EuroGuiderm guideline on lichen sclerosus-introduction into lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness. It may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are redness and oedema, typically followed by whitening of the genital skin; sometimes fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

EuroGuiderm guideline on lichen sclerosus-Treatment of lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness; it may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are usually a whitening of the genital skin, sometimes preceded by redness and oedema; fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS (part 1), highlight important aspects in the care of LS patients, generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

Psychological well-being and sexarche in women with polycystic ovary syndrome.

BACKGROUND: The characteristics of polycystic ovary syndrome (PCOS) such as hyperandrogenism and anovulation can be highly stressful and might negatively affect psychological well-being and sexuality. The objective of this study was to evaluate the association between PCOS characteristics and psychological well-being as well as sexarche. METHODS: Patients (n = 1148) underwent standardized clinical evaluation. Psychological well-being was investigated in 480 patients with the Rosenberg self-esteem scale (RSES), the body cathexis scale (BCS) and the fear of negative appearance evaluation scale (FNAES). Sexarche was also assessed. RESULTS: Amenorrhoea was associated with lower self-esteem (P = 0.03), greater fear of negative appearance evaluation (P = 0.01) and earlier sexarche (P= 0.004). Hyperandrogenism and acne were associated with poorer body satisfaction (P = 0.03, 0.02, respectively). Hirsutism and BMI were negatively associated with all psychological variables (RSES, P = 0.01; BCS, P = 0.05; FNAES, P = 0.02 and RSES, P = 0.03; BCS, P = 0.001; FNAES, P = 0.03, respectively). CONCLUSIONS: Our results suggest that menstrual irregularities might be related to sexarche. Moreover, this study stresses that the treatment of women with PCOS should notably focus on physical but also on psychological and sexual characteristics.

Hyperreninemic hypoaldosteronism: a possible etiological factor of septic shock-induced acute renal failure.

OBJECTIVE: Hyperreninemic hypoaldosteronism has been described in critically ill patients. The present study investigated the plasma aldosterone concentration (PAC) in septic shock patients and its relationship with clinical course. DESIGN AND SETTING: Prospective descriptive study in a medical intensive care unit (ICU) of a university hospital. PATIENTS: Forty-six consecutive patients with septic shock as defined by the ACCP/SCCM criteria. INTERVENTION: A corticotropin stimulation test, followed by treatment with low doses of hydrocortisone and fludrocortisone. MEASUREMENTS AND RESULTS: Plasma renin activity, PAC, and cortisol levels were measured before and after the test. PAC measurements were repeated for 1 week. Relevant clinical and laboratory variables were recorded for ICU stay. Patients were divided into two groups according to PAC/renin activity ratio: above 2 (n=24 patients) and below 2 n=22). Patients with PAC/renin activity less than 2 had higher total volume of infused fluid, serum creatinine level, and fractional excretion of sodium values; aldosterone and serum creatinine were negatively correlated. Hypoaldosteronism was reversible within 1 week. Duration of ICU stay (p=0.0026) and the need for renal replacement therapy (p=0.0021) were greater in the group with PAC/renin less than 2. CONCLUSIONS: Transient hyperreninemic hypoaldosteronism is common in patients with septic shock. These abnormal aldosterone levels are associated with greater sodium and fluid depletion and are followed by enhanced incidence of acute renal failure requiring renal replacement therapy and prolonged length of stay in ICU.

[Vulvodynia caused by vulvar vestibulitis syndrome]. / Vulvodynie met als oorzaak vulvair vestibulitissyndroom.

Vulvodynia is a puzzling disorder. Patients experience clear physical complaints of vulvar burning. Often they have consulted many physicians and tried all kinds of treatment. Vulvodynia is often caused by the vulvar vestibulitis syndrome (VVS). To detect VVS an extensive medical and psychosexual history is necessary. Thorough examination of the vaginal vestibule reveals the typical focal erythematous lesions. The aetiology of VVS is unknown. Of the many causal and perpetuating factors a sexual arousal disorder and pelvic floor hypertonia are the main ones. The psychodynamic aspects of these two core symptoms are principal issues in diagnosis and treatment of VVS. Treatment should include all physical, psychological, relational and sexual aspects of the problem. Surgical interventions should be limited to those rare cases in which an integrative approach fails to free the patient from the vicious circle of pain, anxiety and muscle tension.

[Post-traumatic pulmonary pseudocysts]. / Les pseudokystes pulmonaires post-traumatiques.

BACKGROUND: Pulmonary pseudocyst is an unusual complication of chest trauma. CASE REPORT: A 12-year-old boy suffered from a non penetrating chest trauma. Examination was normal except mild hemoptysis. Chest X-rays and tomodensitometry showed several cystic lesions surrounded by areas of pulmonary contusion in the right lower lobe. X-rays performed a few years before the trauma were normal. The cystic lesions progressively disappeared within a few months. CONCLUSION: This is a new case of post-traumatic pulmonary pseudocyst whose diagnosis may be difficult until spontaneous cure.

The effects of 3g eicosapentaenoic acid daily on recurrence of intrauterine growth retardation and pregnancy induced hypertension.

OBJECTIVE: To study the effects of addition of 3 g eicosapentaenoic acid daily to the diet, on recurrence rate of intrauterine growth retardation and pregnancy induced hypertension in a high risk population. DESIGN: Prospective, double blind, randomised multicentre study. Eicosapentaenoic acid or placebo were given from 12 to 14 weeks of gestation onwards. SETTING: University Hospital and regional hospitals in the north of the Netherlands. SUBJECTS: Sixty-three women with a history of intrauterine growth retardation (birthweight < 10th centile) with or without pregnancy induced hypertension in the previous pregnancy. MAIN OUTCOME MEASURES: Birthweight centiles and signs of pregnancy induced hypertension in current pregnancy. RESULTS: One-third of the women developed pregnancy induced hypertension and one-third of the infants had a birthweight below the 10th centile. There were no differences between eicosapentaenoic acid and placebo group. CONCLUSION: Addition of 3 g eicosapentaenoic acid daily does not prevent recurrence of intrauterine growth retardation or pregnancy induced hypertension in a high risk population.

[Chronic obstructive bronchopneumopathy after acute inhalation of sulfur anhydride. Apropos of a case]. / Bronchopneumopathie chronique obstructive après inhalation aiguë d'anhydride sulfureux. A propos d'un cas.

We report on an acute accidental inhalation of sulfurous anhydride, by a man aged thirty in the course of his work. This intoxication, followed by acute respiratory distress, first showed an improvement, then re-aggravation 26 days after the accident. This episode has not been explained. The patient then developed a chronic obstructive bronchopathy. Ten years of regular observation allows us to maintain that the severe obstructive syndrome which the patient presents has never regressed (FEV1/FVC = 32%), and must be associated with a chronic toxic obstructive bronchopneumopathy.

Fatty acid composition of serum lipids of mothers and their babies after normal and hypertensive pregnancies.

The biochemical essential fatty acid (EFA) status of neonates born after normal and hypertensive pregnancies (PIH) and that of their mothers was assessed by measuring the fatty acid composition of phospholipids (PL), triglycerides (TG) and cholesterol esters (CE) of umbilical cord serum and maternal serum, respectively. Relative contents of linoleic acid of serum PL and CE were significantly lower in mothers with PIH compared to normal pregnancies. Most other (n-6) polyenes in PL tended to be higher under hypertensive conditions. Total maternal (n-3) polyenes of serum PL were significantly higher in PIH, mainly due to clupanodonic acid, 22:5 (n-3), and cervonic acid, 22:6 (n-3). Total maternal (n-7) and (n-9) fatty acids were also significantly higher in PIH (PL and CE). The results indicate that PIH is associated with a relative increased unsaturation of maternal serum PL, which might facilitate the placental transfer of long-chain, polyunsaturated fatty acids. As a result, the neonatal EFA status after PIH only slightly differs from normal.

Biochemical EFA status of mothers and their neonates after normal pregnancy.

The essential fatty acid (EFA) status of neonates was compared with that of their mothers by determining the fatty acid compositions of phospholipids (PL), isolated from umbilical arterial and venous tissue, blood cells (BC) and plasma, from maternal venous plasma and BC, and from non-infarcted placental tissue. The PL of umbilical arterial tissue (efferent fetal vessels) contained fewer fatty acids of the (n-6) family and more of the (n-9) family than umbilical venous tissue (afferent fetal vessel). The relative amounts of (n-6) and (n-3) fatty acids were less in arterial than in venous plasma. Mead acid, 20:3(n-9), the presence of which indicates a poor EFA status, was 5 times higher in the efferent than in afferent cord vessels. In neonatal plasma and BC it was twice as high as compared with maternal levels. In general, the fatty acid composition of the placenta PL showed a comparable pattern as neonatal venous plasma PL. These findings demonstrate that the biochemical EFA status of neonates after a normal pregnancy is not optimal. The significant correlations between neonatal and maternal EFAs indicate that the neonatal EFA status depends on the EFA content of the maternal diet.