Leiomyomatosis peritonealis disseminata in a 50-year old woman: imaging findings

Leiomyomatosis peritonealis disseminata (LPD) – or diffuse abdominal leiomyomatosis – is a very rare benign abdominal entity. Only a little more than 100 cases have been reported in the English literature since its first description in 1965. Middle aged female are typically affected and the clinical presentation is rather aspecific. The differential diagnosis between benign LPD and diffuse peritoneal carcinomatosis or abdominal disseminated malignancy represents the crucial diagnostic challenge that can only definitively be made through biopsy and histologic analysis. Multimodal imaging features (ultrasound, CT, MR and PET) of a case of LPD diagnosed in a 50-year old female are presented with review the literature

Neutropenic colitis

Background: A 66-year-old-woman, undergoing chemotherapy for recently diagnosed acute myeloid leukemia developed increasing abdominal pain over a period of several days. Laboratory results showed, apart from severe neutropenia, increasing infection parameters

IRIS study: a phase II study of the steroid sulfatase inhibitor Irosustat when added to an aromatase inhibitor in ER-positive breast cancer patients

Purpose: Irosustat is a first-generation, orally active, irreversible steroid sulfatase inhibitor. We performed a multicentre, open label phase II trial of the addition of Irosustat to a first-line aromatase inhibitor (AI) in patients with advanced BC to evaluate the safety of the combination and to test the hypothesis that the addition of Irosustat to AI may further suppress estradiol levels and result in clinical benefit. Experimental design: Postmenopausal women with ER-positive locally advanced or metastatic breast cancer who had derived clinical benefit from a first-line AI and who subsequently progressed were enrolled. The first-line AI was continued and Irosustat (40 mg orally daily) added. The primary endpoint was clinical benefit rate (CBR). Secondary endpoints included safety, tolerability, and pharmacodynamic end points. Results: Twenty-seven women were recruited, four discontinued treatment without response assessment. Based on local reporting, the CBR was 18.5% (95% CI 6.3–38.1%) on an intent to treat basis, increasing to 21.7% (95% CI 7.4–43.7%) by per-protocol analysis. In those patients that achieved clinical benefit (n = 5), the median (interquartile range) duration was 9.4 months (8.1–11.3) months. The median progression-free survival time was 2.7 months (95% CI 2.5–4.6) in both the ITT and per-protocol analyses. The most frequently reported grade 3/4 toxicities were dry skin (28%), nausea (13%), fatigue (13%), diarrhoea (8%), headache (7%), anorexia (7%) and lethargy (7%). Conclusions: The addition of Irosustat to aromatase inhibitor therapy resulted in clinical benefit with an acceptable safety profile. The study met its pre-defined success criterion by both local and central radiological assessments

No association between glucocorticoid receptor polymorphisms and long-term respiratory outcome after very preterm birth

Developmen

Gender differences in respiratory symptoms in 19-year-old adults born preterm

Objective: To study the prevalence of respiratory and atopic symptoms in (young) adults born prematurely, differences between those who did and did not develop Bronchopulmonary Disease (BPD) at neonatal age and differences in respiratory health between males and females. Methods: Design: Prospective cohort study. Setting: Nation wide follow-up study, the Netherlands. Participants: 690 adults (19 year old) born with a gestational age below 32 completed weeks and/or with a birth weight less than 1500g. Controls were Dutch participants of the European Community Respiratory Health Survey (ECRHS). Main outcome measures: Presence of wheeze, shortness of breath, asthma, hay fever and eczema using the ECRHS-questionnaire

Maternal outcome following temporizing management of the (H)ELLP syndrome

OBJECTIVE: The aim of the study was to describe the clinical progress and maternal outcome of the (H)ELLP syndrome following temporizing management. METHODS: All women (n = 127) admitted in the Academic Medical Center in Amsterdam between 1984 and 1996 with (H)ELLP syndrome and a live fetus in utero were included. The patients were treated by temporizing management, including the use of antihypertensives and magnesium sulfate. The predominant indication for terminating pregnancy was fetal distress or fetal death, and not maternal condition. MAIN OUTCOME MEASURES: Maternal mortality and morbidity. RESULTS: All serious maternal complications occurred at the onset of the syndrome. Two mothers with HELLP syndrome died following a cerebral hemorrhage. The remaining patients recovered completely. Serious maternal morbidity occurred more often in cases of HELLP than in cases of ELLP syndrome. Seventy-nine (62%) women were not delivered after 3 days and 65 (51%) after 7 days. CONCLUSIONS: Severe complications only occurred at the onset of (H)ELLP syndrome. It is unlikely that a more aggressive approach would have reduced maternal mortality or morbidit