Acute Retroperitoneal Hemorrhage Due to Multi-Vessel Aneurysmal Bleeding As the Initial Manifestation of Polyarteritis Nodosa in a Previously Healthy Female.

Polyarteritis nodosa (PAN) is a heterogeneous disease characterized by constitutional symptoms, multi-organ dysfunction, and a subacute to chronic mode of onset. We describe a rarely encountered manifestation of PAN in a previously healthy 64-year-old woman who developed acute intra-abdominal bleeding due to multi-vessel, vasculitis-induced retroperitoneal aneurysmal ruptures, followed by the spontaneous cessation of abdominal pain and intra-abdominal bleeding within 24 hours after hospitalization, in the absence of immunosuppressive treatment. Aneurysms regressed and eventually normalized coincident with a moderate-term course of oral corticosteroids and six months of monthly intravenous cyclophosphamide. Our report reveals that patients with life-threatening PAN may present acutely with unexpected historical, physical examination and laboratory features not commensurate with the gravity of the clinical findings. We also highlight that long-term survival lasting greater than 11 years can occur when the correct diagnosis and appropriate treatment are introduced early in the course of the illness.

Left Inferior Vena Cava With Infrahepatic Disruption, Azygos Continuation, and Retroaortic Left Renal Vein - A Complex, Symptomatic Caval Anomaly Managed With Endovascular Intervention.

Background: Complex congenital anomalies of the inferior vena cava (IVC) are rare sequelae of inappropriate persistence or regression of embryological precursor veins. These anomalies are typically asymptomatic and generally do not warrant intervention. Case Presentation: Here we present a case of severely symptomatic left IVC with infrahepatic disruption, azygos continuation, and retroaortic left renal vein causing symptoms of severe pelvic congestion and recurrent miscarriages (8 total) in a 41 year old female. The patient was treated with stenting of the compressed retroaortic portion of the IVC/left renal vein. Four months post-procedure, the stent remained patent and the patient reported considerable improvement in their venous congestion symptoms. Most notably, as of the writing of this report, the patient is 38 weeks pregnant. Conclusions: The case is notable for its severe symptomatology of pelvic venous disease including recurrent miscarriage. More importantly, it represents the first documented case of successful retroaortic endovascular management of such a venous anomaly, in which the entirety of the typical IVC drainage occurred via a compressed left-to-right retroaortic crossover.

Image-Guided Biopsy for Relapsed Neuroblastoma: Focus on Safety, Adequacy for Genetic Sequencing, and Correlation of Tumor Cell Percent With Quantitative Lesion MIBG Uptake.

PURPOSE: Many novel therapies for relapsed and refractory neuroblastoma require tumor tissue for genomic sequencing. We analyze our experience with image-guided biopsy in these patients, focusing on safety, yield, adequacy for next-generation sequencing (NGS), and correlation of tumor cell percent (TC%) with quantitative uptake on 123I-meta-iodobenzylguanidine (MIBG) single-photon emission computed tomography with computed tomography (SPECT/CT). MATERIALS AND METHODS: An 11-year retrospective review of image-guided biopsy on 66 patients (30 female), with a median age of 8.7 years (range, 0.9-49 years), who underwent 95 biopsies (55 bone and 40 soft tissue) of relapsed or refractory neuroblastoma lesions was performed. RESULTS: There were seven minor complications (7%) and one major complication (1%). Neuroblastoma was detected in 88% of MIBG- or fluorodeoxyglucose-avid foci. The overall NGS adequacy was 69% (64% in bone and 74% in soft tissue, P = .37). NGS adequacy within neuroblastoma-positive biopsies was 88% (82% bone and 96% soft tissue, P = .11). NGS-adequate biopsies had a greater mean TC% than inadequates (51% v 18%, P = .03). NGS-adequate biopsies had a higher mean number of needle passes (7.5 v 3.4, P = .0002). The mean tissue volume from NGS-adequate soft-tissue lesions was 0.16 cm3 ± 0.12. Lesion:liver and lesion:psoas MIBG uptake ratios correlated with TC% (r = 0.74, r = 0.72, and n = 14). Mean TC% in NGS-adequate samples was 51%, corresponding to a lesion:liver ratio of 2.9 and a lesion:psoas ratio of 9.0. Thirty percent of biopsies showed an actionable ALK mutation or other therapeutically relevant variant. CONCLUSION: Image-guided biopsy for relapsed or refractory neuroblastoma was safe and likely to provide NGS data to guide therapy decisions. A lesion:liver MIBG uptake ratio of ≥ 3 or a lesion:psoas ratio of > 9 was associated with a TC% sufficient to deliver NGS results.

Idiopathic acute mesenteric venous thrombosis causing ischemic enteritis: A case report.

INTRODUCTION: Though mesenteric venous thrombosis (MVT) causes bowel ischemia far less frequently than arterial thrombosis, it still has the potential to cause life-threatening bowel infarction. PRESENTATION OF CASE: Presented here is a case of idiopathic MVT of the superior mesenteric vein and multiple distal venous branches causing diffuse peritonitis secondary to small bowel infarction in a 64 year old male. History and physical exam demonstrated severe persistent abdominal pain, hematochezia, and diffuse abdominal tenderness to palpation with guarding. Venous filling defects and segmental enteritis were noted on CT. The patient was treated with immediate IV heparin therapy with subsequent laparotomy and excision of 45 cm of ischemic ileum. The patient had an uncomplicated recovery. Post-operative thrombophilia screen was negative. The patient was discharged on indefinite warfarin therapy. DISCUSSION: MVT is often idiopathic in nature, with up to 49% having no identifiable cause. Risk factors include abdominal inflammation and systemic thrombophilias. Importantly, bowel infarction is more common with occlusion of more distal, smaller caliber mesenteric vessels. The standard of diagnosis is contrast-enhanced abdominal CT, and management is prompt anticoagulation with surgical intervention if severe. If the cause remains unclear, outpatient anticoagulation is continued indefinitely. CONCLUSION: This case provides a valuable demonstration of several important MVT concepts - specifically the high rate of idiopathic etiology, the need for indefinite anticoagulation in idiopathic cases, and the increased risk of infarction in occlusion of smaller, more distal mesenteric veins.

Demonstrated brain insulin resistance in Alzheimer's disease patients is associated with IGF-1 resistance, IRS-1 dysregulation, and cognitive decline.

While a potential causal factor in Alzheimer's disease (AD), brain insulin resistance has not been demonstrated directly in that disorder. We provide such a demonstration here by showing that the hippocampal formation (HF) and, to a lesser degree, the cerebellar cortex in AD cases without diabetes exhibit markedly reduced responses to insulin signaling in the IR→IRS-1→PI3K signaling pathway with greatly reduced responses to IGF-1 in the IGF-1R→IRS-2→PI3K signaling pathway. Reduced insulin responses were maximal at the level of IRS-1 and were consistently associated with basal elevations in IRS-1 phosphorylated at serine 616 (IRS-1 pS6¹6) and IRS-1 pS6³6/6³9. In the HF, these candidate biomarkers of brain insulin resistance increased commonly and progressively from normal cases to mild cognitively impaired cases to AD cases regardless of diabetes or APOE ε4 status. Levels of IRS-1 pS6¹6 and IRS-1 pS6³6/6³9 and their activated kinases correlated positively with those of oligomeric Aß plaques and were negatively associated with episodic and working memory, even after adjusting for Aß plaques, neurofibrillary tangles, and APOE ε4. Brain insulin resistance thus appears to be an early and common feature of AD, a phenomenon accompanied by IGF-1 resistance and closely associated with IRS-1 dysfunction potentially triggered by Aß oligomers and yet promoting cognitive decline independent of classic AD pathology.