Background and Objective: Emphysematous pyelonephritis (EPN) is a rare, life-threatening necrotizing renal parenchymal infection. Traditional management of EPN with nephrectomy had a mortality of 40-50%. The purpose of this case series was to assess the management, biochemical factors, and outcome of EPN patients. Methods: In this retrospective study, patients admitted to The Indus Hospital, Karachi with a diagnosis of EPN from January 2010 to February 2019, were grouped according to the Huang Tseng Classification (HTC). Their biochemical parameters, sensorium states and outcomes were recorded and analysed. Results: Twenty patients were reviewed (9 males). No mortality was recorded. 11 patients (55%) were treated conservatively with only intravenous antibiotics and eight patients underwent an intervention: minimally invasive with drain placement in six patients, and invasive in four patients (two underwent subsequent nephrectomy, and two patients had nephrectomy only). One patient left against medical advice. All patients had decreased serum creatinine levels and total leucocyte counts on discharge. Conclusion: EPN can be successfully managed conservatively for HTC Grade-1 and 2. Conservative treatment may be tried in higher grades, but poor response should lead to prompt escalation of treatment. doi: https://doi.org/10.12669/pjms.36.ICON-Suppl.1728 How to cite this:Irfaan AM, Shaikh NA, Jamshaid A, Qureshi AH. Emphysematous Pyelonephritis: A single center review. Pak J Med Sci. Special Supplement ICON 2020. 2020;36(1):S83-S86 doi: https://doi.org/10.12669/pjms.36.ICON-Suppl.1728 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background Peutz-Jeghers syndrome is an autosomal dominant disease characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract (GIT). There have also been cases of extra GIT polyps such as the renal pelvis, urinary bladder, lungs and nares. Bladder hamartoma is an extremely rare finding, with only 12 cases described in the literature up to now. The rarity of the condition necessitates a comprehensive compilation of managements up to now so as to provide a better tool for the treatment of such conditions in the future. Case presentation A twenty-year-old male, known to have Peutz-Jeghers syndrome, presented to us complaining of obstructive urinary symptoms. A urethrogram done showed a filling defect at the base of the urinary bladder. The mass was resected transurethrally, and histopathology revealed a hamartoma of the bladder. The patient has since remained tumor-free on follow-up. Conclusions Transurethral resection of the bladder mass proved to be an effective therapy in this patient with no recurrence on the patient’s follow-up till now. There is still, however, a dearth of knowledge regarding the management of bladder hamartomas owing to the extreme rarity of the case.
Leiomyosarcoma (LMS) of the colon accounts for <1% of all colorectal malignancies. Our patient was a 72-year-old man with a history of aortic valvular disorder and congestive heart failure, who presented with an abdominal mass and no constitutional symptoms. The CT scan finding suggested a large tumour with both solid and cystic components. Intraoperatively, a portion of the involved colon was resected along with the tumour. Microscopically, the tumour was found to invade the muscularis propria layer of the transverse colon. The final diagnosis was LMS, FNCLCC grade 2 of 3 based on the histology and immunochemistry.
Background Tuberous sclerosis complex (TSC) is a rare genetic disorder characterized by benign hamartomas in multiple organs of the body. Renal angiomyolipomas (AML) are commonly associated with TSC. They are mostly asymptomatic. But large and rapidly growing AMLs with the presence of an aneurysm cause symptoms and pose a life-threatening risk for hemorrhage. Case presentation Our patient is a 25-year-old female who presented to us as an undiagnosed case of tuberous sclerosis having a large abdominal mass. She fulfilled the clinical criteria required for the diagnosis of TSC. The CT scan revealed an 18 × 13 × 33 cm fat-containing lesion in the right kidney with an adjacent aneurysm measuring around 16 cm in diameter. Due to the large size of the AML and associated aneurysm, surgical exploration was mandated. On the contralateral kidney, multiple contrast-enhancing soft-tissue densities were present that appeared suspicious on radiology. So a percutaneous biopsy of those lesions was done. Fortunately, it had the same histopathology as an Angiomyolipoma. Nephrectomy of the right-sided kidney with AML has been done. The left-sided lesions that are less than 2 cm and asymptomatic are kept on close surveillance. Any change in size will prompt therapy with mTOR inhibitors. Conclusion When dealing with bilateral renal AML, it is important to adopt a conservative approach. When intervention is indicated, the least invasive strategy should be sought and enacted. Radical surgery should be the last resort.
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