Recurrent episodes of hypoglycemia were reported to be a presenting feature in a suspected case of 3βHSD2D but the genotype was not performed to confirm the diagnosis [36]. Another patient presented during second day of life with hypoglycemia, later on, the molecular genetics confirmed 3βHSD2D [31]. In case of SW phenotype, 3βHSD2D is usually diagnosed within the first few weeks of life. In case of non-SW phenotype, patients may be diagnosed at any time before puberty [37]. However, the diagnosis has rarely been further delayed and patients can present with gender role related concerns during adulthood [38]. Overall, the patients tend to be diagnosed at a younger age in 46,XY children due to a higher rate of genital ambiguity compared to females [34, 39]. Also, there seems to be an underrepresentation of 46,XX patients, which might be explained by lack of diagnosis in milder form of 3βHSD2D in females.
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The introduction of glucocorticoid replacement and increased awareness have increased the survival of classic 21OHD [5], and this is most probably also the case in 3βHSD2D. In population studies, patients with CAH had generally an increased mortality rate (hazard ratio 3–5) and died 6.5–18 years earlier, compared to controls [122, 135]. Adrenal crisis was the main cause of death [135], iterating the importance of stress dosing during acute illness [40, 41]. System Bet – comprises more than one accumulator bet of the same perdi meu código size. Glucocorticoid and mineralocorticoid replacement is similar to other forms of CAH. In SW crisis, treatment includes prompt rehydration, correction of hypoglycemia, and parenteral hydrocortisone (intravenous or intramuscular) [15, 40, 41]. For follow-up children are treated with hydrocortisone in a dose of 10–15 mg/m 2 /day. Long-acting glucocorticoids such as dexamethasone and prednisolone, known to suppress growth in children, can be used during adulthood [7, 33, 67]. Jogos dinheiro infinito.
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