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Haloperidol, psikoz, Tourette Sendromu ve agir ajitasyonlarda sikca kullanilan ve intoksikasyon durumunda antikolinerjik etkiler ve ekstrapiramidal belirtilerin gorulebildigi tipik antipsikotik bir ilactir. Babasi tarafindan agri kesici olarak bir adet haloperidol ( © Norodol 20 mg) tablet verildikten 8 saat sonra intoksikasyon bulgulari (yesil renkli gorme ve hemidistoni) ortaya cikan 12 yasindaki erkek hastayi bildiriyoruz. Biz bu olgumuzla, distoninin yanlislikla konvulziyon olarak degerlendirilebilecegini ve haloperidolun tek dozunda bile intoksikasyon belirtilerinin ortaya cikabilecegini, ayrica piyasada bulunan ve hastanin anamnezini alan hekimi yaniltabilecek sonu '–dol' ile biten agri kesicilerle ( © Endol vb.) son ek olarak isim benzerligi gosteren haloperidolun ( © Norodol) acil degerlendirme gereken durumlarda karisikliklara yol acabilecegini vurgulamak istedik.

Cytokines are possibly one of the factors responsible for death due to Crimean-Congo hemorrhagic fever (CCHF). This study aimed to determine the differences between the cytokine levels in children and adult patients with CCHF; the influence of cytokines; and the severity of the course of the disease, which seems to be milder in children. Thirty-four children and 36 adult patients diagnosed with CCHF between 2010 and 2011 were included in this study. Diagnosis was performed by serology or by the polymerase chain reaction for CCHF virus. Levels of IFN-γ, TNF-α, IL-1β, IL-2, IL-4, IL-5, IL-6, IL-9, IL-10, IL-12 p70, IL-13, IL-17A, and IL-22 were measured in all serum samples. Although the disease had a fatal course in three adult patients, there were no deaths in children. Statistically significant differences were not observed between the cytokine concentrations in the adults and children. No differences were detected between the serum cytokine levels in the children with moderate and those with a severe clinical course of the disease. In the adult patients with fatal outcome, significantly higher serum levels of IL-2, IL-5, IL-9, IL-12 p70, and IL-13 were detected as compared to the cytokine levels in patients who survived the infection. No differences were detected between the serum levels of IFN-γ, IL-1β, IL-17A, IL-22, IL-10, IL-6, IL-4, and TNF-α in the patients who died and those who survived. Thus, the milder clinical course in children with CCHF cannot be explained by the cytokine network alone. The incomplete maturation of the immune system and timing and scale of immune responses could change the outcome dramatically.

WOS: 000371909200007, PubMed ID: 26928096, Aim The aim of the study is to discuss clinical effects, treatments, and outcomes of pediatric colchicine poisoning. Method This study was designed as an observational case series study. The medical records of children aged between 0 and 18 years, who were hospitalized for colchicine poisoning at the Department of Pediatric Intensive Care Unit, Cumhuriyet University Faculty of Medicine, between January 2010 and January 2012, were retrospectively evaluated. Results We presented 17 children with colchicine poisoning. The mean (SD, range) age of patients was 71.5 (69.19, 18-204) months. The period to apply to the hospital after taking the medications was 7.3 hours (7.97, 30 minutes-26 hours) on average. The use of colchicine was due to diagnosis of Familial Mediterranean fever (FMF) in the families of 8 patients, diagnosis of Behcet disease in 1 patient's father, diagnosis of Behcet disease in 1 patient herself, and diagnosis of FMF in 6 patients themselves. Thirteen patients had taken colchicine at the dose of less than 0.5 mg/kg known as subtoxic and 1 patient had taken colchicine at the dose of greater than 0.8 mg/kg, and doses taken by 3 patients were not known. Fourteen patients (82.4%) had involuntary drug intake. Fifty percent of them were symptomatic at the moment of application and all had gastrointestinal complaints. All patients were observed in intensive care unit upon first admission and received supportive care. One of patients showed total alopecia, one showed leucocytosis, and another one showed acute abdomen picture. None of the patients showed mortality. Conclusions Mortality of colchicine toxicity is high and quick assessment is absolutely required. In regions where FMF is common and the use of colchicine is high, clinicians should pay attention to symptoms and findings related to colchicine intoxication and keep them in mind in differential diagnosis.

Alkaptonüri, homogentisik asid oksidaz eksikliği ile karakterize ve nadir görülen bir metabolik hastalıktır. Karakteristik özellikleri, idrar renginde koyulaşma, okranozis ve artropatidir. Pediatrik yaş grubunda idrar renginin koyulaşması tek bulgudur ve hastalığın çok erken dönemlerinde aile tarafından belirtilebilir. Goldenhar sendromu, okuloaurikulovertebral displazi olarak da bilinir ve nadir görülen bir tablodur. Kulak, burun, yumuşak damak, dudak ve mandibula gibi organların vücudun tek tarafında yeterince gelişememesi sözkonusudur. Renal ve genitoüriner malformasyonlar bu kompleks içinde bildirilmiştir. Goldenhar sendromu tanılı ondört aylık erkek hasta kliniğimize nefrolitiazis ve idrarının değdiği kısımlardaki çamaşırının siyaha dönmesi yakınması ile başvurdu. Özellikle orta ve geç erişkin dönemde alkaptonüri ile birlikte ürolitiazis olguları bildirilmiştir. Bununla birlikte bu komplikasyonun çocuklarda görülmesi konusunda yeterince veri yoktur. Bu vaka erken çocukluk döneminde alkaptonüri tanısı konması, nefrolitiazis komplikasyonunun gelişmiş olması ve Goldenhar sendromu ile birlikte görülmesi nedeni ile sunulmuştur., Alkaptonuria is a rare disorder of metabolism characterized by deficiency of homogentisic acid oxidase. Characteristic features include darkening of urine, ochronosis, and arthropathy. Darkening of urine is the only sign of the disorder in the pediatric age group. Goldenhar syndrome, also known as oculoauriculovertebral dysplasia, is an uncommon condition, characterized by a incomplete development of the ear, nose, soft palate, lip, and mandible. Renal and genitourinary malformations have also been reported with the complex. A 14-month-old boy with Goldenhar syndrome presented to our clinic with the complaint of nephrolithiasis, and bluish black staining of clothes. There are reports on nephrolithiasis in alkaptonuria (AKU) in middle and late adulthood patients. However, the occurrence of this complication in affected children has not been analysed so far. This report is discussed for the early diagnosis, the development of complication of nephrolithiasis and co-existence with Goldenhar syndrome.

WOS: 000291794100002, PubMed ID: 21511509, Background: Crimean-Congo hemorrhagic fever (CCHF) is a zoonotic viral disease with a high mortality rate, and is one of the viral hemorrhagic fever syndromes. The average mortality rate of CCHF is 3-30%. Research indicates that the longest incubation period after a tick bite is 12 days in CCHF disease. However, in clinical practice, we encounter patients with CCHF as a result of tick bites with much longer incubation periods (max. 53 days) than those reported in the literature. We present herein CCHF cases presumably infected through tick bites and having incubation periods longer than the upper limit reported in the literature. Methods: We analyzed the cases of the 825 CCHF patients admitted to our hospital from 2007 to 2010 and found that 312 of them had undoubtedly been bitten by a tick. We searched the patient records for information on the incubation period and found that 12 patients had experienced an incubation period of over 12 days, which is the longest incubation period stated in the literature for patients definitely bitten by a tick. Results: A total of 12 patients (eight males and four females, with a mean age of 45 years) were recruited into this study. Five (41.7%) of the 12 patients had positive CCHF virus-specific IgM antibodies, three (25%) had a positive reverse transcription polymerase chain reaction test for CCHF virus, and four (33.3%) had positive results in both tests during the acute and/or convalescent phase of the disease. In these cases, the interval between tick bite and the onset of symptoms was a mean of 23.6 days (range 13-53 days). Conclusion: Physicians serving in endemic regions should be aware of these longer incubation periods after a tick bite. It is suggested that they perform more follow-ups on clinically and serologically highly suspected patients than they currently do. (C) 2011 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Springer India, The aim of the present study was to perform a genotype/phenotype and gender correlation in children in addition to investigate distribution and frequency of the Mediterranean fever gene mutations. Records of 700 children with familial Mediterranean fever living in Sivas-Central Anatolia were retrospectively reviewed. Most common clinical features were fever (100 %), abdominal pain (92.7 %), and arthritis (23.3 %). Mutations were detected in 66.7 % of patients. Simple heterozygous, compound heterozygous, and homozygous mutations were detected respectively 66.4, 8.6 and 25 % of patients. The most frequent mutations were M694V, E148Q, M680I (G/C), V726A, and A744S. The mean severity score was higher in homozygous M694V, in heterozygous M694V and in heterozygous E148Q mutations than in others. It was higher in girls than in boys. Arthritis and abdominal pain had a meaningful relation with M694V and E148Q mutations. Appendectomy and rare complications (oral aphthae, scrotal edema, Henoch Schönlein purpura, and amyloidosis) were significantly seen more in M694V and in E148Q than in other mutations. High acute phase reactant response was highest in M694V and in E148Q. Ten patients had amyloidosis and 4 of them had recurrent Henoch Schönlein purpura. This study showed that, in addition to patients with M694V mutations, patients with E148Q mutations also had a more severe clinical course. Apart from that, disease severity score was higher in girls than in boys and the co-existence of Henoch Schönlein purpura and familial Mediterranean fever is significant. © 2014, The National Academy of Sciences, India., Duksal, F.; Division of Pediatric Immunology and Allergy, Department of Pediatrics, Cumhuriyet School of Medicine, Cumhuriyet UniversityTurkey

WOS: 000360090300003, PubMed ID: 25504688, Proinflammatory and anti-inflammatory cytokines have an important role in the pathogenesis of febrile seizures (FS). Adipocytokines like interleukin 6 (IL-6), leptin, and adiponectin released from adipose tissue play a role in inflammation. This study aimed to assess the probable role of adipose tissue in children with FS. We measured serum IL-6, leptin, and adiponectin levels and evaluated clinical and laboratory findings in children with FS (n = 32) and compared the results with the values of children of the same age with febrile illness without seizures (febrile control, FC; n = 26) and healthy control group (HC; n = 29). The serum levels of white blood cells, C-reactive protein, IL-6, leptin, and adiponectin were found to be significantly higher, while serum hemoglobin (Hb) levels were found to be significantly lower in FS and FC groups than in the HC group (p < 0.001). When we compared the FS with the FC group, the serum Hb levels were significantly lower in the FS group than those in the FC group (p = 0.001). There was no significant difference between the FS and FC group with regard to the serum levels of these adipocytokines (p > 0.05). Our data showed that elevated levels of these adipocytokines as acute phase reactants in FS and FC groups did not contribute to the development of FS.

WOS: 000352702000018, PubMed ID: 25866343, Background: Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne viral zoonosis. Clinical reports indicate the severity of CCHF is milder in children than adults. The chemokines are important chemoattractant mediators of the host immune system. Objectives: The main aim of the study was to identify whether or not there were any differences in chemokine levels between the pediatric and adult patients and control groups, and whether there was any correlation with disease severity. Study design: The serum levels of select chemokines including chemokine (C-C) ligand 2 (CCL2), CCL3, CCL4, chemokine (C-X-C) ligand 8 (CXCL8), CXCL9, and granulocyte-colony stimulating factor (G-CSF) in 29 adult and 32 pediatric CCHF patients and in 35 healthy children and 40 healthy adult control groups were studied by flow cytometric bead immunoassay method. Results: Great variability was detected in the serum levels of the chemokines for both the adult and pediatric patients and controls. With the exception of G-CSF, the median serum levels of CCL2, CCL3, CCL4, CXCL8, and CXCL9 were found to be significantly higher in the adult patients compared to adult controls (2364.7 vs. 761 pg/ml; 714.1 vs. 75.2 pg/ml; 88.6 vs. 25.5 pg/ml; 217.9 vs. 18.3 pg/ml; 875 vs. 352.2 pg/ml, respectively, p < 0.0001 for all comparisons). Among the chemokines the median CCL4 and G-CSF levels were significantly higher in the pediatric patients compared to pediatric controls (40.3 vs. 7.1 pg/ml, p < 0.0001; 0.1 vs. 0.1 pg/ml, p = 0.049, respectively). Conclusion: The results of this study showed prominent chemokine raising in adult CCHF patients compared to children CCHF patients. (C) 2015 Elsevier B.V. All rights reserved.

WOS: 000349908800020, PubMed ID: 25170551, Background: Apoptosis is a main regulator in responses of cellular immunity throughout systemic viral infections. Perforin, soluble Fas ligand, caspase-3 and caspase-cleaved cytokeratin-18 (M-30) are mediators of apoptosis. The aim of this study is the evaluation of Crimean-Congo hemorrhagic fever (CCHF) disease changes in the levels of these apoptotic markers and the relation of these changes with disease severity. Methods: Forty-nine hospitalized children with CCHF and 36 healthy controls were enrolled in this prospective study. The CCHF patients were classified into 2 groups based on disease severity (severe group and nonsevere group). Demographic characteristics and clinical and laboratory findings of all patients were recorded on admission. Results: Serum perforin, caspase-3 and soluble Fas ligand levels were found to be significantly higher both in the severe and nonsevere CCHF groups than the healthy control group (P < 0.05), but there was no significant difference in these apoptotic markers between severe and nonsevere CCHF groups (P > 0.05). In addition, serum M-30 levels did not differ significantly among all groups (P > 0.05). There was a positive correlation between serum values for perforin, caspase-3 and M-30 and the disease's severity criteria such as aspartate aminotransferase and/or alanine aminotransferase. The serum levels of all these markers were negatively correlated with disease severity criteria such as the platelet count. Conclusions: In this study, we concluded that the interactions of cytolytic granules containing perforin and caspase cascade and Fas-FasL may play an important role in the pathogenesis of CCHF in children., Cumhuriyet University BAP [T-539], This study was supported by the Cumhuriyet University BAP with project number: T-539. The authors thank the Refik Saydam National Public Health Agency, a national reference laboratory in Turkey, for testing the serum samples.

WOS: 000330621300012, PubMed ID: 23942784, Impaired cardiac myocardial function may contribute to the risk for sudden unexpected death of a patient with epilepsy. This study aimed to investigate the effect of antiepilepsy drugs (AEDs) on cardiac function in pediatric epileptic patients using standard and tissue Doppler imaging (TDI) echocardiography. This hospital-based, prospective cross-sectional study investigated 52 epileptic children (mean age 9.3 +/- A 3.1 years) treated with AEDs (duration 2.4-10.0 years) and 36 healthy children (mean age 9.5 +/- A 4.0 years). In the epilepsy group, standard echocardiography showed increased left ventricular (LV) end-diastolic and end-systolic diameters, an increased LV mass index, and preserved ejection fraction. The patients also exhibited increased mitral peak A-wave velocity and mitral E-wave deceleration time as well as a decreased mitral E/A ratio. The E/Em ratio was significantly higher in the epilepsy group (5.6 +/- A 1.2) than in the control group (5.2 +/- A 1.1) (p = 0.016). In the epilepsy group, TDI showed an increased isovolumetric relaxation time and myocardial performance index (MPI). It also exhibited decreased early diastolic velocity (Em) and a decreased mitral annular displacement index in these patients. There were positive correlations between the LV lateral wall MPI (r = 0.231), septal MPI (r = 0.223), and LV mass index (p < 0.05) but no correlation with the duration of AED treatment. The authors detected subclinical ventricular dysfunction associated with AEDs at a preclinical stage. They suggest that TDI can be useful for determining the short- and long-term cardiac effects of AEDs.

WOS: 000330751500005, PubMed ID: 24451097, In this study, we investigated the pro- and antioxidant status of patients with a pathogenesis of Crimean-Congo hemorrhagic fever (CCHF) in terms of their role in its pathogenesis. During the study period, 34 children and 41 adults were diagnosed with CCHF. The control group consisted of healthy age- and gender-matched children and adults. Serum levels of the total antioxidant capacity (TAC), total oxidant status (TOS), oxidative stress index (OSI), and plasma total thiol (TTL) were evaluated and compared between groups. The difference in mean TAC values between CCHF patients and healthy controls was not statistically significant (P > 0.05). Mean TOS, OSI, and TTL values were significantly lower in CCHF patients than in healthy controls (P < 0.001). Comparisons between the 2 groups revealed that mean TOS and OSI values were significantly lower in adults with CCHF than in their healthy counterparts (P < 0.001). Similarly, mean TTL levels were lower in both children and adults with CCHF when compared separately with healthy controls (P < 0.05). There was no significant difference in the mean serum TTL levels between children and adults with CCHF (P > 0.05). Our results suggest that TTL may play a more important role in CCHF pathogenesis than the other parameters investigated. The mean TOS and OSI values were higher in the control group than in CCHF patients.

WOS: 000329360400008, PubMed ID: 24359422, Objective: We aimed to assess the association between resting heart rate (RHR) and severe infection in children with Crimean-Congo hemorrhagic fever (CCHF). Methods: In all, 121 patients under 18 years of age with a laboratory-confirmed diagnosis of CCHF were enrolled in the study. Patients were classified into two groups based on disease severity (severe group and nonsevere group). RHR was measured by electrocardiography (ECG) on admission. Maximum P-wave duration (Pmax), P-wave dispersion (Pd), QRS duration, corrected QT interval, and QT dispersion were also measured. Results: Mean age was 11.43.9 years and 84 patients were male. Twenty-six patients were classified as severe. Patients in this group had a higher RHR (103.6 +/- 10.4vs. 80.5 +/- 8.1, p=0.001) than those with nonsevere disease. There was no difference in Pmax, Pd, QRS duration, QTcmax, or QTc dispersion. The optimal cutoff value of RHR to predict disease severity was>96 beats per minute (bpm), with 70.6% sensitivity and 50.1% specificity. Bleeding, thrombocytopenia (80x10(9)/L), elevated aspartate transaminase (AST) (>208IU/L), elevated alanine transaminase (ALT) (>87IU/L), elevated lactate dehydrogenase (LDH) (>566IU/L), long activated partial thromboplastin time (aPTT) (>42s), and increased hospitalization days were more frequent in patients with RHR >96bpm. Multivariate logistic regression analysis revealed low platelet count (42s), high LDH (>566IU/L), and elevated RHR (>96bpm) as independent risk factors for severe disease. Conclusions: We conclude that elevated RHR was significantly associated with severe disease in children with CCHF, thus offering the potential to identify patients with increased risk.

Walker Warburg Sendromu (WWS) otozomal resesif geçiş gösteren göz, beyin ve kasların etkilendiği nadir bir doğumsal musküler distrofi tipidir. Bu yazıda Cumhuriyet Üniversitesi Çocuk Sağlığı ve Hastalıkları kliniğinde takip edilen üç WWS olgusu sunulmuştur. Olgularımızdan biri bilgilerimize göre rapor edilmiş en uzun yaşayan olgudur., Walker-Warburg syndrome (WWS), a rare form of congenital muscular dystrophy, is an autosomal recessive disorder affecting the brain, eye, and muscles. In this study, three WWS cases followed in the pediatric clinic of Cumhuriyet University are presented. According to our knowledge, one of our patients is the longest- lived case who has been reported.

Cytokines are possibly one of the factors responsible for death due to Crimean-Congo hemorrhagic fever (CCHF). This study aimed to determine the differences between the cytokine levels in children and adult patients with CCHF; the influence of cytokines; and the severity of the course of the disease, which seems to be milder in children. Thirty-four children and 36 adult patients diagnosed with CCHF between 2010 and 2011 were included in this study. Diagnosis was performed by serology or by the polymerase chain reaction for CCHF virus. Levels of IFN-γ, TNF-α, IL-1β, IL-2, IL-4, IL-5, IL-6, IL-9, IL-10, IL-12 p70, IL-13, IL-17A, and IL-22 were measured in all serum samples. Although the disease had a fatal course in three adult patients, there were no deaths in children. Statistically significant differences were not observed between the cytokine concentrations in the adults and children. No differences were detected between the serum cytokine levels in the children with moderate and those with a severe clinical course of the disease. In the adult patients with fatal outcome, significantly higher serum levels of IL-2, IL-5, IL-9, IL-12 p70, and IL-13 were detected as compared to the cytokine levels in patients who survived the infection. No differences were detected between the serum levels of IFN-γ, IL-1β, IL-17A, IL-22, IL-10, IL-6, IL-4, and TNF-α in the patients who died and those who survived. Thus, the milder clinical course in children with CCHF cannot be explained by the cytokine network alone. The incomplete maturation of the immune system and timing and scale of immune responses could change the outcome dramatically.

SUMMARY Levetiracetam is a novel anticonvulsant drug that was approved by the FDA in adult and pediatric convulsions. There is not sufficient information regarding the use of newborn such as the appropriate dose and interval, its safety, efficacy and adverse effects. We present a case of a 10 day-old male, who was accidentally administered 75 mg/kg/d levetiracetam for 3 days by his mother at home. The patient was discharged 4 days after admission to hospital without any signs and symptoms. The case demonstrated that high dose levetiracetam might be safe in newborn convulsions. Keywords: Levetiracetam, newborn, convulsion, dose, safe OZET Levatirasetam eriskin ve pediatrik konvulziyonlarda FDA tarafindan onaylanan yeni birantikonvulziyon ilactir. Yenidogan kullanimi ile ilgili doz ve araligi, guvenligi, etkinligi ve yan etkileri konusunda yeterli bilgi bulunmamaktadir. Annesi tarafindan evde 3 gun boyunca kaza ile 75 mg/kg/gun levatirasetam verilen 10 gunluk bir erkek bebek olguyu sunuyoruz. Hasta, hastaneye basvurduktan 4 gun sonra hicbir bulgu ve semptom olmadan taburcu edildi. Bu olgu yuksek doz levatirasetam yenidogan konvulziyonlarinda kullanilabilecegini gosterdi. Anahtar sozcukler: Levatirasetam, yenidogan, konvulziyon, doz, guvenlik

WOS: 000293140200008, PubMed ID: 21709631, Background: Tularemia is a zoonotic infection, and the causative agent is Francisella tularensis. A first-line therapy for treating tularemia is aminoglycosides (streptomycin or, more commonly, gentamicin), and treatment duration is typically 7 to 10 days, with longer courses for more severe cases. Material/Methods: We evaluated 11 patients retrospectively. Failure of the therapy was defined by persistent or recurrent fever, increased size or appearance of new lymphadenopathies and persistence of the constitutional syndrome with elevation of the levels of the proteins associated with the acute phase of infection. Results: We observed fluctuating size of lymph nodes of 4 patients who were on the 7(th) day of empirical therapy. The therapy was switched to streptomycin alone and continued for 14 days. The other 7 patients, who had no complications, were on cefazolin and gentamycin therapy until the serologic diagnosis. Then we evaluated them again and observed that none of their lymph nodes regressed. We also switched their therapy to 14 days of streptomycin. After the 14 days on streptomycin therapy, we observed all the lymph nodes had recovered or regressed. During a follow-up 3 weeks later, we observed that all their lymph nodes had regressed to the clinically non-significant dimensions (< 1 cm). Conclusions: All patients were first treated with gentamicin, but were than given streptomycin after failure of gentamicin. This treatment was successful in all patients. The results of our study suggest that streptomycin is an effective choice of first-line treatment for pediatric oropharyngeal tularemia patients.

WOS: 000295899700014, PubMed ID: 21804465, Background: Crimean-Congo hemorrhagic fever (CCHF) involves the multi-organ systems. The involvement of the heart in adult patients has been described previously. We investigated the electrocardiographic and echocardiographic findings of pediatric patients with CCHF. Material/Methods: Patients younger than 16 years of age diagnosed with CCHF were enrolled in the study. The diagnosis of CCHF infection was based upon typical clinical and epidemiological Findings and serological tests. All patients underwent a thorough cardiologic evaluation. A standard 12-lead electrocardiography and echocardiography were performed. Results: Twenty-three consecutive patients who were hospitalized with diagnosis of CCHF were enrolled in the study (mean age: 12 2 years, 6 female). All electrocardiographic parameters were within normal ranges according to age. Seven patients (30%) had minimal (

Dandy-Walker variant is a rare anomaly of the central nervous system that is characterized by partial agenesis of vermis and results in persistence of the 4. ventricule and cisterna magna. Firstly, in 1988 Kohyama et al. [10] reported the coexistence of Dandy-Walker variant and tetralogy of Fallot. Afterwards, it is reported as several case reports in the literature. In this case report, we report a 2 days-old male patient with Dandy-Walker variant and pentalogy of Fallot., ÖzetDandy-Walker varyasyonu, 4. ventrikül ve sisterna magna arasındaki devamlılıkla sonuçlanan vermisin parsiyel agenezisi ile karakterize santral sinir sisteminin nadir bir anomalisidir. Literatürde ilk kez 1988’de Kohyama ve arkadaşları tarafından bildirilen Dandy-Walker varyasyonu ve Fallot Tetralojisi birlikteliği daha sonraki yıllarda birkaç vaka sunumu şeklinde bildirilmiştir. Biz bu yazıda, Dandy-Walker varyasyonu ve Fallot Pentalojisi olan 2 günlük bir erkek hastayı bildiriyoruz.Anahtar sözcükler: Dandy-Walker varyasyonu, Fallot Pentalojisi AbstractDandy-Walker variant is a rare anomaly of the central nervous system that is characterized by partial agenesis of vermis and results in persistence of the 4. ventricule and cisterna magna. Firstly, in 1988 Kohyama et al. reported the coexistence of Dandy-Walker variant and tetralogy of Fallot. Afterwards, it is reported as several case reports in the literature. In this case report, we report a 2 days-old male patient with Dandy-Walker variant and pentalogy of Fallot.Keywords: Dandy-Walker variant, pentalogy of Fallot