Tanıl Kendirli

Ankara University, Engüri, Ankara, Turkey

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Publications (16)15.31 Total impact

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    ABSTRACT: Niemann-Pick disease type C (NPC) is a fatal autosomal recessive lipid storage disease associated with impaired trafficking of unesterified cholesterol and glycolipids in lysosomes and late endosomes. This disease is commonly characterized by hepatosplenomegaly and severe progressive neurological dysfunction. There are two defective genes that cause this illness. One of these genes is NPC1 gene which is the cause of illness in 95% of the patients. The other gene is the rare type NPC2 which is the cause of illness in 5% of the patients. Patients with NPC2 usually present with respiratory distress in early infancy, which is rather unusual with NPC1. This article discusses about a patient who died at an early age from pulmonary involvement and who subsequently was found to have a novel homozygous mutation of NPC2 gene.
    05/2015; DOI:10.1515/jpem-2014-0358
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    01/2015; 2(1):7-12. DOI:10.5505/cayb.2015.29591
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    ABSTRACT: A very rare syndrome of rapid-onset obesity with hypoventilation, hypothalamic dysfunction and autonomic dysregulation (ROHHAD) has been recently described as causing morbidity due to hypothalamic dysfunction and respiratory arrest. Its prognosis is poor and often cardiac arrest occurs due to alveolar hypoventilation. This disorder can mimic genetic obesity syndromes and several endocrine disorders. We present a 13-year-old female patient who was reported to be healthy until the age of 3 years. She was admitted to our emergency department, presenting with respiratory distress. Features matching ROHHAD syndrome such as rapid-onset obesity, alveolar hypoventilation, central hypothyroidism, hyperprolactinemia, Raynaud phenomenon and hypothalamic hypernatremia were detected in the patient. In addition to these features, the patient was found to have hypergonadotropic hypogonadism and megaloblastic anemia. Because of its high mortality and morbidity, the possibility of ROHHAD syndrome needs to be considered in all pediatric cases of early- and rapid-onset obesity associated with hypothalamic-pituitary endocrine dysfunction.
    Journal of Clinical Research in Pediatric Endocrinology 12/2014; 6(4):254-257. DOI:10.4274/Jcrpe.1432
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    ABSTRACT: Neuroleptic malignant syndrome (NMS) is a rare but potentially fatal complication of treatment with antipsychotic medication. NMS has also been associated with non-neuroleptic agents that block central dopamine pathways, such as metoclopramide, amoxapine and lithium. Metoclopromide has antidopaminergic properties and is a rare but well-recognized perpetrator in the development of NMS. NMS has a constellation of signs and symptoms, including hyperthermia, muscle rigidity, autonomic instability, tachycardia, tachypnea, diaphoresis, hypertension and altered mental status. We present a 2-year-old girl who developed neuroleptic malignant syndrome after metoclopromide therapy. High-dose metoclopromide was given to our patient, and it is very likely that she was dehydrated while using metoclopromide, as she developed NMS two hours after treatment. The patient was discharged on the sixth day after admission to our hospital, having been cured. In summary, NMS developed in this patient very soon after metoclopromide treatment. NMS is a life-threatening emergency; if not recognized, or left untreated, it may be fatal. Therefore, early recognition of the developing signs and symptoms, along with a thorough medical history, is of great importance.
    The Turkish journal of pediatrics 09/2014; 56(5):535-7. · 0.56 Impact Factor
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    ABSTRACT: The aims of this study were to review the frequency, characteristics, and the clinical course of primary immunodeficiency (PID) patients admitted to pediatric intensive care unit (PICU) and attempt to identify factors related with mortality that might predict a poor outcome. We performed a retrospective review of children with PID aged 1 month to 18 years and admitted to PICU from January 2002 to January 2012 in our tertiary teaching children's hospital. There were a total of 51 patients accounting for 71 admissions to the PICU. The most common diagnosis was severe combined immunodeficiency. Respiratory problems were the leading cause for admission. A total of 20 patients received hematopoietic stem cell transplantation. Immune reconstitution was achieved in 9 (45 %) patients and eight of them did survive. In all 56 % of all admission episodes resulted in survival. Risk factors for mortality included requirement of mechanical ventilation (P < .001), number of organ system failure (P = .013), need for renal replacement therapy (P < .001), use of inotropes (P < .001), higher Pediatric Logistic Organ Dysfunction (PELOD) score (P = .005), and length of PICU stay (P < .001). This is the first study regarding the outcome and mortality-related risk factors for PID patients requiring PICU admission. We suggest that PICU management is as important as early diagnosis and treatment for these patients. Prediction of those at risk for poorer outcome might be beneficial for accurate intensive care management and survival.
    Journal of Clinical Immunology 02/2014; DOI:10.1007/s10875-014-9994-6 · 2.65 Impact Factor
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    ABSTRACT: Abstract Causes of hyperglycemia in critically ill non-diabetic children may differ from those in adults. The objective of this study was to investigate the pathogenesis of critical illness hyperglycemia (CIH) in terms of insulin resistance and β-cell dysfunction. Critically ill children with blood glucose (BG) levels of >150 mg/dL (8.3 mmol/L) were enrolled in the study. Insulin sensitivity and β-cell function in the hyperglycemic and euglycemic periods were analyzed with BG/insulin and BG/C-peptide ratios, and utilizing homeostasis model assessment (HOMA). A total of 40 patients were enrolled in the study. BG/insulin and BG/C-peptide ratios were significantly higher in the hyperglycemic period. The HOMA-B and S scores for the hyperglycemic period revealed that out of all the patients who survived (n=30), 20 had β-cell dysfunction, while the remaining (n=11) had insulin resistance. β-cell dysfunction was significantly higher in the hyperglycemic period (p<0.001). As in adults, β-cell dysfunction may play a major role in the pathophysiology of CIH in children.
    Journal of pediatric endocrinology & metabolism: JPEM 05/2013; 26(7-8):1-6. DOI:10.1515/jpem-2012-0313 · 0.71 Impact Factor
  • Cağlar Odek · Tanıl Kendirli · Funda Cipe · Banu Katlan · Figen Doğu
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    ABSTRACT: Pressure-induced urticaria is a non-immunoglobulin E-mediated type of urticaria. Some patients only have angioedema, and the term pressure-induced angioedema (PIA) is more appropriate for them. PIA has not previously been reported in association with endotracheal tube. Here we describe two patients who developed PIA after endotracheal intubation. There were no histories of angioedema, drug and food allergy in both patients. Tests for specific aero-allergens and latex were negative. Serum total immunoglobulin E and C4 levels were in normal ranges. Antihistamines and intravenous steroid therapy were ineffective. Angioedema regressed with intravenous epinephrine infusion and did not relapse after extubation. Conclusion: We suggest that endotracheal tubes can cause PIA. Epinephrine therapy should be used early at treatment, especially in the patients who are at great risk for life-threatening airway problems.
    European Journal of Pediatrics 04/2012; 171(10):1573-5. DOI:10.1007/s00431-012-1738-0 · 1.98 Impact Factor
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    ABSTRACT: Hashimoto encephalopathy is a rare, clinically heterogenous condition. Its treatment is based on corticosteroids. A previously normal 12-year-old boy was admitted to our pediatric emergency department with status epilepticus. He experienced a recurrence of status epilepticus after pentobarbital withdrawal, and required repeated resumptions of drug-induced coma. He manifested acute personality changes. His limbic encephalitis markers were normal, but his level of anti-thyroid peroxidase antibody was high. A diagnosis of Hashimoto encephalopathy was considered. Our patient responded to plasmapheresis instead of corticosteroid treatment. This case report is the first, to the best of our knowledge, of plasmapheresis because of Hashimoto encephalopathy in a child.
    Pediatric Neurology 02/2012; 46(2):132-5. DOI:10.1016/j.pediatrneurol.2011.11.009 · 1.50 Impact Factor
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    ABSTRACT: The purpose of our study was to evaluate and analyse the prevalence and association of acute kidney injury (AKI) as defined by paediatric Risk, Injury, Failure, Loss of kidney function and End-stage kidney disease (pRIFLE) and Acute Kidney Injury Network (AKIN) classifications in a paediatric intensive care unit (PICU). A prospective analysis of all patients that were admitted to our PICU between June 2009 and December 2010 was performed. Patients were classified according to AKIN and pRIFLE criteria. One hundred and eighty-nine patients (mean age 45.9 ± 54.7 months; 110 male, 79 female) were enrolled. Sixty-three (33.3%) patients developed AKI by AKIN criteria and 68 (35.9%) patients developed AKI by pRIFLE criteria. All patients that had AKI according to AKIN criteria also had this diagnosis with pRIFLE criteria. Five patients had developed AKI only according to pRIFLE classification, four of them owing to reduction in their estimated creatinine clearance and one of them owing to changes over 1-week period. The mean length of PICU stay was longer, need for mechanical ventilation and mortality rates were higher in patients with AKI when compared to patients without AKI. Although both pRIFLE and AKIN criteria were very helpful in the detection of patients with AKI even in the early stages of it, pRIFLE seems to be more sensitive in paediatric patients.
    Acta Paediatrica 11/2011; 101(3):e126-9. DOI:10.1111/j.1651-2227.2011.02526.x · 1.84 Impact Factor
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    Pediatrics International 10/2011; 53(5):761-3. DOI:10.1111/j.1442-200X.2010.03321.x · 0.73 Impact Factor
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    ABSTRACT: The management of children with diabetic ketoacidosis (DKA) continues to be a controversial issue with regard to amount of intravenous fluid to be given, rate of delivery of fluid, and type of fluid to be used. We aimed to analyze the results obtained by administration of rehydration fluids of two different sodium (Na) concentrations (75 mEq/L vs. 100 mEq/L) in the treatment of children with DKA. Thirty-two children with DKA were assessed for efficacy and safety of fluid treatment. After an initial rehydration time, intravenous fluids were switched to a 5% dextrose solution with a Na content of 75 mEq/L (Group I, n=19) or 100 mEq/L (Group II, n=13). Venous blood samples were collected from all subjects at diagnosis and at the 4th, 8th, 16th and 24th hours of treatment. Changes in blood glucose levels did not differ significantly between the two groups at the 4th, 8th, 16th and 24th hours of the follow-up. Nadir effective plasma osmolality (Peff osm) and Peff osm levels also did not show statistically significant differences. Plasma sodium (PNa) level did not drop lower than the level at diagnosis in both groups. The changes in PNa concentrations in the two groups were not statistically significant at diagnosis or in follow-up samples (p=0.74). pH, anion gap, pCO₂ and HCO₃ levels were also similar in Group I and Group II. The duration of a pH level of <7.3 was shorter in Group II, but this was not statistically significant (p=0.65). None of the patients enrolled in this study developed cerebral edema. The efficacy and safety of rehydration fluids with Na concentrations of 75 or 100 mEq/L did not reveal any differences in children with DKA.
    Journal of Clinical Research in Pediatric Endocrinology 09/2011; 3(3):149-53. DOI:10.4274/jcrpe.v3i3.29
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    ABSTRACT: Angiodysplasia is a frequent cause of gastrointestinal bleeding in adults with chronic renal failure (CRF); however, there is no data about this association in children. The mechanism of this association is not known. We report a 4.5-year-old boy with CRF presenting with hematochezia due to colonic angiodysplasia. He was on hemodialysis for the previous 9 months. Treatment with argon plasma coagulation (APC) was commenced following a short course of octreotide therapy. During the 3 years of follow-up, no occult or gross bleeding occurred. This case illustrates that octreotide and APC therapy seems to be useful for arresting bleeding from angiodysplasia and prevention of recurrent bleeding in children with CRF.
    Renal Failure 03/2011; 33(2):252-4. DOI:10.3109/0886022X.2011.553299 · 0.78 Impact Factor
  • Pediatric Nephrology 11/2008; 24(6). DOI:10.1007/s00467-008-0946-3 · 2.88 Impact Factor
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    ABSTRACT: Respiratory syncytial virus (RSV) is the leading cause of viral respiratory tract infections in infants and young children. Although the course of RSV infection is usually benign, a small proportion of infants require mechanical ventilation for respiratory failure. We describe an eight-month-old previously healthy female who developed bilateral pneumothorax and acute respiratory distress syndrome (ARDS) secondary to RSV infection. Because of the severe hypoxemia, three doses of surfactant were administered and prone positioning was implemented for nine days. After a prolonged course of mechanical ventilation, she was extubated at day 21 and discharged from the pediatric intensive care unit four days later. We conclude that RSV infections can be severe, and some patients may require mechanical ventilation. Supportive therapies like surfactant replacement therapy and prone positioning can be beneficial in patients with ARDS in whom severe hypoxemia persists despite high levels of positive end-expiratory pressure (PEEP) and plateau pressures.
    The Turkish journal of pediatrics 55(5):539-42. · 0.56 Impact Factor
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    ABSTRACT: For many decades, the primary treatment of acute respiratory failure has been mechanical ventilation. Intubation and mechanical ventilation carry complications such as upper respiratory system trauma, barotrauma, volutrauma, oxytrauma, atelectrauma, and nosocomial infection, and they increase mortality. Noninvasive mechanical ventilation (NIMV) applied to a specially selected group of patients has more advantages than invasive ventilation, especially the decreased number of complications. We present a three-year-old girl who had prolonged invasive mechanical ventilation (IMV) and weaning failure due to severe tracheal bleeding after Fontan operation. She could not be extubated for 20 days after admission to our Pediatric Intensive Care Unit (PICU) because of life-threatening airway bleedings from within the endotracheal tube. Owing to the severe bleedings during endotracheal aspirations, it was decided to change her mechanical ventilation to NIMV. NIMV was performed successfully for 12 days without difficulty, and she was discharged from the hospital on the 55th postoperative day with normal clinical findings. Noninvasive ventilation has an increasing use in critically ill children. NIMV should be considered in prolonged mechanical ventilation and weaning failure from IMV in critically ill children.
    The Turkish journal of pediatrics 56(1):111-4. · 0.56 Impact Factor
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    ABSTRACT: We report a rare case of interventricular septal hematoma after patch closure of a perimembranous ventricular septal defect in a 10-month-old infant. Intraoperative transesophageal echocardiography was not performed. Routine transthoracic echocardiography at the 1st postoperative hour showed a huge intramural hematoma causing severe thickening of the ventricular septum. The patient's hemodynamics were stable and surgical revision was not required. The patient recovered well without complication.
    The Turkish journal of pediatrics 55(6):662-664. · 0.56 Impact Factor

Publication Stats

22 Citations
15.31 Total Impact Points

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  • 2008–2014
    • Ankara University
      • • Department of Pediatric Intensive Care
      • • Department of Pediatric Nephrology
      Engüri, Ankara, Turkey