2018
DOI: 10.4274/jcrpe.2017.s002
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Latest Insights on the Etiology and Management of Primary Adrenal Insufficiency in Children

Abstract: Primary adrenal insufficiency (PAI) is a heterogeneous group of disorders characterized by an impaired production of cortisol and other steroid hormones by the adrenal cortex. Most of the causes of PAI in childhood are inherited and monogenic in origin and are associated with significant morbidity and mortality whenever the diagnosis and treatment is delayed. Therefore, early and accurate diagnosis would allow appropriate management for the patients and genetic counselling for the family. Congenital adrenal hy… Show more

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Cited by 21 publications
(23 citation statements)
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“…Presence of bilious vomiting, severe abdominal pain, abdominal distension, failure to pass the meconium may help to differentiate from FPIES and abdominal X‐ray, upper gastrointestinal series and abdominal ultrasound may be necessary to establish proper care (Table ) . Intracranial mass and acute adrenal insufficiency (eg congenital adrenal hyperplasia (CAH)), present with vomiting, dehydration, hyponatremia and hyperkalaemia and can mimic acute FPIES …”
Section: Fpies On a Spectrum Of Gi Food Allergic Disorder‐differentiamentioning
confidence: 99%
“…Presence of bilious vomiting, severe abdominal pain, abdominal distension, failure to pass the meconium may help to differentiate from FPIES and abdominal X‐ray, upper gastrointestinal series and abdominal ultrasound may be necessary to establish proper care (Table ) . Intracranial mass and acute adrenal insufficiency (eg congenital adrenal hyperplasia (CAH)), present with vomiting, dehydration, hyponatremia and hyperkalaemia and can mimic acute FPIES …”
Section: Fpies On a Spectrum Of Gi Food Allergic Disorder‐differentiamentioning
confidence: 99%
“…Primary adrenal insufficiency is a rare clinical condition characterized by the insufficient production of the steroid hormones from the adrenal cortex, such as cortisol, aldosterone, and adrenal sex steroids, affecting both genders equally in childhood [22][23][24]. The main treatment of PAI is replacing glucocorticoids and mineralocorticoids, principally by hydrocortisone and fludrocortisone [21,22]. The recommended dosage for hydrocortisone ranges between 7.5-15 mg/m 2 /day, according to the underlying cause [25][26][27].…”
Section: Discussionmentioning
confidence: 99%
“…For many GN diseases, cellular immunity plays a decisive role, and humoral immunity is also clearly expressed during the pathogenic process of GN diseases ( 14 ). In addition, the disorder of blood coagulation mechanism and the change of blood rheology have an effect on the occurrence and progression of glomerular diseases ( 15 ). In clinic, the pathogenesis and treatment of GN are known, the drugs used to treat GN are immunosuppressive agents and glucocorticoid, but the efficacy of many glomerular diseases is still unsatisfactory ( 16 , 17 ).…”
Section: Discussionmentioning
confidence: 99%