Introduction: Kidney anomalies present a challenge even for the most experienced vascular surgeon in the reconstruction of the aortoilliac segment. The most significant anomalies described in the surgery of the aortoilliac segment are a horse-shoe and ectopic kidney.
Objective: The aim of this retrospective study was to analyze experience on 40 patients with renal anomalies, who underwent surgery of the aortoilliac segment and to determine attitudes on conventional surgical treatment.
Methods: In the period from 1992 to 2009, at the Clinic for Vascular Surgery of the Clinical Centre of Belgrade we operated on 40 patients with renal anomalies and aortic disease (aneurysmatic and obstructive). The retrospective analysis involved standard epidemiological data of each patient (gender, age, risk factors for atherosclerosis, type of anomaly, type of aortic disease, presurgical parameter values of renal function), type of surgical approach (laparatomy or retroperitoneal approach), classification of the renal isthmus, reimplantation of renal arteries and perioperative morbidity and mortality.
Results: Twenty patients were males In 30 (70%) patients we diagnosed a horse-shoe kidney and in 10(30%) ectopic kidney. In the cases of ruptured aneurysm of the abdominal aorta the diagnosis was made by ultrasound findings. Pre-surgically, renal anomalies were confirmed in all patients, except in those with a ruptured aneurysm who underwent urgent surgery. In all patients we applied medial laparatomy, except in those with a thoracoabdominal aneurysm type IV, when the retroperitonal approach was necessary. On average the patients were under follow-up for 6.2 years (from 6 months to 17 years).
Conclusion: Under our conditions, the so-called double clamp technique with the preservation of the kidney gave best results in the patients with renal anomalies and aortic disease.
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Pediatr Nephrol
January 2025
Department of Pediatric Nephrology, Istanbul University- Cerrahpasa, Cerrahpasa Faculty of Medicine, 34098, Istanbul, Turkey.
Autosomal recessive proximal renal tubular acidosis (AR-pRTA) with ocular abnormalities is a rare syndrome caused by variants in the SLC4A4 gene, which encodes Na/HCO3 cotransporter (NBCe1). The syndrome primarily affects the kidneys, but also causes extra-renal manifestations. Pancreatic type NBCe1 is located at the basolateral membrane of the pancreatic ductal cells and together with CFTR chloride channel, it is involved in bicarbonate secretion.
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Emergency Department, Habib bourguiba university hospital, Faculty of Medicine, Sfax University, Majida Boulila Avenue, Sfax, Tunisia.
Introduction: Electrical injuries (EIs) represent a significant clinical challenge due to their complex pathophysiology and variable presentation, ranging from minor burns to severe internal organ damage. Despite their prevalence in both; domestic and occupational settings, there remains a rareness of systematic guidelines and comprehensive literature to aid clinicians in effectively managing these injuries. Understanding these factors is crucial for developing protocols that can mitigate the risk of delayed complications, such as cardiac arrhythmias, in patients who initially appear stable.
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Lamei Yuan, MD, PhD, Health Management Center, the Third Xiangya Hospital, Disease Genome Research Center, Center for Experimental Medicine, the Third Xiangya Hospital, Research Center of Medical Experimental Technology, the Third Xiangya Hospital, Xiangya School of Medicine, Central South University, Changsha 410013, Hunan, China.
Objective: To identify the disease-causing variant in a family with tuberous sclerosis complex (TSC).
Methods: This study including a Han-Chinese pedigree recruited from the Third Xiangya Hospital, Central South University, Changsha, Hunan, China was conducted between February, 2019 and January, 2023. Detailed clinical examinations were performed on the proband and other family members of a Han-Chinese family with TSC.
Sudan J Paediatr
January 2024
Department of Paediatrics, South West Acute Hospital, Enniskillen, UK.
Pseudohypoaldosteronism (PHA) is a rare disorder that mimics congenital adrenal hyperplasia (CAH). Renal type A1A of the disorder has a known gene mutation (NR3C2) and parents may be asymptomatic despite biochemical abnormalities. Meticulous interpretation of hormonal and biochemical data, and early liaison with endocrinology and renal teams are key in diagnosis.
View Article and Find Full Text PDFUrol Case Rep
January 2025
Department of Urology, China Medical University Hospital, Taiwan.
Zinner syndrome is a congenital anomaly characterized by seminal vesicle cysts, ipsilateral renal agenesis, and ejaculatory duct obstruction possibly associated with infertility. Only 200 cases of Zinner syndrome have been reported since its discovery in 1914. We present the case of a 63-year-old man seeking treatment for a ureteropelvic junction stone causing severe hydronephrosis.
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