Elizabeth Silverstone

Everolimus treatment of abdominal lymphangioleiomyoma in five women with sporadic lymphangioleiomyomatosis

Objective: Lymphangioleiomyomatosis (LAM) is a rare systemic disease of young women arising from mutations in the tuberous sclerosis complex (TSC) genes, TSC1 or TSC2. This disrupts the mammalian target of rapamycin (mTOR) pathway, affecting cellular proliferation and growth. mTOR inhibitors are a promising novel therapy in LAM. The mTOR inhibitor sirolimus is reported to produce resolution of lymphatic abnormalities in LAM, but the efficiacy of the mTOR inhibitor everolimus has not been assessed. We aimed to examine the efficacy of everolimus on lymphatic abnormalities in LAM.

Pulmonary necrobiotic nodules: a rare extraintestinal manifestation of Crohn's disease

The present article reports the case of a 22-yr-old female with new onset Crohns colitis, anterior uveitis and multiple pulmonary nodules which, on histological examination, were necrobiotic nodules. This is a rare but recognised pulmonary extraintestinal manifestation of Crohns disease and only the fourth reported case. The present case report is followed by a brief review of the relevant literature.

Oesophageal dissection after thrombolytic treatment for myocardial infarction.

A 62 year old woman admitted with a history suggesting acute myocardial infarction had thrombolytic treatment with anisoylated plasminogen-streptokinase activator complex, which resulted in submucosal haemorrhage in the oesophagus; this caused dissection of the wall of the oesophagus and complete dysphagia. The haematoma resolved spontaneously, leaving behind a diverticulum, with reduced peristalsis and delayed emptying but no obstruction.

Menstrual cycle variation of retroperitoneal lymphangioleiomyomas in lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare disease affecting women, classically involving the lungs. However, extrapulmonary manifestations also occur, including renal angiomyolipomas, retroperitoneal lymphangioleiomyomas and extrathoracic lymphadenopathy. The lung disease is hormone‐responsive, but no information exists regarding sex hormone responsiveness of abdominal LAM. Here, we report two women with LAM whose abdominal lymphangioleiomyomas increased in size with hormonal changes of the menstrual cycle. This is the first description of abdominal lymphangioleiomyomas exhibiting hormone responsiveness in LAM. We describe these cases and summarize the literature on abdominal LAM. Menstrual cycle variation should be taken into account when assessing response to therapy, both clinically and in research studies.

The CT appearances of hepatic oil embolism following lymphography

Four instances of lymphography complicated by hepatic oil embolism are presented. The subsequent computerised tomography appearances can be misleading: the oily contrast medium mimics hepatic calcification and this may be mistakenly thought to indicate calcified hepatic metastases. The correct diagnosis will become apparent if a history of lymphography is sought, and it is important to realise that, even on conventional radiography, hepatic oily contrast medium may remain visible for several months. Computerised tomography is a much more sensitive indicator of the presence of oily contrast medium in the liver, so the absence of detectable oil on conventional radiography is unhelpful. The mechanisms which are thought to lead to hepatic oil embolism are discussed because recognition of the circumstances leading to hepatic oil embolism and appreciation of the associated findings will provide further clues to the correct diagnosis.

Treasure in the chest.

A 41-year-old woman with a background of asthma was preparing for a party on New Years Eve when she developed a mild wheeze. Concerned her symptoms would develop and impact on festivities, she located her uncapped salbutamol inhaler in her handbag. Ignoring the coarse rattle as she shook it, she proceeded to take a deep inspiration. Instantly, she felt a painful scratch in her pharynx followed by a harsh cough, which persisted over the next few minutes and became associated with haemoptysis. She was taken to the accident and emergency department. Chest X-ray revealed a radiodense foreign body in the distal right main bronchus. During endoscopy, an earring, causing subtotal occlusion in the right lower lobe bronchus was found and extracted. Her observations remained within normal limits throughout. The importance of replacing caps on inhalers when not in use is illustrated and should be encouraged when inhaler technique is taught or reviewed.

Complicated silicosis resulting from occupational exposure to engineered stone products

The biopsy specimen (A) shows the whorled appearance of a silicotic lung nodule, consisting of concentric laminated collagen fibres (haematoxylineeosin stain, magnification 10 ). A higher magnification image (B) highlights clefts containing faintly visible silicate particles surrounded by macrophages and giant cells (magnification 20 ). u A 54-year-old man, formerly a smoker, presented with a 6-year history of chronic coughandexertionalbreathlessnesswithout previous respiratory illnesses. Born in Vietnam,hecametoAustraliaasa refugeeat the age of 20 years. A screening chest x-ray was performed on his arrival in Australia; as the patient was not informed about any abnormality, thiswas assumed tobenormal. He commenced work as a labourer; he denied exposure to silica-containing materials and did not participate in activities typically associated with silica exposure (such as jack-hammering) during this period. About 15 years later, the patient started a job manufacturing stone benchtops. He cut, ground,finishedand installed thebenchtops, using a popular brand of engineered stone comprising > 85% crystalline silica. Occasionally, he made benchtops from granite andmarble. During the first 7 years of this work, the patient did not use any respiratory protective equipment, but later used a simple paper mask. Despite some dust extraction facilities in the factory, he reported that the environment was visibly dusty and that dust suppression with water was hardly ever used.

Focal salmonellosis: an unusual presentation of uterine malignancy

Uterine sarcomas are rare, representing approximately 1 % of all female malignant genital tract tumours. Of those arising from muscle, half are leiomyosarcomas. They are highly malignant tumours and usually present with pain, an abdominal mass or vaginal bleeding. Focal salmonellosis is an extremely rare complication of gynaecological malignancy and has once been reported in a benign fibroid (Han et al. 1967).