Feminizing testicular syndrome with multiple hamartomas and bilateral paratesticular leiomyomas

INTRODUCTION: Complete androgen insensitivity syndrome or testicular feminization syndrome (TF) is the most common form of male pseudohermaphrodism, caused by a failure of androgen receptor binding. Patient with male genotype 46 XY, has a female morphotype with well developed external sexual organs. EXEGESIS: - We report the case of a 29 year-old girl with a TF syndrome discovered during the exploration of a primary amenorrhoea. Bilateral orchidectomy was performed. The testis were immature; they showed bilateral leiomyoma of the tunica albuginea and multiple hamartomas on the right side. CONCLUSION: Benign tumors are developped in 80% of cases of TF and they are generally hamartomatous nodules of testis. Association of paratesticular leiomyoma to synchronous hamartoma has never been described, its histogenesis is discussed.     

Unusual tumor of the stomach

Stomach melanomas are exceptional and often secondary to cutaneous tumors. Their symptomatology is not specific. We report the case of a 66-year-old woman, hospitalized with a three month history of epigastric pain and weight loss. Gastroscopy had revealed a polyp of the gastric mucosa. Histopathologic examination confirmed the diagnosis of a gastric infiltration by a melanoma. At exploration, there were lung, liver and bone metastasis and no cutaneous melanoma. The diagnosis of polymetastasis from a melanoma without primitive was kept. The patient died three months later.     

Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre

Central illustration. Time of occurrence and outcome of cardiovascular disorders in patients (pts) with congenital portosystemic shunt (CPSS). Patients with normal anatomy and those with congenital heart disease (CHD) were distinguished. Heart failure (HF) was the main symptom in both the prenatal and neonatal periods, whereas portopulmonary hypertension (PPH) and hepatopulmonary syndrome (HPS) represented the major concerns beyond the first months of life. CV: cardiovascular; NAS: no additional symptoms; PH: pulmonary hypertension; RD: respiratory distress. ^aFetal diagnosis of CPSS. ^bNeonatal diagnosis of CPSS. ^cDiagnosis of CPSS > 1 month of age

     

Tumeur à cellules de la granulosa juvénile de l’ovaire: à propos de deux cas

Juvenile granulosa cell tumor of the ovary is a rare neoplasm that makes up less than 5% of ovarian tumors in childhood and adolescence. The majority of they tumors are diagnosed in stage I (FIGO) with a favorable prognosis. The aim of this work was to describe the pathological, clinical and therapeutic features of this rare tumor of ovary. The authors describe a juvenile cell tumor expressed by an abdominal and genital syndrome occurring in 30 and 35-year-old two young females. The tumor was strictly localized in the ovary in one case; the second patient presented a bilateral ovarian tumor and peritoneal metastasis at the diagnosis. Clinical follow-up of the latter patient showed a recurrence with liver metastasis in spite of a radical surgical treatment followed by chemotherapy. The first patient has a favorable evolution with a follow-up of two years. Various prognostic factors of the juvenile granulosa cell tumor of the ovary are currently being evaluated. Surgery is the treatment for local disease. Patients with locally advanced, recurrent or metastatic tumors require chemotherapy, although the optimal regimen remains to be determined.     

Mammography screening of breast cancer in Tunisia. Results of first experience]

This article reports the results of a mammography screening program of breast cancer, realized in the department of Radiology, Charles Nicolle hospital. A free screening mammography with two incidences was offered to women aged from 40 to 70 years old. 2200 mammographies were realized from May 1995 till July 1997. Women having a positive test benefited of a diagnostic explorations in the same unity. The positive test rate was 24%. Predictive positive value was 31%. This program allowed to detect 10 subclinical cancers, corresponding to a rate of detection of 4.5 cancers for 1000 women. This program is a first experience which demonstrated the feasibility of the mammography screening to wide scale and allowed the medical and paramedical team to acquire an experience.     

Blepharitis due to Demodex: myth or reality?]

PURPOSE: Demodex folliculorum has been incriminated in the development of blepharitis although much controversy persists. Certain authors suggest that Demodex is a direct pathogen in chronic palpebral conditions while others consider the saprophyte to be innocuous to skin. METHODS: We conducted a prospective study of eyelashes in 100 persons, searching for Demodex folliculorum and chronic blepharitis. Microscopy in immersion oil after storage in a moist chamber was performed. RESULTS: The incidence of Demodex folliculorum was very high in patients with blepharitis compared with normal controls. Incidence increased with age. Harmless cuffs around the base of the eyelashes was found in 4% with Demodex irradior. CONCLUSION: Demodex should be considered as the cause of chronic blepharitis. Anti-Demodex treatment is indicated when the parasite is found.     

Topical Ivermectin 10 mg/g and Oral Doxycycline 40 mg Modified-Release: Current Evidence on the Complementary Use of Anti-Inflammatory Rosacea Treatments

Rosacea is a common, chronic inflammatory skin disease that can present with a variety of signs and symptoms. The potentially simultaneous occurrence of different signs and symptoms is due to different underlying inflammatory pathways, emphasizing the need for complementary treatment approaches. Topical ivermectin cream (10 mg/g) and systemic, oral anti-inflammatory doxycycline (40 mg modified-release) are both approved for the treatment of papulopustular rosacea (PPR). Whether or not a combined therapeutic approach may be more beneficial than monotherapy for patients with PPR remains to be tested. Here, we summarize underlying inflammatory pathways implicated in rosacea and clarify the impact of these two agents on selective pathways during inflammation, due to specific characteristics of their individual mechanisms of action (MoA). Based on the complementary MoA of doxycycline modified-release and ivermectin, a scientific rationale for a combined therapy targeting inflammatory lesions in rosacea is given. We propose that topical ivermectin cream is a promising new candidate as first-line treatment to target the inflammatory lesions of rosacea, which can be used in combination with systemic doxycycline modified-release to provide an optimal treatment approach considering all inflammatory pathways involved in PPR. Funding Galderma.