新生儿，生后8天起，反应差，进食少，伴呕 吐、体重下降。査体:两眼凹陷，皮肤弹性 差，皮肤黏膜色窣增深.女性外阴。血钠 120mmol/L，血钾6. 5mmol/L，染色体核型为46，XY,该患儿最可能的诊断是

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