TorontoGiFellow

Normal human A1AT is a 52-kDa glycoprotein of the serpin (serine proteinase inhibitor) family, predominantly produced in the liver and released into the blood.  In affected patients, circulating levels of mutant A1AT are ≤15% of normal protein levels.  A1ATD is unique in that it is a proteinopathy that impacts the lungs and the liver by different mechanisms [ 9 ]. In the lungs, it is a loss-of-function mutation, where A1AT is an inhibitor of neutrophil elastase. Emphysema results when A1AT is not present to inhibit the serine proteinase, allowing it to freely destroy the lung tissue [ 10 ].  The liver disease associated with A1ATD is a gain-of-toxic function mechanism. The misfolded insoluble globular proteins (ATZ) accumulate in the endoplasmic reticulum (ER), leading to hepatic fibrosis and even hepatocellular carcinoma (HCC) [ 11 ] Liver transplant is the only curative treatment. Source:  Alpha-1 antitrypsin deficiency: A re-surfacing adult liver disorder - Journal of Hepatology (jo

  70 patients randomized to each EUS-CDS (6x8 mm axios) and ERCP arm EUS-CDS was quicker (14 mins vs 24 mins), had similar technical and clinical success Same rate of early adverse events, and delayed stent obstruction 5-8% underwent Whipples - no increased difficulty with whipple in EUS-CDS arm (similar surgical duration, similar adverse event, similar post-op hospitalization duration) when compared to ERCP Zero risk of pancreatitis - as expected.

Source: https://www.gastrojournal.org/action/showPdf?pii=S0016-5085%2804%2900195-7 

  Baveno 7   1)     Portal hypertension = HVPG >5 mm hg 2)     In viral & alcoholic hepatitis – CSPH = HVPG >=10 3)     In PBC, CSPH likely develops at lower HVPH, due to (unmeasured) component of pre-sinusoidal PH 4)     Similarly, in NASH, CSPH may develop at HVPG < 10     Reduction in HVPG secondary to Betablockers predicts clinical response!   In patients undergoing TIPS, HVPG <12 affords near-complete protection from PH complications     Compensated advanced chronic liver disease (cACLD) ( a.k.a cirrhosis) <10 kPa = unlikely cACLD      (<1% 3-year risk of decompensation or liver-related death) >15 Kpa = highly likely cACLD     Clinically significant reduction in LSM defined as either: 1)     20% reduction, such that LSM <20 Kpa 2)     Or LSM <10 Kpa   CSPH (based on LSM) LSM <15 + Platelet >150 – unlikely CSPH (>90% negative predictive value) LSM >25 – Definite CSPH   Baveno indicatio

 Refer to UTD Following is an easy excerpt as of July 2023 from UTD ALT = 35 (men), 25(women) regardless of laboratory cut-ff 1 IU = 5 copies /ml

IDA   Ferritin: (BSG 2021 / UTD)   Ferritin is the universal intracellular protein containing Iron – a soluble and non-toxic form. It is the primary iron storage protein in prokaryotes and eukaryotes. Ferritin w/o iron is called apo-ferritin . Each ferritin molecule can store up to 4,500 atoms of iron within it.     Iron deficiency anaemia:   1)     Low MCH is more sensitiv e for Iron deficiency than Low MCV. 2)     Low Ferritin is single most important serological marker of IDA. Serum ferritin assessed by assays is usually apo-ferritin, but reflects intra-cellular ferritin stores in RES (macrophages in liver, spleen, LN etc ) in absence of inflammation.   Fe < 15 – indicative of absent iron stores Fe <45 – indicative of low iron stores   In anaemia of chronic disease / CHF  - TSat <20% with Fe <100 is suggestive of Fe deficiency     Fe > 150 unlikely to represent low iron stores even in face of inflammation .   3)     Transf