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    An unusual case of left-sided hemitruncus arteriosus associated with aortic arch hypoplasia
    (Czech Society of Cardiology Z.S, 2024) Akıllı, Hande; Alhashmi, Hadeil; Babayiğit, Aslan; Yücel, Serap; Öztaş, Didem Melis; Uğurlucan, Murat; Tireli, Emin
    Hemitruncus arteriosus is among the rarest congenital cardiovascular defects. Pathology usually involves the right pulmonary artery. It may occur either isolated or associated with other cardiac defects such as septal defects and tetralogy of Fallot; however, it is seldom associated with aortic arch pathologies. We represent an unusual case of a 15-day-old neonate with left-sided hemitruncus arteriosus associated with aortic arch hypoplasia, coarctation of the aorta, patent ductus arteriosus, and craniofacial abnormalities, who underwent surgical correction.
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    Biliary atresia-splenic malformation (BASM) syndrome: a case report
    (Elsevier Ltd, 2024) Alhashmi, Hadeil; Chawshly, Esra; Çelebi, Süleyman
    Introduction and importance: Biliary atresia (BA) is characterized by the presence of persistent cholestatic jaundice during the neonatal period. This group of patients is at higher risk for liver disease and/or portal hypertension compared with other chronic liver diseases. Case presentation: We present a newborn patient who had biliary stools early postnatally, but her gallbladder was not seen on radiological examination and was referred to our clinic. On examination, polysplenia was seen, and the inferior vena cava was not seen. The patient's stool color changed to a creamy stool without bile three weeks postnatally. The neonate was diagnosed with biliary atresia and splenic malformation (BASM) syndrome. The patient underwent a Kasai portoenterostomy in the 3rd week of age and was preoperatively diagnosed with malrotation and treated with a Ladd procedure at the same time. The patient had a postoperative stool with bile and decreased bilirubinemia with a normal defecation period. Clinical discussion: BASM syndrome, clinically has different causes and a worse prognosis than isolated BA. The syndrome can lead to end-stage cirrhosis and liver failure if left untreated. Although timely Kasai surgery is the standard treatment for BA, the age of the patient at the time of the surgery is different depending on BA groups. Conclusion: The most important prognostic factors for BASM syndrome are early diagnosis and treatment. Despite this, the patients need careful follow-up. Postoperative adjuvant steroid therapy may have a good impact on the outcomes.