Person: SAKAR, MUSTAFA
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SAKAR
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MUSTAFA
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Publication Open Access A Rare Cause of Hypophosphatemia: Raine Syndrome Changing Clinical Features with Age(SPRINGER, 2020-07) DAĞÇINAR, ADNAN; Eltan, Mehmet; Alavanda, Ceren; Yavas Abali, Zehra; Ergenekon, Pinar; Yalindag Ozturk, Nilufer; Sakar, Mustafa; Dagcinar, Adnan; Kirkgoz, Tarik; Kaygusuz, Sare Betul; Gokdemir, Yasemin; Elcioglu, Huriye Nursel; Guran, Tulay; Bereket, Abdullah; Ata, Pinar; Turan, SerapRaine Syndrome (RS) is caused by biallelic loss-of-function mutations in FAM20C gene and characterized by hypophosphatemia, typical facial and skeletal features. Subperiosteal bone formation and generalized osteosclerosis are the most common radiological findings. Here we present a new case with RS. A 9-month-old male patient on a home-type ventilator was referred for hypophosphatemia. He was born with a weight of 3800 g to non-consanguineous parents. Prenatal ultrasound had demonstrated nasal bone agenesis. A large anterior fontanel, frontal bossing, exophthalmos, hypoplastic nose, high arched palate, low set ears, triangular mouth, and corneal opacification were detected on physical examination. Serial skeletal X-rays revealed diffuse osteosclerosis at birth which was gradually decreased by the age of 5 months with subperiosteal undermineralized bone formation and medullary space of long bone could be distinguishable with bone-within-a-bone appearance. At 9 months of age, hand X-ray revealed cupping of the ulna with loose radial bone margin with minimal fraying and osteopenia. Cranial computed tomography scan showed bilateral periventricular calcification and hydrocephalus in progress. The clinical, laboratory, and radiological examinations were consistent with RS. Molecular analyses revealed a compound heterozygous mutation in FAM20C gene (a known pathogenic mutation, c.1645C > T, p.Arg549Trp; and a novel c.863 + 5 G > C variant). The patient died due to respiratory failure at 17 months of age. This case allowed us to demonstrate natural progression of skeletal features in RS. Furthermore, we have described a novel FAM20C variant causing RS. Previous literature on RS is also reviewed.Publication Open Access Effect of the pandemic on surgical procedures in a tertiary care hospital: A retrospective review(2022-09-01) SAÇAK, BÜLENT; KESİMER, MEHMET DENİZ; ŞAHİN, BAHADIR; UĞURLU, MUSTAFA ÜMİT; SAKAR, MUSTAFA; YUMUŞAKHUYLU, ALİ CEMAL; Ozkan M. C. , SAÇAK B., KESİMER M. D. , ŞAHİN B., UĞURLU M. Ü. , Sirzai E. Y. , SAKAR M., Aykut A., Cicek I., YUMUŞAKHUYLU A. C.Objective: The aim of this study was to examine the impact of performing surgeries with necessary precautions and to evaluate demographic characteristics of operated patients during novel coronavirus-2019 (COVID-19) pandemic and the infection rates during hospitalization and within 14 days after surgery.Publication Open Access Langerhans cell histiocytosis located in the spheno-orbital bone and the pons: Illustrative case(2023-07-01) ÇEKİÇ, EFECAN; KARAGÖZ, AHMET; SAKAR, MUSTAFA; KAÇAR, KADRİYE EBRU; BOZKURT, SÜHEYLA; DAĞÇINAR, ADNAN; ÇEKİÇ E., Karagoz A., SAKAR M., Senay R. E., AKAR K. E., BOZKURT S., DAĞÇINAR A.BACKGROUND This is a case of aggressive Langerhans cell histiocytosis (LCH) with an atypical intracranial location. OBSERVATIONS In this report, the authors present the diagnosis and treatment of a 12-year-old male patient diagnosed with LCH. The patient was admitted to the emergency department with left-sided facial palsy, and a solid lesion with mass effect in the pons was found. A biopsy was performed via suboccipital craniotomy, and the diagnosis was LCH. A chemotherapy regimen was started since the LCH sample was the resistant type. The patient showed improvement in his neurological deficit following treatment. LESSONS This rare localized and aggressive case’s diagnosis process and treatment choices may apply to future cases. https://thejns.org/doi/abs/10.3171/CASE22367.Publication Open Access Continuous hemoglobin measurement during frontal advancement operations can improve patient outcomes(2022-12-01) SARAÇOĞLU, AYTEN; ABDULLAYEV, RUSLAN; SAÇAK, BÜLENT; AYKAÇ, ZEYNEP ZUHAL; SAKAR, MUSTAFA; Saraçoğlu A., Abdullayev R., Sakar M., Saçak B., Incekoy F. G., Aykac Z.Massive hemorrhage in pediatric cranioplasty operations may necessitate blood transfusion, which may cause many complications. Radical-7 Pulse CO-Oximeter (Massimo Corporation, Irvine, CA) can provide continuous hemoglobin concentration (SpHb) measurements noninvasively. In this study, we aimed to evaluate the effects of SpHb measurement on perioperative transfusion management and postoperative patient outcomes. For this retrospective case-control study, we collected the data of pediatric patients undergoing fronto-orbital advancement surgery for plagiocephaly and trigonocephaly between 2018 and 2021. Perioperative SpHb monitoring was performed for patients in the SpHb Group. Other patients that were managed conventionally were considered as the control group (C Group). The data on patients\" demographic and clinical characteristics, intraoperative hemodynamic and laboratory variables such as blood gases, intraoperative blood losses, the amount of the transfused blood products, the length of postoperative intensive care unit (ICU) stay, and the duration of hospital stay were collected. The data of 42 patients were collected, and 29 of these patients were males (69%). In 16 of the patients, SpHb monitoring was performed. The demographic, clinical, and perioperative hemodynamic characteristics of the patients were comparable between the groups. Compared to the C Group, the SpHb Group had significantly lower perioperative packed red blood cell (PRBC) transfusion (136.3 +/- 40.1 vs. 181.5 +/- 74.8 mL, P = 0.015), less postoperative drainage (125.3 +/- 47.7 vs. 185.8 +/- 97.6 mL, P = 0.013), and shorter ICU stay (37.1 +/- 12.0 vs. 64.8 +/- 24.9 h, P < 0.001). There was a positive correlation between the amount of PRBC transfusion and the length of ICU stay (r = 0.459, P = 0.003). Patients with perioperative continuous SpHb measurement have lower intraoperative PRBC transfusion, less postoperative bleeding, and shorter ICU stay. When necessary, SpHb, together with clinical judgment and laboratory confirmation, can be used in decision-making for perioperative PRBC transfusion.Publication Open Access A new method for quantification of frontal retrusion and complex skull shape in metopic craniosynostosis: a pilot study of a new outcome measure for endoscopic strip craniectomy(2022-06-01) SAÇAK, BÜLENT; ERDOĞAN, ONUR; BAYRİ, YAŞAR; DAĞÇINAR, ADNAN; SAKAR, MUSTAFA; SÖNMEZ, ÖZCAN; SAKAR M., Haidar H., SÖNMEZ Ö., ERDOĞAN O., SAÇAK B., BAYRİ Y., DAĞÇINAR A.OBJECTIVE The objective of this study was to propose a new skull outline–based method to objectively quantify complex 3D skull shapes and frontal and supraorbital retrusion in metopic craniosynostosis using 3D photogrammetry. METHODS A standard section from 3D photogrammetry, which represents the trigonocephalic shape, was used in this study. From the midpoint of the area of this section, half diagonals were calculated to the skull outline at 5° increments in the anterior half of the head. These half diagonals were used to create a sinusoidal curve, and the area under the sinusoidal curve (AUC) was used to represent the mathematical expression of the trigonocephalic head shape. The AUC from 0° to 180° (90° from the midline to each side) was calculated and is referred to as AUC0→180. The AUC from 60° to 120° (30° from the midline to each side) was also calculated and is referred to as AUC60→120. A total of 24 patients who underwent endoscopic strip craniectomy and 13 age- and sex-matched controls were included in the study. The AUC values obtained in patients at different time points and controls were analyzed. RESULTS The mean preoperative AUC60→120 and AUC0→180 in the patients were significantly lower than those in control individuals. The increase in both AUC60→120 and AUC0→180 values is statistically significant at the discontinuation of helmet therapy and at final follow-up. Receiver operating characteristic curve analysis indicated that AUC60→120 is a more accurate classifier than AUC0→180. CONCLUSIONS The proposed method objectively quantifies complex head shape and frontal retrusion in patients with metopic craniosynostosis and provides a quantitative measure for follow-up after surgical treatment. It avoids ionizing radiation exposurePublication Open Access Impact of the COVID-19 pandemic on patients with paediatric cancer in low-income, middle-income and high-income countries: a multicentre, international, observational cohort study(2022-04-01) SAKAR, MUSTAFA; Bandyopadhyay S., Peter N., Lakhoo K., Vieira Abib S. d. C. , Abdelhafeez H., Wilson S., Pachl M., Martin B., Nagras S., Sheth M., et al.Objectives Paediatric cancer is a leading cause of death for children. Children in low-income and middle-income countries (LMICs) were four times more likely to die than children in high-income countries (HICs). This study aimed to test the hypothesis that the COVID-19 pandemic had affected the delivery of healthcare services worldwide, and exacerbated the disparity in paediatric cancer outcomes between LMICs and HICs. Design A multicentre, international, collaborative cohort study. Setting 91 hospitals and cancer centres in 39 countries providing cancer treatment to paediatric patients between March and December 2020. Participants Patients were included if they were under the age of 18 years, and newly diagnosed with or undergoing active cancer treatment for Acute lymphoblastic leukaemia, non-Hodgkin\"s lymphoma, Hodgkin lymphoma, Wilms\" tumour, sarcoma, retinoblastoma, gliomas, medulloblastomas or neuroblastomas, in keeping with the WHO Global Initiative for Childhood Cancer. Main outcome measure All-cause mortality at 30 days and 90 days. Results 1660 patients were recruited. 219 children had changes to their treatment due to the pandemic. Patients in LMICs were primarily affected (n=182/219, 83.1%). Relative to patients with paediatric cancer in HICs, patients with paediatric cancer in LMICs had 12.1 (95% CI 2.93 to 50.3) and 7.9 (95% CI 3.2 to 19.7) times the odds of death at 30 days and 90 days, respectively, after presentation during the COVID-19 pandemic (p<0.001). After adjusting for confounders, patients with paediatric cancer in LMICs had 15.6 (95% CI 3.7 to 65.8) times the odds of death at 30 days (p<0.001). Conclusions The COVID-19 pandemic has affected paediatric oncology service provision. It has disproportionately affected patients in LMICs, highlighting and compounding existing disparities in healthcare systems globally that need addressing urgently. However, many patients with paediatric cancer continued to receive their normal standard of care. This speaks to the adaptability and resilience of healthcare systems and healthcare workers globally.Publication Open Access An Astonishing Extrarenal Wilms Localisation; Spinal Cord(2022-06-01) TOKUÇ, AYŞE GÜLNUR; BOZKURT, SÜHEYLA; KAÇAR, KADRİYE EBRU; EKER, NURŞAH; SAKAR, MUSTAFA; TOKUÇ A. G., EKER N., Tas B. T., BOZKURT S., SAKAR M., Aras S., AKAR K. E.Wilms\" tumour is a renal tumour mostly seen during the first 5 years of life and it accounts for 95% of renal malignancies during childhood. Its origin is primitive metanephric cells and, very rarely, it may occur in places other than the kidneys. The estimated rate of nephroblastoma outside the kidneys is approximately 0.5 to 1% of Wilms\" tumour cases. In this article, we report on a 3-year-old female patient who first presented with spinal dysraphism and a mass in the lumbar spinal cord with a histopathological diagnosis of nephrogenic rest, and after one year, a Wilms tumour arose in this location. This is a very rare extrarenal Wilms\" tumour location. Here, we report on a case with immature renal cells located in the lumber spinal cord associated with spinal dysraphism and the development of Wilms\" tumour there after one year.Publication Open Access Twelve-month observational study of children with cancer in 41 countries during the COVID-19 pandemic(2022-10-01) SAKAR, MUSTAFA; Bandyopadhyay S., Peter N., Lakhoo K., Abib S. d. C. V. , Abdelhafeez H., Wilson S., Pachl M., Martin B., Nagras S., Sheth M., et al.Introduction Childhood cancer is a leading cause of death. It is unclear whether the COVID-19 pandemic has impacted childhood cancer mortality. In this study, we aimed to establish all-cause mortality rates for childhood cancers during the COVID-19 pandemic and determine the factors associated with mortality. Methods Prospective cohort study in 109 institutions in 41 countries. Inclusion criteria: children <18 years who were newly diagnosed with or undergoing active treatment for acute lymphoblastic leukaemia, non-Hodgkin\"s lymphoma, Hodgkin lymphoma, retinoblastoma, Wilms tumour, glioma, osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, medulloblastoma and neuroblastoma. Of 2327 cases, 2118 patients were included in the study. The primary outcome measure was all-cause mortality at 30 days, 90 days and 12 months. Results All-cause mortality was 3.4% (n=71/2084) at 30-day follow-up, 5.7% (n=113/1969) at 90-day follow-up and 13.0% (n=206/1581) at 12-month follow-up. The median time from diagnosis to multidisciplinary team (MDT) plan was longest in low-income countries (7 days, IQR 3-11). Multivariable analysis revealed several factors associated with 12-month mortality, including low-income (OR 6.99 (95% CI 2.49 to 19.68); p<0.001), lower middle income (OR 3.32 (95% CI 1.96 to 5.61); p<0.001) and upper middle income (OR 3.49 (95% CI 2.02 to 6.03); p<0.001) country status and chemotherapy (OR 0.55 (95% CI 0.36 to 0.86); p=0.008) and immunotherapy (OR 0.27 (95% CI 0.08 to 0.91); p=0.035) within 30 days from MDT plan. Multivariable analysis revealed laboratory-confirmed SARS-CoV-2 infection (OR 5.33 (95% CI 1.19 to 23.84); p=0.029) was associated with 30-day mortality. Conclusions Children with cancer are more likely to die within 30 days if infected with SARS-CoV-2. However, timely treatment reduced odds of death. This report provides crucial information to balance the benefits of providing anticancer therapy against the risks of SARS-CoV-2 infection in children with cancer.Publication Open Access Long-term outcomes of gamma-knife radiosurgery for intracanalicular vestibular schwannomas(2022-01-01) PINAR, ERTUĞRUL; BAYRİ, YAŞAR; BAYRAKLI, FATİH; SAKAR, MUSTAFA; ATASOY, BESTE MELEK; Sakar M., Pınar E., Bayri Y., Bayraklı F., Atasoy B. M., Ziyal M. İ.GİRİŞ ve AMAÇ: Kanal içi vestibüler schwannomalar, tüm vestibüler schwannomalar içerisinde küçük bir yüzdeye sahiptir. Kanal içi vestibüler schwannomalarda işitmenin korunması, hasta yönetiminin temel amaçlarındandır. Bu çalışmanın amacı, kanal içi vestibüler schwannomalarda Gamma-Knife radyocerrahinin (GKR) tümör kontrolü ve işitmenin korunması üzerine etkisinin incelenemesidir.YÖNTEM ve GEREÇLER: Bu geriye dönük çalışmada, Gamma-Knife Radyocerrahi Merkezi’mizde Ocak 2010–Ocak 2020 arasında kanal içi vestibüler schwannoma tanısı ile stereotaksik radyocerrahi almış, klinik, odyometrik ve radyolojik takibi bulunan hastalar değerlendirildi. Tümör kontrolü manyetik rezonans görüntüleme yönteminde tümör boyutlarının ölçülmesi ile takip edildi. İşitme seviyeleri Gardner-Robertson (GR) işitme sınıflaması yöntemine göre değerlendirildi.BULGULAR: Ortalama 48.23 aylık takipte, çalışmaya dahil edilen toplam 45 hastadan 44’ünde tümör kontrolü sağlandığı görüldü (%97.7). Tümör büyümesi görülen bir hastada ise, ek bir tedavi uygulanması gerekmedi. Ek tedavi gerekliliğine göre değerlendirildiğinde tüm hastalarda tümör kontrolü sağlandı (%100). Tedavi öncesi fonksiyonel işitmesi olan (GR derece I ve II) toplam 29 hastadan, ortalama 70.42 aylık takip sonrasında, toplam 13 hastada fonksiyonel işitme korundu (%44.8). GR derecesinde kayıp, ameliyat öncesinde yüksek GR derecesine sahip olmak ile korelasyon gösterdi (Rs=0.459, p=0.002). Tedavi dozu işitme kaybı ile korelasyona sahipti ve daha yüksek doz alan hastalar daha kötü sonuçlara sahipti (Rs=0.459, p=0.002).TARTIŞMA ve SONUÇ: Kanal içi vestibüler schwannomalarda GKR uzun dönemli takiplerde mükemmel tümör kontrolü sağlamaktadır. Beş yılın üzerindeki takip sürelerinde fonksiyonel işitmenin korunma oranı azalabilir. Kanal içi vestibüler schwannomalarda GKR sonrası işitmenin seyrini ortaya koyabilmek için uzun takip süreli daha fazla çalışma gerekmektedir.Anahtar Kelimeler:Gamma-knife radyocerrahi, işitmenin korunması; kanal içi; tümör kontrolü; vestibüler schwannoma.Publication Open Access Hereditary spastic paraplegia with recessive trait caused by mutation in KLC4 gene(NATURE PUBLISHING GROUP, 2015-12) DAĞÇINAR, ADNAN; Bayrakli, Fatih; Poyrazoglu, Hatice Gamze; Yuksel, Sirin; Yakicier, Cengiz; Erguner, Bekir; Sagiroglu, Mahmut Samil; Yuceturk, Betul; Ozer, Bugra; Doganay, Selim; Tanrikulu, Bahattin; Seker, Askin; Akbulut, Fatih; Ozen, Ali; Per, Huseyin; Kumandas, Sefer; Torun, Yasemin Altuner; Bayri, Yasar; Sakar, Mustafa; Dagcinar, Adnan; Ziyal, IbrahimWe report an association between a new causative gene and spastic paraplegia, which is a genetically heterogeneous disorder. Clinical phenotyping of one consanguineous family followed by combined homozygosity mapping and whole-exome sequencing analysis. Three patients from the same family shared common features of progressive complicated spastic paraplegia. They shared a single homozygous stretch area on chromosome 6. Whole-exome sequencing revealed a homozygous mutation (c.853_871del19) in the gene coding the kinesin light chain 4 protein (KLC4). Meanwhile, the unaffected parents and two siblings were heterozygous and one sibling was homozygous wild type. The 19 bp deletion in exon 6 generates a stop codon and thus a truncated messenger RNA and protein. The association of a KLC4 mutation with spastic paraplegia identifies a new locus for the disease.