Advertisement

Hypopituitarism Related Cholestatic Jaundice: Important to Recognise, Rewarding to Treat but Difficult to Diagnose!

  • Rishi Bolia
  • Anshu SrivastavaEmail author
Editorial Commentary
  • 9 Downloads

Congenital Hypopituitarism (CH) is defined as a deficiency of ≥2 pituitary hormones and can vary in severity and age at presentation. It has an incidence of 1–11/100,000 in the general pediatric population and may present in 1/3rd of cases as neonatal cholestasis (NC) [1, 2]. CH accounted for 1% of infants with NC in a pediatric hepatology centre in UK [1].

In this issue Altay et al. discuss the clinical features, laboratory parameters and outcomes of 6 children with CH who presented with NC at a median age of 2.5 mo. While 3 patients were diagnosed in their initial presentation, the remaining were diagnosed later in life [3].

Establishing a diagnosis of CH is challenging. Some of the clinical features like poor feeding, hypoglycemia, jaundice, apnea, jitteriness and poor weight gain are non-specific and one needs a high index of clinical suspicion to diagnose this condition. However, presence of visual problems, nystagmus, midline facial dysmorphism, and undescended testes/ micropenis...

Notes

Compliance with Ethical Standards

Conflict of Interest

None.

References

  1. 1.
    Grammatikopoulos T, Deheragoda M, Strautnieks S, et al. Reduced hepatocellular expression of canalicular transport proteins in infants with neonatal cholestasis and congenital hypopituitarism. J Pediatr. 2018;200:181–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Ellaway CJ, Silinik M, Cowell CT, et al. Cholestatic jaundice and congenital hypopituitarism. J Paediatr Child Health. 1995;31:51–3.CrossRefPubMedGoogle Scholar
  3. 3.
    Altay D, Eren E, Ozkan TB, Ozgur T, Tarım O. Liver involvement in congenital hypopituitarism. Indian J Pediatr. 2019.  https://doi.org/10.1007/s12098-018-2833-7.
  4. 4.
    Spray CH, Mckiernan P, Waldron KE, Shaw N, Kirk J, Kelly DA. Investigation and outcome of neonatal hepatitis in infants with hypopituitarism. Acta Paediatr. 2000;89:951–4.CrossRefPubMedGoogle Scholar
  5. 5.
    Parks JS. Congenital hypopituitarism. Clin Perinatol. 2018;45:75–91.CrossRefPubMedGoogle Scholar
  6. 6.
    Alatzoglou KS, Dattani MT. Genetic forms of hypopituitarism and their manifestation in the neonatal period. Early Hum Dev. 2009;85:705–12.CrossRefPubMedGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2019

Authors and Affiliations

  1. 1.Division of Pediatric Gastroenterology Department of GastroenterologyPGIMERChandigarhIndia
  2. 2.Department of Pediatric GastroenterologySGPGIMSLucknowIndia

Personalised recommendations