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