Preeminence of Lesser Splanchnic Blood Flow in Selected Patients With Generalized Portal Hypertension

Although restricted transhepatic portal flow is necessary for development of generalized portal hypertension (GPH), increased splanchnic arterial inflow also contributes to GPH and its clinical sequelae. In this context, we describe 7 male and 6 female patients (mean age 48 years) in whom the lesser splanchnic (gastrosplenic) system played a key role in the signs and symptoms of GPH. These 13 patients (9 with hepatic cirrhosis, 3 with primary myeloproliferative disorder, and 1 with extrahepatic portal block) shared common features of massive splenomegaly, huge splenofundic gastric varices, often with a prominent natural shunt to the left renal vein. Total or near total splenectomy alone or combined where appropriate with coronary vein ligation was effective in controlling varix hemorrhage (10 patients), ascites (3), or complications of an enlarged spleen-anorexia and abdominal pain (3), hemolytic anemia (1) and profound thrombocytopenia with severe epistaxis (1). Intraoperative jejunal portal venography was crucial in operative management in order to establish definitively the presence or absence of coronary venous collaterals, and when present, to verify their operative ligation

Immunoarchitecture of the Regenerating Rat Spleen: Effects of Partial Splenectomy and Heterotopic Autotransplantation

To investigate the microstructure of in situ (eutopic) and autotransplanted (ectopic) splenic remnants, adult Sprague-Dawley rats were studied 60 days after 1) subtotal (~80%) splenectomy, 2) total splenectomy followed by single or multiple remnant intraperitoneal autotransplantation, or 3) sham operation. Total nucleated cell counts were determined in excised splenic remnants, and immunohistochemical staining using monoclonal antibodies to rat B-and T-cell antigens was performed in serial tissue sections. Immunoarchitecture of eutopic remnants was indistinguishable from that of intact spleens and total nucleated cell counts remained proportional to weight. In contrast, ectopic remnants showed sparsity and abnormal mixing of B and T lymphocyte subpopulations with widespread loss of follicles and periarteriolar lymphoid sheaths in addition to lower density and marked reduction of total nucleated cells. These findings provide immunohistologic evidence that preservation of intact vasculature is critical to splenic architecture, which may account in part for the demonstrable functional inferiority of ectopic remnants

Lymphangiogenesis, lymphatic systemomics, and cancer: context, advances and unanswered questions

Ever since it was discovered that endothelial cells line lymphatic vessels, investigators have been working on unraveling the mechanisms that control the growth of this distinctive endothelium and its role in normal physiology and human disease. Recent technological advances have ushered in a new era of "omics" research on the lymphatic system. Research on the genome, transcriptome, proteome, and metabolome of lymphatics has increased our understanding of the biology of the lymphatic vasculature. Here, we introduce the context-lymphatic "systemomics," then briefly review some of the latest advances in research on tumor-associated lymphatic vessels highlighting several "omic" studies that have shed light on mechanisms controlling the growth and function of tumor-associated lymphatic vessels. We conclude by returning, with unanswered questions, to the larger context of cancer and the lymphatic system as a vasculature, circulation, route of entry and transport, and control center of the immune network.12 month embargo; published online: 28 May 2018This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.Open access journalThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle

Patients with single ventricle congenital heart disease are at risk of unpredictable protein-losing enteropathy (PLE) after surgical palliation. Based on prior reports of physiologic differences for patients with single morphologic right versus left ventricles, we hypothesized that those with right ventricular morphology would have a higher incidence of PLE. We performed a retrospective review of > 15 million pediatric hospitalizations from the Healthcare Cost and Utilization Project KID 2000-2012 databases for admissions 5-21 years old with ICD-9 codes for hypoplastic left heart syndrome (HLHS) and tricuspid atresia (TA) with and without PLE. Incidence of PLE was compared between those with HLHS and TA. In addition, outcomes and costs were compared between admissions with and without PLE and between HLHS and TA. Of 1623 HLHS admissions, 289 (17.8%) had PLE, and of 926 TA admissions, 58 (5.9%) had PLE (p < 0.001). Admissions with PLE were older compared to those without PLE (12 vs 10 years, p < 0.001) and PLE onset occurred at a younger age for HLHS than TA (11 vs 14 years, p < 0.001). There were no differences in hospital outcomes or costs. Review of this large administrative database suggests a higher incidence of PLE in patients with HLHS and a younger age of onset compared to those with TA. These data suggest that a single systemic right ventricle may be an independent risk factor for developing PLE.12 month embargo; published 25 September 2020This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]