What is the evidence for the use of adrenaline in the treatment of neonatal hypotension?

AIM: The authors of this review present the current evidence of the physiology, indications and use of adrenaline in neonates, with particular focus on the treatment of hypotension. METHOD: A structured literature search was performed across selected electronic databases, reference lists and related articles. Abstracts arising from the search were screened for relevance according to predefined inclusion criteria. Full articles for the selected abstracts were obtained and then reviewed. Articles were analysed through a two stage process until agreement was reached between the research team on the studies for inclusion. RESULTS: We identified 187 animal and human studies (published between 1924-2011) using various methodologies but with two main themes: the physiology of endogenous adrenaline in neonates and the therapeutic uses of this hormone in neonatal medicine. The physiological studies measured catecholamine levels in cord blood, neonatal urine and blood, some in response to interventions such as suctioning, skin massage or morphine infusion. Within the therapeutic studies there was only one randomised controlled trial (RCT): a comparison of dopamine versus adrenaline involving 60 infants of < 32 weeks gestational age. CONCLUSION: Despite the number of studies identified, we found few adequately-controlled studies on the therapeutic use of adrenaline in neonates. Future research should focus on RCTs comparing adrenaline to other commonly used inotropes.

Difficulties in diagnosis and treatment of 5alpha-reductase type 2 deficiency in a newborn with 46,XY DSD.

BACKGROUND/AIMS: Steroid 5alpha-reductase deficiency (MIM*607306) caused by mutations in the SRD5A2 gene is characterized by a predominantly female phenotype at birth and significant virilization at puberty. The undermasculinization at birth results from low dihydrotestosterone (DHT) levels during fetal development as the type 2 isoenzyme activity is reduced. In puberty, when the type 1 isoenzyme activity increases, significant virilization occurs. Most 46,XY individuals with 5alpha-reductase 2 deficiency develop a male gender identity. CASE REPORT AND RESULTS: We present a case with a predominantly female phenotype and ambiguous external genitalia but a normal 46,XY karyotype. Plasma steroid analysis after beta-hCG stimulation at 8 days of age revealed a steroid profile estimated as normal with a testosterone (T)/DHT ratio of 9.5 initially misleading to the exclusion of 5alpha-reductase deficiency. However, mutation analysis of the SRD5A2 gene revealed a homozygote point mutation (Leu55Gln) confirming the diagnosis of 5alpha-reductase deficiency. A male phenotype was successfully achieved by hormone treatment with T and DHT after diagnosing 5alpha-reductase deficiency and a masculinization operation. As a side effect skeletal age accelerated temporarily. CONCLUSION: In individuals with predominantly female phenotype and suspected 5alpha-reductase deficiency, a T/DHT ratio during the neonatal period >8.5 might point to 5alpha-reductase deficiency. After confirmation of the diagnosis by molecular analysis of the SRD5A2 gene, a satisfactory change to a male phenotype can be achieved by hormone treatment preceding surgery.