Sandbox:Parastoo

Please practice here!

Screening for AA in Asymptomatic person with Marfan's syndrome
An echocardiogram is recommended at the time of diagnosis of Marfan syndrome to determine the aortic root and ascending aortic diameters and 6 months thereafter to determine the rate of enlargement of the aorta.(Class I, Level of Evidence: C)
Annual imaging is recommended for patients with Marfan syndrome if the stability of the aortic diameter is documented.
If the maximal aortic diameter is 4.5 cm or greater, or if the aortic diameter shows significant growth from baseline, more frequent imaging should be considered.

Screening for AA in patients with Loeys-Dietz syndrome or a confirmed genetic mutation known to predispose ( TGFBR1, TGFBR2, ACTA2, or MYH11)
Patients with Loeys-Dietz syndrome should undergo complete aortic imaging at the time of diagnosis and 6 months thereafter to establish if enlargement is happening. (Class I, Level of Evidence: C). Patients with Loeys-Dietz syndrome should have yearly magnetic resonance imaging from the cerebrovascular circulation to the pelvis. ( Class I, Level of Evidence: B)

Screening for AA in patients with Turner syndrome
patients with Turner syndrome should undergo imaging of heart and aorta at the initial diagnosis.
If the initial imaging is normal and there are not any risk factors for aortic dissection, repeat imaging is performed every 5–10 years.(Class I, Level of Evidence: C) Annual imaging is recommended if abnormalities exist
patients with Turner syndrome, and additional risk factors (bicuspid aortic valve, coarctation of the aorta, pregnant or desiring pregnancy, and/or hypertension), it may be reasonable to perform imaging of the heart and aorta to help determine the risk of aortic dissection. (Class I, Level of Evidence: C)

Screening for AA in family members of patients with AA or genetic mutations associated with aortic aneurysm
First-degree relatives of patients with thoracic aortic aneurysm or dissection should undergo aortic imaging.(Class I,Level of Evidence: C). If thoracic aortic aneurysm or dissection found in one or more first-degree relatives, then imaging of second-degree relatives is reasonable. Also, then referral to a geneticist may be considered reasonable (Class IIa, Level of Evidence: B) to screen for the following aneurysm/dissection-associated genes: FBN1, TGFBR1, TGFBR2, COL3A1, ACTA2, and MYH11 reasonable. (Class I,Level of Evidence: C)
Sequencing of the ACTA2 gene will determine if ACTA2 mutations are responsible for the inherited predisposition reasonable. Also,maybe sequencing of these additional genes: TGFBR1, TGFBR2, and MYH11.(Class IIa, Level of Evidence: B)
first-degree relatives of patients with a bicuspid aortic valve, premature onset of thoracic aortic disease with minimal risk factors, and/or a familial form of thoracic aortic aneurysm and dissection should be evaluated for the presence of a bicuspid aortic valve and asymptomatic thoracic aortic disease. (Class I, Level of Evidence: C)both the root and ascending aorta needs to be evaluated in patients with bicuspid aortic valves. (Class I, Level of Evidence: B)

Refrences for Jaundice

Scrrening for TAA in Marfan's syndrome

An echocardiogram is recommended at inital visit Marfan syndrome to determine the aortic root and ascending aortic diameters and 6 months later to determine the rate of enlargement of the aorta

Annual imaging is recommended if stability of the aortic diameter is documented.

If the maximal aortic diameter is 4.5 cm or greater, or if the aortic diameter shows significant growth from baseline, more frequent imaging should be considered.

Refer to surgeon,if Aortic root or ascending aorta > 5.0 cm aortic cross-sectional area-to-height ratio>10cm2 Descending aorta diameter >6 cm

Treatment

Type of hyperbilirubinemia	Diagnostic Indicators	Management Recommendations
Managment of isolated unconjugated jaundice, hemolytic	Any H/O recent trauma, hematoma or blood transfusion Any recent travel Inquire about medications that can cause hemolysis Any positive family history of hemolytic anemia	 Work-up and detect the cause of hemolysis, if low Hb, High LDH, Low haptoglobin, and reticulocytes present  G6PD deficiency - mostly recover on its own, if progresses to hemolytic anemia, oxygen therapy or blood transfusion may be required. Avoid of precipitants and etiological factors  Spherocytosis, phototherapy and/or exchange transfusion for infants, Folic acid for maintaining erythropoiesis. Splenectomy is the definitive treatment  Sickle cell anemia reduce pain and prevent complications, blood transfusions and supplemental oxygen, as well as a bone marrow transplant.  Immune-related hemolysis – corticosteroids, folic acid is the main line of treatment  Parasitic Infections like malaria are treated with antimalarial drugs like chloroquine, artesunate, lumefantrine, amodiaquine  Ineffective erythropoiesis- iron and folic acid & B12 supplementation, repeated blood transfusions
Managment of isolated unconjugated jaundice, Non-hemolytic	 Gilbert's syndrome does not produce symptoms or adverse effects and patients have a normal life span Crigler-Najjar type I is fatal in early life due to development of kernicterus. Crigler-Najjar type II is compatible with a normal life.	 Phenobarbital can decrease serum bilirubin by enzymatic induction of UDPGT in Crigler-Najjar type II.
Managment of isolated conjugated jaundice	❑Dubin- Johnson syndrome doesn't produce any symptoms and compatible with a normal life span. ❑Rotor's syndrome is a harmless chronic hyperbilirubinemia	❑Suspect for Dubin-Johnson syndrome before considering surgery if the healthy patient with long-standing conjugated hyperbilirubinemia, other normal liver function tests, and a non visualized gallbladder. Hepatic architecture is normal but there is an accumulation of hepatic pigment compatible with melanin in patients with Dubin-Johnson syndrome ❑In Rotor's syndrome the gallbladder opacifies normally with cholecystographic dye and no pigmentation be seen in the liver.
Managment of conjugated & unconjugated hyperbilirubinemia jaundice with ⇈AST/ALT out of proportion to ALK-P'	❑H/O recent travel ❑ Social history: Alcohol, sexual history ❑ Family history of Wilson's disease or hemochromatosis ❑Review medications ❑Pregnancy test HELLP ,AFLD ❑Toxicology screen Acetaminophen level ❑Consider work-up for rare cases Liver biopsy if results negative	❑Viral hepatitis Hepatitis A: mostly self-limiting Hepatitis B treated with antiviral medications Hepatitis C is treated with interferons Other Viral infections like EBV, CMV, HSV are treated with Antiviral medications Alcohol hepatitis: Alcohol abstinence, glucocorticoids, pentoxifylline  Wilson"s disease: chelating agents such as D-penicillamine  Drug toxicity treatment(e.g. Acetaminophen, Isoniazid  Autoimmune hepatitis treatment with glucocorticoids  Avoid drugs associated if any(e.g.kavakava) ❑Mild elevation in aminotransferase levels in female patients with concomitant autoimmune disorders (e.g., autoimmune thyroiditis, connective tissue diseases) is suggestive of autoimmune hepatitis.
Managment of conjugated & unconjugated hyperbilirubinemia jaundice with ⇈ Alk-P out of proportion to AST/ALT	H/O intermittent right upper quadrant pain radiating to the back or right shoulder favors gallstones, fever and chills suggest cholangitis. H/O biliary tract surgery within 2 years should alert the physician to possible biliary stricture. H/o recent weight loss, constant epigastric or right upper quadrant pain radiating to the back suggests malignancy icteric patient with extrahepatic obstruction due to gallstones or postsurgical biliary stricture has usually had acute symptoms for less than 2 weeks Those with carcinoma, chronic pancreatitis, or primary sclerosing cholangitis have had symptoms of longer duration ❑ A middle-aged women with a history of itching and autoimmune disease raises the suspicion of primary biliary cirrhosis More than half the people with primary biliary cholangitis do not have any symptoms when diagnosed. Symptoms develop over the next five to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. Commonly used drugs such as antihypertensives (e.g., angiotensin-converting enzyme inhibitors) or hormones (e.g., estrogen) may cause cholestasis Abnormal ALk-p levels may be a sign of metastatic cancer of the liver, lymphoma or infiltrative diseases such as sarcoidosis. H/O inflammatory bowel disease (most commonly ulcerative colitis) suggest the presence of primary sclerosing cholangitis since about 70% of these cases are associated with inflammatory bowel disease	Obstruction removal by ERCP, PTC, Surgery (e.g.Cholecystectomy or Palliative Bypass procedures such as hepaticojejunostomy if stenting has failed in patients with tumors) Primary biliary cholangitis management: no cure for primary biliary cholangitis, but medications are available for slow the progression and prevent complications of the disease: Ursodeoxycholic acid (UDCA), Obeticholic acid (Ocaliva), Fibrates, Liver transplantation may help prolongs life.
Managment of conjugated & unconjugated hyperbilirubinemia jaundice with ↑ INR,↓ Alb,↓ PLt		Compensated Cirrhosis Mangament Alochol abstinence Antiviral medications for viral hepatitis avoidance of hepatotoxic medications vaccination Decompensated Cirrhosis Managment Managment of complications: Varices,Asciets,Hepatorenal syndrome,Hepatic encephalopathy