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