Sepsis and septic shock represent important causes of morbidity and mortality in children, and adrenal dysfunction may play a role in the cardiovascular and immunological response. According to existing reports, the incidence of adrenal dysfunction in critically ill children varies significantly between 4 and 52% of patients. This article reviews the concept of adrenal insufficiency and the role it may play in a pediatric septic shock. Also discussed are the diagnosis, prognosis, and treatment of adrenal insufficiency in septic shock in adults and children. Finally, the latest recommendations about steroid use in pediatric septic shock are summarized.
One of the major causes of congenital hypophosphatemic rickets is the X-linked hypophosphatemic rickets (XHR), due to a defect on PHEX gene. The XHR increases the renal elimination of phosphate, that condition leads a defective mineralization of bones and also affects the growth in children. Clinical diagnosis should be suspected in children with signs of rickets and hypophosphatemia with normal calcium levels. We describe clinical characteristics and genetic results of four patients diagnosed and treated in our Nephrology Section. All patients have a "de novo" XHR as none familiars are affected. Early diagnosis should be suspected before the bone deformities have been submitted and the growth would have been impaired.
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