46,XX male syndrome with mullerianosis of the urinary bladder: A new clinical entity.

Müllerianosis of the urinary bladder is a rare entity characterized by the presence of an admixture of at least two types of müllerian tissue in the muscularis propria of the bladder. We report the case of a 43-year-old male patient presented for urological evaluation due to episodes of total gross hematuria. Physical examination revealed ambiguous external genitalia. Abdomiopelvic CT scan revealed a tumor at the level of the bladder floor. Transurethral resection of the bladder lesions was done and histopathological studie confirmed the diagnosis of müllerianosis. Karyotype analysis showed a 46 XX male syndrome.

[Male sexuality after external continent urinary diversion type Mitrofanoff]. / Étude de la sexualité masculine après dérivation urinaire externe continente de type Mitrofanoff.

OBJECTIVE: To evaluate the influence of continent external urinary diversion type Mitrofanoff on male sexuality. MATERIAL AND METHODS: Between 1992 and 2011, 140 patients underwent continent urinary diversion type Mitrofanoff at an academic hospital. Among 76 men, 46 were interviewed about their sexuality after this operation. This study was performed using a set of validated questionnaires (IIEF, DAN PSS and Urolife), grouped by the model of the CTMH. Patients were divided according to their marital status: group 1: patients married before surgery (15 cases), group 2: patients married after surgery (7 cases) and group 3: singles (24 cases). RESULTS: In the first group, the functional dimension of sexuality was positive with an overall score of 81%, the sexual discomfort score was assessed at 26 % and the sexual satisfaction score was 77%. In the second group, sexual function was considered conserved in all cases with a satisfaction score estimated at 98%. These patients reported a feeling of well-being following the disappearance of urinary incontinence with integrity of their body images. In contrast, in the last group, relatively impaired sexual function was noted (65%) with a satisfaction score estimated at 59%. These disorders were multifactorial, mainly related to neurological causal pathology. CONCLUSION: To our knowledge, this is the first study about male sexuality in patients with a continent urinary diversion type Mitrofanoff. Marital status has a major role in the sexuality of these patients. A prospective study with pre- and postoperative evaluation will better clarify the factors affecting sexuality in these patients.

[Fatal postoperative crisis in acute hereditary porphyria]. / Crise post-operatoire fatale d'une porphyrie aiguë héréditaire.

The acute porphyria is an autosomal dominant disorder of the héme biosynthesis enzyme. The aim of this work is to determin a diagnostic step in order to anticipate porphyric crises. We report the observation of a child which was hospitalized in the pédiatric service three years ago for peripheric arthralgies and myalgies where the diagnosis of an acute porphyria was not posed. He wase admitted in our service for appendicitis, he was operated, the anesthesie was carried out by the thiopenthal and the succinylcholine. At the first post-operative day, a respiratory insufficiency was the cause of death. The rate of coproporphyrines and of uroporphyrines in the urine had confirmed the diagnosis of acute porphyria. The positive diagnostic of acute porphyria is difficult, the association of abdominal, psychological and neurologic signs must suggest the diagnostic, all the more, if the urines have a dark colour. The presence of uroporphyrines and coproporphyrines and certain precursors in the urines or in the high-rated stools confirm the diagnostic. The family survey and dosage of porphyric compounds in the sickness-bearing patient's family members allow to reveal certain asymptomatic forms of the heriditary acute porphyria, it is caused by certain products used in neuropsychiatry, in aneasthesic or in certain circunstances, such as, the infection or the trauma. The treatment of the acute porphyria is an emergency wich requires the transfert of the patient to a unit of intensive care.