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Conn's Syndrome Print E-mail
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Contributed by Jim Newton   
Thursday, 08 March 2007
Untitled Document

 

Primary hyperaldosteronism secondary to adenoma of the adrenal cortex

Leads to sodium retention, potassium loss

Hypokalaemic Alkalosis with hypertension is seen

adrenal adenoma (more in females), tumours involve the zona glomerulosa of the cortex
Primary hyperaldosteronism can also occur as a result of bilateral adrenal hyperplasia (40%, more in elderly men)

 

Features:

Hypertension and muscle weakness, myalgia, nocturia and tetany are seen

 

Investigations:

Check potassium off diuretics
Aldosterone levels (remain high despite IV N saline or fludrocortisone), renin levels are reduced (an increase in th aldosterone:renin ratio)

Postural samples:

  • Aldosterone increases on standing in adenoma
  • Aldosterone decreases on standing in hyperplasia

CT and MRI can be used to demonstrate tumours

Rare form of glucocorticoid responsive hyperaldosteronism

 

Management

Surgical removal of tumour
Spironolactone (Aldosterone antagonist)
Calcium channel blockers are also helpful for hypertension but do not affect aldosterone levels

 

Last Updated ( Thursday, 08 March 2007 )
Churg-Strauss Syndrome Print E-mail
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Contributed by Jim Newton   
Thursday, 08 March 2007
Untitled Document

 

Otherwise known as allergic granulomatosis

A small vessel arteritis affecting mainly respiratory and neurological systems

Males, usually >30

Features

  • Rhinitis
  • Asthma
  • Eosinophilia
  • Vasculitis
  • Tender skin nodules
  • Rarely involves kidneys

Probably allergic in origin

Eosinophilic infiltrate seen in affected areas

Transient lung shadows are seen

Diagnosis

ANCA is positive (MPO type)

Treatment

steroids
Last Updated ( Thursday, 08 March 2007 )
Cheidak-Higashi Syndrome Print E-mail
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Contributed by Jim Newton   
Thursday, 08 March 2007
Untitled Document

 

Autosomal recessive

Giant granules in myeloid cells leads to...

Defective function and abnormal microbial killing

Features

Albinism

Recurrent infections

 
Last Updated ( Thursday, 08 March 2007 )
Chaga's Disease Print E-mail
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Contributed by Jim Newton   
Thursday, 08 March 2007
Untitled Document

 

American Trypanosomiasis

Trypanasomiasis Cruri (transmitted by the Reduviid bug)

Insect transmission

Occurs in South and Central Americas (compare with African Trypanosomiasis or sleeping sickness transmitted by the Tsetse fly)

 

Features:

2 weeks incubation

Acute disease:

  • Usually in children
  • Erythematous papule at bite (chagoma)
  • Lymphadenopathy
  • Fever
  • Hepatosplenomegaly
  • Usually recover
  • Rarely develop heart failure

Chronic disease:

  • After many years
  • Probably autoimmune
  • Heart involved in most
  • Right heart failure and arrhythmia, conduction abnormalities
  • Dysphagia due to myenteric plexus disease
  • Thromboembolism
  • Aspiration

 

Investigations:

Trypanosomes can be seen in blood in acute phase

Chronic phase diagnosed by complement fixation tests, indirect antibodies and haemagglutination

 

Management:

Benzimidazole for 60 days is main treatment

Cytotoxic drugs are ulikely to be effective in chronic Chaga's disease due to its probably autoimmune aetiology

 
Last Updated ( Thursday, 08 March 2007 )
Budd-Chiari Syndrome Print E-mail
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Contributed by Jim Newton   
Thursday, 08 March 2007
Untitled Document

 

Occlusion of the hepatic vein

Classically spares caudate lobe as this drains directly into IVC

 

Causes:

  • Hypercoagulable states (Polycythaemia Rubra Vera, the OCP, leukaemia)
  • Adrenal tumours
  • Web
  • Sarcoma
  • Hydatid cyst
  • Radiotherapy

 

Presentation:

  • Acute abdominal pain
  • Nausea and vomiting
  • Tender enlarged liver
  • Ascites with high protein content

Can also present chronically with enlarged caudate lobe, jaundice ascites and splenomegaly

 

Investigations

Exclude RVF and IVC obstruction

Drain and analyse ascites

US / CT / MRI to demonstrate obstructed hepatic vein

Doppler ultrasound to indicate reversal of flow within the hepatic vein

Radioisotope can show spared caudate lobe

 

Treatment

Treat underlying condition

Thrombolysis

Surgical decompression with shunts (e.g. the transjugular intrahepatic portosystemic shunt or TIPS)

 
Last Updated ( Saturday, 26 January 2008 )
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