BACKGROUND: Gynecologic anomalies, including uterine agenesis and ovarian dysgenesis, are some of the several differential diagnoses in adolescent females with primary amenorrhea and delayed puberty. Primary ovarian insufficiency is reported in the clinical practice of reproductive endocrinology can be determined by conducting sex hormone tests to evaluate the hypothalamic-pituitary-ovarian axis. However, confirmation of Mullerian agenesis by image modalities can be extremely challenging. Once the diagnosis is established, breakthrough bleeding usually occurs 2 to 3 years after hormonal replacement therapy.
METHODS: We report a case of a seventeen year old Taiwanese female, 46 XX karyotype, with ovarian dysgenesis and an initial tentative diagnosis of uterine agenesis who experienced a breakthrough bleeding after a month of hormonal replacement therapy.
CONCLUSIONS: The breakthrough bleeding after a month of estrogen therapy in primary ovarian insufficiency is uncommon, and the diagnosis of the absent uterus can have an extensive psychological impact on patients and their families.

BACKGROUND: Mooren\'s ulcer is a painful, inflammatory chronic keratitis that affects corneal periphery, progressing centripetally, ultimately ending in perforation. The first line of treatment includes systemic immunomodulators, with surgery being the last option. We present a case of bilateral Boston keratoprosthesis implantation for severe Mooren\'s ulcer that responded differently in each eye.
METHODS: A 32-year-old male with corneal opacification, anterior staphylomas, vision of hand movement, was started on systemic immunosuppression with cyclosporine. After two failed penetrating keratoplasties in each eye, high intraocular pressure despite diode cyclophotocoagulation, and cystic macular edema, we performed Boston keratoprosthesis type 1 in both eyes. The right eye responded initially well, with a best-corrected visual acuity of 20/80 and normal intraocular pressure. The left eye presented high intraocular pressure, which required cyclophotocoagulation, ultimately resulting in hypotony. Boston keratoprosthesis was performed but had peripheral corneal necrosis that progressed despite amniotic membrane transplantation and aggressive intensive treatment with medroxyprogesterone, autologous platelet-rich-in-growth-factors eye drops, and oral doxycycline. Thus, replacement of the semi-exposed Boston keratoprosthesis with tectonic penetrating keratoplasty was necessary. However, both eyes developed phthisis bulbi with final visual acuity of perception of light with poor localization.
CONCLUSIONS: Mainstay treatment of Mooren\'s ulcer is systemic immunomodulation. Surgical treatment must be considered only when risk of perforation, preferably with inflammation under control. Penetrating keratoplasty frequently fails, and Boston keratoprosthesis may be a viable option. However, postoperative complications, especially uncontrolled high intraocular pressure, corneal necrosis, and recurrence of Mooren\'s ulcer may jeopardize the outcomes and need to be addressed promptly with intensive topical and systemic treatment.

In the Himalayan region, there is a prevalence of unknown bites (not much data except media) including snakes with high range of mortality among victims because hilly terrain leads to delay in transportation and delayed initiation of proper treatment due to lack of developed tertiary care centers. These bites can present from local hypersensitivity reactions to neurological, cardiological, respiratory, hematological, musculoskeletal, and renal manifestations. We highlight two cases that presented with delayed and varied manifestations, recovered but delayed with dedicated supportive care. A 25-year-old female presented 3 days after bite from an unknown snake, possibly krait, developed cardiotoxicity, neuroxotoxicity, rhabdomyolysis, and hemolytic features and was managed with antivenom and anticholinesterase therapy along with medroxyprogesterone to facilitate recovery from bite-associated neurotoxicity. A 75-year-old male subjected to an unknown bite possibly a scorpion developed shock which was most likely cardiogenic in nature secondary to toxin and was managed initially using inotropic support. Prazosin was started, and he recovered completely though at a later time. Hence, apart from krait bite presenting as multisystem involvement, anticholinesterase and medroxyprogesterone acetate are vital for survival. Similarly, prazosin has a vital role in the recovery of scorpion bite-induced cardiotoxicity. Many such unknown venomous bites go unreported. Further case studies and case reports are necessary to help redefine the epidemiology of such bites in the Himalayan region that poses a diagnostic and therapeutic challenge.

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OBJECTIVE: To retrospectively examine the utility of high-dose oral medroxyprogesterone (MPA) for the treatment of inappropriate hypersexuality (IH) in elderly men with dementia.
METHODS: Ten men aged 65 years or older (median 79.5 years, range 65-93 years) were identified from all admissions at a 170-bed tertiary referral psychiatric hospital between December 2005 and January 2011. Admission records were used to identify subjects who received at least 100 mg daily of oral MPA. The primary outcome of successful treatment was chart documentation of a substantial decline in IH, such that subjects could return to preadmission residence. Data were collected to assess trends in dose, adverse effects, use of other symptom-modifying medications prior to MPA initiation, and successful return to preadmission placement. A trial serotoneric agent was used in 70% of subjects prior to MPA initiation. Sixty percent of subjects failed a trial of an antipsychotic, while 40% did not have response to the use of both a serotonergic agent and an antipsychotic before MPA was initiated. The average daily dose of MPA was 300 mg (range 100-400 mg/day). No adverse effects were documented from physician, nursing, or behavioral health rounding notes; however, adverse effects may not have been systematically assessed at the time of MPA administration. Seventy percent of subjects experienced favorable changes in target behaviors from MPA.
CONCLUSIONS: Few data exist on effective therapy options for treatment of IH. The minimum concentration of MPA needed to suppress IH in the male body is unknown. MPA was titrated upward, with the efficacy measure being a decrease in inappropriate behaviors. Use of MPA likely contributed to decreased IH; however, other factors involved in hospitalization could have contributed to improved behavior.
CONCLUSIONS: While requiring further study, high-dose (100-400 mg/day) oral MPA may represent an effective and well-tolerated treatment option for subjects displaying IH.

A 5 6-year-old woman, who underwent breast-conserving surgery and radiation (60 Gy) therapy in July, 1992, at the age of 40, was diagnosed with pT1aN0M0, pStage I. She was administered tamoxifen (TAM) as adjuvant therapy. However, she underwent microdochectomy for DCIS in her contralateral breast in June, 1998. TAM was given till August, 1999. In June, 2006, at the age of 54, 14 years after initial surgery, CT revealed extensive liver masses which were diagnosed as liver metastasis by liver biopsy. Receptor status was positive for ER and PgR, and negative for HER2. AC was started as a first-line chemotherapy ( 4 courses), but did not prove effective. She refused second-line chemotherapy, so letrozole was selected, and subsequently resulted in PR of the liver metastasis. However, 8 months later, with a liver metastasis relapse, exemestane followed by tamoxifen, medroxyprogesterone acetate, and high-dose toremifene were administered sequentially, resulting in long-time disease control. In conclusion, endocrine therapy might be an effective option even in a visceral crisis, if metastatic tumors have showed slow growth and there is positive hormone receptor status.

BACKGROUND: Adenosarcomas are rare tumors usually derived from the endometrium. About 50 cases of adenosarcomas of the ovary have been reported. The relationship between adenosarcoma and CA125 has not been described. The authors present a case of adenosarcoma with elevated CA125 because of the unusual presentation of this pathology and also because elevation of the CA125 antigen has not been reported in the literature.
METHODS: A 42-year-old woman presented for consultation for incidental right ovarian tumor and CA125 of 1100 U/mL. Histology revealed a homologous Müllerian adenosarcoma of the right ovary with sarcomatous overgrowth. CA125 decreased to 16 U/mL after surgery. Sixteen months post-surgery, the patient is disease free and with normal CA125.
CONCLUSIONS: Ovarian adenosarcomas are more aggressive than adenosarcomas of the uterus. Because of the embryological origin, ovarian adenosarcomas are able to produce CA125 antigen, especially in the presence of sarcomatous overgrowth. With these facts, CA125 antigen may be useful as a prognostic factor because it may represent an indirect marker of sarcomatous overgrowth.
CONCLUSIONS: CA125 may be useful for follow-up of ovarian adenosarcomas. Elevated CA125 antigen in adenosarcomas of the ovary may be indicative of sarcomatous overgrowth and poor prognosis.

OBJECTIVE: To observe the influence of percutaneous estradiol + micronized progesterone or medroxyprogesterone on endometrium and vaginal bleeding in Chinese post-menopausal women.
METHODS: A 3-year open randomized clinical study was designed. The estrogen and progestin were used continuous-combined together in a cyclic regimen. The percutaneous estradiol was regarded as estrogen. The microprogesterone (MP) and medroxyprogesterone (MPA) were served as progestin. Sixty healthy women (naturally menopausal for 1 to 5 years) were recruited and divided into four groups. The regimen as follows: (1) percutaneous estradiol gel (gel) containing 1.5 mg of 17 beta-estradiol (E2)/d plus micronized progesterone (MP) 100 mg/d in group 1 (G1, E2 1.5 + MP); (2) gel, 1.5 mg E2/d plus medroxyprogesterone acetate (MPA), 2 mg/d in group 2 (G2, E2 1.5 + MPA); (3) gel, 0.75 mg E2/d plus MP, 100 mg/d in group 3 (G3, E2 0.75 + MP); and (4) gel, 0.75 mg E2/d plus MPA, 2 mg/d in group 4 (G4, E2 0.75 + MPA). All were given for 25 d/month, and 5 days free. The transvaginal sonography was performed 0, 12, 24 and 36 months. The thickness of endometrium were measured. The endometrium biopsy by Pipelle tube was conducted at 24th or 36th months. The vaginal bleeding was recorded.
RESULTS: Fifty-nine patients (98.3%) finished 1 year and 56 patients (93.3%) for 2 years, 51 (85%) for 3 years. There was no significant changes of endometrium. The thickness of endometrium before treatment in four groups were 0.25 cm, 0.27 cm, 0.22 cm, 0.28 cm and after treatment 0.37 cm, 0.30 cm, 0.326 7 cm and 0.3 cm respectively. No significant differences was found in four groups (P > 0. 05). Fifty -three patients had the Pipelle biopsy. The pathological result showed: 16 cases (25.8%) proliferated, 1 case (1.6%) secretary; 11 cases (17.7%) atrophy; 23 cases (37.1%) mixed cell; tendency to hyperplasia in 2 cases (3.2%). During the treatment, the vaginal irregular bleeding rate was highest in G2 and lowest in G3.
CONCLUSIONS: Daily estrogen containing 0.75 mg and 1.5 mg E2 combined with 100 mg progesterone and 2 mg medroxyprogesterone do not increase the thickness of endometrium significantly. The vaginal bleeding rate was low when combined with 100 mg micronized progesterone. Addition of natural progesterone will low down the bleeding rate and improve the HRT compliance.

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