Cryptococcosis in sarcoidosis: cryptOsarc, a comparative study of 18 cases.

AIM: To describe the main characteristics and the treatment of cryptococcosis in patients with sarcoidosis. DESIGN: Multicenter study including all patients notified at the French National Reference Center for Invasive Mycoses and Antifungals. METHODS: Retrospective chart review. Each case was compared with two controls without opportunistic infections. RESULTS: Eighteen cases of cryptococcosis complicating sarcoidosis were analyzed (13 men and 5 women). With 2749 cases of cryptococcosis registered in France during the inclusion period of this study, sarcoidosis accounted for 0.6% of all the cryptococcosis patients and for 2.9% of the cryptococcosis HIV-seronegative patients. Cryptococcosis and sarcoidosis were diagnosed concomitantly in four cases; while sarcoidosis was previously known in 14/18 patients, including 12 patients (67%) treated with steroids. The median rate of CD4 T cells was 145 per mm(3) (range: 55-1300) and not related to steroid treatment. Thirteen patients had cryptococcal meningitis (72%), three osteoarticular (17%) and four disseminated infections (22%). Sixteen patients (89%) presented a complete response to antifungal therapy. After a mean follow-up of 6 years, no death was attributable to cryptococcosis. Extra-thoracic sarcoidosis and steroids were independent risk factors of cryptococcosis in a logistic regression model adjusted with the sex of the patients. CONCLUSIONS: Cryptococcosis is a significant opportunistic infection during extra-thoracic sarcoidosis, which occurs in one-third of the cases in patients without any treatment; it is not associated to severe CD4 lymphocytopenia and has a good prognosis.

[Personality tests and gender identification in male transsexuals]. / Tests de personnalité et identification sexuée chez des transsexuels masculins.

The aim of this research project was to study gender identification in male transsexuals compared to male and female controls, using the Rorschach test and the MMPI. In the international literature, many researches have shown that the nature of the human response on Rorschach card III is linked to gender identification, as is the MMPI Mf scale. Ten untreated male homosexual transsexuals and 18 treated and operated male homosexual transsexuals were compared to 10 male and 12 female controls regarding verbal IQ, human content on Rorschach card III and the MMPI Mf scale. Absence of hormonal treatment for the first group of transsexuals was checked by a blood test at the time of the psychological testing. Responses on Rorschach card III were scored according to different kinds of human contents: male (M), female (F), gender-unidentified/neutral (N), bisexual (B), feminine then masculine or the opposite (M/F), and nonhuman (NH). N, B, M/F and NH responses were rare in all Rorschach protocols. As expected, responses given by participants in the control group were significantly more consistent with their anatomical sex than with the opposite sex. Untreated transsexuals do not differ from treated and operated transsexuals on Rorschach data, and both transsexual groups give significantly more female human representations than male controls. Transsexuals' results are similar to female controls. Untreated transsexuals' mean score on the MMPI Mf scale is significantly higher than that of treated and operated transsexuals' score, in the male profile (biological sex). Both groups of transsexuals score higher on the Mf scale in the male profile than in the female profile. The mean Mf score in the male profile is significantly higher than that of male controls, whereas, in the female profile, the mean Mf score is similar to that of female controls. This study shows that for both groups of transsexuals, results are homogenous in respect of Rorschach and MMPI, showing hyper-conformism to self-perceived gender. Results in both groups are similar to results of female controls, but tend to show even more feminine gender identification. The absence of any significant difference between untreated and treated and operated transsexuals seems surprising, suggesting that the hormonal treatment has not had a major impact on gender identification processes. It would doubtless be interesting to study gender identification using even more kinds of data: all human contents in the Rorschach protocol (not just the responses given to card III), MMPI Mf scale, Draw-A-Person Test and Animal-and-Opposite Drawing Test. This would enhance result liability and could provide useful information about how gendter identification processes evolve after surgical sex reassigment.

Assessment of hypothalamic-pituitary-adrenal axis function after corticosteroid therapy in inflammatory bowel disease.

OBJECTIVES: In patients with inflammatory bowel disease (IBD), little is known about the effect of long term corticosteroid therapy (CT) on the hypothalamic-pituitary-adrenal (HPA) axis function. Our aim was to assess HPA axis function in IBD, before the end of CT, during the tapering phase. METHODS: HPA axis function was assessed with cortisol (ng/ml) measurement before (T0) (normal > 100) and 60 min (normal > 210) after 0.25 mg tetracosactide (Synacthen immédiat) injection (T60) in 55 consecutive cases of IBD attacks. Abnormal response was defined as a T60 <210. The attacks were separated into two groups according to the result of the Synacthen test (ST). RESULTS: In all, 36 of 55 ST were abnormal. The time for recovery normal HPA axis function was 7.2+/-1.3 months. Duration of disease since onset, past history of surgical or immunosuppressive treatment, severity and extension of the attack, need for surgical or immunosuppressive treatment, total cumulative and mean daily corticosteroid dose, total duration of CT, and steroid dose at the time of ST were not significantly different in the two groups. In multivariate analysis a past history of CT was predictive of abnormal ST (OR = 8.4, 95% CI = 2.2-31.5, p = 0.0009). Among patients with a past history of CT, the time (months) elapsed between the last course of CT was significantly longer in those with normal ST than in those with abnormal ST (45.5+/-13.5 vs 15.4+/-6.0; p = 0.02), and in multivariate analysis a duration free of CT < 15 months was predictive of abnormal ST (OR = 15.00, CI = 1.23-183.00, p = 0.03). CONCLUSIONS: In all, 65% of the ST were abnormal. These results suggest that ST should be performed before corticosteroid withdrawal, especially in patients with recent past history of CT.

[Diabetes insipidus with a hypothalamo-hypophyseal morphologic anomaly during a pregnancy]. / Diabète insipide avec anomalie morphologique hypothalamo-hypophysaire au cours d'une grossesse.

BACKGROUND: Diabetes insipidus is uncommon in pregnancy. Despite physiological modifications in hydroelectrolytic balance during normal pregnancy, the capacity of the kidney to concentrate urine is preserved, partially due to lower vasopressin secretion. CASE REPORT: A young woman developed diabetes insipidus during the third trimester of normal pregnancy. The disease regressed totally after delivery. However, magnetic resonance imaging revealed a persistent expansive intrasellar image with a high-intensity signal. DISCUSSION: Onset of diabetes insipidus is usually rapidly progressive in pregnancy. Occurring generally during the third trimester in normal pregnancies, diabetes insipidus is generally well tolerated and responds to dDAVP, usually without pituitary abnormally, and regresses after delivery. Two types are distinguished: partially latent diabetes insipidus occurring during pregnancy and due to a central rather than nephrogenic origin; and excessive vasopressinase activity leading to diabetes insipidus usually associated with liver anomalies and high frequency of pre-eclampsia. During normal pregnancy, the size of the anterior pituitary increases and the normal high-intensity signal in the posterior pituitary seen on MRI usually regresses or disappears. In diabetes insipidus, the posterior pituitary hypersignal image generally disappears, reflecting reduced vasopressin storage. Few observations of diabetes insipidus occurring during pregnancy have been reported with morphological explorations. Most have described a "normal" aspect of the pituitary, specifically in the post partum period. In our patient, the weak vasopressin response to the end of water restriction at post partum when the diabetes insipidus symptoms had disappeared would suggest partial central diabetes insipidus revealed by pregnancy. Other pathologies involving this region could also be involved due to the unusual and persistent sellar image, with an expansive process showing a high intensity signal on MRI. An asymptomatic craniopharyngioma cyst was hypothesized and would be more compatible with the observed symptoms.

[Cushing's syndrome]. / Syndromes de Cushing.

The adrenal origin of hypercorticism is established by usual hormonal and morphological investigations. Adrenal causes of Cushing syndrome are mainly due to unilateral tumours. In some cases, the remaining difficulty is evaluation of prognosis. Here, the contribution of molecular biology is likely to be major. To better understand the mechanisms of adrenal tumourigenesis, it seems essential to seek the factors modulating steroidogenesis and adrenal cells proliferation.

[The place of endocrinology in the management of transsexualism]. / La place de l'endocrinologie dans la prise en charge du transsexualisme.

Transsexualism is not a sexual perversion but rather a gender identity disorder. The patient claim for his personal and public image and condition. The diagnosis problems arise from the lack of clinical, biological or hormonal typical signs. The pathogenesis is unclear but works in progress suggest somatic alterations. These alterations might be pre or perinatal hormonal changes and/or alterations in cerebral structures involved in sexual differentiation. Transsexualism is not a treatable disease using currently available treatments. Only hormonal and surgical treatments with sex reassignment might answer to the patient's request and improve his mental condition. Hormonal treatment might only prescribed after a consensus statement of about the sex reassignment of the patient and under medical follow-up. An early and strict medical follow-up of the patients by a multi specialist medical team is required and have to warrant the etic aspects. This is mostly important for the approval for sex reassignment from the authorities.

[Cushing's disease]. / Maladie de Cushing.

Patients with Cushing's disease usually present typical clinical and biological features easily leading to the diagnosis. However very atypical presentations of the disease do exist especially in the intermittent forms and several investigations are often necessary to detect pituitary microadenoma wich sometimes is inconspicuous. The course of the disease can be serious and might responsible of definitive sequellas wich can threaten patient's life. Therefore rapid treatment is recommended. Pituitary surgery should be considered as the major therapeutic approach; however, in severe cases or when pituitary-directed treatments or OP'DDD have failed, total bilateral adrenalectomy should be proposed.

[Agranulocytosis induced by synthetic antithyroid drugs: efficacy of hematopoietic growth factors]. / Agranulocytose aux antithyroïdiens de synthèse: efficacité des facteurs de croissance hématopoïétique.

Agranulocytosis is the most serious problem, potentially fatal, that can occur during antithyroid drug therapy. The use of hematopoietic growth factors is an attractive approach to reduce the period of this drug-induced neutropenia. We report two cases of severe antithyroid drug-related agranulocytosis (granulocyte count < 0.1 x 10(9)/L) treated with Granulocyte Colony-Stimulating Factor (G-CSF) or Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF). The delay to observe a granulocyte count superior to 1 x 10(9)/L was respectively 1 and 5 days. Our results, with others, clearly show that hematopoietic growth factors are effective in severe antithyroid drug-induced agranulocytosis.