scholarly journals Liver lymphatic anatomy and role in systemic lymphatic disease

2021 ◽  
Author(s):  
Christopher L. Smith ◽  
Mandi Liu ◽  
Madhumitha Saravanan ◽  
Aaron G. Dewitt ◽  
David M. Biko ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Length Of Hospital Stay ◽  
Flow Patterns ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
Persistent Symptoms ◽  
Key Points ◽  
Substantial Correlation ◽  
Abnormal Pattern ◽  
Lymphatic Imaging

Abstract Objectives To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. Methods In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 – April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed. Results During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics. Conclusion We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal. Key Points • We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal – chylothorax/PB, hepatoperitoneal – ascites/chylothorax, hepatopulmonary – chylothorax/PB, hepatoduodenal – PLE). • Abnormal imaging patterns correlated with increased morbidity.

scholarly journals Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

2018 ◽  
Vol 38 (6) ◽  
pp. e5-e12 ◽  
Author(s):  
Christine Peyton
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Complex Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Venous Hypertension ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
Fontan Physiology

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Patients with single-ventricle physiology who have undergone the Fontan procedure are at risk for these conditions. Fontan physiology predisposes patients to chronically low cardiac output, increased central venous pressure, and congestive heart failure. These altered hemodynamics lead to increased mesenteric vascular resistance, resulting in venous hypertension and congestion in protein-losing enteropathy. Plastic bronchitis is a complex disease in which chronic high lymphatic pressures from Fontan physiology cause acellular bronchial casts to develop. These entities may also occur in patients with normal Fontan hemodynamics. This article also covers medical and surgical interventions for protein-losing enteropathy and plastic bronchitis. (Critical Care Nurse. 2018;38[6]:e5–e12)

scholarly journals 665. Key Clinical and Laboratory Features in Early Diagnosis of Ehrlichiosis in an Endemic Area of Long Island, New York

2018 ◽  
Vol 5 (suppl_1) ◽  
pp. S240-S241
Author(s):  
Olga Kaplun ◽  
Kalie Smith ◽  
Teresa Khoo ◽  
Eric Spitzer ◽  
Fredric Weinbaum ◽  
...  
Keyword(s):  
New York ◽  
Clinical Presentation ◽  
Average Length ◽  
Kidney Injury ◽  
Length Of Hospital Stay ◽  
Retrospective Chart Review ◽  
Suffolk County ◽  
University Hospital ◽  
Laboratory Findings ◽  
Significant Difference

Abstract Background Human monocytic ehrlichiosis (HME) is a tick-borne disease caused by Ehrlichia chafeensis in the northeast United States. Suffolk County, New York has the highest amount of HME cases in NY (176 from 2010 to 2014). Our aim is to identify risk factors for HME and compare clinical presentation and laboratory findings of young vs. older adults. Methods A retrospective chart review from January 1, 2014 to December 31, 2017 was performed on all patients ≥18 years who presented to the ER at Stony Brook University Hospital (SBUH) or Stony Brook Southampton Hospital (SBSH) with (i) ICD-9 code 082.4 or ICD-10 code A77.40 and (ii) a positive E. Chafeensis PCR. Data were collected on demographics, clinical presentation, and laboratory results. Results Twenty-seven cases of HME were found and separated into Group 1 (G1, n = 10) or Group 2 (G2, n = 17) based on age (Table 1). G1 had a significantly higher chance of being Hispanic than G2. Twenty-four of the 27 patients (89%) were hospitalized with an average length of stay of 3.4 days (range 1–14 days).The only significant difference in clinical presentation was that G1 was more likely to have myalgia (P = 0.02). 40% or more of patients in both groups presented with an acute kidney injury and the average length of hospital stay in days was 4.0 ± 2.9 and 3.2 ± 3.1 for G1 and G2, respectively. The number of cases overall have increased 6.0% per year between 2014 and 2017. Thrombocytopenia presented in all cases. Conclusion. HME is prevalent in Suffolk County. Clinical presentation and laboratory findings were largely similar between the two groups, except the younger population more often presented with myalgia. A risk factor in this study was to be young and Hispanic, likely due to occupational exposure. Disclosures All authors: No reported disclosures.

scholarly journals Eosinophilic Enterocolitis Diagnosed by Means of Upper Endoscopy and Colonoscopy with Random Biopsies Treated with Budenoside: A Case Report and Review of the Literature

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Ghulamullah Shahzad ◽  
Duane Moise ◽  
Seth Lipka ◽  
Kaleem Rizvon ◽  
Paul J. Mustacchia
Keyword(s):  
Inflammatory Process ◽  
Colonic Mucosa ◽  
Clinical Presentation ◽  
Age Group ◽  
Pediatric Age ◽  
Peripheral Eosinophilia ◽  
Review Of The Literature ◽  
Unique Case ◽  
Protein Losing Enteropathy ◽  
Pediatric Age Group

Intense infiltration of gastrointestinal and colonic mucosa with eosinophils or acidophilic gastroenteritis (EG) is a relatively uncommon picture for a pathologist endoscopist especially outside the pediatric age group and is highly suggestive of an ongoing chronic inflammatory process. Existing literature projected a hypothetical association with allergy but the exact pathophysiology is still unknown. Association with malabsorption, protein losing enteropathy, and refractory ulcers with gastrointestinal bleeding makes the clinical presentation more complicated. We present a unique case of diarrhea and abdominal pain in the clinical presentation with associated peripheral eosinophilia, asthma, and gastroesophageal reflux disease (GERD). The patient's symptoms abated after initiation of budesonide.

Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii84-iii94 ◽  
Author(s):  
Giorgio Trivioli ◽  
Benjamin Terrier ◽  
Augusto Vaglio
Keyword(s):  
B Cells ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Mononeuritis Multiplex ◽  
Persistent Symptoms ◽  
Tissue Eosinophilia ◽  
Eosinophilic Granulomatosis

Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.

scholarly journals The clinical spectrum of Erdheim-Chester disease: an observational cohort study

2017 ◽  
Vol 1 (6) ◽  
pp. 357-366 ◽  
Author(s):  
Juvianee I. Estrada-Veras ◽  
Kevin J. O’Brien ◽  
Louisa C. Boyd ◽  
Rahul H. Dave ◽  
Benjamin H. Durham ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Age Of Onset ◽  
Observational Cohort Study ◽  
World Health ◽  
Key Points ◽  
The World ◽  
Observational Cohort ◽  
Health Organization ◽  
Erdheim Chester ◽  
Chester Disease

Key Points ECD varies in terms of age of onset, clinical presentation, manifestations, organ involvement, disease severity, and survival. ECD is a neoplasm and should be adopted by the field of hematology-oncology following the World Health Organization reclassification.

scholarly journals Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease

2020 ◽  
Vol 22 (12) ◽  
Author(s):  
Benjamin Kelly ◽  
Sheyanth Mohanakumar ◽  
Vibeke Elisabeth Hjortdal
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Treatment Options ◽  
Future Research ◽  
Protein Losing Enteropathy ◽  
Current Review ◽  
Lymphatic Complications ◽  
And Function ◽  
Lymphatic Imaging

Abstract Purpose of Review Lymphatic disorders have received an increasing amount of attention over the last decade. Sparked primarily by improved imaging modalities and the dawn of lymphatic interventions, understanding, diagnostics, and treatment of lymphatic complications have undergone considerable improvements. Thus, the current review aims to summarize understanding, diagnostics, and treatment of lymphatic complications in individuals with congenital heart disease. Recent Findings The altered hemodynamics of individuals with congenital heart disease has been found to profoundly affect morphology and function of the lymphatic system, rendering this population especially prone to the development of lymphatic complications such as chylous and serous effusions, protein-losing enteropathy and plastic bronchitis. Summary Although improved, a full understanding of the pathophysiology and targeted treatment for lymphatic complications is still wanting. Future research into pharmacological improvement of lymphatic function and continued implementation of lymphatic imaging and interventions may improve knowledge, treatment options, and outcome for affected individuals.

New concepts: development of a survivorship programme for patients with a functionally univentricular heart

2011 ◽  
Vol 21 (S2) ◽  
pp. 77-79 ◽  
Author(s):  
David J. Goldberg ◽  
Kathryn Dodds ◽  
Jack Rychik
Keyword(s):  
Fontan Operation ◽  
Univentricular Heart ◽  
Plastic Bronchitis ◽  
Complete Understanding ◽  
Protein Losing Enteropathy ◽  
New Concepts ◽  
Fontan Physiology ◽  
The Impact ◽  
Valuable Service

AbstractChildren with functionally univentricular hearts are now surviving into their third and fourth decades of life. Although survival alone is a remarkable achievement, a lot must still be done to improve the quality and duration of life after the Fontan operation. Challenges that may be faced by these patients include the impact of the Fontan operation on the liver and the density of bone, protein-losing enteropathy, and plastic bronchitis. Paediatric cardiologists are familiar with the haemodynamic issues inherent in Fontan physiology; however, training in cardiology is often not sufficient to give us a complete understanding of the pathophysiology of the complications or of the options for treatment. Collaboration with other subspecialists including gastroenterologists, endocrinologists, and pulmonologists is essential in order to provide the rigorous and nuanced care that our patients need and deserve. A clinic in which a patient can see multiple subspecialists, and in which the subspecialists, as a group, can discuss each patient, can provide a unique and valuable service for patients with a functionally univentricular heart.

scholarly journals Post-acute sequelae of COVID-19 in a non-hospitalized cohort: results from the Arizona CoVHORT

2021 ◽  
Author(s):  
Melanie L Bell ◽  
Collin J Catalfamo ◽  
Leslie V. Farland ◽  
Kacey C Ernst ◽  
Elizabeth T Jacobs ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Severe Disease ◽  
Median Number ◽  
Population Based ◽  
Diverse Population ◽  
Shortness Of Breath ◽  
Persistent Symptoms ◽  
Estimate Prevalence ◽  
Hispanic White

AbstractClinical presentation, outcomes, and duration of COVID-19 has ranged dramatically. While some individuals recover quickly, others suffer from persistent symptoms, collectively known as post-acute sequelae of SAR-CoV-2 (PASC). Most PASC research has focused on hospitalized COVID-19 patients with moderate to severe disease. We used data from a diverse population-based cohort of Arizonans to estimate prevalence of various symptoms of PASC, defined as experiencing at least one symptom 30 days or longer. There were 303 non-hospitalized individuals with a positive lab-confirmed COVID-19 test who were followed for a median of 61 days (range 30-250). COVID-19 positive participants were mostly female (70%), non-Hispanic white (68%), and on average 44 years old. Prevalence of PASC at 30 days post-infection was 68.7% (95%CI 63.4, 73.9). The most common symptoms were fatigue (37.5%), shortness-of-breath (37.5%), brain fog (30.8%), and stress (30.8%). The median number of symptoms was 3 (range 1-20). Amongst 157 participants with longer follow-up (≥60 days), PASC prevalence was 77.1%.

scholarly journals Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A78-A79
Author(s):  
Thomas Uslar ◽  
Ignacio San Francisco ◽  
Roberto Ignacio Olmos ◽  
Stefano Pietro Macchiavello ◽  
Alvaro Zuñiga ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Latin American ◽  
Clinical Presentation ◽  
Length Of Hospital Stay ◽  
Female Gender ◽  
Perioperative Outcomes ◽  
Multidisciplinary Program ◽  
Academic Center ◽  
Frequent Mutations ◽  
Biochemical Genetic

Abstract Objective: Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies have shown changes in both clinical presentation and management of these patients. We aimed to assess the main characteristics of PPGL patients in a single academic center over the last four decades. Experimental design: Cohort study. Patients and methods: Demographic, clinical, biochemical, genetic and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980–2019 period. Patients were categorized into four groups (14 patients in the 1st, 25 patients in the 2nd, 27 patients in the 3th and 39 patients in the 4th decade) according to the date of diagnosis. Results: The mean age at diagnosis was 46±19 years, and the tumor size was 5.3±2.2 cm, female gender was 63%, bilateral tumor of 15%, paragangliomas 9% and metastatic disease in 15%. The aforementioned parameters remained stable across the four decades. During the study period we observed significant increases in doxazosin dosing (2.7±2.6 mg vs. 8.0±4.5 p&lt;0.003) and laparoscopic procedures (28% vs. 84% p&lt;0.001) along with a decrease in the length of hospital stay (10.0±8.9 vs. 3.8±1.7 days p=0.007). Among the 24 genetic tests performed, we identified 59% germline mutations. The most frequent mutations were RET (18%) and SDHX (18%), followed by VHL (14%), MAX (5%) and NF1 (4%). Notably, in the last decade we observed a dramatic increase in the proportion of incidental PPGL diagnosis (0% vs. 53% p&lt;0.001) and genetic testing analyses (0 vs. 19 p&lt;0.001). When comparing incidental diagnosis (n=25) versus clinically suspicious cases(n=50), incidentalomas had fewer adrenergic symptoms (38 vs. 62%; p&lt;0.001), and lower rates of hypertension (64 vs. 80%; p=0.01), hypertension crises (28 vs. 44%; p=0.02), functionality (79 vs. 100%; p=0.01) and total catecholamines and/or metanephrine levels (8.4 vs. 12.5 fold above the upper normal limit; p=0.04). Conclusions: The implementation of a multidisciplinary program increased diagnosis and genetic testing and also optimized anesthesia and surgical procedure, translating into a notorious improvement in perioperative outcomes. In addition, we observed a change in the clinical presentation of PPGL in recent decades with a marked increase in incidental cases, which highlights the importance of early diagnosis and treatment.

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