Cystic angiomatosis of the bone, liver, and spleen

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S125-S126
Author(s):  
J Fleckenstein ◽  
John T Milliken ◽  
J S Lee ◽  
S J Ballentine
Keyword(s):  
Hepatitis C ◽  
Vascular Endothelial Cells ◽  
Liver Involvement ◽  
Bone Lesions ◽  
Vascular Endothelial ◽  
Organ Systems ◽  
Vascular Proliferation ◽  
History Of ◽  
Cystic Angiomatosis

Abstract Introduction/Objective Cystic angiomatosis is a very rare entity characterized by multifocal cystic angiomatous lesions of the skeletal system, occasionally involving visceral organ systems. Clinical presentation and progression are extremely variable. Cases with liver involvement are even rarer, and have been described as invariably fatal. The pathogenesis of this disorder is poorly understood, and no treatments are currently available. Methods/Case Report A 24 year old man with history of polysubstance abuse and untreated hepatitis C presented with acute abdominal pain. Imaging revealed hepatosplenomegaly and multiple liver, spleen, and skeletal lesions. Differential diagnoses included metastatic disease, lymphoma, and infection. Apart from known untreated chronic hepatitis C, all infectious, autoimmune and hypercoagulability work-ups were negative. Biopsies of the liver and iliac bone lesions showed areas of benign-appearing vascular proliferation in a background of fibrosis. The vascular endothelial cells were positive for CD34 and CD31 but negative for D2-40. On follow-up approximately eight months later, the patient was asymptomatic. Results (if a Case Study enter NA) NA Conclusion Liver involvement by cystic angiomatosis is exceedingly rare and has been associated with increased mortality. This case provides an example of a patient with incidentally discovered cystic angiomatosis involving liver, spleen, and bone who remains asymptomatic at follow-up. Additional cases are required to understand the pathophysiology and disease course in this group of patients, and to investigate possible therapeutic targets.

scholarly journals Natural History of Hepatitis C Virus among Apparently Normal Schoolchildren: Follow‐up after 7 Years

2003 ◽  
Vol 49 (6) ◽  
pp. 384-385 ◽  
Author(s):  
Azza El‐Sherbini ◽  
Wafaa Hassan ◽  
Mohamad Abdel‐Hamid ◽  
Ahmad Naeim
Keyword(s):  
Hepatitis C Virus ◽  
Hepatitis C ◽  
Natural History ◽  
History Of

Cardiovascular Effects of Endothelin

Physiology ◽  
1997 ◽  
Vol 12 (3) ◽  
pp. 113-117
Author(s):  
JD Grossman ◽  
JP Morgan
Keyword(s):  
Endothelial Cells ◽  
Heart Disease ◽  
Vascular Endothelial Cells ◽  
Cardiovascular Effects ◽  
Multiple Organ ◽  
Vascular Endothelial ◽  
Cellular Calcium ◽  
Organ Systems

Endothelin is a potent vasoconstricting agent released by vascular endothelial cells. Endothelin affects multiple organ systems and occurs at high levels with heart disease. Changes in cellular calcium levels and Ca2+ sensitization of myofilaments mediate the endothelin pathway. The ability to modulate endothelin levels may lead to many effective therapies.

scholarly journals Prevalence of Hepatitis B and C among Tuberculosis Patients at Local Community of Sindh, Pakistan

2021 ◽  
Vol 15 (7) ◽  
pp. 1794-1797
Author(s):  
Khalil Ahmed Memon ◽  
M. Khan ◽  
Sarah Azhar ◽  
Jai Kershan ◽  
Partab Puri ◽  
...  
Keyword(s):  
Hepatitis C ◽  
Hepatitis B ◽  
Urban Areas ◽  
Local Community ◽  
Age Group ◽  
Tuberculosis Patients ◽  
Key Words Hepatitis ◽  
History Of ◽  
N 93

Objective: To assess the prevalence rate of Hepatitis B and C among those patients who had tuberculosis in local community of Sindh, Pakistan. Study Design:Survey-based study Place and Duration of Study: Department of Pathology, Liaquat University of Medical & health Sciences Jamshoro from 1stJanuary 2020 to 31stDecember 2020. Methodology: Five hundred and eighty nine confirmed cases of tuberculosis patients were enrolled. The patients were further analyzed to assess either HBV, HCV or both are present or absent. Results: Three hundred and forty one (57.8%) were males and 248 (42.1%) were females. The majority of participants were in the age group of 45-54 years 147 (24.9%).The residence detail showed that 167 (28.3%) belonged to urban areas. Further 143 (24.2%) had sickness history of 2-6 months, 239 (40.5%) had history of 6-12 months, The prevalence of hepatitis B and C among tuberculosis patients showed, 17.8% (n=105) with Hepatitis B, 26.3% (n=155) were diagnosed with hepatitis C, 15.7% (n=93) had Both Hepatitis B and C, however 236 (40.0%) had no history with hepatitis. Hepatitis C was most frequently found age of above 54 years, 55 (9.3%). Conclusion:The control of tuberculosis has remained one of the greatest goals globally till date, the higher risk of liver complications, along with the Hepatitis B and Hepatitis C. Although the complications of Tuberculosis patients remain unsolved yet the possible efforts can be made to identify the earlier problems for the clinical prospective and a complete follow up of the records can optimize the management of Tuberculosis in co-existing conditions of hepatitis B and C. Key Words: Hepatitis B, Hepatitis C, Tuberculosis, Liver diseases

Cystic Hygroma with Multiple Benign bone Lymphangiomas in an Adult Patient: a rare Entity in the Differential Diagnosis of Multiple Osseous Lesions in Oncology Practice

2020 ◽  
Vol 17 ◽  
Author(s):  
Ferit Aslan ◽  
İnanç Güvenç ◽  
Aydın Aslan ◽  
Elif Günaydın
Keyword(s):  
Differential Diagnosis ◽  
Adult Patient ◽  
Cystic Hygroma ◽  
Cystic Lymphangioma ◽  
Bone Lesions ◽  
Lytic Bone Lesions ◽  
History Of ◽  
Oncology Practice ◽  
To Receive

: Cystic lymphangioma presenting with multiple bone lesions in an adult patient is a rare occurrence, with a limited number of reported cases in the literature. In this case report, we describe a 32-year old female patient with chronic neck and pelvic pain, and multiple lytic bone lesions on radiological imaging, which were eventually discovered to originate from cystic hygroma and widespread bone lymphangiomas that were present for more than 10 years. It should be kept in mind that there may be benign causes in the differential diagnosis in patients presenting with findings suggestive of diffuse bone metastasis.Because misdiagnosis may cause the patient to receive unnecessary treatments, especially radiotherapy. In this case, we reached the diagnosis of benign disease, diffuse bone lymphangiomatosis, and the long and stable medical history of the patient with the findings of BT, ultrasound and bone scintigraphy. We think that as long as there are no stable and serious results in the company with the clinical and radiological findings of the patient, it should be approached with medical follow-up without treatment.

Dilated cardiomyopathy in a case of Shwachman–Diamond syndrome

2011 ◽  
Vol 21 (5) ◽  
pp. 588-590 ◽  
Author(s):  
Liliane Kopel ◽  
Paulo S. Gutierrez ◽  
Silvia G. Lage
Keyword(s):  
Dilated Cardiomyopathy ◽  
Cardiac Failure ◽  
Bone Marrow Failure ◽  
Pancreatic Insufficiency ◽  
Exocrine Pancreatic Insufficiency ◽  
Bone Marrow Failure Syndrome ◽  
Organ Systems ◽  
History Of ◽  
Shwachman Diamond Syndrome

AbstractThe Shwachman–Diamond syndrome is an autosomal recessive bone marrow failure syndrome with exocrine pancreatic insufficiency. Additional organ systems, such as the liver, heart and bone, may also be affected. We report a patient with a long history of cardiac failure and diagnosis of dilated cardiomyopathy with intermittent neutropenia. Periodic follow-up revealed progressive cardiac failure and pulmonary hypertension. A diagnosis of Shwachman–Diamond syndrome was made at the autopsy.

Clinical efficacy of Direct Acting Antivirals (DAAs) for Treatment of Recurrent Hepatitis C Virus infection After Liver Transplantation

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Mohamed Saleh Mohamed Ahmed Ismail ◽  
Osama Aboelfotoh El Sayed ◽  
Mohamed Mohamed Bahaa El-Din ◽  
Wael Ahmed Yousry ◽  
Maha Mohsen Kamal El-Din ◽  
...  
Keyword(s):  
Hepatitis C Virus ◽  
Hepatitis C ◽  
Treatment Failure ◽  
Hcv Infection ◽  
Post Transplantation ◽  
Recurrent Hepatitis ◽  
History Of ◽  
Recurrent Hepatitis C ◽  
Hcc Recurrence

Abstract BACKGROUND & AIMS Recurrent hepatitis C virus (HCV) infection of transplanted liver allografts is universal in patients with detectable HCV viremia at the time of transplantation. Directacting antiviral (DAA) therapy has been adopted as the standard of care for recurrent HCV infection in the post-transplant setting. However, there are insufficient data regarding its efficacy in liver transplant (LT) recipients with a history of hepatocellular carcinoma (HCC), and the risk of HCC recurrence after DAA therapy is unknown. In this study, we aimed to demonstrate predictors of DAA treatment failure and HCC recurrence in LT recipients. METHODS A total of 106 LT recipients given DAAs for recurrent HCV infection from 2015 to 2019 were identified (68 with and 38 without HCC). Descriptive statistics and logistic regression models were used to estimate the multivariate odds ratios and respective 95% confidence intervals for predictors of treatment failure and HCC recurrence. RESULTS Six patients (6%) experienced DAA therapy failure and 100 (94%) had a sustained virologic response at follow-up week 12 (SVR12). A high alanine transaminase level >35 U/L at treatment week 4 was a significant predictor of treatment failure. DAA failure at follow-up week 12 was significantly associated with post-transplantation HCC recurrence, (odds ratio, 10.6 [95% confidence interval, 1.0-121.6]; P = .05). CONCLUSIONS DAAs are effective and safe in the treatment of recurrent HCV infection in LT recipients with history of HCC. Lack of SVR12 is a predictor of post-transplantation HCC recurrence.

scholarly journals Natural History of the Bruise: Formation, Elimination, and Biological Effects of Oxidized Hemoglobin

2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
Author(s):  
Viktória Jeney ◽  
John W. Eaton ◽  
György Balla ◽  
József Balla
Keyword(s):  
Actin Polymerization ◽  
Vascular Endothelial Cells ◽  
Biological Effects ◽  
Scavenger Receptor ◽  
Oxidation States ◽  
Vascular Endothelial ◽  
Disease States ◽  
Macrophage Scavenger Receptor ◽  
Single Chemical ◽  
History Of

Numerous disease states are associated with hemolysis or hemorrhage. Because red cells in the extravascular space tend to lyse quickly, hemoglobin (Hb) is released and is prone to autoxidation producing MetHb. Inorganic and organic peroxides may convert Hb and MetHb to higher oxidation states such as ferrylHb. FerrylHb is not a single chemical entity but is a mixture of globin- and porphyrin-centered radicals and covalently cross-linked Hb multimers. Oxidized Hb species are potent prooxidants caused mainly by heme release from oxidized Hb. Moreover, ferrylHb is a strong proinflammatory agonist that targets vascular endothelial cells. This proinflammatory effect of ferrylHb requires actin polymerization, is characterized by the upregulation of proinflammatory adhesion molecules, and is independent of heme release. Deleterious effects of native Hb are controlled by haptoglobin (Hp) that binds cell-free Hb avidly and facilitates its removal from circulation through the CD163 macrophage scavenger receptor-mediated endocytosis. Under circumstances of Hb oxidation, Hp can prevent heme release from MetHb, but unfortunately the Hp-mediated removal of Hb is severely compromised when Hb is structurally altered such as in ferrylHb allowing deleterious downstream reactions to occur even in the presence of Hp.

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