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Hepatitis, fulminant viral, susceptibility to(FVH)

MedGen UID:
1684882
Concept ID:
C5231406
Finding
Synonyms: FVH; HEPATITIS, FULMINANT VIRAL, SUSCEPTIBILITY TO
 
Gene (location): IL18BP (11q13.4)
 
Monarch Initiative: MONDO:0032809
OMIM®: 618549

Clinical features

From HPO
Fatigue
MedGen UID:
41971
Concept ID:
C0015672
Sign or Symptom
A subjective feeling of tiredness characterized by a lack of energy and motivation.
Hepatomegaly
MedGen UID:
42428
Concept ID:
C0019209
Finding
Abnormally increased size of the liver.
Jaundice
MedGen UID:
43987
Concept ID:
C0022346
Sign or Symptom
Yellow pigmentation of the skin due to bilirubin, which in turn is the result of increased bilirubin concentration in the bloodstream.
Nausea
MedGen UID:
10196
Concept ID:
C0027497
Sign or Symptom
A sensation of unease in the stomach together with an urge to vomit.
Liver failure
MedGen UID:
88444
Concept ID:
C0085605
Disease or Syndrome
A disorder characterized by the inability of the liver to metabolize chemicals in the body. Causes include cirrhosis and drug-induced hepatotoxicity. Signs and symptoms include jaundice and encephalopathy. Laboratory test results reveal abnormal plasma levels of ammonia, bilirubin, lactic dehydrogenase, and alkaline phosphatase.
Poor appetite
MedGen UID:
68562
Concept ID:
C0232462
Sign or Symptom
A reduced desire to eat.
Fulminant hepatitis
MedGen UID:
86223
Concept ID:
C0302809
Disease or Syndrome
Acute hepatitis complicated by acute liver failure with hepatic encephalopathy occurring less than 8 weeks after the onset of jaundice.
Coma
MedGen UID:
1054
Concept ID:
C0009421
Disease or Syndrome
The complete absence of wakefulness and consciousness, which is evident through a lack of response to any form of external stimuli.
Gingival bleeding
MedGen UID:
42218
Concept ID:
C0017565
Pathologic Function
Hemorrhage affecting the gingiva.
Hashimoto thyroiditis
MedGen UID:
151769
Concept ID:
C0677607
Disease or Syndrome
Hashimoto's disease is a condition that affects the function of the thyroid, which is a butterfly-shaped gland in the lower neck. The thyroid makes hormones that help regulate a wide variety of critical body functions. For example, thyroid hormones influence growth and development, body temperature, heart rate, menstrual cycles, and weight. Hashimoto's disease is a form of chronic inflammation that can damage the thyroid, reducing its ability to produce hormones.\n\nOne of the first signs of Hashimoto's disease is an enlargement of the thyroid called a goiter. Depending on its size, the enlarged thyroid can cause the neck to look swollen and may interfere with breathing and swallowing. As damage to the thyroid continues, the gland can shrink over a period of years and the goiter may eventually disappear.\n\nOther signs and symptoms resulting from an underactive thyroid can include excessive tiredness (fatigue), weight gain or difficulty losing weight, hair that is thin and dry, a slow heart rate, joint or muscle pain, and constipation. People with Hashimoto's disease may also have a pale, puffy face and feel cold even when others around them are warm. Affected women can have heavy or irregular menstrual periods and difficulty conceiving a child (impaired fertility). Difficulty concentrating and depression can also be signs of a shortage of thyroid hormones.\n\nHashimoto's disease usually appears in mid-adulthood, although it can occur earlier or later in life. Its signs and symptoms tend to develop gradually over months or years.
Diabetes mellitus type 1
MedGen UID:
41522
Concept ID:
C0011854
Disease or Syndrome
Type 1 diabetes mellitus (T1D), also designated insulin-dependent diabetes mellitus (IDDM), is a disorder of glucose homeostasis characterized by susceptibility to ketoacidosis in the absence of insulin therapy. It is a genetically heterogeneous autoimmune disease affecting about 0.3% of Caucasian populations (Todd, 1990). Genetic studies of T1D have focused on the identification of loci associated with increased susceptibility to this multifactorial phenotype. The classic phenotype of diabetes mellitus is polydipsia, polyphagia, and polyuria which result from hyperglycemia-induced osmotic diuresis and secondary thirst. These derangements result in long-term complications that affect the eyes, kidneys, nerves, and blood vessels.
Elevated circulating hepatic transaminase concentration
MedGen UID:
338525
Concept ID:
C1848701
Finding
Elevations of the levels of SGOT and SGPT in the serum. SGOT (serum glutamic oxaloacetic transaminase) and SGPT (serum glutamic pyruvic transaminase) are transaminases primarily found in the liver and heart and are released into the bloodstream as the result of liver or heart damage. SGOT and SGPT are used clinically mainly as markers of liver damage.

Professional guidelines

PubMed

Mackinney-Novelo I, Barahona-Garrido J, Castillo-Albarran F, Santiago-Hernández JJ, Méndez-Sánchez N, Uribe M, Chávez-Tapia N
Ann Hepatol 2012 Sep-Oct;11(5):652-7. PMID: 22947525
Czaja AJ
Minerva Gastroenterol Dietol 2007 Mar;53(1):43-78. PMID: 17415344
Hussain Z, Kar P, Husain SA
Indian J Exp Biol 2003 Nov;41(11):1226-32. PMID: 15332488

Recent clinical studies

Etiology

Lin KY, Chen GJ, Lee YL, Huang YC, Cheng A, Sun HY, Chang SY, Liu CE, Hung CC
World J Gastroenterol 2017 May 28;23(20):3589-3606. doi: 10.3748/wjg.v23.i20.3589. PMID: 28611512Free PMC Article
Holmes JA, Yu ML, Chung RT
Expert Opin Drug Saf 2017 Jun;16(6):651-672. Epub 2017 May 19 doi: 10.1080/14740338.2017.1325869. PMID: 28471314Free PMC Article
Blechová Z, Trojánek M, Kynčl J, Cástková J, John J, Malý M, Herrmannová K, Marešová V
Wien Klin Wochenschr 2013 Feb;125(3-4):83-90. Epub 2013 Jan 29 doi: 10.1007/s00508-012-0316-9. PMID: 23358879
Gea-Banacloche JC
Semin Hematol 2010 Apr;47(2):187-98. doi: 10.1053/j.seminhematol.2010.01.002. PMID: 20350666
Onwubalili JK
J Infect 1983 Jul;7(1):2-20. doi: 10.1016/s0163-4453(83)90863-0. PMID: 6313809

Diagnosis

Esumi M, Ishibashi M, Yamaguchi H, Nakajima S, Tai Y, Kikuta S, Sugitani M, Takayama T, Tahara M, Takeda M, Wakita T
Hepatology 2015 Feb;61(2):437-46. doi: 10.1002/hep.27426. PMID: 25203900Free PMC Article
Blechová Z, Trojánek M, Kynčl J, Cástková J, John J, Malý M, Herrmannová K, Marešová V
Wien Klin Wochenschr 2013 Feb;125(3-4):83-90. Epub 2013 Jan 29 doi: 10.1007/s00508-012-0316-9. PMID: 23358879
Mackinney-Novelo I, Barahona-Garrido J, Castillo-Albarran F, Santiago-Hernández JJ, Méndez-Sánchez N, Uribe M, Chávez-Tapia N
Ann Hepatol 2012 Sep-Oct;11(5):652-7. PMID: 22947525
Gea-Banacloche JC
Semin Hematol 2010 Apr;47(2):187-98. doi: 10.1053/j.seminhematol.2010.01.002. PMID: 20350666
Locasciulli A, Nava S, Sparano P, Testa M
Bone Marrow Transplant 1998 Apr;21 Suppl 2:S75-7. PMID: 9630333

Therapy

Lin KY, Chen GJ, Lee YL, Huang YC, Cheng A, Sun HY, Chang SY, Liu CE, Hung CC
World J Gastroenterol 2017 May 28;23(20):3589-3606. doi: 10.3748/wjg.v23.i20.3589. PMID: 28611512Free PMC Article
Holmes JA, Yu ML, Chung RT
Expert Opin Drug Saf 2017 Jun;16(6):651-672. Epub 2017 May 19 doi: 10.1080/14740338.2017.1325869. PMID: 28471314Free PMC Article
Gea-Banacloche JC
Semin Hematol 2010 Apr;47(2):187-98. doi: 10.1053/j.seminhematol.2010.01.002. PMID: 20350666
Vento S, Cainelli F, Longhi MS
Lancet Oncol 2002 Jun;3(6):333-40. doi: 10.1016/s1470-2045(02)00773-8. PMID: 12107020
Houglum JE
Clin Pharm 1983 Jan-Feb;2(1):20-8. PMID: 6192965

Prognosis

Csernalabics B, Marinescu MS, Maurer L, Kelsch L, Werner J, Baumann K, Zoldan K, Panning M, Reuken P, Bruns T, Bengsch B, Neumann-Haefelin C, Hofmann M, Thimme R, Dao Thi VL, Boettler T
J Hepatol 2024 Apr;80(4):564-575. Epub 2023 Dec 26 doi: 10.1016/j.jhep.2023.12.016. PMID: 38154741
Baruah V, Tiwari D, Hazam RK, Bose M, Bujarbaruah D, Saikia AK, Kar P, Dutta S, Bose S
J Med Virol 2021 Jun;93(6):3656-3665. Epub 2020 Oct 8 doi: 10.1002/jmv.26557. PMID: 32975838
Blechová Z, Trojánek M, Kynčl J, Cástková J, John J, Malý M, Herrmannová K, Marešová V
Wien Klin Wochenschr 2013 Feb;125(3-4):83-90. Epub 2013 Jan 29 doi: 10.1007/s00508-012-0316-9. PMID: 23358879
Navaneethan U, Al Mohajer M, Shata MT
Liver Int 2008 Nov;28(9):1190-9. Epub 2008 Jul 25 doi: 10.1111/j.1478-3231.2008.01840.x. PMID: 18662274Free PMC Article
Vento S, Cainelli F, Longhi MS
Lancet Oncol 2002 Jun;3(6):333-40. doi: 10.1016/s1470-2045(02)00773-8. PMID: 12107020

Clinical prediction guides

Csernalabics B, Marinescu MS, Maurer L, Kelsch L, Werner J, Baumann K, Zoldan K, Panning M, Reuken P, Bruns T, Bengsch B, Neumann-Haefelin C, Hofmann M, Thimme R, Dao Thi VL, Boettler T
J Hepatol 2024 Apr;80(4):564-575. Epub 2023 Dec 26 doi: 10.1016/j.jhep.2023.12.016. PMID: 38154741
Baruah V, Tiwari D, Hazam RK, Bose M, Bujarbaruah D, Saikia AK, Kar P, Dutta S, Bose S
J Med Virol 2021 Jun;93(6):3656-3665. Epub 2020 Oct 8 doi: 10.1002/jmv.26557. PMID: 32975838
Holmes JA, Yu ML, Chung RT
Expert Opin Drug Saf 2017 Jun;16(6):651-672. Epub 2017 May 19 doi: 10.1080/14740338.2017.1325869. PMID: 28471314Free PMC Article
Mackinney-Novelo I, Barahona-Garrido J, Castillo-Albarran F, Santiago-Hernández JJ, Méndez-Sánchez N, Uribe M, Chávez-Tapia N
Ann Hepatol 2012 Sep-Oct;11(5):652-7. PMID: 22947525
Vento S, Cainelli F, Longhi MS
Lancet Oncol 2002 Jun;3(6):333-40. doi: 10.1016/s1470-2045(02)00773-8. PMID: 12107020

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