Transient pseudohypoaldosteronism in infancy secondary to urinary tract infection

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    Abstract

    Aim: Hyponatraemia with hyperkalaemia in infancy is a typical presentation of congenital adrenal hyperplasia. In the presence of pyelonephritis, the same biochemical picture can occur with transient type 1 pseudohypoaldosteronism (PHA-1) also termed type 4 renal tubular acidosis. Recognition of PHA-1 enables appropriate management thus avoiding unnecessary investigations and treatment. To improve awareness of this condition, we present a case series to highlight the clinical and biochemical features of PHA-1. Methods: A retrospective chart review of patients diagnosed with transient PHA-1 at a tertiary children's hospital in Western Australia was conducted. Results: Five male infants (32days to 6 months) with transient PHA-1 were identified. Failure to thrive was the most common symptom with hyponatraemia on presentation. Two infants had antenatally diagnosed bilateral hydronephrosis and urinary tract infection (UTI) on admission. Two infants were treated for congenital adrenal hyperplasia and received hydrocortisone. All infants had UTI and required parenteral antibiotics. The condition was transient and hyponatraemia corrected by day 4 in all infants. There was no correlation between plasma sodium and aldosterone levels. The severity of PHA-1 was independent of the underlying renal anomaly. Four infants had hydronephrosis and vesicoureteric reflux. Surgical intervention was required in two infants. Conclusions: PHA-1 may be precipitated by UTI or urinary tract anomalies in early infancy. Urine analysis should be performed in infants with hyponatraemia. Diagnosis of PHA-1 facilitates appropriate renal investigations to reduce long-term morbidity.

    Original languageEnglish
    Pages (from-to)458-463
    JournalJournal of Paediatrics and Child Health
    Volume53
    Issue number5
    DOIs
    Publication statusPublished - May 2017

    Fingerprint

    Pseudohypoaldosteronism
    Urinary Tract Infections
    Hyponatremia
    Congenital Adrenal Hyperplasia
    Hydronephrosis
    Hypoaldosteronism
    Kidney
    Failure to Thrive
    Western Australia
    Hyperkalemia
    Pyelonephritis
    Aldosterone
    Urinary Tract
    Tertiary Care Centers
    Hydrocortisone
    Sodium
    Urine
    Anti-Bacterial Agents
    Morbidity

    Cite this

    @article{746c4f1ea1024a4690fdc839b0093eb5,
    title = "Transient pseudohypoaldosteronism in infancy secondary to urinary tract infection",
    abstract = "Aim: Hyponatraemia with hyperkalaemia in infancy is a typical presentation of congenital adrenal hyperplasia. In the presence of pyelonephritis, the same biochemical picture can occur with transient type 1 pseudohypoaldosteronism (PHA-1) also termed type 4 renal tubular acidosis. Recognition of PHA-1 enables appropriate management thus avoiding unnecessary investigations and treatment. To improve awareness of this condition, we present a case series to highlight the clinical and biochemical features of PHA-1. Methods: A retrospective chart review of patients diagnosed with transient PHA-1 at a tertiary children's hospital in Western Australia was conducted. Results: Five male infants (32days to 6 months) with transient PHA-1 were identified. Failure to thrive was the most common symptom with hyponatraemia on presentation. Two infants had antenatally diagnosed bilateral hydronephrosis and urinary tract infection (UTI) on admission. Two infants were treated for congenital adrenal hyperplasia and received hydrocortisone. All infants had UTI and required parenteral antibiotics. The condition was transient and hyponatraemia corrected by day 4 in all infants. There was no correlation between plasma sodium and aldosterone levels. The severity of PHA-1 was independent of the underlying renal anomaly. Four infants had hydronephrosis and vesicoureteric reflux. Surgical intervention was required in two infants. Conclusions: PHA-1 may be precipitated by UTI or urinary tract anomalies in early infancy. Urine analysis should be performed in infants with hyponatraemia. Diagnosis of PHA-1 facilitates appropriate renal investigations to reduce long-term morbidity.",
    keywords = "Hyponatraemia, Pseudohypoaldosteronism, Type 4 renal tubular acidosis, Urinary tract infection",
    author = "Abraham, {Mary B.} and Nicholas Larkins and Choong, {Catherine S.} and Shetty, {Vinutha B.}",
    year = "2017",
    month = "5",
    doi = "10.1111/jpc.13481",
    language = "English",
    volume = "53",
    pages = "458--463",
    journal = "Journal of Paediatric and Child Health",
    issn = "1034-4810",
    publisher = "John Wiley & Sons",
    number = "5",

    }

    TY - JOUR

    T1 - Transient pseudohypoaldosteronism in infancy secondary to urinary tract infection

    AU - Abraham, Mary B.

    AU - Larkins, Nicholas

    AU - Choong, Catherine S.

    AU - Shetty, Vinutha B.

    PY - 2017/5

    Y1 - 2017/5

    N2 - Aim: Hyponatraemia with hyperkalaemia in infancy is a typical presentation of congenital adrenal hyperplasia. In the presence of pyelonephritis, the same biochemical picture can occur with transient type 1 pseudohypoaldosteronism (PHA-1) also termed type 4 renal tubular acidosis. Recognition of PHA-1 enables appropriate management thus avoiding unnecessary investigations and treatment. To improve awareness of this condition, we present a case series to highlight the clinical and biochemical features of PHA-1. Methods: A retrospective chart review of patients diagnosed with transient PHA-1 at a tertiary children's hospital in Western Australia was conducted. Results: Five male infants (32days to 6 months) with transient PHA-1 were identified. Failure to thrive was the most common symptom with hyponatraemia on presentation. Two infants had antenatally diagnosed bilateral hydronephrosis and urinary tract infection (UTI) on admission. Two infants were treated for congenital adrenal hyperplasia and received hydrocortisone. All infants had UTI and required parenteral antibiotics. The condition was transient and hyponatraemia corrected by day 4 in all infants. There was no correlation between plasma sodium and aldosterone levels. The severity of PHA-1 was independent of the underlying renal anomaly. Four infants had hydronephrosis and vesicoureteric reflux. Surgical intervention was required in two infants. Conclusions: PHA-1 may be precipitated by UTI or urinary tract anomalies in early infancy. Urine analysis should be performed in infants with hyponatraemia. Diagnosis of PHA-1 facilitates appropriate renal investigations to reduce long-term morbidity.

    AB - Aim: Hyponatraemia with hyperkalaemia in infancy is a typical presentation of congenital adrenal hyperplasia. In the presence of pyelonephritis, the same biochemical picture can occur with transient type 1 pseudohypoaldosteronism (PHA-1) also termed type 4 renal tubular acidosis. Recognition of PHA-1 enables appropriate management thus avoiding unnecessary investigations and treatment. To improve awareness of this condition, we present a case series to highlight the clinical and biochemical features of PHA-1. Methods: A retrospective chart review of patients diagnosed with transient PHA-1 at a tertiary children's hospital in Western Australia was conducted. Results: Five male infants (32days to 6 months) with transient PHA-1 were identified. Failure to thrive was the most common symptom with hyponatraemia on presentation. Two infants had antenatally diagnosed bilateral hydronephrosis and urinary tract infection (UTI) on admission. Two infants were treated for congenital adrenal hyperplasia and received hydrocortisone. All infants had UTI and required parenteral antibiotics. The condition was transient and hyponatraemia corrected by day 4 in all infants. There was no correlation between plasma sodium and aldosterone levels. The severity of PHA-1 was independent of the underlying renal anomaly. Four infants had hydronephrosis and vesicoureteric reflux. Surgical intervention was required in two infants. Conclusions: PHA-1 may be precipitated by UTI or urinary tract anomalies in early infancy. Urine analysis should be performed in infants with hyponatraemia. Diagnosis of PHA-1 facilitates appropriate renal investigations to reduce long-term morbidity.

    KW - Hyponatraemia

    KW - Pseudohypoaldosteronism

    KW - Type 4 renal tubular acidosis

    KW - Urinary tract infection

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    U2 - 10.1111/jpc.13481

    DO - 10.1111/jpc.13481

    M3 - Article

    VL - 53

    SP - 458

    EP - 463

    JO - Journal of Paediatric and Child Health

    JF - Journal of Paediatric and Child Health

    SN - 1034-4810

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    ER -