Fakülteler / Faculties
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Item Liver Transplant in a Child With an Uncommon Co-Occurrence of Biliary Atresia and Bilateral Vesicoureteral Reflux(2014) Deniz, Emine Ebru; Terzi, Aysen; Ozdemir, Binnaz Handan; Haberal, Nihan; Baskin, Esra; Haberal, Mehmet; https://orcid.org/0000-0002-1225-1320; https://orcid.org/0000-0002-7528-3557; https://orcid.org/0000-0001-9852-9911; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0002-3462-7632; 24635820; F-7546-2013; X-8540-2019; AAK-4587-2021; B-5785-2018; AAJ-8097-2021We report the clinicopathologic findings of a patient with biliary atresia associated with vesicoureteral reflux who underwent a liver transplant and nephroureterectomy simultaneously. The patient was a 22-month-old female infant born of a nonconsanguineous marriage, who was reported to be icteric from first day of life. Her antenatal history was significant for bilateral hydronephrosis. The results of a liver biopsy showed findings of biliary atresia, and the results of a radiograph showed bilateral vesicoureteral reflux. Therefore, the patient underwent a liver transplant and a right nephroureterectomy simultaneously. In the days after the operation, the patient died because of extrahepatic hematoma. In conclusion, a child having biliary atresia must remain for investigation of associated anomalies including vesicoureteral reflux.Item Acute Renal Injury in Liver Transplant Patients and Its Effect on Patient Survival(2014) Kirnap, Mahir; Colak, Turan; Baskin, Esra; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Haberal, Mehmet; https://orcid.org/0000-0002-8372-7840; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0002-8726-3369; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-3462-7632; 24635816; AAH-9198-2019; AAJ-8554-2021; B-5785-2018; AAA-3068-2021; AAE-1041-2021; AAJ-8097-2021Objectives: Acute renal injury is a common complication in liver transplant patients. Acute kidney injury is due to nephrotoxic drugs used after liver transplant, infections, and hemorrhage. Though it is generally reversible, it has effects on grafts and patients survival. In this retrospective observational study carried out at a single center, the effects of acute renal disease on liver recipient's survival were investigated. Materials and Methods: Liver transplant recipients of live-donor and deceased-donor transplants between January 2002 and May 2013 were included in this study; there were 310 liver transplant patients (mean age, 28 y; age range, 6 mo-62 y; 165 males, 145 females). The acute kidney disease diagnosis and staging was based on the nephrology department evaluation and daily serum creatinine levels. Patients with acute kidney injury before undergoing liver transplant and those undergoing a transplant for the second time were excluded. Kidney functions were evaluated by the nephrology department 1 week, 3 months, and 1 year after the liver transplant. Results: Acute kidney disease rates in these patients were 5%, 8%, and 12%. Four patients developed chronic kidney failure during follow-up. The mortality rate was higher (18%) in acute renal failure patients compared with those that did not have acute renal failure. The mortality rate was 11% in patients without acute renal failure. Conclusions: Acute renal injury is common after liver transplant and has an effect on mortality.Item Success of Eculizumab in the Treatment of Atypical Hemolytic Uremic Syndrome(2015) Baskin, Esra; Gulleroglu, Kaan; Kantar, Asli; Bayrakci, Umut; Ozkaya, Ozan; 0000-0003-1434-3824; 0000-0003-1434-3824; 0000-0003-4361-8508; 25384530; F-3294-2013; AAJ-8833-2021; B-5785-2018Disorders of complement regulation are the most important etiology of atypical hemolytic uremic syndrome (aHUS). Recent studies demonstrate that eculizumab is beneficial in long-term aHUS treatment. We present a series of children with aHUS resistant to/dependent on plasma exchange (PE) who were treated with eculizumab. This was a retrospective study in which data were retrieved from the medical files of children who had received PE as treatment for aHUS. The data retrieved included age, sex, presenting symptoms, presence of diarrhea/vomiting, hospitalization duration, laboratory data on admission and follow-up, need for transfusion or dialysis, response to PE, response to eculizumab and outcome. Of the 15 children diagnosed with aHUS in 2011 and 2012 in our departments, ten were resistant to, or dependent on, plasma therapy and treated with eculizumab; these children were enrolled in the study. Three patients had relapses, and seven had a new diagnosis. Nine children had oliguria or anuria, and eight required dialysis. Hypertension was observed in six patients. Neurologic involvement developed in six patients, with the symptoms including seizures, loss of balance, vision loss and severe confusion. Five and five patients were resistant to and dependent on plasma therapy, respectively. Following the start of eculizumab treatment, all patients achieved full recovery of renal function and hematologic parameters. In our ten pediatric patients with aHUS who did not respond to PE, eculizumab was a lifesaving therapy and improved their quality of life. Early eculizumab use was a rescue therapy for renal function. Our results show that eculizumab is an effective treatment for aHUS. However, more studies are needed on the long-term efficacy and safety of eculizumab in children with aHUS and to determine the optimal duration of treatment.Item Liver and Kidney Transplant in Primary Hyperoxaluria: A Single Center Experience(2015) Moray, Gokhan; Tezcaner, Tugan; Ozcay, Figen; Baskin, Esra; Akdur, Aydincan; Kirnap, Mahir; Yildirim, Sedat; Arslan, Gulnaz; Haberal, Mehmet; 0000-0002-5735-4315; 0000-0003-2498-7287; 0000-0002-5214-516X; 0000-0002-8726-3369; 0000-0003-4361-8508; 0000-0002-3462-7632; 0000-0002-3641-8674; 25894144; AAF-4610-2019; AAE-1041-2021; ABG-5684-2020; AAA-3068-2021; B-5785-2018; AAJ-8097-2021; AAH-9198-2019; AAD-9865-2021Objectives: Primary hyperoxaluria, especially type 1, is a severe disease with multisystem morbidity and high mortality. We present 3 primary hyperoxaluria type 1 patients who underwent liver transplant, including living-donor liver transplant or combined liver and kidney transplant in our institution. Case Reports: Patients who underwent liver transplant or combined liver/kidney transplant at our institution were evaluated, retrospectively. Between January 2002 and 2013, there were 3 patients who underwent transplant for primary hyperoxaluria. All 3 patients had disease onset in childhood, and the definitive diagnosis was established at age < 1, 6, and 8 years. Although early diagnosis was made, primary hyperoxaluria resulted in end-stage renal disease in 2 patients, and hemodialysis was introduced before liver transplant. All 3 patients underwent living-donor liver transplant. Case 1 was a 10-year-old girl who had an uneventful course after living-donor liver transplant, and she received a living-donor kidney transplant from the same donor 4 months after living-donor liver transplant. Case 2 was a 7-year-old boy who was the younger brother of the first patient; he did not have end-stage renal disease or any renal disorder after successful living-donor liver transplant. Case 3 was a 3-year-old boy who was diagnosed at age 2 months with renal disorders; although he was discharged from the hospital after living-donor liver transplant, he was readmitted because of unconsciousness that developed 1 day after discharge, and he died because of intracranial hemorrhage 2 months after liver transplant, unable to receive a kidney transplant. Conclusions: Primary hyperoxaluria is a rare disorder that is difficult to diagnose until end-organ damage is severe. Outcomes may be improved with early and accurate diagnosis, aggressive supportive treatment, and correction of the enzyme defect by liver transplant before systemic oxalosis develops. However, kidney transplant or combined liver and kidney transplant is required in many primary hyperoxaluria type 1 patients because of the delayed diagnosis or long organ waiting time.Item HPSE2 Mutations in Urofacial Syndrome, Non-Neurogenic Neurogenic Bladder and Lower Urinary Tract Dysfunction(2015) Bulum, Burcu; Ozcakar, Z. Birsin; Duman, Duygu; Cengiz, Filiz Basak; Kavaz, Asli; Burgu, Berk; Baskin, Esra; Cakar, Nilgun; Soygur, Tarkan; Ekim, Mesiha; Tekin, Mustafa; Yalcinkaya, Fatos; 0000-0003-4361-8508; 25924634; B-5785-2018Background: Urofacial syndrome (UFS) is characterised by congenital bladder dysfunction accompanied by a characteristic abnormal grimace upon smiling and crying. In recent years, biallelic mutations of HPSE2 and LRIG2 have been reported in UFS patients. Non-neurogenic neurogenic bladder (NNNB) has a bladder identical to UFS without typical facial features. The aim of this study was to analyse HPSE2 mutations in patients with UFS and NNNB or severe lower urinary tract dysfunction (LUTD) without abnormal facial expression. Methods: Patients with UFS, NNNB and severe LUTD were enrolled in the study. We examined a total of 35 patients from 33 families. There were seven UFS patients from five different families, 21 patients with NNNB and seven with LUTD. HPSE2 gene mutation analysis was performed using the polymerase chain reaction protocol followed by Sanger sequencing in these patients. Results: A twin pair with UFS was found to be homozygous for c.457C>T (p.Arg153*) mutation. No other pathogenetic variant was detected. Conclusion: HPSE2 mutations were found in one UFS family but not detected in patients with NNNB and severe LUTD. Considering the increasingly recognised cases of NNNB that were diagnosed in early childhood period, genetic factors appear to be responsible. Thus, further genetic studies are needed to discover novel associated gene variants in these bladder anomalies. (C) 2015 S. Karger AG, BaselItem Neuroblastoma Accompanied by Hyperaldosteronism(2014) Gulleroglu, Kaan; Bayrakci, Umut; Kinik, Sibel Tulgar; Uslu, Nihal; Atilgan, Alev O. K.; Sarialioglu, Faik; Baskin, Esra; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0002-6733-8669; https://orcid.org/0000-0001-8595-8880; https://orcid.org/0000-0002-8257-810X; https://orcid.org/0000-0003-4361-8508; 25340174; AAJ-8833-2021; ABC-5258-2020; AAK-3333-2021; AAL-7766-2021; B-5785-2018Background: Tumors known derived from kidneys which take place in secondary hyperaldosteronism etiology are juxtaglomerular cell tumor and Wilms' tumor. Neuroblastoma presenting with hyperaldosteronism is rare. Case: A 15-month-old girl who had been having diarrhea and fever for 2 weeks presented with a 3 day history of bilious vomiting, metabolic acidosis and severe hypokalemia. She was referred to our hospital with the pre-diagnosis of unknown manifest hypertension etiology, diarrhea, and paralytic ileus after having therapy-resistant hypokalemia and severe resistant acidosis. On her examination after being admitted to our clinic, she was weak, unwell and lethargic with a blood pressure of 140/93 mmHg. Due to the hypertension and severe hypokalemia, the patient was considered to be hyperaldosteronism. Serum aldosterone level, plasma renin activity and cortisol level were elevated. Radiologic findings were compatible with neuroblastoma. The patient underwent an abdominal surgery and the mass excision. The histopathological examination was proved neuroblastoma. Conclusion: Hyperaldosteronism can be presented by unexpected atypical forms as in our patient.Item Acidosis and Hyperkalemia Caused By Losartan and Enalapril in Pediatric Kidney Transplant Recipients(2014) Sakalli, Hale; Baskin, Esra; Bayrakci, Umut Selda; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-3462-7632; 24447308; B-5785-2018; AAE-1041-2021; AAJ-8097-2021Objectives: To evaluate the efficacy and safety of losartan and enalapril in pediatric kidney transplant recipients. Materials and Methods: A retrospective review was performed in 31 pediatric kidney transplant recipients who were treated with losartan (50 mg/d, oral) for 1 to 6 months because of mild hypertension and persistent proteinuria. All patients were treated concurrently with enalapril (5 or 10 mg daily, oral), and 12 patients (39%) also were treated with amlodipine (5 or 10 mg daily, oral). Demographic and clinical characteristics of the patients were reviewed. Results: Losartan use was associated with a significant decrease in mean systolic (before losartan was started, 123 +/- 14 mm Hg; before losartan was stopped, 111 +/- 10 mm Hg; P <= .001) and diastolic blood pressure (before losartan was started, 78 +/- 11 mm Hg; before losartan was stopped, 69 +/- 10 mm Hg; P <= .001) and urinary protein excretion (before losartan was started, 51 +/- 45 mg/m(2)/h; before losartan was stopped, 28 +/- 34 mg/m(2)/h; P <= .001). However, losartan therapy was associated with a significant mean increase in serum potassium level (before losartan was started, 4.0 +/- 0.4 mmol/L; before losartan was stopped, 5.7 +/- 0.5 mmol/L; P <= .001) and decrease in pH (before losartan was started, 7.35 +/- 0.0; before losartan was stopped, 7.23 +/- 0.0; P <= .001). Losartan was stopped because of hyperkalemia and acidosis earlier in patients who were on tacrolimus than cyclosporine immunosuppression (tacrolimus, 3 +/- 1 mo; cyclosporine, 4.7 +/- 0.8 mo; P <= .001). Conclusions: Losartan and enalapril may be beneficial in pediatric kidney transplant recipients by decreasing blood pressure and proteinuria, with maintenance of stable graft function, but may be associated with serious adverse events including hyperkalemia and life-threatening acidosis.Item The Effect of Anti-hla Antibodies on Renal Graft Functions(2014) Baskin, Esra; Gulleroglu, Kaan; Kantar, Asli; Kirnap, Mahir; Karakayali, Feza; Haberal, Aysegul; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0002-1874-947X; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-3462-7632; B-5785-2018; F-3294-2013; AAH-9198-2019; AAB-3888-2021; AAE-1041-2021; AAJ-8097-2021Item Association Between Heat Shock Protein-72 Gene Polymorphism and Chronic Renal Failure in Children(2014) Gulleroglu, Kaan; Baskin, Esra; Kantar, Asli; Sahin, Feride; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0001-7308-9673; F-3294-2013; B-5785-2018; AAC-7232-2020Item Fractalkine Receptor Gene Polymorphisms and Chronic Renal Disease(2014) Gulleroglu, Kaan; Baskin, Esra; Kantar, Asli; Sahin, Feride; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0003-4361-8508; AAJ-8833-2021; B-5785-2018