In conclusion, the up-regulated expression of TNF-α, IL-6 and IFN-γ in the luminal fluid of ES, in the absence of any aberrant systemic pro-inflammatory response, has provided direct evidence for an increased local inflammatory response at the ES in these patients with unilateral MD, and this phenomenon was validated by demonstrating a positive expression of TNF-α, IL-6 and IFN-γ in the epithelial cells lining the sac in human ES specimens from patients with MD using immunohistochemical and immunofluorescent analysis. Here, TNF is linked to Menkes disease.