Left-Sided Congenital Diaphragmatic Hernia with Multiple Congenital Cardiac Anomalies, Hernia Sac, and Microscopic Hepatic Heterotopia: A Case Report

Congenital diaphragmatic hernia is a common congenital anomaly of uncertain etiology. Its association with multiple congenital anomalies in various organs is well recognized and antenatal radiological evidence of congenital diaphragmatic hernia warrants thorough evaluation to detect other anomalies, some of which can be life threatening. Rarely, heterotopic hepatic tissue is identified in the hernia, a rare pathological finding, exhibiting more than one macroscopic and microscopic characteristics, and always associated with cardiac congenital anomalies. Herein, we report a case of left-sided microscopic heterotopic hepatic tissue in a congenital diaphragmatic hernia in an infant with multiple cardiac congenital anomalies, but with preserved pericardium

Fatal Subacute Hepatic Failure in a Patient with AA-Type Amyloidosis: Case Report

Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as subacute hepatic failure and resulted in a fatal clinical course in a 69-year-old man. To the best of our knowledge, this is the fifth case of hepatic amyloidosis of AA type that clinically presented as fatal subacute hepatic failure, an unusual clinical presentation for hepatic involvement by systemic AA-type amyloid

Salvaging of severely ruptured living-related renal allograft secondary to acute antibody mediated rejection

AbstractINTRODUCTIONSpontaneous renal allograft rupture (RAR) is a serious and potentially life-threatening complication of kidney transplantation. Debate on the management of RAR has focused on graft nephrectomy versus salvaging in cases where: the allograft rupture site is surgically manageable; the bleeding can be controlled; and/or leaving the renal allograft in situ does not compromise patient survival.PRESENTATION OF CASEA 45-year-old, living-related, female, kidney allograft recipient experienced RAR on the fourth day post transplantation. Surgical exploration showed 12cm laceration along the convex border of the graft. Histologically the graft demonstrated mild acute kidney injury and linear deposition of C4d along the cortical peritubular capillaries; morphological features for violent humoral or cellular rejection were not identified. The graft was surgically salvaged with excellent clinical and biochemical improvement.DISCUSSIONObservations arising from this case are: (1) RAR caused by rejection is still encountered in clinical practice despite effective immunosuppressive management; (2) the severity of the histopathological features of rejection does not necessarily correlate with the extent of graft rupture; and (3) salvaging the graft should be attempted whenever possible as current immunosuppression and advances in surgical techniques may have an impact on long-term graft function and survival, differing from those previously published.CONCLUSIONWith modern immunosuppression therapy and proven surgical procedures, the efficacy of salvaged renal grafts and graft survival rates may improve substantially

Skin adnexal neoplasms—part 2: An approach to tumours of cutaneous sweat glands

Tumours of cutaneous sweat glands are uncommon, with a wide histological spectrum, complex classification and many different terms often used to describe the same tumour. Furthermore, many eccrine/apocrine lesions coexist within hamartomas or within lesions with composite/mixed differentiation. In addition to the eccrine and apocrine glands, two other skin sweat glands have recently been described: the apoeccrine and the mammary‐like glands of the anogenital area. In this review (the second of two articles on skin adnexal neoplasms), common as well as important benign and malignant lesions of cutaneous sweat glands are described, and a summary for differentiating primary adnexal neoplasms from metastatic carcinoma is outlined, striving to maintain a common and acceptable terminology in this complex subject. Composite/mixed adnexal tumours are also discussed briefly

Treatment of Light Chain Deposition Disease Using Bortezomib-Based Regimen Followed by Thalidomide-Based Regimen in a Saudi Male

Light chain deposition disease (LCDD) is a rare illness with, as yet, no clear evidence-based guidelines for its treatment. To the best of our knowledge, LCDD has not been previously reported from Saudi Arabia. We present in this report, a 38-year-old Saudi male who presented with clinical features suggestive of hypertensive nephropathy but kidney biopsy later revealed the diagnosis of LCDD. His serum creatinine at presentation was 297 μmol/L which came down to 194 μmol/L on treatment with Bortezomib, Cyclophosphamide and Dexamethasone. His 24-hour protein excretion at presentation was 6 g/L which also came down to less than 1 g/day. He was later placed on Cyclophosphamide, Thalidomide, and Dexamethasone regimen because of persistent high titres of serum free light chains. He went into remission with undetectable serum free light chains and remained so for three years at the time of writing this report. We conclude that LCDD, though rare, does occur in Saudi population. The treatment of LCDD is challenging but the use of Bortezomib, a proteosome inhibitor, is promising. However, suboptimal response may require further treatment with other therapeutic options such as chemotherapy with alkylating agents or high-dose Melphalan with autologous stem cell transplant

Incidental Eosinophilic Chromophobe Renal Cell Carcinoma in Renal Allograft

The incidence of renal cell carcinoma (RCC) in renal allograft in transplant recipients is 0.22–0.25%. De novo clear cell, papillary, and chromophobe RCCs and RCCs with sarcomatoid differentiation originating in renal allograft have been reported. Routine surveillance for graft tumours is not routinely practiced and these tumours are commonly asymptomatic and incidentally discovered. We describe a case of incidental, eosinophilic chromophobe RCC in a 31-year-old, long-term renal transplant male recipient, who presented with acute gastroenteritis 11 years after transplantation. The graft was nonfunctional at the time of presentation. Abdominal ultrasound and computed tomography scan demonstrated 1.8 cm well-defined, round enhancing lesion, confined to the renal allograft and suspicious for malignancy. Pathological examination of graft nephrectomy specimen showed gross, histopathological, and immunohistochemical features of eosinophilic chromophobe RCC. Fifty-five months after surgery, the patient was alive and free of malignancy. To the best of our knowledge, only five chromophobe RCCs originating in a renal allograft were previously described in English literature. We suggest that chromophobe RCC should be considered in the differential diagnosis of renal allograft mass, including eosinophilic tumours, and emphasise the importance of periodic screening of renal allograft in all renal transplant recipients

Late Renal Allograft Rupture Associated with Cessation of Immunosuppression following Graft Failure

A 29-year-old man developed chronic allograft nephropathy 63 months after renal transplantation. He became symptomatic with advanced chronic graft failure; his immunosuppressive medications were reduced and he was commenced on haemodialysis. Two months following the withdrawal of immunosuppression, he presented with abdominal pain, haematuria, and a marked drop in haemoglobin. The patient was taken to the operating room, where the renal allograft was found to be ruptured, and graft nephrectomy was subsequently performed. Histological examination of the graft specimen showed severe haemorrhagic acute vascular cellular rejection in a background of marked chronic allograft vasculopathy. Immunostaining for C4d showed diffuse, strong, linear circumferential staining of the peritubular capillaries, indicating a concurrent antibody-mediated rejection. We report herein an unusual case of spontaneous renal allograft rupture that occurred long time after transplantation due to severe acute rejection following cessation of immunosuppressive medications for advanced chronic allograft failure. To the best of our knowledge, the time interval between transplantation and the rupture of this allograft is the longest of those reported in the literature

Community-associated methicillin-resistant Staphylococcus aureus causing diffuse xanthogranulomatous pyelonephritis in a neonate

Xanthogranulomatous pyelonephritis (XGP) is an uncommon variant of chronic pyelonephritis; often associated with ipsilateral urological obstructive pathology and infection. It occurs rarely in the pediatric population and is caused usually by gram-negative bacteria. We herein present a case of a 6-week old male patient who presented with fever, gross hematuria and left flank tenderness. Urine and blood cultures were negative. Radiological investigations suggested an infiltrating malignant neoplasm of the kidney. There was no evidence of nephrolithiasis or obstructive pathology. A left radical nephrectomy was performed and histopathological examination revealed diffuse XGP. Microbiological culture of the perinephric purulent discharge proved positive for methicillin-resistant Staphylococcus aureus (MRSA). To the best of our knowledge, this is the first reported case of MRSA-induced XGP in a neonate emphasizing the expanding spectrum of disease secondary to community-associated MRSA

Collagenofibrotic (Collagen Type III) glomerulopathy in association with diabetic nephropathy

Collagenofìbrotic (collagen type III) glomerulopathy (CG) is a rare nonimmune-mediated glomerular disease. It is characterized by massive deposition of organized collagen type III fibers, which is localized in the mesangial and subendothelial glomerular areas and associated with increased serum levels of procollagen type III peptide. Association with systemic diseases and malignancies is extremely rare. Herein, we present a case of a nine-year-old girl, known case of type I diabetes mellitus, who presented with fever, nephrotic range proteinuria, generalized edema, and hypertension. Clinical examination did not show nail abnormalities or bone abnormalities. Renal biopsy revealed mesangial expansion and remarkable narrowing and obliteration of the glomerular capillaries by pale, amorphous material. Immunohistochemical study demonstrated diffuse linear glomerular capillary and tubular basement membrane staining for immunoglobulin G (IgG) and albumin. Ultrastructural examination identified massive mesangial and sub-endothelial deposition of dense frayed, curvilinear banded fibers with characteristic features of type III collagen. The patient was diagnosed to have combined CG and diabetic nephropathy (DN). This is the first report of CG in association with diabetic changes in renal biopsy. In this report, we describe the clinicopathological characteristics of this disease, review CG in pediatric population, and explore its association with DN