scholarly journals Hepatic involvement in patient with asia syndrome

2021 ◽  
Vol 44 (1) ◽  
pp. 9-11
Author(s):  
V Martos Ruiz ◽  
AI Cascales Vallejo ◽  
E García Cortacero
Keyword(s):  
Hepatic Involvement ◽  
Asia Syndrome

Resumen El nombre de síndrome autoinmune /inflamatorio inducido por adyuvantes (Síndrome ASIA o de Shonefeld) se relaciona con la exposición típicamente de alguna de estas sustancias: vacunas (especialmente papiloma humano), silicona, escualeno, hidróxido de aluminio, parafina líquida, acrilamidas, ácido hialurónico o metacrilato. Puede dar manifestaciones inflamatorias tanto locales como sistémicas. Presentamos el caso de una paciente con prótesis de silicona en mamas y tatuaje de piel y labios, en estudio por hepatitis de perfil colestásico, en la que aparecen lesiones cutáneas que se biopsian (granulomas no necrotizantes), siendo esto clave para el diagnóstico de reacción sarcoidítica.

Hypereosinophilic syndrome: Clinical, laboratory, and imaging manifestations in patients with hepatic involvement

1993 ◽  
Vol 29 (4) ◽  
pp. 757
Author(s):  
Gi Beom Kim ◽  
Ok Hwoa Kim ◽  
Jong Min Lee ◽  
Yeong Soon Sung ◽  
Duk Sik Kang
Keyword(s):  
Clinical Laboratory ◽  
Hypereosinophilic Syndrome ◽  
Hepatic Involvement

scholarly journals Cystic fibrosis in disguise – the wolf in sheep’s clothing, a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Friederike Wilbert ◽  
Sarah C. Grünert ◽  
Andrea Heinzmann ◽  
Sebastian F. N. Bode
Keyword(s):  
Cystic Fibrosis ◽  
Systemic Disease ◽  
Gastrointestinal Symptoms ◽  
Chloride Concentration ◽  
Failure To Thrive ◽  
Sweat Test ◽  
Upper Airways ◽  
Inborn Errors ◽  
Hepatic Involvement ◽  
Number Of Patients

Abstract Background Childhood hypoglycemia in combination with hepatomegaly is suspicious for inborn errors of metabolism. Cystic fibrosis typically presents with failure to thrive, pulmonary and gastrointestinal symptoms. Hepatic involvement and hypoglycemia can occur in a significant number of patients, although hepatomegaly is uncommon. Case presentation A 28 months old boy was presented with recurrent upper airways infections, progressive lethargy and weight loss. Clinically hepatomegaly was the main presenting feature and hypoglycemia (minimum 1.4 mmol/l) was noted as were elevated transaminases. The patient did not produce enough sweat to analyze it. Infectious causes for hepatitis were excluded and a broad metabolic work-up initiated. A therapy with starch was initiated to control hypoglycemia. In further course loose stools were reported and pancreatic elastase was found to be reduced. A further sweat test yielded pathological chloride concentration and genetic testing confirmed the diagnosis of cystic fibrosis. Conclusions Cystic fibrosis is a systemic disease and less common presentations need to be considered. Even in the age of CF-newborn screening in many countries CF needs to be ruled out in typical and atypical clinical presentations and diagnostics need to be repeated if inconclusive.

scholarly journals Fatal Subacute Hepatic Failure in a Patient with AA-Type Amyloidosis: Case Report

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Ibrahim Altraif ◽  
Fayaz A. Handoo ◽  
Khaled O. Alsaad ◽  
Adel Gublan
Keyword(s):  
Alkaline Phosphatase ◽  
Case Report ◽  
Hepatic Failure ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Clinical Findings ◽  
Systemic Amyloidosis ◽  
Hepatic Involvement ◽  
Reactive Amyloidosis ◽  
Hepatic Amyloidosis

Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as subacute hepatic failure and resulted in a fatal clinical course in a 69-year-old man. To the best of our knowledge, this is the fifth case of hepatic amyloidosis of AA type that clinically presented as fatal subacute hepatic failure, an unusual clinical presentation for hepatic involvement by systemic AA-type amyloid.

scholarly journals A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement

2014 ◽  
Vol 200 (8) ◽  
pp. 490-493 ◽  
Author(s):  
Elita Santosaputri* ◽  
Elizabeth J Ellis* ◽  
Sureshkumar Nagiah ◽  
Anugrah Chrispal ◽  
Anthony Thomas
Keyword(s):  
Granulomatous Disease ◽  
Hepatic Involvement

scholarly journals Coagulation Disturbances in Patients with Argininemia

2018 ◽  
Vol 140 (4) ◽  
pp. 221-225 ◽  
Author(s):  
Ertugrul Kiykim ◽  
Tanyel Zubarioglu ◽  
Mehmet Serif Cansever ◽  
Tiraje Celkan ◽  
Johannes Häberle ◽  
...  
Keyword(s):  
Autosomal Recessive ◽  
Urea Cycle ◽  
International Normalized Ratio ◽  
Factor Ix ◽  
Factor Vii ◽  
Clotting Factor ◽  
Clotting Factors ◽  
Hepatic Involvement ◽  
Prolonged Aptt ◽  
Liver Transaminases

Background: Argininemia is an autosomal recessive urea cycle disorder (UCD). Unlike other UCD, hyperammonemia is rarely seen. Patients usually present in childhood with neurological symptoms. Uncommon presentations like neonatal cholestasis or cirrhosis have been reported. Although transient elevations of liver transaminases and coagulopathy have been reported during hyperammonemia episodes, a permanent coagulopathy has never been reported. Methods: In this retrospective study, coagulation disturbances are examined in 6 argininemia patients. All of the patients were routinely followed up for hepatic involvement due to argininemia. Laboratory results, including liver transaminases, albumin, prothrombin time (PT), international normalized ratio (INR), activated partial thromboplastin time (aPTT), and clotting factor levels, were assessed in all of the patients. Results: All of the patients had a prolonged PT and an increased INR, while none of the patients had a prolonged aPTT. Five patients had slightly elevated liver transaminases. A liver biopsy was performed in 1 patient but neither cirrhosis nor cholestasis was documented. Five of the 6 patients had low factor VII and factor IX levels, while other clotting factors were normal. Conclusions: Argininemia patients should be investigated for coagulation disorders even if there is no apparent liver dysfunction or major bleeding symptoms.

Expression of blood hepatocyte-derived microRNA-122 in canine multicentric lymphoma with hepatic involvement

2019 ◽  
Vol 43 (4) ◽  
pp. 231-238 ◽  
Author(s):  
Eman S. Ramadan ◽  
Adel Abdelbaset Kubesy ◽  
Taher Ahmed Baraka ◽  
Faisal Abdelsamad Torad ◽  
Shaymaa Ismaiel Salem ◽  
...  
Keyword(s):  
Hepatic Involvement ◽  
Multicentric Lymphoma

Hereditary Hemorrhagic Telangiectasia: Multi–Detector Row Helical CT Assessment of Hepatic Involvement

Radiology ◽  
2004 ◽  
Vol 230 (1) ◽  
pp. 250-259 ◽  
Author(s):  
Amato Antonio Stabile Ianora ◽  
Maurizio Memeo ◽  
Carlo Sabbà ◽  
Anna Cirulli ◽  
Antonio Rotondo ◽  
...  
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Helical Ct ◽  
Hepatic Involvement

A case of Myelofibrosis with Splenohepatomegaly

1965 ◽  
Vol 51 (4) ◽  
pp. 237-247
Author(s):  
Annamaria Monti
Keyword(s):  
Bone Marrow ◽  
Liver Cirrhosis ◽  
Myeloid Differentiation ◽  
Post Mortem ◽  
Hepatic Biopsy ◽  
Hepatic Involvement ◽  
Rapid Enlargement ◽  
Atypical Cells ◽  
Arteriolar Hyalinosis ◽  
Normal Formula

A 63-year old woman affected by splenomegaly, anemia and moderate leukocytosis was submitted to splenectomy for a supposed Banti disease. Histological examinations showed splenic invasion by undifferentiated mesenchymal cells with partial myeloid differentiation and with arteriolar hyalinosis; hepatic biopsy revealed only a moderate phlogosis. Following splenectomy a rapid enlargement of the liver was observed, with increase of erythrocytes to 5.6 millions per mm3 and appearance of erythroblasts in the circulating blood; neutrophilic leukocytosis to 200,000 cells per mm3 with presence of myelocytes and metamyelocytes and of occasional atypical cells; increase of platelets to 500,000 elements per mm3 with giant thrombocytes; bone marrow showed areas of fibrosis and a normal formula. The patient died 27 months after splenectomy with a terminal period of profuse enterorrhagia and anemia; post-mortem examination of the liver showed infiltration of cells of erythroblastic type and diffuse cirrhotic fibrosis. The case has been ascribed to the myelofibrosis and defined as a « panmyelosis with fibrotic evolution », with prevailing spleno-hepatic involvement, erythro-leuko-thrombocytemia and terminal liver cirrhosis.

scholarly journals An Unusual Case of “Conjugal” Polymyalgia Rheumatica after SARS-CoV-2 Vaccination

Rheumato ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 17-21
Author(s):  
Elena Vanni ◽  
Jacopo Ciaffi ◽  
Luana Mancarella ◽  
Francesco Ursini
Keyword(s):  
Environmental Factors ◽  
Polymyalgia Rheumatica ◽  
Temporal Correlation ◽  
Married Couples ◽  
Laboratory Findings ◽  
Immune Mediated ◽  
Asia Syndrome ◽  
Causative Effect ◽  
Genetic And Environmental Factors

The rare occurrence of polymyalgia rheumatica (PMR) in married couples has been reported in the literature. Susceptibility to PMR is contributed by genetic and environmental factors and cases of PMR developing after influenza vaccine have also been described, in a debated phenomenon known as ‘ASIA’ syndrome. We report the case of two cohabitating married patients developing PMR few weeks after the first dose of ChAdOx1-S SARS-CoV-2 vaccine. Both patients presented with typical symptoms suggestive of PMR. Laboratory findings and ultrasound examination confirmed the diagnosis. Glucocorticoid therapy led to rapid improvment of symptoms. Anti-receptor-binding domain IgG titre was tested and, eight weeks after vaccination, both patients showed no antibody response. It has been suggested that vaccines might trigger autoimmune or inflammatory states in predisposed individuals and various hypotheses have been made regarding the pathogenesis of PMR. Although the causative effect of vaccines cannot be determined, the close temporal correlation observed in our case supports the potential role of environmental factors in triggering the onset of PMR. However, the literature indicates that post-COVID19 vaccination immune-mediated or inflammatory adverse events are extremely rare and vaccination should be encouraged since the benefit largely outweighs possible risks.

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