Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
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J Coll Physicians Surg Pak · Aug 2022
Case ReportsTwo Cases of Interrupted Inferior Vena Cava with Azygos / Hemiazygos Continuation.
Interrupted inferior vena cava (IVC) is a rare disease, occurring either in isolation or in association with asplenia or polysplenia syndromes. Infrahepatic part of the IVC is absent representing the failure of fusion of the vitelline and subcardinal embryological portions of the IVC. ⋯ We present two cases of interrupted IVC, one occurring in isolation with hemiazygos continuation and discovered incidentally, and the second one is a child with azygos continuation, associated with polysplenia syndrome. Key Words: Inferior vena cava, Polysplenia, Azygous vein, Hemiazygos vein.
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J Coll Physicians Surg Pak · Aug 2022
Case ReportsA Rare Case of Female Bladder Outlet Obstruction: Severe Introital Stenosis.
Herein, we report a rare case of severe introital stenosis in a 22-year female who presented with obstructive voiding symptoms and inability to have sexual intercourse. Introital stenosis had been caused by the emergency primary closure after major vulvoperineal trauma 15 years ago. To our knowledge, this is the first case report in the literature that describes a very long time from primary closure to dilation and reconstruction. ⋯ Our technique was effective and did not lead to any complications. The sexual dysfunction and voiding complaints of the patient completely resolved within a post-operative period of 11 months. Key Words: Introital stenosis, Bladder outlet obstruction, Dilation, Female, Reconstruction.
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J Coll Physicians Surg Pak · Aug 2022
Case ReportsA Rare Case of Propionic Acidemia in a Six Months Female Child.
Propionic Acidemia (PA) is a rare metabolic disorder caused by the defect in enzyme (propionyl-coenzyme A (CoA) carboxylase) leading to the abnormal accumulation of metabolites of branched-chain amino acid catabolism in blood and urine. We describe the first ever diagnosed case in our setup of early onset PA in a 06 months old baby girl who presented with the complaints of decreased feed intake, lethargy, vomiting, failure to thrive, and intermittent seizures. ⋯ Finally, urine organic acid analysis by gas chromatography-mass spectrometry (GCMS) showed marked elevation of 3-hydroxy propionate, methyl citrate, and 3-hydroxy, 2 methylbutyric acid with moderate rise in 3-hydroxy butyric acid without an elevation of methylmalonate in urine, thus giving the diagnosis of PA. Key Words: Propionic acidemia, Propionyl-CoA Carboxylase deficiency, Gas chromatography-mass spectrometry.
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J Coll Physicians Surg Pak · Aug 2022
Case ReportsSolid Renal Mass in a Transplanted Allograft Kidney: Mucinous Tubular and Spindle Cell Renal Cell Carcinoma.
Renal transplantation is the main treatment for end-stage kidney disease. Transplantation reduces the mortality rate and increases life quality in this population but cancer in allograft kidney is a major problem. Here, we report a 39-year male who had renal transplantation 10 years ago. ⋯ After partial nephrectomy, histopathologic examination revealed mucinous tubular and spindle cell variant of renal cell carcinoma(MTSCC). To our best knowledge, this is the first case in the literature that underwent partial nephrectomy in the transplanted kidney and was diagnosed with MTSCC. Key Words: Renal mass, Mucinous tubular and spindle cell tumor, Kidney transplantation.
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J Coll Physicians Surg Pak · Aug 2022
Case ReportsCutaneous T-cell Lymphoma Predisposing to Recurrent Infective Endocarditis by Methicillin-resistant Staphylococcus Aureus (MRSA) in a Patient with Intracardiac Defibrillator (ICD).
Cutaneous T-cell lymphoma (CTCL) is a rare group of extra-nodal non-Hodgkin's lymphomas resulting in infiltration of the skin by the malignant cells. Sézary syndrome (SS) and mycosis fungoides (MF) are the most common subtypes, and infectious complications are the major cause of death in such patients. ⋯ Herein, we report a unique case of non-ischemic cardiomyopathy with an implantable cardiac defibrillator (ICD), who later developed CTCL, complicated by the recurrent right-sided IE which is caused by MRSA. Key Words: Cutaneous T-cell lymphoma, Methicillin-resistant staphylococcus aureus (MRSA), Infective endocarditis.