Gastroenterology (Quesmed) Flashcards
What is Achalasia?
It is a condition of unknown cause which causes Failure of the Lower Oesophageal Sphincter
What are the 5 signs of Achalasia?
Remember 3 of them are LINKED directly
Dysphagia to both SOLIDS and LIQUIDS (which gradually increases over Months and Years)
Regurgitation of undigested foods
Aspiration
RETROSTERNAL Chest Pain/ Heartburn (which usually doesn’t respond to PPIs)
Weight Loss (often mild)
What investigations should be ordered in Achalasia?
Endoscopy (Dilated Oesophagus containing residual material)
GOLD STANDARD- Oesophageal Manometry (High Pressure and Incomplete Lower Oesophageal Sphincter Relaxation)
Barium Swallow (Bird’s Beak Appearance)
Chest Xray- Widened Mediastinum and Fluid Levels
What is the management of Achalasia?
FIRST LINE OPTION- BALLOON DILATATION
Surgery- Oesophageal Dilatation and Surgical Cleavage of the Lower Sphincter so that food can pass through (called !!!Heller’s Myomotomy)
!!!!Medical- Botox (if this fails or if they are not suitable for surgery, Calcium Channel Blockers/ Nitrates)
What are Porphyrias?
What are the signs and the management of Acute Intermittent Porphyria (Autosomal Dominant) (Porphyria caused by Triggers)?
NO DIARRHOEA, but may be constipation
Defects in Haem Synthesis due to alterations in Enzyme structure and function. This leads to the accumulation of PORPHYRINS
Signs-
1) Abdominal Pain and Vomiting
2) Motor Neuropathy
3) Hypertension and Tachycardia
4) Depression AND CONFUSIONNNNNNN
!!!!!5) ALSO Urine turns RED
Management
- Avoid the Triggers
- IV HAEM otherwise IV glucose
What are the 3 types of Acute Porphyrias?
Acute Intermittent Porphyria (Autosomal Dominant)
Variegate Porphyria (Autosomal Dominant)- suspect if SKIN is affected
ALA Deficiency (Autosomal RecessivE)
What are the medications that trigger Acute Porphyrias?
Antibiotics (Rifampicin, Isoniazid, Nitrofurantoin)
Anaesthetic Agents (Ketamide and Etomidate)
Sulfonamides
Barbiturates
Antifungal Agents
What are the signs of Acute Porphyria?
Suspect if Abdo pain, Confusion and recent Antibiotics/ Anaesthetics etc.
Remember CONFUSION
Generic Abdominal Symptoms
- Abdominal Pain
- Nausea
- Confusion
- Hypertension
What is needed for the diagnosis of Acute Porphyria?
What must you be careful about with the urine sample?
Measure Urinary Porphobilinogen levels which is a product of Haem Metabolism
Urine Samples needed to be protected from sunlight to prevent breakdown of PBG
What is the management of Acute Porphyria?
Largely supportive
IV Haem Arginate can be given to replenish Haem levels
What is Alpha 1 Antitrypsin Deficiency?
WHAT is the INHERITANCE PATTERN?
What Lobes of the lungs are affected in Alpha 1 vs COPD
What is the management?
It is an INHERITED AUTOSOMAL RECESSIVE condition that affects the Lungs, causing Emphysema- so this is a CAUSE of COPD in YOUNG PEOPLE/ NON SMOKERS
LOWER LOBES= Alpha 1, UPPER LOBES= COPD
It also affects the Liver which causes Cirrhosis and Hepatocellular Carcinoma
Without Alpha-1 Antitryptase, there is less defence against Neutrophil Elastase which destroys the Alveoli
This process is exacerbated in Smokers
Management-
1) Physiotherapy, Bronchodilators and Alpha 1 Antitrypsin
2) LUNG REDUCTION SURGERY
What is the presentation of Alpha 1 Antitrypsin Deficiency?
COPD presenting in a 30-40 year old
Neonatal JAUNDICE at Birth
Deranged LFTs in an adult with no other identifiable cause
What investigations should be ordered for Alpha 1 Antitrypsin Deficiency?
Test for Alpha 1 Antitrypsin Levels
Genotyping
Liver Biopsy (look for Periodic-acid-Schiff (PAS) positive globules)
What is the management of Alpha 1 Antitrypsin Deficiency?
Stop Smoking
Intravenous A1AT pooled from human donors is expensive and NOT widely used
If Decompensation, Liver Transplant may be needed
What is seen in patients who have been vomiting?
They appear dehydrated and unwell
There is Metabolic Hypochloraemic Alkalosis on a blood gas due to the loss of Stomach Acid when vomiting
What are the 4 types of Anti-Emetics?
H1 Receptor Antagonist
- Cyclizine
D2 Receptor Antagonist
- Domperidone, Metoclopramide (The ones with M at the start of their name) and Prochlorperazine
5HT3 Receptor Antagonist
- Ondansetron
Anti-Muscarinic
- Hyoscine hydrobromide
What is Ascites?
It is the accumulation of fluid within the Peritoneal Cavity
It may be seen in patients with Cirrhosis- though the mechanism is complex and not fully understood
It is thought to involve PORTAL HYPERTENSION causing an increased Hydrostatic Pressure which leads to the Transudation of Fluid
What is the Serum Ascites Albumin Gradient (SAAG)?
It can help determine the cause of Ascites
Subtract the Albumin concentration of the Ascites Fluid from the Serum Albumin Concentration (S-A)
What are the causes of a high (>1.1g/dL) SAAG?
2 organ failures plus 2 other conditions related to those organs plus 1 extra
Cause is Raised Portal Pressure
- Cirrhosis
- Heart Failure
- Budd Chiari Syndrome
- Constrictive Pericarditis
- Hepatic Failure
Increased Hydrostatic Pressure forces water into the Peritoneal Cavity whilst Albumin remains in the vessels
What are the causes of a low (<1.1g/dL) SAAG?
5 causes
- Cancer of the Peritoneum
- Tuberculosis and other infections
- Pancreatitis
- Hypoalbuminaemia- like Nephrotic Syndrome or Kwashiokor Malnutrition
- Bowel Obstruction
What is the management of Ascites?
SFSF Drain
- Address the underlying cause
- Salt-restricted diet
- Fluid Restriction (not recommended if Uncomplicated Ascites)
- Spironolactone
- Adjunctive Diuretic therapy like Furosemide may be needed if Spironolactone is insufficient
- Patients with Ascites refractory to Medical Management may require Regular Therapeutic Paracentesis, where the Fluid is drained from the abdomen
What is the main contraindication to Paracentesis (Ascites management)?
Signs of Disseminated Intravascular Coagulation
What are the signs of Autoimmune Hepatitis?
!!!!!THERE is AMENORRHOEA and FEVER AS WELL BTW
Remember the Arthralgia
Signs of OTHER AUTOIMMUNE CONDITIONS
!!!!!!AMENORRHOEA AND FEVER
Jaundice
Fatigue
Loss of Appetite
Hepatomegaly
Splenomegaly
Abdominal pain
Pruritus and Spider Naevi
Arthralgia in their Small Joints as well
What are the LFTs in Autoimmune Hepatitis?
What is the CRP like in Autoimmune Hepatitis?
What else is seen in Autoimmune Hepatitis in the blood that is raised?
Hepatic Pattern of Disease- Raised ALT and Bilirubin and Normal/ Mildly Raised ALP (by 10-20)- Significantly raised in Cholangitis
CRP is usually NOT raised
They may have IgG Predominant Hypergammaglobuminaemia