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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.
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Human genetics

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Synopsis

Explain inheritance, penetrance, genomic variation and testing limits; calculate family probabilities; and use genomic results only when phenotype, test scope and classification support action.

  • Separate genotype, phenotype, penetrance and variable expression: inheriting a pathogenic variant may not predict whether, when or how severely disease appears.
  • Autosomal dominant, autosomal recessive, X-linked and mitochondrial patterns generate probabilities per pregnancy, conditional on the parental genotypes and variant mechanism.
  • A negative result excludes only variants the chosen assay could detect in genes and regions relevant to the tested indication.

Reasoning priorities

01
Three-generation pedigree

Represent affected status, ancestry, reproductive history and biological relationships across a family.

Record ages and uncertainty, verify diagnoses where possible and avoid inferring inheritance from a small or incomplete family alone.

Worked reasoning

Worked exampleCalculate autosomal-recessive recurrence risk

Inputs: both parents are confirmed heterozygous carriers of the same pathogenic recessive variant; their first child is affected; calculate outcomes for the next pregnancy.

  1. Represent each parent as A/a, where a is the disease-associated allele; each produces A and a gametes with probability one half.
  2. Combine independent gametes: AA probability one quarter, A/a plus a/A total probability one half, and a/a probability one quarter.
  3. State the next-pregnancy outcomes: 25% affected, 50% unaffected carrier and 25% unaffected non-carrier, assuming the model and parentage are correct.
  4. Explain that the affected first child does not change the next conception's probabilities because each meiosis is a new event.
  5. Discuss reproductive options and test limitations through genetics services without treating probability as a prediction of the individual outcome.
  6. Verify by enumerating four equally likely gamete pairs—A/A, A/a, a/A and a/a—which reproduces the one-two-one distribution.
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Sources and review status5 sources · checked 7 Sept 2026 · clinical review pending
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Apply principles in context and verify current guidance when a decision affects care. Source check completed 7 Sept 2026; clinical approval remains outstanding.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom