign of pathology. The purpose of this study was to analyze the frequency of syncope due to cardiac, neurocardiogenic, neurologic, situational, psychiatric, and other causes and make a differential diagnosis of syncope types according to detailed medical history and further investigations. We examined prospectively 268 children presented to pediatric polyclinics as well as cardiology and neurology departments (age range, 1-18 years) with a primary complaint of syncope for the study. Cardiac syncope was diagnosed in 12 patients, neurocardiogenic syncope in 232, neurologic syncope in 9, psychiatric syncope in 9, situational in 4, and benign paroxysmal positional vertigo in 2. The neurologic syncope group consists of patients diagnosed with epilepsy after evaluation. Eight patients in the cardiac syncope group were found to have diseases such as long QT syndrome, and the remaining patients had hypertrophic cardiomyopathy, atrioventricular nodal reentry tachycardia, ventricular tachycardia, and a second-degree heart block that can cause sudden death. In conclusion, syncope is a common problem in childhood that requires hospitalization. Because it may be the first finding of an underlying malignant cardiac or neurologic disease, clinicians must be very careful during medical evaluation. An electrocardiogram and a medical history including the details of the event, chronic diseases, and familial diseases are among the most important steps for the right diagnosis and prognosis. Instead of a routine procedure, further diagnostic workup should be directed according to medical history for high yield. Convulsive movements may be defined in all types of syncope related with cerebral hypoxia, and this may lead to a misdiagnosis of seizure by the clinician.

Viral etiology in infants hospitalized for acute bronchiolitis.

Acute bronchiolitis is predominantly a viral disease. Respiratory syncytial virus is the most common agent, but other newly identified viruses have also been considered as causes. The aim of the present study is to determine the respiratory viruses causing acute bronchiolitis in hospitalized infants. Infants younger than 2 years of age who were hospitalized for acute viral bronchiolitis in a children's hospital between November 2011 and May 2012 were evaluated for the presence of viruses as etiologic agents using a realtime polymerase chain reaction method.A total of 55 infants were included in this study. The mean age of the children was 6.98±5.53 months, and 63.6% were male. In the 55 children, 63 viruses were detected. A single viral pathogen was detected in 47 (85.5%) patients, and two viruses were co-detected in 8 (14.6%) patients. Respiratory syncytial virus was the most common virus identified, accounting for 25 (45.5%) cases, followed by rhinovirus (n=9, 16.4%), and human metapneumovirus (n = 8, 14.5%).Although respiratory syncytial virus remains the major viral pathogen in infants hospitalized for acute broncholitis, more than half of bronchiolitis cases are associated with other respiratory viruses.

The association between vitamin D status and recurrent wheezing.

OBJECTIVE: To investigate association between vitamin D status and recurrent wheezing in infants. METHODS: Thirty infants with recurrent wheezing and 45 healthy, similar aged infants without any history of acute or chronic illness were included in the study. The clinical features of infants were recorded and serum 25-hydroxyvitamin D [25(OH)D] levels were measured. Data analysis was performed using SPSS 13 package program. RESULTS: The mean value of 25 (OH) D vitamin levels were 22.1 ± 8.9 IU/L and 18.8 ± 11 IU/L for the control and recurrent attack group respectively. Seventy-three percent of subjects with recurrent wheezing had vitamin D levels in the deficient range (<20 ng/ml) and 48.9 % had vitamin D levels under < 20 ng/ml in the control group. The percentage of insufficient vitamin D levels (<30 ng/ml) were 90 and 77.8 for the patient and control group respectively. Eight patients had extremely deficient vitamin D (<10 ng/ml) levels. There was no statistical significance between the groups in terms of the distribution of 25 (OH)D level. CONCLUSIONS: The present study did not demonstrate significant association between vitamin D status and recurrent wheezing in the infants.

Differences in iron deficiency anemia and mean platelet volume between children with simple and complex febrile seizures.

OBJECTIVE: The relationship between iron deficiency anemia and febrile seizures (FSs) were examined in several studies before. The aim of our study is to find out the differences regarding iron deficiency anemia, demographic characteristics and mean platelet volume (MPV) which is an inflammatory marker between simple and complex febrile seizure groups. METHODS: In this study, the authors investigated the recordings of 493 children with a diagnosis of simple and complex febrile seizure, aged between 6 months and 6 years, followed between 2002 and 2010 retrospectively. RESULTS: Mean age and male/female ratio were similar in two groups. There was no significant difference regarding with age, gender and family history of FS between two groups. We found significant difference statistically with respect to gestational age, consanguinity, family history of epilepsy and birth weight between two groups. The mean levels of Hb, Htc, MCV were lower and Plt and RDW levels were higher in children with CFS than SFS group, the differences were statistically significant (p: 0.001). A higher proportion of children with CFS (16.2%) had iron deficiency anemia compared to SFS group (12.1%). Mean platelet volume (MPV) of CFS (7.99±0.96fL) were significantly lower than that of SFS group (8.77±0.75) (p<0.001). CONCLUSIONS: The results of this study suggests that iron deficiency anemia is more frequently seen among the patients with CFS than the patients with SFS. The lower levels of MPV as an inflammatory marker, supports the idea that CFS is a brain inflammatory disease and the consequence of this inflammatory mechanism is the development of the epilepsy. Further studies are necessary to highlight the relationship between iron metabolism, inflammation and seizures.

Assessment of bone density in children with cerebral palsy by areal bone mineral density measurement.

The aim of this cross-sectional study was to investigate the frequency of decreased areal bone mineral density (aBMD) among patients with cerebral palsy (CP), as estimated by using various aBMD Z-score adjustment methods. In addition, this study examined factors related to decreased aBMD scores. One hundred and two children between the ages of 3.2 and 17.8 years were examined. In patients with severe CP, the incidences of decreased aBMD according to various adjusting methods based on decimal age, bone age, height age, and height-for-age Z-score (HAZ) were 79.5%, 69.5%, 51.9%, and 38.3%, respectively. Abnormal levels of calcium, phosphorus, alkaline phosphatase, parathyroid hormone, or anticonvulsant were not predictive for a decreased aBMD. Mean aBMD Z-scores were significantly lower in all aBMD Z-score adjustment methods in patients with severe CP compared to patients with mild-to-moderate CP, except for the adjustment method based on HAZ.

Multilevel botulinum toxin type a as a treatment for spasticity in children with cerebral palsy: a retrospective study.

INTRODUCTION: Cerebral palsy is the most common cause of physical disability in children. Spasticity is a disabling clinical symptom that is prevalent among patients suffering from cerebral palsy. The treatment of spasticity with botulinum toxin type A (BTX-A) is a well-established option in the interdisciplinary management of spasticity, providing focal reductions in muscle tone in cerebral palsy patients. OBJECTIVE: The aim of this retrospective study was to describe the effect of multilevel BTX-A injections in the lower extremities, focusing mainly on gross motor function and functional status in cerebral palsy patients. METHODS: Data from 71 cerebral palsy patients (64% male, 36% female, mean age 6.7 +/-3.2 years) were analyzed retrospectively. We used the Ashworth and Tardieu scales to evaluate the degree of spasticity. Motor function was measured by the Gross Motor Function Measure (GMFM-88), and functional status was classified by the Gross Motor Function Classification System (GMFCS I-V). Multilevel BTX-A injections were applied after sedation and with electrostimulation guidance. The evaluations were repeated every three months, and the patients were followed for six months. RESULTS: We found that the Ashworth and Tardieu scores decreased significantly at the three-month evaluation (p<0.05) but not at the six-month evaluation (p>0.05). Although the improvement in spasticity was not maintained at the six-month evaluation, GMFM-88 scores increased significantly at the three- and six-month assessments. GMFSC levels showed no change in the three- and six-month assessments. CONCLUSION: We believe that a single multilevel BTX-A injection reduces spasticity and improves motor function in children with cerebral palsy.

Cardiac effects of carbamazepine treatment in childhood epilepsy.

OBJECTIVE: Hypotension, bradycardia, and atrioventricular block are well-known cardiovascular effects of carbamazepine (CBZ). However, direct effects of CBZ on ventricular functions have been rarely encountered. We investigated the effect of CBZ on ventricular functions in pediatric patients without previous cardiac disease to determine whether CBZ causes ventricular dysfunction or electrocardiographic changes in therapeutic doses in children. METHODS: The study includes 40 patients (31 boys, 9 girls) with epilepsy who had been treated with carbamazepine. Electroencephalography, electrocardiography, echocardiography, and cranial imaging were performed on all patients before treatment and were repeated at the end of the third and 12th months of treatment. The systolic and diastolic thickness of the interventricular septum and the posterior wall of the left ventricle and the systolic and diastolic diameter of the left ventricle were measured during M-mode investigation. The end-systolic and end-diastolic volumes (mL), the stroke volume (mL), fractional shortening (FS), (%) and ejection fraction (EF) (%) measurements were obtained from the computer on the echocardiography device. RESULTS: In 1-year follow-up, no electrocardiographic abnormalities were detected. The FS and EF values showing left ventricular function did not show a significant difference (P > 0.05). CONCLUSIONS: CBZ seems to be a safe drug in pediatric epileptic patients without any preexisting cardiac disease. We suggest that the risk factors should be defined and followed-up regarding cardiac function when treatment is initiated in groups with risk factors.