Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
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J Coll Physicians Surg Pak · Dec 2019
Case ReportsRobot-assisted Laparoscopic Nephroureterectomy for a Nephrogenic Adenoma of Ureter.
Nephrogenic adenoma is a rare and benign tumour of the urinary tract thought to be caused by metaplastic change of native urothelial tissue. The majority of cases arise in the bladder, with very few cases affecting the ureter reported in the literature. Herein, we describe the presentation, diagnostic challenges, and the eventual management of a nephrogenic adenoma of the ureter by robot-assisted laparoscopic nephroureterectomy. Urologists and pathologists should be aware of the potential diagnostic and management pitfalls associated with this rare tumour, as well as the sparsity of evidence with respect to follow-up.
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Angiosarcomas constitute less than 1% of primary bone malignancies. Their association with prosthetic material has been described, while their etiology remains unclear. We present a case of a 64-year woman with severe bleeding and hip joint osteolysis after an accidental fall. ⋯ Despite supportive treatment, she died. Autopsy revealed evidently dissemanted angiosarcoma of pelvic bones. Angiosarcoma of the bone should be considered in the differential diagnosis of patients with uncontrollable bleeding and osteolysis, due to the increase of life expectancy when prompt and combined treatment is initiated.
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Kallmann syndrome is a rare genetic disorder marked by hypogonadotropic hypogonadism (HH) and anosmia, affecting 1 in 50,000 females. It is due to a defect of gonadotropin-releasing hormone (GnRH)-secreting neurons migration from the nasal olfactory epithelium to the basal hypothalamus. Non-reproductive, non-olfactory symptoms can also be present, depending on the genetic form of disease. ⋯ MRI of brain was normal. Treatment was started with cyclic conjugated estrogen and progestin with good response. She is now on regular follow-up to monitor treatment.
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J Coll Physicians Surg Pak · Dec 2019
Case ReportsA Single Coronary Artery with an Absent Right Coronary and a Superdominant Left Circumflex Giving off a Branch Supplying the Right Coronary Artery Territory.
An absent right coronary artery (RCA) with single left coronary artery (LCA) originating from left aortic sinus with a superdominant left circumflex (LCX) and giving off an RCA branch is one of the rarest coronary artery anomalies. It occurs with an incidence of less than 0.1%. Usually, patients are asymptomatic and abnormality is found incidentally on cardiac catheterisation or CT angiography. ⋯ Patient was subjected to coronary angiography, which revealed absent RCA originating from distal LCX artery, supplying the base of heart and RCA territory. He underwent primary percutaneous intervention (PCI) with stenting to the left anterior descending (LAD) artery. This type of anomaly, in which single LCA from which dominant LCX continues as RCA, is important to diagnose and manage if diseased, as stenosis of the dominant LCX artery in such cases can jeopardise a large portion of myocardium, which can lead to increased morbidity and mortality, if left untreated.
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J Coll Physicians Surg Pak · Dec 2019
Case ReportsAccessory Cardiac Conduction Pathway with an Unusual Presentation.
Pre-excitation syndrome (PES) is a congenital abnormality in which there is conduction through accessory pathway in addition to atrioventricular (AV) node between the atria and the ventricles. Conduction through accessory pathway is without any delay and; hence, results in early excitation of ventricles. This dual connection provides substrate for atrioventricular reciprocating tachycardia (AVRT), a type of supra-ventricular tachycardia. ⋯ However, ECG monitoring revealed CHB with intermittent conduction through accessory pathway. It highlights the critical evaluation of PES patients with syncope because therapeutic strategy is entirely different. This case was managed with dual chamber pacemaker implantation instead of accessory pathway ablation.