November 2024 Flashcards
Most common complications of Hypertension (5)
- CV disease (stroke)
- CHD, LVH, AF.
- Renal disease: Focal segmental glomerulosclerosis (FSGE)
- HT Retinopathy.
- Peripheral vascular disease (Aortic aneurism/dissection)
Clinical features of Aortic Dissection
- Diastolic murmur if Aortic Regurgitation occurred
- Abrupt chest pain, sharpen,
migrating / irradiating to the
back. - Unequal or absent pulses.
- Difference of BP in arms (more than 20mmHg).
Aortic Dissection Types
- Type A: Ascending aorta.
- Type B: Descending aorta.
Aortic Dissection FIRST Investigation
Transesophageal Echocardiogram
Aortic Dissection BEST Investigation
CT angiogram
Aortic Dissection Treatment
- Beta blockers (to reduce shear stress)
- Immediate Qx for type A
(ascending aorta)
ABI values
> 1.4: Calcification → Refer
1-1.4: Normal
0.9-1: Acceptable
0.8-0.9: Mild Arterial disease → treat risk factors
0.5-0.8: Moderate Arterial disease →
- Treat risk factors
- Refer if signs of gangrene or pain on rest.
< 0.5: Severe → Refer
Aortic dissection/aneurysm vs myocardial infarction investigation
Check if patient stable
if stable:
Initial: ECG
Best: CT angio
If unstable: fast US
Cardiac Tamponade classic quartet
- Hypotension
- Increased JVP
- Tachycardia
- Pulsus paradoxus ( ↓ BP after inhalation)
Beck’s triad
- Hypotension
- Increased JVP
- Muffled heart sounds
Main cause of dysphagia
achalasia
most common oesophageal disorder
achalasia
Dysphagia to solids and liquids + Heartburn unresponsive to PPI + Retained food in the oesophagus on upper endoscopy + Unusually increased esophagogastric junction sphincter tone + failure of muscle relaxation + weight loss + regurgitation getting worse at night/lying down
Achalasia
Achalasia diagnostic feature
Dysphagia for both solids and liquids
Achalasia initial investigation
Plain X -ray
- air fluid levels to see absence of gastric bubble
Barium swallow
- Birds beak/rat tail appearance
OGD endoscopy
- exclude other causes of dysphagia
Achalasia best investigation
oesophageal manometry
- increased tension in lower end of oesophagus
Achalasia complications
- strictures
- oesophageal cancer
Achalasia management
Mild symptoms
- CCB (Nifedipine)
- nitrates
Young px
- Endoscopic Pneumatic dilation of LES
Old px
- Botulinum injection (may need to be repeated every 3 - 12 months) + mild symptoms management
Best
- Laparoscopic Myotomy (Heller’s)
dysphagia + hoarseness + hx of achalasia + thoracic inlet mass
Oesophageal cancer
Progressive dysphagia + Weight loss >10% + Elderly
Rule out Oesophageal cancer
Oesophageal cancer features
▪ Dysphagia progressive continuous - first solids then liquids → odynophagia
▪ Striking unintentional weight loss ( >10%)
▪ Hiccoughs (early sign – phrenic nerve irritation)
▪ Hoarseness and cough (upper 1/3 cancer – recurrent
laryngeal nerve irritation – vocal cord palsy)
▪ Progressive chest discomfort or pain in locally invasive cancer
Oesophageal cancer types
▪ SCC (most common)
▪ Adenocarcinoma
Oesophageal cancer risk factors
SCC:
▪ Smoking & OH → Triple S
(smoking - spirits – SCC)
Adeno:
▪ Barrett’s oesophagus & smoking
Oesophageal cancer investigation
1st test: Barium swallow to locate lesion
▪ Narrowing of oesophagus
▪ Irregular oesophageal borders
▪ apple core appearance
THEN
Endoscopy w/biopsies
Oesophagogastroduodenoscopy
Oesophageal cancer ddx
Dysphagia intermittent = Achalasia
Hoarseness and cough = also in Pancoast tumour but Horner is present and no GI symptoms
Complete oesophageal rupture causes
▪ Iatrogenic - 56% due to an endoscopy or
paraesophageal surgery
▪ Boerhaave’s syndrome- 10%
▪ Spontaneous perforation include:
- Caustic ingestion
- Pill esophagitis
-Barrett’s oesophagus
-Infectious ulcers in AIDS patients
- oesophageal strictures dilation
dysphagia + iron deficiency anaemia + glossitis ‘rings’ (oesophageal webs)
Plummer Vinson Syndrome/Syderopenic dysphagia
Eosinophilic oesophagitis causes
history of atopy (genetic tendency to develop allergic diseases):
- eczema
- asthma
- rhinitis
- hay fever
- cow’s milk allergy
- dietary allergens or aeroallergens
- chronic, immune-mediated esophageal inflammation
Plummer Vinson Syndrome/Syderopenic dysphagia biggest risk factor
Oesophageal SCC
Oesophageal varices management
- Prophylaxis: Beta blocker (propranolol or nadolol)
- Endoscopic variceal/band ligation
- If failed endoscopic or medical management TIPS (trans-jugular intrahepatic portosystemic shunt)
- Active bleeding: Octreotide
What is the major cause of morbidity and mortality in patients with cirrhosis?
Oesophageal varices 30%-60%
Abdominal aortic aneurysm (AAA) size screening
3-3.9cm = 2 yearly US
4-4.5cm = 1 yearly US
4.6-5cm = 6m US
> 5cm = 3m US
> 5.5cm (male) = surgical intervention
Abdominal aortic aneurysm (AAA) screening
- family history 20%
- >50 years US
Abdominal aortic aneurysm (AAA) investigation
- >50 years fast US
- CT angiogram (before elective surgical repair)
Acute limb ischaemia causes
- Cardiac/arterial embolus (eg, AF, LV thrombus, IE)
- Local thrombosis from disruption of a preexisting atherosclerotic plaque (PAD)
- Iatrogenic/blunt trauma
Arterial emboli origin
Cardiac
Acute limb ischemia requiring surgical treatment
- Paralysis
- Paraesthesia
- Rest pain
Abdominal aortic aneurysm (AAA) mortality rate
- 80%
Abdominal aortic aneurysm (AAA) surgical intervention
- Rapid growth > 1cm yearly
- Males > 5.5cm
- Females > 5cm
- Symptomatic (abdominal/back pain, tenderness, distal embolization)
Acute limb ischemia treatment
embolectomy
Acute limb ischaemia reperfusion syndrome
not enough oxygen causes muscles to die releasing potassium outside the cell but restricted to the limb.
When restoriung blood flow all the potassium will get into circulation causing hyperkalaemia in the heart and px dies due to AF/VF
The same thing happens with the myoglobin gets into the circulation and gets into the kidneys causing blockage leading to AKI. Px doesn’t pass any urine.
Patients with PAD and intermittent claudication have the risk of developing which diseases in the future?
- nonfatal myocardial infarction and stroke in the next 5 years (20%)
- death due to cardiovascular causes in the next 5 years (15%—30%)
- The risk rises exponentially PAD progression 1-year risk of cardiovascular mortality in patients with critical limb ischemia (25%)
Acute limb ischemia reperfusion injury features
– Metabolic acidosis
– Acute renal failure
– Hyperkalaemia.
– Myoglobinemia.
– CK elevation
Acute limb ischemia most common cause
Thrombosis
- lower limbs 80%
Abdominal aortic aneurysm (AAA) symptoms
Triad:
1. abdominal and/or back pain
2. pulsatile abdominal mass
3. hypotension
Acute limb ischemia clinical features/symptoms
- Context of a patient with: Thrombosis (most common cause) or Embolus from AF.
- Pain (progressive)
- Calf: Common femoral art / Superficial femoral art (MC site of occlusion).
- Buttock: Common iliac/external iliac Thrombosis. - Paralysis - Foot drop = Peroneal nerve paralysis - Most reliable sign requiring Emergency Qx intervention
- Pulselessness
- Paraesthesia
- Pallor
- Poikilothermia
Acute limb ischemia investigation
- Measure ABI
- Doppler US
- CT angiogram (gold standard) for surgical
intervention
Acute limb ischaemia initial treatment
Golden time: 4 hrs
1. IV Unfractionated Heparin: 5000 IU then 1250IU/hour. APTT guides further adjustment.
- Surgical treatment: Embolectomy - Can cause reperfusion injury (HyperK, metabacid, myoglobinuria, increased CK). Keep pt hydrated and perfused.
- Arterial bypass is helpful if it is chronic limb ischemia.
- Amputation is required only if there are irreversible ischemic changes. - After acute, give warfarin for 3m
Angioplasty/Catheter
Acute limb ischaemia reperfusion syndrome heart management
If Potassium >= 5.5:
- Stop all K+ supplementation
- Stop medication causing hyperK+
- Cardiac monitoring
If px asymptomatic + normal EKG + K >= 5.5 <= 6:
- Consider if treatment necessary.
- ± Salbutamol Neb
- ± Polystyrene sulfonate PR or oral
- ± Bicarbonate IV if metabolic acidosis
Patient Asymptomatic and Normal ECG and K+ >6 <=7mmoI/L:
- Salbutamol NEB
- Insulin/Glucose perfusion IV
- ± Resonium PR or oral
- Bicarbonate IV if metabolic acidosis
Patient unstable or Symptomatic or Clearly abnormal ECG or K+>7mmoI/L:
- Discuss dialysis
- Consider transfer to tertiary centre.
↓
- Calcium IV
- Salbutamol Neb
- Insulin / Glucose IV
- Bicarbonate IV if metabolic
acidosis - ± Resonium PR or oral
Aortic dissection (AD) confirmation investigation
CT angio
Aortic dissection/aneurysm vs myocardial infarction
Check if patient stable
if stable:
Initial: ECG
Best: CT angio
If unstable: fast US
Acute limb ischemia LMWH vs UFH
- Preceding surgery/renal impairment: UFH
- Medical Mangement only: LMWH (enoxaparin)
Pseudoaneurysm management
Ultrasound-guided thrombin injection
Pseudoaneurysm complication
femoral artery catheterization 7.5%
Clinical features of Chronic Lower Limb Ischemia
- Claudication (pain w/ exercise and relieved by rest)
- if pain at rest: 🚩🚩🚩
- Shiny hairless legs
- Muscles atrophied
Chronic limb ischemia diagnostic symptoms
- Poor pulses (hallmark)
- Shiny, hyperpigmented skin.
-Hair loss & ulceration on the legs
-Thickened nails.
-Muscle atrophy.
-Vascular bruits.
Chronic limb ischemia symptoms that require surgical intervention
– Rest Pain
– Ischemic ulceration
– Gangrene
– Claudication symptoms limiting day to day life & work, no improvement with risk factor modifications, exercises, medical management > 6 months
Chronic Lower Limb Ischaemia MEDICAL Treatment
Measure ABI:
1-1.4: Normal
0.9: Borderline. Nothing
<0.9: Manage risk factors
- Smoke cessation
- Antiplatelets (aspirin or clopidogrel) ONLY if clinically evident CVD
- Statins (even in the absence of dyslipidemia)
- ACE Inhibitors or ARBs.
- Supervised exercise program.
beta-blockers should be avoided until and unless they are commenced for cardioprotection(sys/dia cardia insufficency).
For mixed ulcers (Do not use compression bandage if ABI <0.8)
<0.4: Urgent referral
Chronic Lower Limb Ischaemia SURGICAL Treatment
– Endovascular angioplasty or stenting
– Open surgical reconstruction by bypass or endarterectomy
Chronic Lower Limb Ischaemia initial investigation
- Measure ABI
- Duplex US (often the only imaging required to plan endovascular interventions)
Chronic Lower Limb Ischaemia BEST investigation
CT Angiography w/contrast (Contraindicated in RF)
Exposure to what can trigger an asthma attack?
Sulphites
Prophylactic treatment of asthma in children?
Inhaled corticosteroids ICS : Fluticasone
NOTE: Sodium cromoglycate in some cases
Asthma best initial treatment
Short-acting β2 agonist (salbutamol)
Asthma treatment progression
Oxygen and short-acting beta-2 agonists (salbutamol)
- 6 puffs for children 0-5 and up to 12 puffs for 6 years
- wait 20 mins
If the attack does not subside, up to 6 puffs can be given every 20 minutes for one hour.
If the attack still does not subside, ipratropium bromide
- 4 puffs by MDI + mask or 250 mcg by nebuliser for children 0-5 years
- 8 puffs by MDI + mask or 500 mcg by nebuliser for children > 6 years
Oral prednisolone 1 mg/kg for first 3 days after the asthma attack (prophylactic)
Asthma in children definitive dx
- 2 and 5 years
-Bilateral wheezing - viral wheezing, pre-school wheeze, episodic viral wheeze and multiple
trigger wheeze
Chronic cough cause in children
asthma
most common
allergens that can cause allergy and asthma
dust mites 90%
sheepskin 2nd most common
Asthma LABA
Infrequent Intermittent asthma in children
- less than one episode of asthma in 6
weeks and no symptoms in between the flare-ups - inhaled short-acting beta2 agonist
(SABA)
indications for preventer therapy in asthma
symptoms at night
>1 canister b2 agonists every 3 months
using beta 2 agonists >2days /week
asthma attacks >2/month
infrquent asthma attacks but severe
spirometry with reversible airflow obstruction
Raised JVP is a feature of
Right heart failure
Cardiac tamponade in terms of heart pathogenesis
fluid accumulation in the pericardial cavity → ↑intrapericardial pressure > diastolic ventricular pressure
↓
This restricts venous return to the heart and lowers right and left ventricular filling →
↓preload ↓stroke volume ↓cardiac output.
Pericarditis Clinical Features
Chest pain + SOB+ viral infection
Kussmaul sign.
S4 Gallop: Cardiac Tamponade
Continuous Murmurs List
◦ Patent Ductus Arteriosus – Paeds
Has a harsh, machinery-like quality (Gibson murmur)
◦ Pericardial friction rub: Pericarditis or pericardial effusion. Not a real murmur. Has scratchy, scraping quality
Pericarditis Initial Investigations
- ECG:
- ST elevation except in AVR & V1
- Reciprocal PR - CXR:
- Pericardial fluid
- Pulmonary congestion - Echocardiogram: Is diagnostic! Chest FAST scan should be done ASAP.
- Cardiac CT
Pericarditis Causes
- Viral infection: Coxsackie B, CMV, influenza, EBV, COVID, HIV
- After a major heart attack or heart surgery: Dressler syndrome.
- Systemic inflammatory disorders: Lupus, rheumatoid arthritis.
- Trauma
Pericarditis Best Investigation
Echocardiogram with drainage and culture (Pericardiocentesis)
Pericarditis Complications
Constrictive pericarditis.
Cardiac tamponade
Pericarditis Medical Treatment
Mild to moderate Pericarditis
- AAS/Ibuprofen (7 to 10 days)
- Colchicine x 3 months. Indication: Recurrent symptoms (Side Effects: Diarrhea, abd pain)
- Prednisone
If infection: ATBs and drainage
Pericarditis Surgical Treatment
Severe Pericarditis, include admision
- Cardiac tamponade: Pericardiocentesis
- Severe, Recurrent or Constrictive:
Pericardiectomy
Pericarditis Pathophysiology
Restrictive Cardiomyopathy
Diastolic Dysfunction with impaired filling – relaxation
Normal Ejection fraction + S4 gallop
Dressler’s Syndrome risk factors
- Young age
- B-negative blood type
- Prior history of pericarditis
- Prior treatment with prednisone
Dressler’s Syndrome Treatment
1st LINE: NSAIDs in high doses (aspirin, ibuprofen, naproxen) tapered over 4 to 6 weeks.
2nd LINE: Corticosteroids (prednisone) tapered over a 4-week period
3rd LINE: Colchicine.
Dressler’s Syndrome investigations
Gold Standard: Echocardiogram
UNSTABLE patient: bedside ultrasonographic (E-FAST)
ECG: Same pattern as pericarditis (global ST segment elevation and T wave inversion)
Dressler’s Syndrome COMPLICATION
Cardiac Tamponade
Pericarditis Duration: Acute & Chronic timeline
Acute (<6w)
Chronic (>6w).
Kussmaul sign physical exam
Paradoxical: ↑ JVP with insp and ↓ JVP with exp)
Means: constrictive and/or cardiac tamponade.
Dressler’s Syndrome definition
Pericarditis in the context of major heart attack or heart surgery
Takotsubo Cardiomyopathy ECG:
STEMI or pericarditis pattern.
Cardiac Tamponade is an early complication of which surgical procedure post-op?
coronary artery bypass grafting
painless + elderly + recurrent pneumonia + dysphagia + solids & liquids undigested food regurgitation + coughing immediately after eating + halitosis
Zenker’s diverticulum (pharyngeal pouch)
Zenker’s diverticulum investigation (pharyngeal pouch)
Initial: Barium swallow/Contrast oesophagography
Best: Upper gastrointestinal endoscopy
Zenker’s diverticulum management (pharyngeal pouch)
Surgery: cricopharyngeal myotomy ± diverticulectomy
Laparoscopic surgery
acquired bleeding disorders other causes
- malignancy
- infection
- liver disease
- DIC
Autosomal dominant haemotological diseases
- spherocytosis
- hemorrhagic telangiectasia
- Von Willebrand disease
Autosomal recessive haemotological diseases
- Factor 5 diseases
- Fanconi’s anaemia
- haemochromatosis
Coagulation issue + low platelet
Immunocompromised - suspect HIV
delayed bleeding in trauma is due to
coagulation factor deficiency
DIC causes
- Sepsis
- Transfusion reactions
- Neoplasm (adenocarcinoma, leukemia).
- Trauma.
- Obstetric complications (placental abruption, preeclampsia, septic abortion, retained dead fetus).
DIC management
Treat underlying disorder (see causes)
Platelets
Packed RBC
FFP
Cryoprecipitate
Consider IV heparin for thrombotic complications.
DIC physical symptoms
Epistaxis
Petechiae purpura
Bleeding from venipuncture site or incisional wounds
Haematemesis
Tachycardia
Tachypnoea
Respiratory failure
drugs to avoid in haemophilia
aspirin
warfarin
heparin
early beeding in trauma is due to
platelet issue
Haemophilia features
X - linked recessive = only males affected (1 in 5000)
Factor VIII/IX deficiency
Physical symptoms:
Haemarthroses + muscle bleeds + delayed bleeding.
haemoarthrosis: especially knees, ankles, elbows
* Mild/moderate bleeding: after major trauma or surgery.
* Severe: spontanaeous bleeding
Haemophilia management
Recombinant factor VIII/IX concentrates infusion
Henoch Schonlein Purpura management
Supportive – bed rest and analgesia.
* Corticosteroids – may reduce the duration of abdominal pain, but it is uncertain if they significantly affect other features.
* Refer to a specialist if renal dysfunction, hypertension or surgical complications develop
Henoch Schonlein Purpura features
- Onset 2–8 years most common.
- lgA—mediated small vessel vasculitis
- Post URTI
- Large joint migratory arthritis of variable duration and severity.
- Fever and fatigue
- Non-TP
- Evolving crops of palpable purpura (buttocks and legs)
- Abdominal pain (occasionally melaena) may precede rash.
- High risk of Intussusception
- Nephritis w/ oedema: dorsum of the feet and hands, acute scrotal swelling and bruising
Henoch Schonlein Purpura investigation
- Full blood examination (NO thrombocytopenia).
- Urinalysis: haematuria/proteinuria.
- Renal function: urea/creatinine and urinary protein estimation.
ITP adult features
- Nearly always chronic
- Duration > 14 days
- 50% associated with HIV, SLE, CLL
- Rarely recovers without treatment
- Splenectomy usually required
ITP child features
- Acute onset
- Follows viral infection or drug hx
- Nearly always self limiting (90%)
- 10% pass onto chronic ITP
ITP investigation
Exclude ALL/AML
Bone marrow aspiration: increased megakariocytes
ITP management
Conservative management:
- platelet count > 20 x 109/L
- no active bleeding
- isolated brusiing and petechiae
- without mucousal, GI or renal tract bleeding
Inpatient management:
- Prednisolone for 2 weeks
- IV Ig
- Splenectomy in chronic cases
Most common cause of acquired bleeding disorder
drug therapy (nsaids, anticaogulants)
Prolonged aPTT causes
Increased aPTT only:
- Haemophilia A
- Haemophilia B
- Von Willebrand
Increased aPTT + PT:
- DIC
- Heparin/Warfarin
- Vit K deficiency
Prolonged aPTT is due to which pathway deficiency?
intrinsic pathway deficiency
Factors:
VIII
IX
XI
XII
prolonged PT causes
- Heparin/Warfarin
- DIC
- Vit K deficiency
- Liver disease
Prolonged PT is due to which pathway deficiency?
extrinsic pathway deficiency
Factors:
II
VII
IX?
X
TTP causes
- ADAMTS 13 deficiency
- bleeding disorders: Von Willebrand
- cocaine
- chronic rhinitis/sinusitis
- nasal cavity ulcers/ perforation
- nasal polyps
TTP investigation
Blood film:
- Prolonged bleeding time
- schistocytes (helmet cells)
- High creatinine
- Normal coagulation profile
FBC:
- anaemia
- thrombocytopenia.
TTP management
- Platelet transfusion is contraindicated.
- Plasma exchange transfusion
- FFP (maybe)
TTP symptoms
Clinical symptoms:
- Easy bleeding/epistaxis
- Neurological symptoms: confusion, seizures, headache
- Thrombosis
- Renal dysfunction
On examination:
- Fever
- Pallor
- Purpura/petechiae
- Jaundice
- Splenomegaly
TTP triggers
ADAMTS 13 deficiency
▪ Haemolytic uraemic syndrome
▪ Drugs (quinine, ticlopidine, clopidogrel,cyclosporine).
▪ SLE
▪ Infections
▪ AIDS
▪ Malignancies
Vit K deficiency management
IV Vit K
Severe bleeding: FFP
Vit K deficiency physical symptoms
Easy bruising.
Oozing from nose or gums.
Excessive bleeding from wounds, injection or surgical sites.
Heavy menstrual periods.
Bleeding from the gastrointestinal (GI)tract.
Blood in the urine and/or stool.
Vit K deficiency required for & assiciated with
Required for the function of factors II, VII, IX, X, protein C, protein S.
Associated with malabsorption, cholestatic disease.
Von Willebrand disease features
- Autosomal dominant
- Most common in girls (although genders equal)
- vWF defect resulting in decreased factor VIII + platelet adhesion to each other/ blood vessel wall
- Impaired platelet-endothelial binding
- bleeding exacerbated by NSAIDs/aspirin
- PT normal
-aPTT prolonged
Physical symptoms:
- minor surgical bleeding: dental, incisions
- epistaxis (common)
- menorrhagia
- bruising
- gingival bleeding
Von Willebrand best investigation
Ristocetin co-factor assay (best test)
Von Willebrand treatment
SURGERY
- A single dose of DDAVP (Desmopressin)
- vWF concentrate
- Menorrhagia: Tranexamic acid
DENTAL EXTRACTION:
- Tranexamic acid preoperatively and continue for 1-5 days.
- Single dose of DDAVP or/and single dose of vWF concentrate
X-linked recessive
Haemophilia A
Haemophilia B
- Glucose-6-phosphate dehydrogenase deficiency
fever + maculopapular rash+ serum AST, bilirubin and alkaline phosphatase elevation post transplant
Graft-versus-host disease
B12 deficiency conditions
- Autoimmune gastritis
- Terminal ileum disease
- Gastrectomy
- Metformin therapy
- Coeliac disease
- H.pyelori infection
- Crohn disease
- Postgastrectomy
B12 deficiency causes what type of anaemia
pernicious anaemia
fever + thrombocytopenia + low haemoglobin + acute renal failure + hallucination + haemolytic anaemia
Thrombotic thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura (TTP) treatment
- replacement fluid
- fresh frozen plasma
decreased serum albumin + hypercalcaemia + normocytic anaemia + bony lytic lesions/osteoporosis
Multiple myeloma
Multiple myeloma investigation
Initial: & diagnostic: serum and urinary
electrophoresis
haemochromatosis investigation
Serum transferrin saturation
- >60% men
- 50% women
haemophilia A in pregnancy
- measure Factor 8 level at 1st antenatal
visit and at 32 weeks - majority develop normal level of factor 8 and do not require replacement
congenital developmental anomalies + progressive pancytopenia
Fanconi’s anaemia
-Malaise and pallor + Recurrent infection + Gingival hypertrophy + Fever, night sweats + -Normocytic normochromic anaemia + Thrombocytopenia + myeloblasts more than 20%
acute myeloid leukaemia (AML)
fatigue + prolonged PT/active PTT + hypofibrinogenemia + and easy bruising + pancytopenia + coagulopathy + elevated lactate dehydrogenase
Acute Promyelocytic myeloid leukaemia (APML)
vegan vegetarian deficiency
B12 deficiency
Fanconi anaemia investigation
Diagnostic: Chromosome fragility test
Most common nutrition al deficiency in children?
Iron deficiency anaemia
Most common cause of iron deficiency anaemia in developed countries?
Occult blood loss
rise in platelet count causes
– Polycythemia vera.
– Hyposplenism due to splenectomy.
– Iron deficiency anemia.
– Correction of vitamin B12 and folic acid deficiency
pruritis on hot bath + vertigo + tinnitus + headache + visual disturbances + hypertension + facial plethora + transient ischemic strokes
Polycythaemia vera
Polycythaemia Vera lab findings
- JAK2 mutation positive
- Increased blood viscosity
- Elevated haemoglobin
- Leucocytosis & thrombocytosis
- Low erythropoietin level
Polycythaemia Vera treatment
- Phlebotomy
- Hydroxyurea (if ↑ risk of thrombus)
acute haemolysis following ingestion of cotrimoxazole/trimethoprim-sulfamethoxazole, nitrofurantoin, primaquine
Glucose-6-phosphate dehydrogenase deficiency
sudden onset of jaundice + pallor, +
dark urine + with or without abdominal and back pain + fall in the haemoglobin concentration + “bite” cells
Glucose-6-phosphate dehydrogenase deficiency
The outcome of splenectomy in a patient with spherocytosis
1- Spherocytosis persists after splenectomy, the cells survive longer in the circulation.
2-Decrease in reticulocytosis.
3- RBC fragility remains high resulting short life span of red blood cells
4- Fall in elevated bilirubin
5- In severely affected patients, life-threatening anaemia
Fanconi anaemia congenital abnormalities
- Hand and arm anomalies (misshapen,missing or extra thumbs or abnormalities of
the radius) - Skeletal anomalies of the hips, spine or ribs
- Skin discolouration (café au lait spots, hypopigmentation)
- Small head or eyes
- Low birth weight and subsequent short stature
- Missing or horseshoe kidney
- Gastrointestinal abnormalities including abnormal development of the
oesophagus
Fanconi’s anaemia treatment
Hematopoietic stem cell transplant
Spherocytosis gene defect
spectrin
ancyrin
protein 4.1
CRAB
C - hypercalcaemia 13%
R - renal impairment 20–40%
A - anaemia 70%
B - bony lesions
hepatomegaly + weakness+ hyperpigmentation + atypical
arthritis + diabetes + impotence + unexplained chronic abdominal pain + cardiomyopathy + Decreased production of FSH and LH + gynaecomastia + 90% are C282Y homozygotes
haemochromatosis
Vitamin B12 bsorbed by binding to
intrinsic factor
conditions which can lead to the development of AML
-Trisomy 21 noted in Down syndrome.
-Fanconi anaemia.
-Ataxia-telangiectasia.
-Myeloproliferative syndromes.
urticaria + stridor + hypotension during blood transfusion+ hx of recurrent infections
Immunoglobulin A deficiency
> 60y/o + fatigue and weakness, dyspnea, and pallor + dysplasia + Anaemia with/o f bi- or
pancytopenia+ Macrocytosis + absolute neutropenia
Myelodysplasia
common feature of autoimmune thrombocytopenia in
childhood
Antecedent viral illness
microcytic anemia + increased concentration of hemoglobin A2
β-Thalassemia
weakness + fatigue + dizziness + palpitations + exercise intolerance + craving of ice/clay
Iron deficiency
increased MCV + neutropenia + thrombocytopenia + history of alcohol abuse + hypersegmentation of
the neutrophils + taking phenytoin
Folate deficiency
Causes of macrocytic anemia
High MCV
megaloblastic anemias
- Vitamin B12 (cobalamin)
- folate deficiency
- vigorous reticulocytosis
- hypothyroidism
- chronic liver disease
- myelodysplastic syndrome
Common causes of microcytic anaemia
Low MCV
iron deficiency
thalassemia,
anaemia of chronic disease
sideroblastic anaemia (Vitamin B6 (pyridoxine) deficiency)
Iron studies in microcytic anaemia
Cause | MCV | Iron | TIBC | Ferritin | Transferrin Saturation
Iron Deficiency | ↓ | ↓ | ↑ | ↓ | ↓
Thalassemia | ↓↓ | ↑ | ↓ | ↑ | ↑↑
Anaemia of |Normal/↓ | ↓ | ↓ | Normal/↑ | Normal/↓
chronic disease
microcytic anaemia + . Seizures + peripheral neuritis + dermatitis + isoniazid
Vitamin B6 (pyridoxine) deficiency
Pyridoxine anaemia lab findings
hypochromic and normochromic RBC’s
cheilosis (welling and fissuring of the lips) + glossitis+ a variety of ocular problems + hx of biliary atresia/hepatitis
Folate deficiency
pallor + sweats + weight loss + bilateral axillary lymphadenopathy + massive Spleen and liver megaly
Chronic lymphocytic leukaemia (CLL)
mature lymphocytes with the presence of smudge cells means
Chronic lymphocytic leukaemia (CLL)
Most common manifestation of cardiac
involvement in hemochromatosis
Congestive cardiac failure
difference between haemolytic anaemia vs anaemia of chronic disease
Low haptoglobin
Glucose-6-phosphate dehydrogenase deficiency dx
Heinz Bodies
decreased red cell, white cell and platelets counts
pancytopenia/aplastic anemia
aplastic anaemia causes
- NSAIDs
- Sulphonamides.
– Gold.
– Anti-epileptic drugs (carbamazepine, valproic acid, phenytoin).
– Nifedipine.
– Chloramphenicol.
sickle cell anaemia how to reduce iron overload
- Subcutaneous deferoxamine
- Splenectomy
- Erythrocytopheresis
Sickle cell anaemia maintenance management
- Vaccination
- Penicillin (until age 5)
- Folic acid supplementation
- Hydroxyurea (for patients with recurrent vaso-occlusive crises)
Sickle cell anaemia pain crisis management
- Hydration
- Analgesia
- +/- Transfusion
Sickle cell anaemia first line medication
Hydroxyurea
NOTE: side-effect is myelosuppression
neutropenia, anaemia, thrombocytopenia
poor prognosis of multiple myeloma
- higher levels of beta-2 microglobulin
- lower levels of albumin
px around 70 + fatigue + tiredness + macrocytic anaemia
myelodysplasia
myelodysplasia investigation
Bone marrow biopsy
generalised weakness, low-grade fever and sore throat + normal haemoglobin + low neutrophil + low platelets + thyroid medicatiob
agranulocytosis
spherocytosis clinical features
1-Haemolytic Anaemia and jaundice.
2-Splenomegaly.
3- elevated mean corpuscular hemoglobin
concentration (MCHC)
4-Positive family history of anaemia, jaundice, or gallstones.
5-Spherocytosis with increased reticulocytes.
6-Increased osmotic fragility.
7-Negative direct antiglobulin test (DAT).
most common causes of death in hemochromatosis
- Liver disease
- cardiomypathy
most profound adverse effect of chronic
transfusional iron overload in children with thalassemia?
Progressive hepatic cirrhosis
Long-standing history of prolonged bleeding after trauma and history of menorrhagia in an otherwise healthy woman
Von Willebrand disease
earliest complication of sickle cell disease
Bone infarction (can lead to avascular necrosis)
progressive, painless lymphadenopathy + fever, night sweats + weight loss + elevated LDH
non-Hodgkin lymphoma
non-Hodgkin lymphoma features
GI tract most common site
- 3% of all malignant gastric tumors
- Surgery alone can be considered adequate treatment that does not infiltrate beyond the submucosa
-
β-Thalassemia investigation
diagnostic: hemoglobin electrophoresis
incidence of thrombosis and bleeding in patients with myeloproliferative neoplasms?
- typically mucocutaneous
- Bleeding is usually less severe and less frequent than thrombosis
- Arterial
thromboses are more common than venous thrombosis - Strokes are more frequent followed by myocardial infarction and peripheral arterialocclusion
best test to monitor heparin therapy
Activated partial thromboplastin time (aPTT)
DVT in the presence of thrombocytopenia
heparin induced thrombocytopenia
types of heparin induced thrombocytopenia
Type 1 : first 2 days after exposure, platelet count normalizes with continued heparin therapy. non-immune
Type 2: typically occurs 4-10 days after
exposure, immune-mediated disorder
- Venous limb gangrene
- Bilateral adrenal hemorrhagic infarction
- Skin lesions at injection sites
- Acute systemic reactions following an i- ntravenous heparin bolus
heparin induced thrombocytopenia management
discontinue and avoid all heparin products immediately
CLL management
1st line: corticosteroids such as prednisolone
chemotherapy if needed
immediate haemolytic reaction secondary to a blood transfusion investigation
Positive Coombs test
helmet cells + Artificial (mechanical) valves placemenent
schistocytes Hemolytic anemia
schistocytes Hemolytic anemia lab findings
Decreased serum haptoglobin level
Renal failure reflected by oliguria and abdominal pain following invasive diarrhoea
hemolytic uremic syndrome (HUS).
Preceding bloody diarrhoea + Fatigue, pallor + Bruising, petechiae + Oliguria, oedema + Decreased platelet count
Haemolytic uremic syndrome (HUS)
respiratory distress + hypotension + bilateral pulmonary infiltrates minutes to hours after blood transfusion
Transfusion-related acute lung injury (TRALI)
age 15-35 & >60 + fever, night sweats, weight loss + painless generalized lymphadenopathy which becomes painful after alcohol consumption + Mediastinal mass
Hodgkin lymphoma (HL)
Hodgkin lymphoma epidemiology
- Bimodal peak incidence: age 15-35 & > 60
- Association with EBV in the immunosuppressed
Hodgkin lymphoma (HL) physical symptoms
- Painless lymphadenopathy
- Mediastinal mass
- Fever, sweats, weight loss
- Pruritus
Hodgkin lymphoma (HL) highest risk of malignancy
Supraclavicular lyphadenopathy
Hodgkin lymphoma (HL) diagnostic investigation
Lymph node excision biopsy
- Reed-Sternberg cells on histology
Normal pH
7.35-7.45
CURB-65 Score
– Confusion of new onset.
– Urea greater than 7 mmol/l.
– Respiratory rate of 30 breaths per minute or higher.
– Blood pressure less than 90 mmHg systolic or diastolic blood pressure 60 mmHg
or less.
– Age 65 or older
CURB-65 score equal or >3, an
inpatient treatment for community acquired pneumonia is necessary.
Treatment for community acquired pneumonia
IV benzyl penicillin and azithromycin
periodontal disease + cough + foul-smelling sputum + immobilised (hiatal, post-op)
Aspiration pneumonia
Aspiration pneumonia treatment
1st line: ampicillin-sulbactam (1.5 to 3 g IV every 6 hours)
Aspiration pneumonia pathogen
oral streptococci
Bronchiectasis treatment
IV ticarcillin-clavulanate
pigmentation of skin + lung mass + hypokalaemia + hirsutism
Small cell lung carcinoma
-Ectopic production of adrenal corticotropin (ACTH)
Small cell lung carcinoma complication
Ectopic production of ACTH can lead to Cushing’s syndrome
- hypokalaemia
Most common causes pulmonary hypertension and cor-pulmonale
- COPD
- Emphysema
cough + mucopurulent sputum lasting for months to years + finger clubbing
bronchiectasis
Bronchiectasis investigation of choice
High-res CT
best bed-side measure to assess the severity of asthma
Use of accessory muscles upon inspiration
abdominal / trans-thoracic procedure post-op + cough and fever + within 48 hours
Postoperative atelectasis
Postoperative atelectasis management
- Removal of impacted secretions by coughing, managed by physiotherapists, and involves active chest percussion and breathing exercises.
- oxygen supplementation
- Passive postural drainage.
- bronchoscopy if px still hypoxic
FEV1/FVC ratio
0.75-0.85
FEV1/FVC ratio > 0.85
Restrictive airway disease
FEV1/FVC ratio < 0.75
Obstructive airway disease
dyspnea + cough + fever + productive sputum +
occasionally pleuritic chest pain
pneumonia
Small cell lung carcinoma treatment
chemotherapy and radiation
pleural fluid analysis
- pH always below 7.30
- Pleural/serum protein ratio greater than 0.5
- Pleural/Serum lactic dehydrogenase ratio of greater than 0.6
- Glucose concentration (30-50 mg/dL)
- Bloody pleural fluid is strongly suggestive of malignancy
High altitude pulmonary oedema cause
Hypoxic pulmonary vasoconstriction
clubbing of nails can be found in
– Cystic fibrosis.
– Interstitial pulmonary fibrosis.
– Lung cancer
– Interstitial lung disease
- bronchiectasis
middle/older age + high grade fever + dry cough + severe headache + diarrhoea/vomiting/confusion + hyponatremia + deranged liver function tests
Legionnaires disease
recurrent asthma exacerbations + patchy opacities + central bronchiectasis + eosinophilia+ elevated immunoglobulin E
allergic bronchopulmonary aspergillosis
latent tuberculosis (LTB) treatment
Isoniazid (5mg/kg up to a maximum 300mg daily) for 9 months
superior vena cava syndrome investigation
chest CT angiogram
cough + weight loss + lung nodule in X-ray
adenocarcinoma
adenocarcinoma investigation
FNAC gold standard
Difference between crackles associated with alveolar fluid vs interstitial lung disease
Egophony
smoker + pulmonary nodule + hypercalcemia (increased thirst, abdominal pain, constipation, and polyuria)
squamous cell carcinoma
(fever, malaise, cough + acute SOB + bilateral crackles + asymptomatic between exacerbations
hypersensitivity pneumonitis (alveolitis)
IV drug abuse + dyspnoea + bilateral pleuritic chest pain + multiple partners sex hx + hypoxemic
Pneumocystis jiroveci pneumonia
family hx of emphysema/liver disease + + weight loss, + jaundice + dyspnoea
Alpha-1 antitrypsin (AAT) deficiency
idiopathic pulmonary fibrosis (IPF) treatment
Antifibrotic therapy
black + 20-30 years + erythema nodosum + arthritis + hilar lymphadenopathy
Sarcoidosis Lofgren syndrome
Unprocessed raw cotton + bacterial endotoxin
Byssinosis
doesn’t cause COPD
Normal PaO2
75-100 mmHg
Normal PaCO2
35-45 mmHg
Normal HCO3
22-26 meq/L
black + 20-30 years + fever + parotid gland enlargement + uveitis + facial palsy
Sarcoidosis
pneumothorax in the absence of trauma and no history of lung disease
Primary spontaneous pneumothorax (PSP)
Pneumothorax management
PSP < 15%: observation
PSP > 15%: needle aspiration
SSP: mechanical ventilation (can’t put needle into an already damaged lung)
Symptomatic SSP: Catheter drainage/water sealed thorcostomy
Pneumothorax as well as other lung disease is labelled as
Secondary spontaneous pneumothorax (SSP)
Most common symptom in pleural effusion
Dyspnoea
Most effective preventative therapy for asthma
ICS
- fluticasone
- beclomethasone
- budesonide
Montelukast if ICS contraindicated
NOTE: Montelukast is associated with nightmares in children
X-ray pleural thickening + significant weight loss + pleural effusion
mesothlioma
when to add preventer
using preventer if they use salbutamol more than 3x week
Residual lung volume increased
obstructive disease
Residual lung volume decreased
restrictive disease
Asthma causes
emotional changes
gord
allergies
pregnancy
occupational hazards
dust mites
List of restrictive diseases
- pulmonary fibrosis
- pneumothorax
List of obstructive diseases
- asthma
- COPD
Asthma dx
triad: dyspnoea + wheeze + cough
fev1/fevc ratio < 75
PEF: improvement in fev1 more > 12% after medication (salbutamol)
- prolonged expiration
- resonant
- expiratory wheeze
respiratory acidosis
Types of asthma
mild:
moderate:
severe:
4 hallmark indicators of severe asthma
- <94% oxygen saturation
- altered mental state
- effort on breathing
- struggling to talk
role of oral steroids in asthma
can only be used in exacerbation, not for long term
prolonged use of oral steroids (prednisolone):
- Cushing syndrome,
- osteoporosis,
- immunosuppression
Exercised induced asthma management
Cromolyn
physical symptoms that are not indicated in assessing severe asthma
- wheezing
- respiratory rate
fever + malaise + hepatosplenomegaly + exudative pharyngitis + cervical lymphadenopathy
Infectious mononucleosis
Indications for splenectomy
- Trauma
- Spontaneous rupture (mononucleosis)
- Hypersplenism (ITP)
- Neoplasia
Infectious mononucleosis cause
EBV
Infectious mononucleosis inv
Initial: FBC - Atypical lymphocytosis (>50%)
- ESR: Differentiate between IM (elevated) & Strep pharynitis (normal)
Diagnostic: Positive heterophile antibody (Monospot) test
Transient hepatitis
Infectious mononucleosis management
- Supportive treatment: rest, fluids, analgaesia
- Symptoms resolve within 1-2 weeks
- Avoid sports due to risk of splenic rupture
Symptoms 1-2 weeks after exposure + Fever + skin rash + polyarthralgia + urticaria
Serum sickness
Serum sickness sensitivity type
type III
Serum sickness management
- Remove/avoid offending agent
- Supportive care
- Steroids or plasmapheresis if severe
Malar rash (butterfly rash),
- highly specific for
the diagnosis of SLE - flat, scaly, non-pruritic and
characteristically spares the nasolabial fold
metacarpophalangeal joint + wrists are warm and tender + swollen
RA
SLE investigation
(ANA) is indicated as the best initial screening test.
The dsDNA is 90% specific
highest specificity for rheumatoid arthritis
Anti-citrullinated peptide antibody
Anti-CCP
SLE clinical features
- Anaemia, leukopenia, thrombocytopenia
- most common presentation of this arthritis is symmetrical polyarthritis affecting the hands, wrist and knees.
- affects women in high oestrogen period
- more common in females of African American, Hispanic, and Asian descent
- weight loss
- malar rash and oral ulcers
Thrombotic thrombocytopenic purpura
- thrombotic microangiopathy
- severely reduced activity of the von Willebrand factor-cleaving protease
ADAMTS13 - small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia, and sometimes organ
damage
colchicine contraindications
1- Renal insufficiency.
2-Cytopenias.
3-Abnormal liver functions.
4-Sepsis.
Bradycardia in a patient with SLE antibody
Anti-Ro
Acute rheumatic fever clinical features
- Peak incidence: age 5-15
- Twice as common in girls
- Major symptoms: JONES
*Joints (migratory arthritis) - ❤️ (Carditis)
- Nodules (subcutaneous)
- Erythema marginatum
- Sydenham chorea
- Minor symptoms: FEAP
- Fever
- Arthralgias
- Elevated ESR/CRP
- Prolonged PR interval
Acute rheumatic fever late sequelae
Mitral regurgitation/stenosis
Acute rheumatic fever diagnosis
group A streptococcal infection along with 2 major
or
1 major plus 2 minor (J❤️NES)
ARF can also present with
carditis
chorea
elevated ESR
Coxsakie virus also known as
Hand foot and mouth disease
coxsackie can lead to what condition?
myocarditis
Coxsackie seasonal onset
late summer early autumn
fever + lesions on hands/feet + oral sores/ulcerations
Coxsackie (HFM)
Rinne test in relation to AC/BC
AC>BC = Normal/SNHL
BC>AC= CHL
Development of pubic hair
Girls: 8 years
Boys: 9 years
SIADH in children
- hyponatremia
- excessive amount of ADH from hypothalamus-pituitary
Hyponatremia in children
Dehydration: hypernatremia
Cardiac: Pseudohyponatremia
Addisons:
- Low aldosterone
- Hyperkalaemia
- hypotension
SIADH in children symptoms
- nausea and vomiting
- headache
- problems with balance
- mental changes (confusion, memory problems)
- seizures or worst case, coma
SIADH in children causes
- type 2 DM medication:
- antiepileptic
- antidepressant
- surgery under general anaesthesia
- brain disorders, injury, infections, stroke
- lung disease (pneumonia, tuberculosis, cancer, chronic infections)
- cancer of lung, small intestine, brain, leukaemia
Weber’s test
Normal = lateralizes equally to both ears
CHL= lateralizes to abnormal ear
SNHL= lateralizes to the normal ear
Acute Otitis media risk factors
- Age (6-18 months)
- Lack of breastfeeding
- Day care attendance
- Smoke exposure
Pathogens that give arise to acute otitis media
- Streptococcus pneumoniae (most common)
- Nontypeable (?) Haemophilus influenzae
- Moraxella catarrhali
Complications of Otitis media
Acute mastoiditis
Acute mastoiditis initial treatment
sample from the ear discharge should be taken for culture DONE BY ENT
flucloxacillin + a third-generation cephalosporin
Acute otitis media treatment
- Amoxicillin 1st choice (used for 1 week)
- 2nd line: amoxicillin-clavulanate
Acute otitis media treatment px that’s allergic to penicillin
- clindamycin
- azithromycin
Chronic suppurative otitis media treatment
Assess if tympanic membrane intact or perforated:
If intact: ciprofloxacin ear drops to treat on going infection
If perforated: initial treatment ear toilet with povidone-iodine solution, followed by
dry mopping with rolled toilet papers2 to 3 times a day using 20ml syringe with plastic tubing. In addition, ciprofloxacin ear drops
Untreated a tympanic membrane perforation secondary to chronic otitis media
- Marginal perforation with discharge
- Perforation that is surrounded by granulation tissue
- Continuously discharging central perforation
- Perforation associated with a cholesteatoma
Cholesteatoma risk factors
- history of recurrent acute otitis media
- chronic middle ear effusion
- tympanostomy tube placement
- history of cleft palate
Lymph nodes red flags
Weight loss
Sustained fever
Night sweats Generalized lymphadenopathy
Signs and symptoms of pancytopenia
Mass persisting> 6 weeks
Lymph node> 3 cm
Thyroid mass
Supraclavicular mass
Hard, irregular mass
Fixed mass
1 standard deviation above/below the 50th percentile
Weight: overweight/underweight
2 standard deviation above/below the 50th percentile
Weight: obese/severely underweight
Marfan’s syndrome (MFS) features
- autosomal dominant connective tissue
disorder (FBN1) - affects height
- does not affect weight
- aortic aneurysm
dissection, aortic regurgitation (Decrescendo high-pitched diastolic murmur at the left sternal edge) - Myopia and ectopic ocular lens.
Most common neurological symptoms in children with cancer
- Nausea
- Vomiting
Most common neurological cancers in children
Lymphadenopathy in children
viral upper respiratory tract infection
TB, Infectious
mononucleosis cytomegalovirus infection
Cat scratch disease
haematological and nonhematological malignancies, Kawasaki
disease.
Growing pains
Growing pains refers to pain in the lower
extremities of growing active children.
children between 2 and 12 years with the peak
incidence in preschool children.
mostly felt in the thighs, calves and
behind the knee.
intermittent and in mostly present in the afternoon and evening after activity during the day.
Pain may also wake up the child at night.
Perthes
- avascular necrosis of the femoral head.
4 -8 years of age
associated with hip pain and intermitent limp.
Transient synovitis
- 3-8 years
- URTI hx
- sudden onset unilateral hip or groin pain most common sx
- medial thigh or knee
pain 2nd common sx
Arthralgia and arthritis secondary to synovium inflammation - no fever, or only a mild fever
Features of Epiglottitis
– H. influenzae
High fever, sore throat,
dysphagia, odynophagia,
drooling due to inability to swallow the saliva because swallowing
is painful and difficult.
child miserable adopts a tripod position with a hyperextended neck to maintain the airway open. Muffled voice (hot potato voice)
Lungs are clear on auscultation initiallt later expiratory stridor (ominous sign.)
