B Gürakan

American Hospital, İstanbul, Istanbul, Turkey

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Publications (45)122.43 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Factor V (FV) deficiency is a rare coagulation disorder, characterized by a bleeding phenotype varying from mild to severe. To date, 115 mutations have been described along the gene encoding for FV (F5) but only few of them have been functionally characterized. Aim of this study was the identification and the molecular characterization of genetic defects underlying severe FV deficiency in a 7-month-old Turkish patient. Mutation detection was performed by sequencing the whole F5 coding region, exon-intron boundaries and about 300 bp of the promoter region. Functional analysis of the identified missense mutation was conducted by transient expression of wild-type and mutant FV recombinant molecules in COS-1 cells. Two novel mutations: a missense (Pro132Arg) and a 1-bp deletion (Ile1890TyrfsX19) were identified in the F5 gene. While the frameshift mutation is responsible for the introduction of a premature stop codon, likely triggering F5 mRNA to nonsense-mediated mRNA degradation, the demonstration of the pathogenic role of the Pro132Arg mutation required an experimental validation. Expression experiments showed that the missense mutation causes a significant reduction in FV secretion and in the specific activity of the residual secreted molecule (77% and 78% decrease, respectively). This paper reports the identification of two novel mutations responsible for FV deficiency, thus widening the mutational spectrum of the F5 gene. The Pro132Arg mutation adds to the only other two functionally characterized missense defects in the FV A1 domain.
    Haemophilia 07/2011; 18(2):205-10. · 3.17 Impact Factor
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    ABSTRACT: An iron regulatory peptide hormone, hepcidin, is also part of the innate immune system and is strongly induced during infections and inflammation. The aim of the present study was to determine serum levels of the 60 aa pro-hormone form of hepcidin (pro-hepcidin) in full-term and preterm newborns with sepsis and to determine the possible relationships between pro-hepcidin levels and serum iron and complete blood count parameters. Fifteen preterm newborns with sepsis, 17 healthy preterm, six full-term newborns with sepsis and 16 healthy full-term newborns were included the study. Blood samples were collected from patients with sepsis at the time of clinical diagnosis. Each blood sample was analyzed for complete blood count, serum iron and ferritin concentrations, iron-binding capacity, and pro-hepcidin level. The mean serum pro-hepcidin level (mean +/- SD) in preterm neonates with sepsis and in healthy preterm newborns was 565.4 +/- 519.5 ng/mL and 279.8 +/- 227.6 ng/mL, respectively (P < 0.05). The mean serum pro-hepcidin level in full-term newborns with sepsis and in healthy full-term neonates was 981.4 +/- 415.4 ng/mL and 482 +/- 371.9 ng/mL, respectively (P < 0.05). Although the mean serum ferritin levels in the two groups with sepsis were higher when compared with the healthy groups, the difference was not statistically significant in full-term newborns. No statistically significant correlations were found between serum pro-hepcidin levels and any other parameters in each group. Serum pro-hepcidin levels were higher in newborns with sepsis (either premature or full-term) than they were in healthy newborns at the time of clinical diagnosis.
    Pediatrics International 04/2009; 51(2):289-92. · 0.88 Impact Factor
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    ABSTRACT: This article presents the case of a female newborn with irritability, increased tonus, jitteriness, and eating difficulties who was referred to our institution. Her mother had been taking fluoxetine (20 mg daily) during the second and third trimesters of her pregnancy. The infant's signs began on the first day after birth and persisted for 6 weeks. Extensive investigations excluded infective, genetic, and metabolic causes, and a provisional diagnosis of fluoxetine withdrawal was made. There have been few reports of neonatal complications following maternal fluoxetine hydrochloride treatment. According to these, signs occurred within a few days after birth and lasted up to 4 weeks. To our knowledge, our patient demonstrated the longest duration of signs reported to date. We recommend that physicians be aware of these complications in newborns after fetal exposure in utero to selective serotonin reuptake inhibitors. These neonates should be followed-up closely for adverse signs during the first days of life so that signs can be recognized and treated if necessary.
    The journal of maternal-fetal & neonatal medicine: the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 12/2008; 21(12):921-3. · 1.36 Impact Factor
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    ABSTRACT: Small gestational age (SGA) is one of the major causes of fetal mortality and morbidity. Altered maternal homeostasis as a result of point mutations in the coagulation cascade has been reported as an important risk factor for this adverse pregnancy outcome. This study aims to investigate the relationship between mother's thrombophilic mutations and SGA deliveries in our population. The study group was consisted of sixty-six women who gave birth to one or more SGA babies. 104 women who gave birth to appropriate-for-gestational age (AGA) babies were sampled for the control group. Restriction fragment size analysis were performed by visualizing digested PCR products for Factor V Leiden (G1691A), Factor V Cambridge (A1090G), Factor V A1299G, prothrombin G20210A, methylene tetrahydropholate reductase C677T, A1298C and T1317C mutations. The results of this study indicate that maternal C677T (p=0.01) and A1298C (p<0.01) mutations in MTHFR gene may be suggested as risk factors for SGA outcome in our population. Therefore, maternal screening of these two mutations in the first trimester of pregnancy could help in the assessment of patients.
    Thrombosis Research 02/2008; 122(2):175-8. · 3.13 Impact Factor
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    ABSTRACT: Congenital leukemia is rare, and is frequently diagnosed as a form of acute myeloid leukemia at, or immediately after birth. Some infections, viral in particular, can mimic clinical signs and/or laboratory findings of congenital leukemia. This is the first documented case of candidemia resembling leukemia.
    Journal of Maternal-Fetal and Neonatal Medicine 08/2007; 20(7):555-7. · 1.52 Impact Factor
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    ABSTRACT: The pathogenetic mechanisms of hepatic injury in perinatal asphyxia (PNA) are similar to those in ischemic hepatitis, yet liver involvement is currently not considered a component of multi-organ failure in PNA. A retrospective study was done on 56 newborns with PNA. Hepatocyte injury was diagnosed based on elevated serum alanine transaminase level (>100 U/L, twice upper normal) with subsequent normalization. Twenty-two of the patients had hepatocyte injury. Fetal distress, thrombocytopenia, convulsions, pathologic findings on imaging of the central nervous system, and a high rate of intrauterine growth retardation were the factors significantly associated with hepatocyte injury. This damage was also associated with high mortality.
    Journal of Maternal-Fetal and Neonatal Medicine 05/2007; 20(5):407-10. · 1.52 Impact Factor
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    ABSTRACT: No previous study has investigated the full range of complete blood count (CBC) parameters in small-for-gestational-age (SGA) newborns. The main aim of this study was to compare CBC and peripheral smear parameters in term, healthy SGA neonates and appropriate-for-gestational-age (AGA) neonates, and to establish CBC reference values for full-term SGA newborns. One hundred thirty-two healthy, term newborns (73 SGA and 59 AGA) were included. On day 1, we obtained 109 samples and on day 7 we obtained 77 samples. A CBC and peripheral smear were analyzed for each sample collected and group data were compared. We observed higher mean values for normoblast count, hemoglobin, hematocrit, and red blood cell (RBC) count in the SGA babies than in the AGA babies on day 1. The mean corpuscular hemoglobin and mean corpuscular hemoglobin concentration values for the SGA babies were decreased because of the relatively high RBC count and relatively high mean corpuscular volume we observed in this group. Of the SGA newborns, 21.9% had neutropenia and 4.7% had absolute neutrophil counts lower than 1500/microl on day 1. On both day 1 and day 7, the SGA newborns had higher mean absolute metamyelocyte counts and higher mean I : T (immature : total neutrophil ratio) values than the AGA group. The SGA babies had a lower mean absolute lymphocyte count on day 7 than the AGA group. We detected thrombocytopenia in almost one-third of the 64 SGA newborns tested on day 1. In summary, our study clearly demonstrates that CBC parameters for healthy, full-term, SGA newborns are different from those of healthy, term AGA newborns. This is the first study that has documented different mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, metamyelocyte counts, lymphocyte counts, and I : T in SGA babies compared with AGA babies.
    Clinical & Laboratory Haematology 05/2006; 28(2):97-104. · 1.11 Impact Factor
  • Acta Paediatrica 05/2005; 94(4):509-10. · 1.97 Impact Factor
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    ABSTRACT: The aim of this study was to determine what proportion of newborns admitted with idiopathic non-hemolytic hyperbilirubinemia exhibit severe weight loss and hypernatremia. The prospective study involved 115 infants >48 h old who were admitted with jaundice between July 2002 and July 2003, and had unconjugated bilirubin levels >12 mg/dL. Premature babies (gestational age <37 weeks) and those with hemolytic jaundice and other pathologic causes of non-hemolytic jaundice were excluded. Postnatal age (days) at admission, bodyweight at admission, weight change since birth (percentage weight loss calculated at admission) and mode of feeding (breast-feeding, formula feeding, mixed feeding) were recorded. Severe weight loss was defined in babies who showed >10% weight loss or had not regained enough to reach birthweight by postnatal day 10. Serum Na levels and breast-milk Na levels were also measured. Twenty-eight (33%) of the 86 newborns with idiopathic hyperbilirubinemia in the study exhibited severe weight loss. Almost all the 86 babies were exclusively breast-fed, and 10 babies (12%) had severe weight loss combined with hypernatremia. The group with severe weight loss and hypernatremia had higher breast-milk Na levels than the other infants. The results indicate that a large proportion of babies with non-hemolytic jaundice have severe weight loss, and that breast-fed newborns with the combination of weight loss and hypernatremia may present with non-hemolytic jaundice.
    Journal of Paediatrics and Child Health 01/2005; 41(9-10):484-7. · 1.25 Impact Factor
  • A Tarcan, B Gürakan, N Ozbek
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    ABSTRACT: A preterm infant with renovascular hypertension who developed significant trilineage bone marrow suppression after receiving captopril is reported. Captopril-associated pancytopenia is a very rare complication that was thought to be dose-related and usually caused by accumulation of the drug through defective renal tubular excretion. In our patient, it appears that the combination of renal artery stenosis and renal tubular dysfunction of prematurity might have led to pancytopenia. Captopril should be used with caution especially in premature babies and newborns with underlying renal or renovascular disease even if they do not have overt renal dysfunction. Monitorization of these babies with creatinine clearance or free serum captopril levels may help to establish accumulation of the drug before causing pancytopenia.
    Journal of Paediatrics and Child Health 08/2004; 40(7):404-5. · 1.25 Impact Factor
  • Aylin Tarcan, Berkan Gürakan, Filiz Tiker
    Annals of Tropical Paediatrics International Child Health 07/2004; 24(2):187-8. · 0.92 Impact Factor
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    ABSTRACT: Acute renal failure (ARF) is a common problem in the neonatal intensive care unit (NICU). In most cases, ARF is associated with a primary condition such as sepsis, metabolic diseases, perinatal asphyxia and/or prematurity. This retrospective study investigated the course of illness, therapeutic interventions, early prognosis and risk factors associated with development of ARF in the neonatal period. A total of 1311 neonates were treated in our NICU during the 42-month study period, and 45 of these babies had ARF. This condition was defined as serum creatinine level above 1.5 mg/dL despite normal maternal renal function. The data collected for each ARF case were contributing condition, cause and clinical course of ARF, gestational age and birth weight, age at the time of diagnosis, treatment, presence of perinatal risk factors and need for mechanical ventilation. The frequency of ARF in the NICU during the study period was 3.4%. Premature newborns constituted 31.1% of the cases. The mean birth weight in the group was 2863 +/- 1082 g, and the mean age at diagnosis was 6.2 +/- 7.4 days. The causes of ARF were categorized as prerenal in 29 patients (64.4%), renal in 14 patients (31.1%) and postrenal in 2 patients (4.4%). Forty-seven percent of the cases were nonoliguric ARF. Asphyxia was the most common condition that contributed to ARF (40.0%), followed by sepsis/metabolic disease (22.2%) and feeding problems (17.8%). Therapeutic interventions were supportive in 77.8% of the cases, and dialysis was required in the other 22.2%. The mortality rate in the 45 ARF cases was 24.4%. Acute renal failure of renal origin, need for dialysis, and need for mechanical ventilation were associated with significantly increased mortality (p<0.05). There were no statistical correlations between mortality rate and perinatal risk factors, oliguria, prematurity or blood urea nitrogen and creatinine levels. The study showed that, at our institution, ARF in the neonatal period is frequently associated with preventable conditions, specifically asphyxia, sepsis and feeding problems. Supportive therapy is effective in most cases of neonatal ARF. Acute renal failure of renal origin, need for dialysis, and need for mechanical ventilation were identified as indicators of poor prognosis in these infants. Early recognition of risk factors and rapid effective treatment of contributing conditions will reduce mortality in neonatal ARF.
    Renal Failure 05/2004; 26(3):305-9. · 0.94 Impact Factor
  • Aylin Tarcan, Berkan Gürakan, Filiz Tiker, Namik Ozbek
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    ABSTRACT: It is well known that breast-feeding protects the newborn from infectious diseases. This is especially important for very low birth weight preterm infants, whose immune systems are immature. In this study we investigated how a milk fortifier and replacement formula affected lymphocyte subsets in preterm infants. The study assessed the effects of different types of feeding (human milk, n = 14; fortified human milk, n = 16; formula, n = 14) on lymphocyte subsets in 44 very low birth weight preterm infants. For each baby, two consecutive blood samples were collected 7-10 days apart during the full enteral feeding period. For each sample, the percentages of CD3+ (pan-T), CD19+ (B-cell), CD4+ (T-helper), CD8+ (T-suppressor), and CD3-CD16/56+ (natural killer cell) lymphocytes were measured in a flow cytometer, and the absolute count for each subset was calculated based on the total lymphocyte count. Within each feeding group, the absolute numbers of each lymphocyte subset in the two consecutive samples were compared. Also, the mean absolute counts for each cell type were compared among the 3 groups for the first set of blood samples, and the same comparisons were made for the second set. The mean number of CD3-CD16/56+ cells in the formula-fed infants was significantly lower than the corresponding means in the groups fed human milk alone and fortified human milk (p = 0.037). The findings suggest that babies fed formula have different lymphocyte subset compositions than those fed breast milk or fortified breast milk.
    Biology of the Neonate 02/2004; 86(1):22-8. · 1.90 Impact Factor
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    ABSTRACT: Premature constriction of the fetal ductus arteriosus has been described with long-term indomethacin therapy, but not in fetuses who have been exposed to the drug for less than 72 hours. The sensitivity of the ductus to extended indomethacin tocolysis increases with advancing gestational age. For this reason, it is recommended that indomethacin not be used beyond 31 weeks of gestation. In the present case the gestational age of the patient was 27 weeks and the period of indomethacin exposure was only 16 hours. Our observations of pulmonary hypertension in this case suggest that administration of indomethacin even hours before delivery can significantly affect the ductus arteriosus and the pulmonary vasculature.
    Journal of Perinatal Medicine 02/2004; 32(1):98-9. · 1.95 Impact Factor
  • A Tarcan, B Gürakan, S Arda, F Boybat
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    ABSTRACT: H-type tracheoesophageal fistula is a rare congenital condition. Its rarity and concurrent problems, such as respiratory distress, may delay its detection. We report delay in the diagnosis in a preterm small-for-gestational-age baby who showed symptoms of apnea and recurrent pneumonia, even when she was being fed by orogastric tube.
    Journal of Maternal-Fetal and Neonatal Medicine 05/2003; 13(4):279-80; discussion 280-1. · 1.52 Impact Factor
  • S M Kayiran, N Ozbek, M Turan, B Gürakan
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    ABSTRACT: The normal capillary and venous hematologic values for neonates have not been defined clearly. It is well known that capillary blood has higher hemoglobin (Hb) and hematocrit (Hct) values than venous blood. In a recent study, we reported differences between capillary and venous complete blood counts (CBC) in healthy term neonates on day 1 of life. The aim of this study was to extend our previous investigation. Term neonates (n=141) were stratified into four groups by days of postnatal age: group 2 (day 7, n=38), group 3 (day 14, n=35), group 4 (day 21, n=32) and, group 5 (day 28, n=36). Data from our previous study were included in the statistical analysis as group 1 (day 1, n=95). A CBC and differential count were carried out on each capillary and venous sample drawn simultaneously. Within each group, the mean and standard deviation for each parameter in capillary and venous blood were calculated and then compared using the paired sample t-test. In all groups, the capillary blood samples had higher Hb, Hct, red blood cell (RBC), white blood cell (WBC), and lymphocyte counts. In each group, venous platelet counts were significantly higher than the corresponding capillary values. There was also a trend toward higher venous mean corpuscular volume, higher capillary polymorphonuclear leukocyte (PML) count and mean platelet volume in all groups. In both capillary and venous blood, Hb, Hct, RBC, MCV values and WBC, lymphocyte, PML counts decreased and platelet counts increased steadily during neonatal period. This study reveals that CBC parameters and differential counts may differ depending on the blood sampling used. The findings underline the importance of considering the sample source when using hematologic reference ranges for healthy or septic neonates. When interpreting results, the term 'peripheral blood' should be replaced with 'capillary blood' or 'venous blood' so that an accurate assessment can be made.
    Clinical & Laboratory Haematology 03/2003; 25(1):9-16. · 1.11 Impact Factor
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    ABSTRACT: Persistent pulmonary interstitial emphysema (PPIE) is a chronic form of pulmonary interstitial emphysema. The disease is histologically distinguished by large cysts and giant cells. Our patient was a female twin who was born at 31 weeks of gestation with a birth weight of 1,450 g. A chest X-ray at 2 hr after delivery was normal. At 12 hr, respiratory distress developed, and nasal continuous positive airway pressure (CPAP) was initiated. A chest film revealed left-sided pneumothorax. A chest tube was inserted, and the baby continued on nasal CPAP for 5 days. Her chest X-ray on postnatal day 4 showed diffuse cystic changes in the left lung. Thoracic computed tomography revealed multiple thick-walled cysts, the largest measuring 3 cm in diameter. Our case confirms that localized PIE may occur in preterm infants who have been treated with nasal CPAP only. Since this method is being used increasingly to avoid mechanical ventilation and in the postextubation period, it is very important that clinicians be aware of its complications.
    Pediatric Pulmonology 12/2002; 34(5):409-11. · 2.38 Impact Factor
  • Teratology 09/2002; 66(2):57-8.
  • Filiz Tiker, Berkan Gürakan, Aylin Tarcan
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    ABSTRACT: This study investigated bilirubin levels in 282 1-month-old, healthy, term infants from the Adana region in southern Turkey. Total bilirubin was > 5 mg/dl in 20.2% of the infants and > 10 mg/dl in 6% of the group. Thyroid function and levels of alanine aminotransferase, aspartate aminotransferase and glucose-6-phosphate dehydrogenase were determined in babies with bilirubin levels > 5 mg/dl. The results were normal in all but one case, an infant with a bilirubin level of > 10 mg/dl and glucose-6-phosphate dehydrogenase deficiency. The results indicate that in this population a 5-mg/dl cut-off level for further investigation would mean that 20% of all infants would require further evaluation. This is not cost-effective. Based on our findings, we suggest that the cut-off level for investigating prolonged jaundice in term, 1-month-old, healthy infants in the Turkish population should be > 5 mg/dl.
    Annals of Tropical Paediatrics International Child Health 09/2002; 22(3):225-8. · 0.92 Impact Factor
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    ABSTRACT: Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder well-known for its association with intracranial aneurysms. Recently, intracranial arachnoid cysts have also been reported to be associated with ADPKD. We describe a father and daughter who each has a posterior fossa arachnoid cyst and asymptomatic ADPKD. To our knowledge, this is the first report of familial occurrence of arachnoid cysts in association with ADPKD.
    PEDIATRICS 08/2002; 110(1 Pt 1):e13. · 4.47 Impact Factor

Publication Stats

294 Citations
122.43 Total Impact Points

Institutions

  • 2011
    • American Hospital
      İstanbul, Istanbul, Turkey
  • 1999–2009
    • Baskent University
      • • Department of Pediatrics
      • • Faculty of Medicine
      Ankara, Ankara, Turkey
  • 1996
    • Ankara Güven Hospital
      Engüri, Ankara, Turkey
  • 1994
    • Hacettepe University
      • Department of Pediatrics
      Ankara, Ankara, Turkey