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]

The articles in this issue will review best practices for managing and treating patients who present with these chronic problems, like hepatitis, nonalcoholic fatty liver, end stage liver disease, and drug-induced injuries. [books.google.com]

• 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]

• Arm Pain

  Weakness 331 Muscular Dystrophy 332 Mycosis Fungoides 333 Myelodysplastic Syndrome 334 Myocarditis 335 Myoglobinuria 336 Myopathies Toxic 337 Myotonia 338 Myxedema Coma 339 Nail Koilonychia 340 Nail Striations 341 Narcissistic Personality 342 Neck and Arm [books.google.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]

• Pruritus Ani

  Ani 404 Pseudofolliculitis Barbae Ingrown Hairs Razor Bumps 405 Pseudoinfarction 406 Psittacosis 407 Ptosis 408 Puberty Delayed 409 Puberty Precocious 410 Pulmonary Hypertension Primary 411 Pulmonary Nodule Solitary 412 Purpura 413 Pyogenic Granuloma [books.google.com]

• Shoulder Pain

  432 Seasonal Affective Disorder 433 Seizures Absence 434 Septicemia 435 Serotonin Syndrome 436 Sexually Transmitted Diseases Anorectal Region 437 Shoulder Pain 438 Sialadenitis 439 Silicosis 440 Small Bowel Obstruction 441 Smallpox 442 Sore Throat 443 [books.google.com]

• Testicular Pain

  Failure 466 Testicular Neoplasm 467 Testicular Pain 468 Tetanus 469 Thromboangiitis Obliterans 470 Thrombocytopenia Inherited Disorders 471 Thrombocytopenia in Pregnancy 472 Thrombophlebitis Superficial 473 Thyroid Nodule 474 TickRelated Infections 475 [books.google.com]

• Vaginal Bleeding

  Bleeding Pregnancy 492 Varicocele 493 Vasculitis Diseases That Mimic Vasculitis 494 Venous Lake 495 Ventricular Septal Defect 496 Vesiculobullous Diseases 497 Vesiculopustules Newborn Infants 498 Vision Loss Chronic Progressive 499 Vocal Cord Paralysis [books.google.com]

• Trichotillomania

  Disorders 471 Thrombocytopenia in Pregnancy 472 Thrombophlebitis Superficial 473 Thyroid Nodule 474 TickRelated Infections 475 Tinea Corporis 476 Tinea Versicolor Pityriasis Versicolor 477 Toxic Epidermal Necrolysis 478 Toxoplasmosis 479 Tracheitis 480 Trichotillomania [books.google.com]

• Mild Cognitive Impairment

  Cognitive Impairment 325 Mitral Regurgitation 326 Monoarthritis Acute 327 Mononucleosis 328 Mucormycosis 329 Mumps 330 Muscle Weakness 331 Muscular Dystrophy 332 Mycosis Fungoides 333 Myelodysplastic Syndrome 334 Myocarditis 335 Myoglobinuria 336 Myopathies [books.google.com]

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]

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]

• Right Axis Deviation

  Axis Deviation 427 Rosacea 428 Salivary Gland Enlargement 429 Sarcoidosis 430 Schistosomiasis 431 Scrotal Pain 432 Seasonal Affective Disorder 433 Seizures Absence 434 Septicemia 435 Serotonin Syndrome 436 Sexually Transmitted Diseases Anorectal Region [books.google.com]

• Left Axis Deviation

  Axis Deviation 298 Leg Length Discrepancies 299 Leg Ulcers 300 LeggCalvéPerthes Disease 301 Leptomeningeal Lesions 302 Leukoplakia Oral Hairy Oral Hairy Cell Leukoplakia 303 Lichen Simplex Chronicus 304 Listeriosis 305 Liver Lesions Benign Often Confused [books.google.com]

• Ovarian Mass

  Mass 364 Pagets Disease of Bone 365 Pain Midfoot 366 Pain Posterior Heel 367 Palpitations 368 Pancreatitis Acute 369 Panic Disorder 370 Papilledema 371 Paraneoplastic Neurologic Syndromes 372 Paranoid Personality Disorder 373 Paraplegia 374 Parkinsons [books.google.com]

• Microcytosis

  Macrothrombocytopenia Inherited 312 Malaria 313 MalloryWeiss Tear 314 Mastoiditis 315 Mediastinitis Acute 316 Melanocytic Nevi Moles 317 Melanonychia 318 Menieres Disease 319 Meningitis Chronic 320 Menopause 321 Metabolic Syndrome 322 Metatarsalgia 323 Microcytosis [books.google.com]

• 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]