Part 2 Set D

• RA
• Pneumoconiosis

Autoimmune destruction of the exocrine glands,

• LISTERIA!!
• Ampicillin and GENT

EAE

• Abdo Pain
• Postural drops in BP
• Hyperpigmentation
• Wt loss
• Hyperkalemia
• Hyponatremia
• Hypoglycaemia
• Hypercalcaemia
• Neutropenia
• Lymphocytosis
• Eosinophilia
• Anaemia
• Abnormal LFTs

• Autoimmune destruction
• TB
• Haemorrhage
• Sepsis (inc Waterhouse-friedrichson due to N.men)
• Shock
• Mets
• HIV and opportunistic infections

• Cerebral aneurysms
• Hepatic + pancreatic cysts
• Colonic diverticulae
• Mitral valve prolapse

• Autosomal Dominant
• Intestinlal polyposis
• Colon cancer (90% by age 45)
• Osteomas
• Soft tissue tumors (Epidermoid, lipomas fibromas)
• Coloectomy advised once polyps appear.

Phenoxybenzamine. Pure Alpha blocker. If you start beta blockade alone you leave alpha unopposed to cause a Hypertensive crisis.

• Autosomal Recessive
• Sensorimotor peripheral neuropathy

Caused by defective alpha oxidisation of phytanic acid - leading to its accumulation in tissue and high serum levels.

• Also presents with sensorineural deafness, anosmia, cerebellar ataxia, pes cavus and nightblindnessvisual disturbance secondary to retinitis pigmentosa.
• Epiphyseal dysplasia causes characteristic SHORTENING of the 4th toe.

Either CDKN2A positive or BRCA - 2 positive

• Polyneuropathy - bilat starting distally
• Organomegaly
• Endocrinopathy
• MGUS
• Skin changes - bilat

Occurs in the setting of plasma cell dyscrasias - inc mgus

• TD can become chronic on DA therapy.
• It can persist for many years even after the offending agent has been stopped. 
• Stopping the offending agent can sometimes worsen symptoms!
• In 23 of cases the TD resolves at 3 years.

• Skin Rash (can progress to erythroderma)
• Diarrhoea (sometimes bloody)
• Liver dysfunction (cholestatic)

• Aggresive Methylpred
• Dont stop the immunosuppressant
• Give TPN if diarrhoea is bad and theyre not absorbing

• Malabsorption, due to trophyerema whippeli.
• Villi in GIT expand due to macrophage infiltrates which are PAS positive Acid-schiff positive.
• Wt loss, steatorrhoea, Joint pains, dementia

• pANCA = UC
• ASCA = Crohns
• Anti Saccharomyces cerevisae antibodies

• Serum sodium 125 or less
• High urine osmolality
• Low plasma osmolality
• High urinary sodium
• In a euvolemic patient with no evidence of renal, adrenal, thyroid, hepatic or cardiac disease

• Dimethylchlorotetracycline
• If that doesnt work. Hypertonic saline

• Women in their 30s who are on the OCP and have a solitary liver mass.
• Single, Subcapsular, Hypoechoic, asymptomatic.
• Resect themLeave them - monitor with aFP.

Spread between animals by ticks.

Humans get it via unpasteurised milk

• Multiple exacerbations
• Chronic pseudomonal infection
• FEV<60%

Otherwise chances are fairly good!!

Dose adjustment for Normal eating.

Education program for IDDM patients to titrate insulin accordingly

• Asthma - Type 1 hypersenstitivity
• Aspergilloma
• Invasive aspergillosis
• Allergic Bronchopulmonary Aspergillosis - Type 3 hypersensitivity
• Extrinsic allergic Alveolitis

Pathognomic for Whipples

Contraction of the jaw on pendular eye movements (which are constant) and up gaze palsy

Trivalent antiserum + guanidine hydrochloride

• Migraine with aura
• Stroke like episodes
• Positive family history for migraine and EARLY dementia.

All in the absence of vascular risk factors.

Encephalopathy secondary to small vessel arteriopathy caused by hypertension.

• Elevated blood and CSF lactate which increase dramatically on exercise.
• Short stature
• Diabetes
• Heart disease
• Optic nerve problems

Drug-induced lupus

Isoniazid, Phenytoin, Hydralazine, procainamide, chlorpromazine

• Systemic disease
• Granulomatosis with polyangitis
• Goodpastures
• Polyarteritis nodosa
• Microscopic polyangitis
• SLE
• HSP
• Churg-strauss

• Primarily pulmonary
• Bacterial pneumonia with AKI
• Legionella pneumonia with interstitial nephritis

• others
• Uremic pneumonitis
• Right sided bacterial endocarditis causing glomerulonephritis
• ARF causing pulmonary oedema

Von-hipple Lindau

• GI symptoms
• Eye symptoms
• Resp symptoms

• Alpha galcatosidase A deficiency leading to creamed trihexose accumulation.
• Xlinked recessive
• Intermittent lancinating pains in extremeties.
• Triggered by exercise, hot weather and febrile illness
• HTN, cardiomegaly, renal failure and thrombotic infarction.

PTHreceptor is resistant to PTH. therefore high levels of PTH and low CA2+

Short stature, short 45 metacarpals, Hypocalcaemia, Learning difficulties

Treat with calcium and vit d supplements

• Assoc with:
• Iron deficiency anaemia
• COPD
• ESRF
• Gastric surgery
• Chronic venous insufficiency
• hypothyroidism

Medications = Bblocker, H2antags, neuroleptics

Extrinsic allergic alveolitis. Go back and take a good history.

• B-blockade
• Then ICD

Digoxin!!

And you'll be tempted to use it because they'll come in with restrictive cardiomyopathy and AF and you'll want to give it....DONT give it. The dig binds to the amyloid and these patients are really sensitive to it.

• Age onset 50-70
• Antihistone bodies positive in drug induced not in SLE
• C3/4 normal in drug induced, low in SLE

Juvenile myoclonic epilepsy

• Seizures types include
• Absences
• Myoclonic jerks and
• Tonic-clonic seizures which tend to occur within an hour of waking.

Precipitating factors include alcohol, menstruation and sleep deprivation.

The condition is genetically linked to the short arm of chromosome 6.

Prognosis is extremely favourable if the condition is treated correctly, with many patients becoming seizure-free. 

Treatment options include sodium valproate, lamotrigine and topiramate.

Lifelong drug treatment is usually necessary to avoid relapses in patients who achieve seizure-free status on medication.

Liver failure, renal failure, Metastatic cancer

Allopurinol inhibits xanthine oxidase which stops the breakdown of 6Mercaptopurine.

Aza needs to be dose reduced by 25%

Patient characteristics = 

• Old age
• Presence of prosthetic valve endocarditis
• Insulin dependent diabetes mellitus
• Severe co-morbidities.

Disease factors

• Endocarditis due to fungus or Gram negative bacilli
• Endocarditis complications including heart failure, renal failure, stroke, septic shock and periannular complications
• Echocardiographic findings including severe left sided valve regurgitation, low left ventricular ejection fraction, pulmonary hypertension, large vegetations and severe prosthetic valve dysfunction.

Cytotoxic T Lymphocyte Antigen 4 Homologue

Anti IL 1

• Adalimumab,
• etanercept,
• infliximab,
• golimumab
• certolizumab

If PaO2 is <60

• Happens to people who have a spinal injury above C6.
• Some Bladder or bowel disturbance results in a poorly understood cascade which increases vagal tone resulting in:

• Bradycardia
• Flushing
• Severe hypertension

Lactate of course!!