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Öğe Beta-ketothiolase deficiency brought with lethargy: Case report(Sage Publications Ltd, 2011) Arica, Vefik; Arica, Secil Gunher; Dag, Huseyin; Onur, Hatice; Obut, Omer; Gulbayzar, SayatBeta-ketothiolase deficiency is a rare autosonnal recessive disorder of isoleucine and ketone body metabolism. This disorder is clinically characterized by ketoacidotic attacks. Ketoacidosis, vomiting, and dehydration, lethargy and coma may be seen during attacks. A 9-month-old girl was admitted to our hospital with acidosis and dehydration. The patient was lethargic. Ketoacidosis was suspected because of acetone odor on her breath. Her blood glucose level was 262 mg/dL and urine ketone was (++++). Branched chain amino acid levels were elevated in her blood sample. Organic acid analysis of urine revealed 2-methylacetoacetyl-CoA thiolase deficiency. This was reported because of rarity of the disease and we should consider it in the differential diagnosis of ketoacidotic episodes.Öğe Closed lip schizencephaly: A case report(Dr Behcet Uz Cocuk Hastaliklari Ve Cerrahisi, 2012) Arica, Vefik; Karakus, Ali; Silfeler, Ibrahim; Arica, Secil Gunher; Altas, Murat; Tutanc, Murat; Basarslan, FatmagulSchizencephaly, has been defined as an almost symmetrical bilateral cleft along the cerebral hemisphere. Clinical findings in patients with schizencephaly depends on the type of clefts (on of off type) and the amount of malformed parenchyma. Incidence of schizencephaly is unclear, while a population-based study performed in the United States reported its incidence as 1.54: 100000. These types of cortical developmental malformations had variable clinical signs depending on the function of the affected area which range widely from mental-motor retardation and epilepsy to focal neurological disorders. We also presented a very rare case with bilateral cleft palate aged 4 years who was referred with recurrent afebrile convulsive episodes and diagnosed as schizencephaly, subependimal heterotopia and temporal lobe epilepsy by cranial MRIÖğe Comparison of Lung graphy and thorax computed tomography findings in children with Tuberculin skin test positive(Medknow Publications & Media Pvt Ltd, 2012) Efe, Evrim; Arica, Vefik; Dogan, Murat; Arica, Secil GunherAim: Benefit of thorax computed tomography was investigated when lung graphy remains insufficient in the diagnosis of tuberculosis. Materials and Methods: 55 patients across children with tuberculin skin test (TST) positive nonspecific symptom who applied to the tuberculosis polyclinic of our hospital were investigated between ages of 0-18, determined positivity in their TST test or with tuberculosis contact story. Age, gender, complaining, TST results, existence/number of BCG scar, underlying disease, contact status, cigarette exposure, laboratory findings, Lung graphy and thorax CT findings of patients were compared. Results: Average age of cases was 110, 38 +/- 42, 8 months. Lung graphy results of 36.4% of cases were abnormal while 63.6% were normal. Results of 30.9% (n=17) were abnormal while 69.1% (n=38) of cases were normal. No compliance is observed between results of CT and Lung graphy (p<0.05). Lung graphy was found to have a low positive true diagnosis value, a higher negative true diagnosis value. A significant difference is observed between tuberculosis sighting case and scar numbers of BCG (p<0.05). Conclusion: Taking lung graphy and thorax CT is beneficial and significant in the investigation of tuberculosis disease in patients with positivity of tuberculin skin test. Computed tomography is more sensitive method in comparison to lung graphy in terms of all findings.Öğe Comparison of Quantiferon Test with Tuberculin Skin Test for the Detection of Tuberculosis Infection in Children(Springer/Plenum Publishers, 2012) Onur, Hatice; Hatipoglu, Sami; Arica, Vefik; Hatipoglu, Nevin; Arica, Secil GunherThe efficacy of Quantiferon-TB gold test (QFT-GIT) remains to be documented in pediatric population. Tuberculin skin test (TST) is a conventional test available for the diagnosis of latent tuberculosis infection (LTBI). We aimed to investigate the concordance between QFT-GIT and TST in children with and without tuberculosis infection. Ninety-seven patients, aged 3 months-14 years, admitted to pediatric outpatient clinics of Dr. Sadi Konuk Training Hospital BakA +/- rkoy, Turkey between March 2008 and April 2009 were recruited. Demographic features, TST results, history of exposure to active tuberculosis (TB), chest X-ray findings, clinical history, presence of Bacillus Calmette Guerin (BCG) vaccination scar were recorded. Patients were categorized into four groups namely, active TB, LTBI, no TB and healthy. It was found that BCG scar positivity did not influence QFT-GIT results. There was a statistically significant agreement between QFT-GIT and TST results (kappa = 0.486; p < 0.01). In patients a parts per thousand yen5 years of age, TST positivity and QFT positivity had a significant relationship (p < 0.01). In all patient groups, sensitivity and specificity was 65.85 % and 82.14 %, respectively. In active TB group, TST and QFT-GIT results demonstrated significant agreement ratio of 40.8 % (kappa = 0.364; p < 0.01). Sensitivity and specificity was 100 % and 30 %, respectively. Utilization of QFT-GIT in the diagnosis of LTBI reduces false-positive results and prevents unnecessary treatment with INH and its adverse effects.Öğe Congenital Agenesis of the Left Lung: A Rare Case(Medknow Publications & Media Pvt Ltd, 2011) Yetim, Tulin Durgun; Bayarogullari, Hanifi; Yalcin, Hlya Polat; Arica, Vefik; Arica, Secil GunherPulmonary agenesis is a rare congenital anomaly, the etiology of which is not clearly known. Other systemic comorbidities such as cardiovascular, gastrointestinal, musculoskeletal, and urogenital system anomalies can be observed in more than half of the patients. It is usually diagnosed during childhood. Diagnosis in adulthood is very rare. We present a case of pulmonary agenesis diagnosed in an adult.Öğe The effect of the length of the prodromal period on the metabolic control within the first 2 years in children with diabetic ketoacidosis manifestation(Walter De Gruyter & Co, 2011) Murat, Zehra; Arica, Vefik; Dogan, Murat; Arica, Secil GunherPurpose: We investigated the effects of clinical and laboratory properties at the time of the initial application of patients recently diagnosed and presenting metabolic indicators of diabetic ketoacidosis who were given disease prognoses in years 1 and 2 after discharge. Materials and methods: A total of 94 patients admitted to Bakirkoy Maternity and Children's Diseases Training and Research Hospital with diabetic ketoacidosis and recently diagnosed with type 1 diabetes mellitus were investigated. Patient files were examined within 2 years following discharge. Findings: All 94 study patients (53.2% male and 46.8% female) presented acidosis, ketonuria and hyperglycemia. While a moderate correlation was detected between the prodromal period and HbA(1c) values in year 1, only a slight correlation was seen in HbA(1c) values in year 2. In addition, a slight correlation was observed between the prodromal period and the number of hospitalizations due to diabetic ketoacidosis in the first year. Again, while a moderate correlation was observed between HbA(1c) values and the number of hospitalizations due to diabetic ketoacidosis in year 1, only a slight correlation was seen in year 2. The prodromal period was directly proportional to patient age. Results: Hospital admissions may be reduced through appropriate treatment, follow-up and metabolic control of patients with type 1 diabetes mellitus. In addition, we report a relationship between the prodromal period and HbA(1c) values in type 1 diabetes patients.Öğe Evaluation of hemoglobinopathy screening results of a six year period in Turkey(Drunpp-Sarajevo, 2012) Arica, Secil Gunher; Turhan, Ebru; Ozer, Cahit; Arica, Vefik; Silfeler, Dilek Benk; Silfeler, Ibrahim; Altun, Ayse BetulBackground and Objectives: Hemoglobinopathies are autosomal recessive inherited diseases more commonly seen in Mediterranean countries. Hereditary blood diseases including Bthalassemia and sickle cell anemia are important health problems. In our study we aimed to analyze the results of the premarital hemoglobinopathy screening test for a 6 years period in Hatay region. Material and Methods: The study sample comprised the couples attending to the Mother and Child Health Care Center in Hatay for premarital hemoglobinopathy screening from 2004 to 2009. Hemoglobin chain analyses of 87.830 couples were evaluated. Results: 175.660 people were screened at total. The prevalence of beta thalassemia trait, sickle cell anemia trait, sickle cell anemia, beta thalassemia major, beta-thalassemia intermedia, alphathalassemia, alpha-thalassemia trait was found as 13.921 (7,9%), 6.074 (3,4%), 631 (0.35%), 132 (0.07%), 118 (0.06%), 9 (0.005%), 150 (0.08%) respectively. 72 newborns with betathalassemia were diagnosed as a result of the marriage of the carrier couples in 6 years. Conclusions: Hatay is a high risk region for beta-thalassemia and sickle cell anemia trait. In countries with high prevalence of hemoglobinopathies, a premarital screening program and counseling is needed to decrease the prevalence.Öğe Knowledge, attitude and response of mothers about fever in their children(Bmj Publishing Group, 2012) Arica, Secil Gunher; Arica, Vefik; Onur, Hatice; Gulbayzar, Sayat; Dag, Huseyin; Obut, OmerAim This study was conducted to determine mothers' knowledge about fever, and their attitudes and responses to fever in children, along with the influence of demographic characteristics on the level of knowledge, and to identify occurrences of fear of fever. Materials and Methods The study consisted of 4500 mothers with children 0-12 years of age who were referred to the pediatric health and disease polyclinic and primary care polyclinic over a period of 24 months. Data were collected using a questionnaire containing 32 open-ended, multichoice questions, and assessed using number, percentage and chi(2) analyses. Results In this study, 36% of mothers regarded body temperatures lower than 37 degrees C as fever; 83% believed that fever was harmful for their children, and 92.3% experienced fear and concern due to their child's fever. The most significant reason for fear was the belief that the child may have a seizure. Moreover, 12% of mothers feared that their child would die due to fever. The level of fear triggered by fever was lower as the education level of the mothers increased. Meanwhile, when their child had a fever, 28.9% of mothers used antifebrile drugs without consulting a physician, while 19% applied cold water and 7.7% applied water with alcohol or vinegar, among other inappropriate practices. Conclusion Increased information about fevers geared towards the caregivers of children, particularly mothers, would prevent the unnecessary treatment of children, as well as minimising delayed and insufficient responses to fever.Öğe Methemoglobinemia due to prilocaine administration(Galenos Yayincilik, 2012) Arslan, Mehmet Tayip; Arica, Vefik; Tutanc, Murat; Arica, Secil GunherPrilocaine is the one of the most utilized agents during dorsal penile block in order to prevent pain during circumcision. It is known that prilocaine may lead to methemoglobinemia in neonates and infants. Here we present a 56 days old male patient with prilocaine induced methemoglobinemia who had been referred to intensive care unit for his frank and instant cyanosis after circumcision. Detailed history taking revealed that he needed 1 mg/kg prilocaine administration twice for adequate anesthesia. Patient had frank cyanosis at administration with no other sign. During admission rate of methemoglobin was 16,2% in the blood of the patient and conservative measures lead to complete resolution of all symptoms. Since infants below six months of age are particularly prone to methemoglobinemia due to their low cytochrome b5 levels and fetal hemoglobine is closer to ferric state than adult hemoglobine, it is emphasized in this paper that other alternative anesthetic agents should be utilized instead of prilocaine for regional anesthesia in infants under six months age. (Turk Arch Ped 2012; 47: 305-6)Öğe Neurobrucellosis as a rare complication of brucellosis: three pediatric case reports(Aves, 2012) Arica, Vefik; Silfeler, Ibrahim; Altas, Murat; Tutanc, Murat; Arica, Secil Gunher; Evirgen, Omer; Davran, RamazanBrucellosis is a major worldwide zoonosis. The disease is endemic in developing countries, particularly in the Mediterranean basin. Brucellosis is frequently seen in Eastern and South-Eastern Anatolia regions in Turkey. Around 18 000 new cases are reported annually in Turkey. Any organ or system of the body can be involved. Brucellosis is an infection due to Brucella species and is characterized by acute febrile illness, sweating, weakness, and headache. Involvement of the central nervous system is rare in brucellosis. Brucella is continuing to be an important health problem especially who live in the countryside. The clinicians usually rule out neurobrucellosis in the differential diagnosis of brucellosis. The three neurobrucellosis cases presented in this paper referred with various non-specific symptoms. Only the first patient was found to have hydrocephalus on CT imaging, while the CT images of the other patients were normal. The second case had a history of consumption of fresh herby cheese, however the other cases had no such history. These three patients were found to have recovered without sequela during their long term follow up. The aim of this presentation is to draw attention to the importance of neurobrucellosis especially in endemic regions including our country. (Turk Arch Ped 2012; 47: 218-21)Öğe Role of proper physical examination in diagnosis of cutaneous manifestation (Erythema Nodosum) of primary tuberculosis(Professional Medical Publications, 2011) Arslan, Mehmet Tayip; Arica, Vefik; Tutanc, Murat; Arica, Secil GunherErythema nodosum (EN), which is a rare skin manifestation among children, is associated with a wide variety of disease processes. Here a 10 year old female patient with EN is presented. She was initially suspected to have coagulation abnormality by many physicians. She was diagnosed to have primary tuberculosis. This paper stresses the importance of proper physical examination in cutaneous lesions and of ruling out primary tuberculosis in endemic countries in children presenting with EN.Öğe TYPE 1 CROUZON SYNDROME WITH HEADACHE(Nobel Ilac, 2013) Arica, Vefik; Tutanc, Murat; Bayarogullari, Hanifi; Arica, Secil Gunher; Basarslan, Fatmagul; Davran, Ramazan; Korkmaz, InanCrouzon syndrome defined by French neurosurgeon Crouzon in 1912. Crouzon syndrome which is characterized by craniosynostosis and dysmorphic facial appearance. This autosomal dominant disease has an incidence rate of 16/1,000,000. Craniosynostosis premature closure of cranial sutures, results in craniofacial anomalies. 4.5% of cases with craniosynostosis have Crouzon syndrome. Craniosynostosis can occur in utero or in the first three years of life. It rarely occurs later Phenotypically specific types of craniosynostosis have been linked to fibroblast growth factor receptor gene (FGFR) mutations. Clinical findings of Crouzon, Apert and Pfeifer syndromes are secondary to FGFR-2 gene mutations. In Crouzon syndrome brachycephaly, ptosis, exophthalmos, hypertelorism, acanthosis nigricans, rostrate type nose, ear and palate anomalies can occur of as a result of premature closure of cranial sutures. In this report we present a 5 years old male with headache. He has Crouzon Syndrome and beaten copper appearance in craniography.Öğe Vitamin B12 deficiency with presenting nutritional difficulty: A case report(Dr Behcet Uz Cocuk Hastaliklari Ve Cerrahisi, 2012) Arica, Vefik; Silfeler, Ibrahim; Arica, Secil Gunher; Tutatanc, Murat; Dogan, Murat; Gucuk, SebahatVitamin B12 deficiency is among important causes of megaloblastic anemia. In developing countries, the major reason of Vitamin B12 deficiency is deficient dietary intake. Although vitamin B12 deficiency is a rare condition, early diagnosis and treatment are important to prevent permanent neurological sequela. Anorexia is seen frequently in children. We want to remind Vitamin B12 deficiency with this case presented with anorexia. A 12 month-old male patient presented with vomiting, weight loss, paleness, malnutrition, repulsion as well as growth retardation. Physical examination revealed paleness and loss of interest to environment. Hematologic test results were as follows: WBC: 6500 gr/dl, Hgb: 8.8 gr/dl, MCV: 96 fl, platelet count: 162.000/mm(3). Mean serum folic acid level of the patient was 19.4 ng/mL (3-22.4 ng/mL), and vitamin B12 level 72 pg/mL (200-1210 pg/mL), while mean vitamin B12 level of the mother was 96 pg/mL. The patient was diagnosed as vitamin B12 deficiency and intramuscular Vitamin B12 treatment was initiated. One month later, his vomiting ceased, his appetite was well and he gained 850 gr. His interest to environment was improved. We want to impress with this case that infants with anorexia might have vitamin B12 deficiency. Treatment of vitamin B12 deficiency is important because it might lead to permanent neurological sequela and vitamin B12 deficiency respond to treatment dramatically.
