دورية أكاديمية
Severe Intracranial Hemorrhage in a Newborn with Autosomal Recessive Polycystic Kidney Disease
| العنوان: | Severe Intracranial Hemorrhage in a Newborn with Autosomal Recessive Polycystic Kidney Disease |
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| المؤلفون: | Stojanović, Vesna, Spasojević, Slobodan, Barišić, Nenad, Radovanović, Tanja, Doronjski, Aleksandra |
| المصدر: | Journal of Pediatric Nephrology; Vol. 6 No. 2 (2018): Summer ; 1-3 ; 2345-3176 ; 10.22037/jpn.v6i2 |
| بيانات النشر: | Shahid Beheshti University of Medical Sciences |
| سنة النشر: | 2018 |
| المجموعة: | Portal of Scientific Journals at Shahid Beheshti University of Medical Science |
| مصطلحات موضوعية: | Polycystic Kidney, Autosomal Recessive, Intracranial Hemorrhages, Intracranial aneurysm |
| الوصف: | Autosomal recessive polycystic kidney disease (ARPKD)is an autosomal recessive inherited developmental kidney disorder characterized by bilateral cystic dilatation of renal collecting ducts. The liver is always affected, with various degrees of biliary dysgenesis and periportal fibrosis. Intracranial aneurysms (ICA) can be associated with autosomal dominant polycystic kidney disease (ADPKD) in 5-10% of the cases, but extremely rarely with ARPKD.We described a full-term male infant with ARPKD born with an extremely distended abdomen and anuria. Bilateral nephrectomy and open liver biopsy were performed during the first month of hospitalization, as well as the insertion of a peritoneal dialysis catheter. On the 59th day of life, the baby`s condition abruptly deteriorated and he showed a decreased level of consciousness, coma (GCS=3), and severe anemia. Head ultrasound and head CT scan revealed large hemorrhage in the left cerebral hemisphere. Hemostasis parameters, as well as clinical and laboratory signs of infection, were in referral ranges at the time of neurological deterioration. MRI showed large hemorrhages in the left ventricle and left cerebral hemisphere but a preexisting intracranial aneurism (ICA) in the site of hemorrhage could not be diagnosed for sure. The baby died on the 66th day of life. Keywords: Polycystic Kidney, Autosomal Recessive; Intracranial Hemorrhages; Intracranial aneurysm; Newborn. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| العلاقة: | https://journals.sbmu.ac.ir/jpn/article/view/22221/15727Test; https://journals.sbmu.ac.ir/jpn/article/view/22221Test |
| DOI: | 10.22037/jpn.v6i2.22221 |
| الإتاحة: | https://doi.org/10.22037/jpn.v6i2.22221Test https://doi.org/10.22037/jpn.v6i2Test https://journals.sbmu.ac.ir/jpn/article/view/22221Test |
| حقوق: | Copyright (c) 2018 Journal of Pediatric Nephrology |
| رقم الانضمام: | edsbas.7E9A72F8 |
| قاعدة البيانات: | BASE |
| DOI: | 10.22037/jpn.v6i2.22221 |
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