BK polyomavirus nephropathy with systemic viral spread: Whole genome sequencing data from a fatal case of BKPyV infection.

BK polyomavirus (BKPyV) infections with multi-organ involvement are rare. Here, we report for the first time whole genome sequencing data from a patient with systemic BKPyV disease. She presented post stem cell transplantation with graft-vs-host disease, suffered from profound immunosuppression, and developed fatal BKPyV disease of kidneys, lungs, and pancreas. The lytic infection was caused by an episomal BKPyV-Ib strain with canonical structural and receptor encoding gene sequences. However, DNA from all infected tissue sites showed diverse BKPyV-NCCR rearrangements (rr-NCCR) involving the P, Q, and R domains, while largely sparing O and S, carrying initiation sites for early and late BKPyV gene transcripts crucial for viral replication and assembly. Common to all rr-NCCR variants was a break point in Q (position 17-27) that can form the nidus for double DNA strand break formation and gene rearrangements. Metastatic clonal BKPyV spread from kidneys to other organs was not detected. We hypothesize that lack of immune surveillance and a specific NCCR break point promote profound gene rearrangements of NCCR-P, Q, and R with alterations of regulatory feedback loops. As a result, viral replication and pathogenicity are enhanced leading to severe, often fatal systemic disease not caused by the common archetypical BKPyV strains.

Erdheim-Chester disease presenting as meningitis with hypoglycorrhachia: A case report.

INTRODUCTION: Erdheim-Chester disease (ECD) commonly has neurologic manifestations but rarely presents with meningitis and hypoglycorrhachia. Here, were present a case of ECD with a clinical and laboratory presentation initially thought to be bacterial meningitis with sepsis. METHODS: We report a case of a 79-year-old with history of enigmatic bone pain and peritoneal nodules who presented with meningitis. After failure to improve on antibiotic therapy other etiologies of hypoglycorrhachia including sarcoid, tuberculosis, and fungal and carcinomatous meningitis were considered. However, no definite diagnosis could be made based on radiologic, serologic, microbiologic, and molecular testing and the patient failed to improve on empiric therapy including antibiotics, antifungals, and tuberculosis and steroid therapy. RESULTS: Ultimately, autopsy revealed a new diagnosis of ECD manifesting as meningitis, a rare presentation of a rare disease. CONCLUSION: Although only reported in one other case to our knowledge, ECD can present with meningitis with hypoglycorrhachia.

Strategies in the management of renal tumors amenable to partial nephrectomy.

PURPOSE: The laparoscopic approach to radical and partial nephrectomy is becoming the standard of care for treating patients with renal tumors. Hand-assisted laparoscopic partial nephrectomy (HALPN) provides some advantages over the pure laparoscopic approach which include manual manipulation of the kidney, tactile feedback, and timely specimen removal. MATERIALS AND METHODS: We describe our technique for HALPN and emphasize the implementation of an in-room pathologist to examine gross margins during the period of renal arterial occlusion. Between 2004 and 2007, 46 patients underwent HALPN performed by the same surgeons. Mean patient age was 59.5 years and mean tumor size was 2.55 cm. Twelve of these patients underwent significant concomitant procedures. RESULTS: Our mean operating time was 173.26 min (range 90-306 min) and our mean warm ischemic time was 28.32 min (range 14-54 min). Average estimated blood loss was 116.82 ml (range 10-1000 ml) with no transfusions. Thirty-six (78%) tumors were renal cell carcinoma, seven (15%) were oncocytomas, and three (7%) were angiomyolipomas. The average length of stay was 5.17 days (range 3-9 days) and there were no positive margins. There was one postoperative bleed (2%) and two postoperative urine leaks (4.3%). DISCUSSION: In our institution, the hand-assist approach to laparoscopic partial nephrectomy has resulted in favorable perioperative outcomes. The use of an in-room pathologist to provide real-time assessment of gross tumor margins has allowed us to achieve a 0% positive final margin rate. We believe that the use of an in-room pathologist during the timely extraction of the specimen made possible by the hand-assisted approach provides a great advantage over pure laparoscopic partial nephrectomy. This low positive margin rate is also the result of maintaining a bloodless field of resection with temporary renal arterial occlusion as well as the avoidance of visual tissue distortion with cold, sharp scissor dissection.

Incidental finding of bilateral renal and adrenal anastomosing hemangiomas: A rare case report.

Anastomosing hemangiomas are rare variants of vascular tumors found in adrenal, hepatic, and gastrointestinal tissue. Frequently, renal anastomosing hemangiomas are misdiagnosed on computed tomography (CT) as kidney cancers, resulting in unnecessary workups and detrimental treatments. We present a rare case of bilateral renal and adrenal anastomosing hemangioma found incidentally on renal biopsy. Patient is a 39 year-old African American male on hemodialysis with a history of end-stage renal disease secondary to lupus who presented with acute pericarditis and worsening renal insufficiency.