Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is an unknown congenital etiology disorder characterized by agenesia or hypoplasia of the Müller ductal system, including the upper vagina, uterus and fallopian tubes. The occurrence of an associated ovarian tumor is rare, with fewer than 20 cases reported to date according to the literature. We report the case of a 14-year-old girl, virgin, who had not yet seen her menarche, complaining of an abdomino-pelvic mass associated with pain. The ultrasound performed revealed a large left ovarian tumor and an absence of uterus. The indication of a laparotomy confirmed the ovarian mass and a complete absence of uterus associated with vaginal hypoplasia. The contralateral ovary was present, and of normal appearance. The pathological examination was in favor of a mature benign multi-tissular teratoma. This is the first case described in our service. The mode of transmission of this entity appears to be autosomal dominant with low penetrance and variable expressivity, suggesting that the incidence of this syndrome is likely underestimated. With the development of techniques of medical assistance to procreation, maternity remains possible, particularly through gestational surrogacy.

Prognostic factors of early sequelae and fatal outcome of Japanese encephalitis.

A clinical case control study to identify prognostic factors present at hospital admission associated with early sequelae and fatal outcome of acute Japanese encephalitis (JE) was carried out in Gusi county, Henan Province, central China from June to September 1991. A total of 70 patients with laboratory-confirmed acute JE were studied, of whom 3 cases died and 33 cases had neurological or psychiatric sequelae at the end of three months follow-up. The results showed that acute JE at younger age, with higher body temperature, high white cell count in CSF, and deep coma present at hospital admission were markers for unfavorable outcomes (sequelae or fatal). A history of the vaccination was not correlated with the early sequelae and fatal outcome of the disease. The paper suggests that early diagnosis and treatment and universal JE vaccination for all susceptible populations are keys for decreasing incidence of sequelae and fatal outcome of acute JE.

Socio-economic status and micro-environmental factors in relation to the risk of Japanese encephalitis: a case-control study.

In a population-based case-control study in Southern Henan Province, central China, children suffering from Japanese encephalitis (JE) were compared with neighborhood controls matched by age and sex in terms of several social and environmental variables. Factors found by crude analysis to be associated with an increased risk of JE included lower family income, lower parental education, living in houses near the periphery of villages and poor quality of houses. When adjustment was made for confounding variables, only the association of house location within village remained of borderline significance (OR = 0.51, 95% CI = 0.15 approximately 1.03). It is suggested that the beneficial effect of higher family income and parental education was partly due to the fact that those parents might be more conscious about having their children vaccinated in the situation where there was a shortage of JE vaccine in the study area.