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The blind kidney: disorders affecting kidneys and eyes

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Abstract

There are many disorders that can affect both the kidneys and the eyes. Awareness of the ocular manifestations of kidney disorders is important as it can guide the diagnosis and facilitate the choice of a specific treatment. Conversely, ophthalmologists need to be aware of potential renal manifestations in disorders presenting initially with visual failure. We review disorders affecting both of these organ systems, based upon cases from our clinical practice to highlight the importance of interdisciplinary collaboration.

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Acknowledgements

Case 4 scenario is based upon a case of Professor Christopher Lyons, paediatric ophthalmologist at British Columbia Childrens Hospital, Burnaby, B.C., Canada, who kindly also provided illustration Fig 2f.

Author information

Authors and Affiliations

  1. Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Foundation Trust London UK and UCL Institute of Child Health, London, WC1N 3JH, UK

    Isabelle Russell-Eggitt

  2. Department of Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust London UK and UCL Institute of Child Health, London, WC1N 3JH, UK

    Detlef Bockenhauer

Authors
  1. Isabelle Russell-Eggitt
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Corresponding author

Correspondence to Isabelle Russell-Eggitt.

Additional information

Answers:

Question 1: d) all of the above, as an eye examination can help establish specific diagnosis such as renal-coloboma syndrome, reveal subclinical uveitis associated with tubulointerstitial nephritis and assess chronicity and target organ damage in hypertension.

Question 2: c) urine low-molecular weight protein. An elevated level in a neonate with glaucoma is consistent with a diagnosis of Lowe syndrome

Question 3: e) all of the above. a, b and c are all typical findings of Alport syndrome, but may not be seen in children with Alport syndrome as these findings typical develop in young adults.

Question 4: d) b and c. The most common cause of renal Fanconi syndrome in children is cystinosis, and the finding of corneal crystals is usually the quickest way to establish the diagnosis. Early treatment with cysteamine is important to defer long-term complications, such as end-stage kidney disease. The corneal crystals can be dissolved with cysteamine eye drops.

Multiple Choice questions (answers are provided following the reference list)

Multiple Choice questions (answers are provided following the reference list)

  1. 1)

    In a child presenting with kidney disease, an eye examination can help:

    1. a)

      establish a specific diagnosis

    2. b)

      reveal subclinical complications

    3. c)

      assess chronicity of the disease

    4. d)

      all of the above

  2. 2)

    In a neonate presenting with congenital glaucoma, what is the most sensitive test to assess potential kidney involvement?

    1. a)

      plasma creatinine

    2. b)

      urine amino acids

    3. c)

      urine low-molecular weight protein, such as retinol-binding protein

    4. d)

      plasma biochemistries

  3. 3)

    In a child with Alport syndrome, the eye examination can reveal the following:

    1. a)

      anterior lenticonus

    2. b)

      fleck retinopathy

    3. c)

      corneal clouding

    4. d)

      normal findings

    5. e)

      all of the above

  4. 4)

    In a child presenting with renal Fanconi syndrome, eye examination is important, because:

    1. a)

      it can assess chronicity of the disease

    2. b)

      establish a treatable diagnosis

    3. c)

      identify a treatable eye condition

    4. d)

      b and c

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Russell-Eggitt, I., Bockenhauer, D. The blind kidney: disorders affecting kidneys and eyes. Pediatr Nephrol 28, 2255–2265 (2013). https://doi.org/10.1007/s00467-012-2404-5

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  • DOI: https://doi.org/10.1007/s00467-012-2404-5

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