14.03.05 Revision notes

Osler-Weber-Rendu syndrome

A 30-year-old man is investigated for recurrent nose bleeds and iron deficiency anaemia. You notice a number of erythematous lesions on his skin:

• AKA hereditary haemorrhagic telangiectasia (HHT)

• Vascular dysplasia leading to telangiectasia

• AVMs of skin, mucosa, and viscera

• Epistaxis and gastrointestinal bleeding

Notes

• A low-grade temperature is an under appreciated sign of pulmonary embolism

• Howell–Jolly bodies

  • • Basophilic nuclear remnants in circulating erythrocytes

• • Usually signifies a damaged spleen

• Geographic tongue

• • Benign, chronic condition of unknown cause

  • Present in around 1-3% of the population and is more common in females

  • Erythematous areas with a white-grey border (the irregular, smooth red areas are said to look like the outline of a map)

  • Some patients report burning after eating certain food

• Keratoderma blenorrhagica

• • Waxy yellow/brown papules on palms and soles

  • Feature of reactive arthritis

• Burning thigh pain - ? meralgia paraesthetica - lateral cutaneous nerve of thigh compression

• Vitiligo is more common in patients with known autoimmune conditions such as thyrotoxicosis

• Milrinone (PDE3 inhibitor) => + Contractility / Vasodilation (reduced afterload)

• Takyasu's arteritis

• • Inflammatory, obliterative arteritis affecting aorta and branches

  • Females> Males

  • Symptoms may include upper limb claudication

  • Clinical findings include diminished or absent pulses

  • ESR often affected during the acute phase

• Buergers disease

• • Segmental thrombotic occlusions of the small and medium sized lower limb vessels

  • Commonest in young male smokers

  • Proximal pulses usually present, but pedal pulses are lost

  • An acuter hypercellular occlusive thrombus is often present

  • Tortuous corkscrew shaped collateral vessels may be seen on angiography

• Fanconi syndrome is due to impaired function of proximal renal tubule

  • • Abnormal amounts of carbohydrates and amino acids in urine

• • Excessive urination

    • Low serum potassium and phosphate

• Normal range for intraocular pressure: 10 - 21 mmHg

• Magnesium is required for both PTH secretion and its action on target tissues

• Drugs exacerbating psoriasis: Beta blockers, lithium, antimalarials (chloroquine and hydroxychloroquine), NSAIDs, ACE inhibitors

• Stevens-Johnson syndrome severe form of erythema multiforme associated with mucosal involvement and systemic symptoms

• Pityriasis versicolor => Topical antifungal e.g. terbinafine or selenium sulphide

• Patients with aortic stenosis are at risk of profound hypotension with ACE inhibitors

• Oral erythromycin for pertussis

Bacterial vaginosis

• Overgrowth of predominately anaerobic organisms such as Gardnerella vaginalis

• Leads to a consequent fall in lactic acid producing aerobic lactobacilli resulting in a raised vaginal pH

• Seen almost exclusively in sexually active women

• Features

  • vaginal discharge: 'fishy', offensive

  • asymptomatic in 50%

• Amsel's criteria for diagnosis of BV - 3 of the following 4 points should be present

  • thin, white homogenous discharge

  • clue cells on microscopy

  • vaginal pH > 4.5

  • positive whiff test (addition of potassium hydroxide results in fishy odour)

• Management

  • oral metronidazole for 5-7 days

  • 70-80% initial cure rate

  • relapse rate > 50% within 3 months

  • topical metronidazole or topical clindamycin as alternatives

Renal tubular acidosis

Childhood infections