1/188. Ultrapotent topical corticosteroid treatment of childhood genital lichen sclerosus. OBJECTIVE: To observe the clinical effects of short-term application of ultrapotent topical corticosteroid on symptomatic genital lesions of lichen sclerosus in pediatric patients. DESIGN: Case series of 10 prepubertal girls with genital lichen sclerosus. Ultrapotent topical corticosteroids were applied twice daily for 6 to 8 weeks and patients were reexamined at completion of treatment. Long-term follow-up over 6 months to 3 years. SETTING: Pediatric dermatology clinic (referral center). patients: Ten prepubertal girls with typical clinical features of genital and/or perianal lichen sclerosus. INTERVENTION: Topical ultrapotent corticosteroid ointment was applied sparingly to affected areas for 6 to 8 weeks. MAIN OUTCOME MEASURE: Improvement of erythema, whitening erosions, and atrophy. Subjective improvement of symptoms. RESULTS: All patients showed partial or total subsistence of signs and symptoms of lichen sclerosus. Frequency and severity of recurrences varied, but patients responded within a few days to reapplication of ultrapotent topical corticosteroid. No significant adverse effects were noted after the initial 6- to 8-week course of therapy or during the 6-month to 3-year follow-up period. CONCLUSION: A 6- to 8-week course of ultrapotent topical corticosteroid is a safe and effective treatment for genital lichen sclerosus in pediatric patients. ( info) |
2/188. Interferon gamma (IFN-gamma) deficiency in generalized Epstein-Barr virus infection with interstitial lymphoid and granulomatous pneumonia, focal cerebral lesions, and genital ulcers: remission following IFN-gamma substitution therapy. A 26-year-old previously healthy woman developed granulomatous pneumonitis, encephalitis, and genital ulceration during primary Epstein-Barr virus (EBV) infection. EBV dna was demonstrated by polymerase chain reaction analysis of serum, lung tissue, and genital ulcer specimens. serology verified primary EBV infection. The patient lacked lymphocytes cytotoxic to autologous EBV-transformed B lymphocytes. No spontaneous or in vitro EBV-induced interferon gamma (IFN-gamma) production was evident in peripheral blood. The cells had normal IFN-gamma production when stimulated with staphylococcus aureus exotoxin A. In the bone marrow and peripheral blood, the number of large granular CD56 lymphocytes (natural killer cells) increased 39%-55%, but no CD4 or CD8 cell lymphocytosis was initially found. A partial clinical response was achieved with treatment with acyclovir, corticosteroids, and intravenous gamma-globulin. Because of persistent granulomatous central nervous system and lung involvement, subcutaneous IFN-gamma therapy was started but was discontinued after 3 months because of development of fever, pancytopenia, and hepatitis. This therapy initiated a complete clinical recovery, which occurred parallel to development of EBV-specific cytotoxic CD8 T lymphocytes and normalization of natural killer cell lymphocytosis. These findings provide evidence for an EBV-induced lymphoproliferative disorder due to a T lymphocyte dysfunction associated with a selective lack of IFN-gamma synthesis. ( info) |
| factor v deficiency is a rare hereditary disorder. We report a patient with factor v deficiency who presented with menorrhagia and pelvic haematoma. The Haematology Department at the Royal Brisbane Hospital performed the definitive factor assays leading to the diagnosis. The challenges of her management were obtaining adequate supplies of factor V and her socioeconomic circumstances. The main future challenge will be the supervision of her pregnancies. ( info) |
4/188. A bad eye and a sore lip. A 48-year-old woman developed painful visual loss in the left eye, meningismus, and painful oral ulcers. magnetic resonance imaging of the brain with gadolinium demonstrated enhancement of the left optic nerve. Lumbar puncture showed a lymphocytic pleocytosis, and a biopsy specimen of one of the oral ulcerations was consistent with Behcet's disease. epidemiologic factors and diagnostic criteria for Behcet's disease are discussed. ( info) |
| We describe a woman with unusual complications of infection with coccidioides immitis--infection of the genital tract and adrenal insufficiency. The patient also had intestinal coccidioidomycosis (cocci) in conjunction with presumed pulmonary, and asymptomatic central nervous system cocci. To our knowledge, concurrent FGC, intestinal and adrenal cocci have not been reported previously. A medline review from 1966-1997 revealed only 1 case of adrenal insufficiency due to cocci. FGC is rare; we identified 12 reported cases since 1929. No combination of investigations or clinical features is sensitive enough to predict FGC. diagnosis is usually made after microscopy of surgical specimens. FGC presents either as tubo-ovarian disease or endometritis. Treatment generally involves surgical excision and antifungal agents. We hypothesize that an initial trial of antifungals may obviate the need for surgery. ( info) |
| schistosoma haemtobium infection in travelers from endemic areas is usually asymptomatic, or presents with hematuria. Uncommon manifestations include neurological syndromes, genital dysaesthesias and watery or blood stained semen. This organism also causes disease within all structures of the female genital tract because of communications between pelvic venous complexes, and can occur long after return home. schistosomiasis may not be suspected, resulting in delays in diagnosis and treatment. We present two cases which illustrate the diverse nature of this condition. ( info) |
7/188. A systematic history for the patient with chronic pelvic pain. Chronic pelvic pain is a source of frustration to both the physician and the patient. physicians have been ill equipped by their training to confront the multifaceted nature of the complaints of patients with chronic pelvic pain. patients have experienced a repetitive dismissal of their complaints by physicians too busy in their practices to address their problems comprehensively. The approach to the patient with chronic pelvic pain must take into account six major sources of the origin of this pain: 1) gynecological, 2) psychological, 3) myofascial, 4) musculoskeletal, 5) urological, and 6) gastrointestinal. Only by addressing and evaluating each of these components by a very careful history and physical examination and by approaching the patient in a comprehensive manner can the source of the pain be determined and appropriate therapy be administered. This article was developed to provide the clinician with a set of tools and a methodology by which the patient with this complaint can be approached. ( info) |
8/188. Crohn's disease and tubal infertility: the effect of adhesion formation. The purpose of this paper was to assess the management of tubal disease in women with Crohn's disease. We are presenting a case series describing four patients with Crohn's disease who were treated at a tertiary infertility clinic within an university hospital. Fertility investigations and appropriate therapy plans in these patients with Crohn's disease who also had tubal infertility are outlined. The clinical outcome in all four cases demonstrated interference with the management and surgical therapy of the tubal infertility by extensive adhesion formation. We conclude that adhesion formation complicates the investigation and surgical therapy of patients with Crohn's disease. ( info) |
9/188. Mullerian agenesis: etiology, diagnosis, and management. Mullerian agenesis, a congenital malformation of the genital tract is the second most common cause of primary amenorrhea. Its etiology is poorly understood but it may be associated with renal, skeletal, and other abnormalities. The diagnosis is often made either radiologically or laparoscopically in patients in whom hormonal and karyotypic investigations for primary amenorrhea are normal. Two-dimensional ultrasound is not a reliable method of diagnosis, as exemplified by the two cases presented in this review; however, three-dimensional ultrasound may be a more sensitive diagnostic tool. The management is varied, but we conclude that the treatment of choice should be a nonsurgical approach aimed at creating a neovagina. Because of the implications for reproduction, these patients require psychological support, which should be offered as part of therapy. TARGET AUDIENCE: Gynecologists and family physicians learning OBJECTIVES: After completion of this article, the reader will be able to describe the pathophysiology and clinical presentation of mulerian agenesis and to list the other abnormalities associated with this condition and to outline potential treatment options for a patient with mullerian agenesis. ( info) |
10/188. Litigation of laparoscopic major vessel injuries in canada. This study is a review of conditions and circumstances associated with 15 injuries to great vessels during laparoscopic surgery. Thirteen cases were litigated in canada and two occurred in the author's operating room. Body habitus may have been an underlying factor in the injuries. Most injuries were entry related and independent on complexity of surgery. One was caused by the Veress needle and one by a secondary trocar, and three occurred during dissection of adhesions. Ten were primary trocar injuries, 9 after pneumoperitoneum and 1 at direct trocar insertion. Of these, five were caused by reusable and five by disposable trocars with so-called safety shields. In 6 of 15 cases recognition of the injury was delayed; 5 in the recovery room. Eleven women had uncomplicated recovery. Of 13 litigated cases, 8 (62%) resulted in settlement. ( info) |