Staged, Open, No-Ischemia Nephron-Sparing Surgery for Bilateral-Multiple Kidney Tumors in a Patient with Birt-Hogg-Dubé Syndrome

Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. Birt-Hogg-Dubé Syndrome (BHDS) is an autosomal dominant genodermatosis characterized by a triad of benign skin tumors (fibrofolliculomas, trichodiscomas, acrochordons) together with an increased risk of developing malignant renal tumors and pulmonary disease such as pneumothoraces and multiple lung cysts. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. Herein, a patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized

The effect of 3D modeling on family quality of life, surgical success, and patient outcomes in congenital heart diseases: objectives and design of a randomized controlled trial

Background: Understanding the severity of the disease from the parents' perspective can lead to better patient outcomes, improving both the child's health-related quality of life and the family's quality of life. The implementation of 3-dimensional (3D) modeling technology in care is critical from a translational science perspective. Aim: The purpose of this study is to determine the effect of 3D modeling on family quality of life, surgical success, and patient outcomes in congenital heart diseases. Additionally, we aim to identify challenges and potential solutions related to this innovative technology. Methods: The study is a two-group pretest-posttest randomized controlled trial protocol. The sample size is 15 in the experimental group and 15 in the control group. The experimental group's heart models will be made from their own computed tomography (CT) images and printed using a 3D printer. The experimental group will receive surgical simulation and preoperative parent education with their 3D heart model. The control group will receive the same parent education using the standard anatomical model. Both groups will complete the Sociodemographic Information Form, the Surgical Simulation Evaluation Form - Part I-II, and the Pediatric Quality of Life Inventory (PedsQL) Family Impacts Module. The primary outcome of the research is the average PedsQL Family Impacts Module score. Secondary outcome measurement includes surgical success and patient outcomes. Separate analyses will be conducted for each outcome and compared between the intervention and control groups. Conclusions: Anomalies that can be clearly understood by parents according to the actual size and dimensions of the child's heart will affect the preoperative preparation of the surgical procedure and the recovery rate in the postoperative period

Bronşiolit ve dilate kardiyomyopatisi olan bir bebekte sol koroner arterin pulmoner arterden çıkış anomalisi (ALCAPA)

The most common cause of dilated cardiomyopathy (CMP) is viral myocarditis. However, anomalous origin of the left coronary artery is a rare condition that should be evaluated with high suspicion in the differential diagnosis of an infant with dilated CMP. With early surgical correction, an anomalous coronary artery origin has a good prognosis. Awareness of this condition is essential for prompt diagnosis. This article reports a 5 months-old infant with dilated cardiomyopathy in the setting of RSV bronchiolitis. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was the final diagnosis in the infant, who presented as viral myocarditis with RSV infection. She was operated successfully with the direct reimplantation of the coronary artery to the aorta.Dilate kardiyomyopatinin en yaygın nedeni viral myokardittir. Bununla birlikte sol koroner arterin pulmoner arterden çıkış anomalisi nadir bir durum olup dilate kardiyomyopatinin etiyolojisinde yüksek şüphe ile irdelenmelidir. Erken cerrahi düzeltme ile koroner arter çıkış anomalisinin prognozu iyidir. Bu bozukluğun farkındalığında olmak hızlı teşhis için önemlidir. Makalede 5 aylık bir süt çocuğunda RSV bronşioliti zemininde gelişen dilate kardiyomopati olgusu bildirilmektedir. RSV infeksiyonu ile viral myokardit geliştiği düşünülen bebekte kesin tanı sol koroner arterin pulmoner arterden çıkış anomalisi (ALCAPA) idi. Sol koroner arterin aortaya doğrudan reimplantasyon ameliyatı başarı ile sağlandı