This case highlights the need to maintain an elevated index of suspicion for atypical pathogens with unusual clinical presentations among the lung transplant population. [ncbi.nlm.nih.gov]

Cases are presented of two surviving teen-age cousins and two young sisters, subsequently deceased, whose third sibling has just presented with the disease. [pediatrics.aappublications.org]

Sarcoidosis typically presents as non-necrotizing epithelioid granuloma, and presentation as non-infective necrotizing sarcoid granuloma (NSG) is infrequent. The finding of NSG in the liver is rare. [acgcasereports.gi.org]

• Fever

  Associated with pyrexia of unknown origin Sarcoidosis, tuberculosis (miliary and pulmonary, caseation important), atypical mycobacterium, Q fever, brucellosis, cat-scratch disease, mycoses, drugs, idiopathic granulomatous hepatitis Human immunodeficiency [abdominalkey.com]

  Fibrin-ring Epithelioid cells surround a vacuole within an encircling fibrin ring Q fever, Hodgkin's lymphoma, Leishmaniasis, cytomegalovirus, hepatitis A, Toxoplasmosis, giant cell arteritis, Q fever, Boutonneuse fever, allopurinol 4. [onlinelibrary.wiley.com]

  Register Periodic Fever Associated with Increased Plasma Unconjugated Etiocholanolone and Granulomatous Liver Disease: Case Report and Studies in Etiocholanolone and Cortisol Conjugation - 24 Hours access EUR €36.00 GBP £28.00 USD $45.00 Rental This article [academic.oup.com]

  Causes of Hepatic Granulomas Cause Examples Drugs Allopurinol, phenylbutazone, quinidine, sulfonamides Infections, bacterial Actinomycosis, brucellosis, cat-scratch fever, syphilis, TB *, other mycobacterial infections, tularemia, Q fever Infections, [merckmanuals.com]

  Granulomatous hepatitis and prolonged fever of unknown origin. a study of 13 patients. Medicine 1973; 52 : 1–21. PubMed Terplan M. Hepatic granulomas of unknown origin presenting with fever. Am J Gastroenterol 1971; 55 : 43–9. [gastrohep.com]

• Malaise

  Drug-induced cholestasis can be accompanied by nausea, anorexia, malaise, and pruritus. 32 Symptoms can occur weeks to months after beginning treatment. [clevelandclinicmeded.com]

• Coffee Ground Emesis

  He returned to hospital the same night with coffee-ground emesis and in Addisonian crisis requiring parenteral steroids and intensive care unit support. [ncbi.nlm.nih.gov]

Litvinov, bip3{at}georgetown.edu Summary We describe a case of a 23-year-old woman with a history of Crohn's disease (CD), who initially presented with sepsis-like symptoms, subsequently developed severe cholestasis and following extensive inpatient workup [casereports.bmj.com]

AST is especially markedly raised in those with liver cirrhosis.[7] AST can be released from a variety of other tissues and if the elevation is less than two times the normal AST, no further workup needs to be performed if a patient is proceeding to surgery [en.wikipedia.org]

Clinical Features and Investigations The observation of granulomata in a liver biopsy specimen warrants thorough workup to identify a possible cause. [onlinelibrary.wiley.com]

Developing the best treatment plan depends on a good, thorough pathologic evaluation as well as a complete workup of both breasts, as noted above. [medicinenet.com]

[…] families, 78, 79 B-cell activating factor (BAFF) receptor in 2 siblings, 80 and CD20 and CD81 in 1 patient each. 81, 82 Because these events are very rare occurrences and not found in general populations of patients, requesting these genetic tests in a workup [bloodjournal.org]

• Liver Biopsy

  Abstract Hepatic granulomas are often encountered on liver biopsy and may represent a primary hepatic process, a manifestation of a systemic illness, or an innocent finding of no clinical relevance. [ncbi.nlm.nih.gov]

  Otherwise, the liver biopsy showed preservation of the liver architecture and no fibrosis. The features were therefore of a granulomatous hepatitis. [gastrohep.com]

  This is not an uncommon problem as reflected by a 3 to 10% prevalence of granulomas in liver biopsies performed at general hospitals (1-4). [annals.org]

  biopsy.[ 1 ] They can be associated with a variety of systemic conditions or may be an incidental finding on an otherwise normal liver biopsy. [onlinelibrary.wiley.com]

Screening serologies were positive for histoplasmosis, and he was started on itraconazole treatment. [ncbi.nlm.nih.gov]

Baughman RP, Lower EE: Steroid-sparing alternative treatment for sarcoidosis. Sarcoidosis 1997, 18 :853–864. The role of steroid-sparing agents in treatment of sarcoidosis is discussed. Google Scholar 14. [link.springer.com]

Readers will come away with a full overview of the diagnosis and treatment of cholestatic liver diseases. [books.google.com]

Current literature suggests a favorable prognosis in most cases of MAC infection in lung transplant recipients with appropriate treatment. [ncbi.nlm.nih.gov]

Prognosis This disease is variable in terms of severity but prognosis has certainly improved over the last few decades. When it was first described in the 1950s, death before the age of 10 was usual. [patient.info]

Prognosis The prognosis has greatly improved with the use of antibacterial and antifungal prophylaxis therapy, with most patients living well into adulthood. The documents contained in this web site are presented for information purposes only. [orpha.net]

Systemic disease, e.g. chronic granulomatous disease, Crohn's disease, sarcoidosis, systemic lupus erythematosus, IgG4 disease and other connective tissue disorders Idiopathic: 10 - 15% of all hepatic granulomas; a diagnosis of exclusion; excellent prognosis [pathologyoutlines.com]

The benign prognosis of patients with idiopathic hepatic granulomas has been noted previously [ 4,8 ]. [gastrohep.com]

Etiology Found in a wide range of infectious (see granulomatous hepatitis - infectious ) and noninfectious conditions ( Nat Rev Gastroenterol Hepatol 2011;8:455 ) Immune mediated: primary biliary cirrhosis, autoimmune hepatitis Fatty liver disease with [pathologyoutlines.com]

Physical examination of patients with cirrhosis may reveal a variety of findings that necessitate a hepatic- or gastrointestinal-based work-up to determine the etiology. [aafp.org]

Etiology CGD is caused by mutations in any one of the 5 genes encoding the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase subunits. [orpha.net]

We propose that PMR should be considered in patient with epithelioid granulomas on liver biopsy in the absence of a known etiology. [gastrohep.com]

Etiology Hepatic granulomas have many causes (see Table: Causes of Hepatic Granulomas ); drugs and systemic disorders (often infections) are more common causes than primary liver disorders. [merckmanuals.com]

Summary Epidemiology The average worldwide birth prevalence is estimated at 1/ 217,000. Clinical description CGD can present at any age but is most commonly diagnosed before the age of 5 years. [orpha.net]

Epidemiology CGD is rare (prevalence approximately 1 in 250,000) [ 2 ]. [patient.info]

Prevalence and epidemiology of autoimmune hepatitis. Clin Liver Dis 2002; 6 :347–359. 2. Toda G, Zeniya M, Watanabe F, et al. [nature.com]

Epidemiology Frequency United States The exact incidence of chronic granulomatous disease (CGD) is unknown. CGD affects approximately 1 infant per 200,000-250,000 live births. [emedicine.medscape.com]

Veterans with HIV* Cirrhosis: 2% Decompensated liver disease: 1% HCC: 0.3% HCV: 28% HBV: 11% Epidemiology of HIV and Liver Diseases High morbidity and mortality: In the U.S. general population, cirrhosis accounts for 40,000 deaths per year and for the [hiv.va.gov]

[…] involvement of host responses such as the inflammatory response, the production and release of potent cytokines and accelerated homeostatic responses (such as increased rates of crypt cell division seen in some infections), and the role that they play in pathophysiological [ingentaconnect.com]

Pathophysiology A granuloma is a localized collection of chronic inflammatory cells with epithelioid cells and giant multinucleated cells. Caseation necrosis or foreign body tissue (eg, schistosome eggs) may be present. [merckmanuals.com]

Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune [clevelandclinicmeded.com]

Although numerous pathophysiologic mechanisms of injury exist, the final common pathway is persistent wound healing resulting in hepatic parenchymal fibrosis. [aafp.org]

The pathophysiological mechanism of the liver dysfunction in PMR remains unknown. Although giant cell arteritis is strongly associated with PMR, arteritis has never been reported in the liver biopsy of such a patient. [gastrohep.com]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

Antibacterial and antifungal prophylaxis is currently the main method of prevention. Immunomodulatory therapy with interferon gamma (IFN-gamma) is part of the prophylactic regimen in many centres. [patient.info]

Videos: Choosing Wisely » CGD Treatment & Management The best treatment plan for CGD is to prevent infections from occurring. Special preventative antibiotics are a mainstay of treatment for CGD. These greatly reduce the chances of infection. [aaaai.org]

Management Goals: prevention of further liver damage, slowing progression of liver disease, management of cirrhotic complications Prevention of further insults to the liver: HAV vaccination HBV vaccination Pneumococcus vaccination Influenza vaccination [hiv.va.gov]