A renal cause for massive retroperitoneal hemorrhage--renal angiomyolipoma

In any case of intra-abdominal trauma or hemorrhage a preoperative IVP is essential to determine the renal status. Preoperative catheter occlusion of the renal artery in these vascular tumors may be attempted to decrease blood loss. Stigmas of tuberous sclerosis should be sought in patients with vascular renal tumors, especially if they are bilateral. Conservation of renal tissue in tuberous sclerotic patients harboring renal angiomyolipomas is important.     

Dieulafoy lesion as a cause of massive gastrointestinal bleeding

The Dieulafoy lesion, also referred to as exulceratio simplex, caliber-persistent artery anomaly, or cirsoid aneurysm, is a relatively rare, yet possibly fatal cause of gastrointestinal bleeding. Recent journal articles suggest that this pathological entity is not as uncommon as once thought. Advances in endoscopic technique and esophagogastroduodenoscopy (EGD) have greatly assisted in earlier diagnosis and added options to the treatment regimen for this lesion. The relationship of this anomaly to possible exsanguination makes it essential that both medical and surgical endoscopists be knowledgeable of the anatomy, diagnosis, and management of this pathology. Several therapeutic approaches to Dieulafoy's lesion are available and are described.     

Intratumor and retroperitoneal massive hemorrhage caused by retroperitoneal liposarcoma]

It is now widely accepted that snoring causes significant social dysfunction. In the absence of obstructive sleep apnoea syndrome, palatal surgery offers a very good chance of eliminating or reducing snoring. The traditional operation of uvulopalatopharyngoplasty remains the 'gold standard', but may be complicated by velopharyngeal incompetence, severe post-operative pain and even nasopharyngeal stenosis. A newer technique to reduce snoring caused by palatal flutter by using a neodymnium:yttrium aluminum garnet laser to stiffen the soft palate has been introduced recently by another unit. We show that this procedure can be carried out using a CO2 laser, and present the initial results of the first 29 patients operated on at The Royal National Throat, Nose and Ear Hospital.     

Jejunal varices as a cause of massive gastrointestinal bleeding--a case report

This study investigated regulatory volume increase (RVI) in rat pancreatic beta-cells. Volume changes in isolated beta-cells were measured by a video-imaging method. Cell shrinkage was induced by exposure to solutions made hypertonic by the addition of 100 mM mannitol. In HEPES-buffered solutions, beta-cells exhibited an RVI which was almost completely abolished by 10 microM bumetanide. These data indicate that Na+-2Cl--K+ cotransporters make a major contribution to RVI in beta-cells. In HCO3--buffered solutions, however, an RVI was observed in the presence of 10 microM bumetanide. This bumetanide-insensitive component of RVI was inhibited by 100 microM amiloride or 100 microM 4,4'-diisothiocyanatostilbene-2,2'-disulphonic acid (DIDS). These data suggest that, in addition to the Na+-2Cl--K+ cotransporter, functionally coupled Na+-H+ exchangers and Cl--HCO3- exchangers may also contribute to RVI in pancreatic beta-cells.     

Renal angiomyolipoma: a study of 13 cases

Arteriographic studies are valuable in the differentiation of angiomyolipoma from adenocarcinoma. Herein are described 13 cases of angiomyolipoma, in 10 of which angiographic studies were done. A correct preoperative diagnosis was made in 3 of the 10 cases and partial nephrectomy was done in 2. Since angiomyolipomas are benign tumors therapy differs greatly from that for adenocarcinoma. Early diagnosis of angiomyolipomas can prevent total or extensive nephrectomy and postoperative radiotherapy or cytostatic therapy, which are not necessary and are harmful to the patient.     

Carcinoid tumor associated with vascular malformation as a cause of massive gastric bleeding

Massive gastric bleeding in a 28-yr-old woman requiring emergency surgical treatment was found to originate from a polypoid carcinoid tumor 1.3 cm in diameter. Histologically, the tumor was found to be associated with a complex vascular malformation apparently originating from the underlying submucosa, crossing the tumor and ending in large mucosal sinusoids that opened on the mucosal surface. A similar clinical presentation was reported in three previous cases of small gastric carcinoids, one of which revealed an anomalous intratumoral bleeding artery. We recommend that in the absence of more common causative lesions of gastric bleeding, gastric carcinoid be considered in cases of focal massive hemorrhage requiring emergency treatment.     

Idiopathic aneurysm of the inferior vena cava as a cause of massive penile bleeding

The effect of droloxifene (3-hydroxytamoxifen) given as first-line endocrine treatment was evaluated in 39 postmenopausal women with advanced receptor-positive or receptor-unknown breast cancer. The patients had not received any previous anticancer therapy apart from adjuvant treatment. The overall response rate (CR + PR) was 51% (8% CR, 43% PR), 95% confidence interval+/-15.7%. Median time to progression (all patients) was 8 months, the median time to response 2 months, while the median duration of response was 10 months. The drug was well tolerated with no major side effects recorded; 16% of the patients experienced hot flushes. The response to droloxifene recorded in the present study is in accordance with the response rates to tamoxifen as first-line treatment in identical groups of patients.     

Renal angiomyolipoma: embolotherapy with a mixture of alcohol and iodized oil

A monoclonal antibody, D5G2, which reacts in a balloon angioplasty damage model with unfixed damaged but not with unfixed undamaged human endothelial cells, was used to screen a human endothelial cDNA library in an Escherichia coli/lambda gt11 expression system. Sequences of DNA inserts in D5G2+ phage clones matched those reported for a laminin-binding protein, LBP-32. Both D5G2 and purified laminin bound to a polypeptide of 55 kD on PVDF membranes carrying electrophoretically separated endothelial cell lysates, D5G2 also bound to recombinant LBP expressed in E. coli, and showed similar staining patterns on human and bovine endothelial cells to another characterized anti-LBP antibody. Increased staining of unfixed endothelial cells on detergent permeabilization suggests that D5G2 binds to intracellular laminin-binding protein made accessible by cell membrane injury. Antibodies to intracellular targets exposed by cell damage may be useful in anchoring therapeutic agents at sites of vascular damage.     

Perianeurysmal retroperitoneal fibrosis. An unusual cause of renal failure

Retroperitoneal fibrosis causing ureteral obstruction in association with an abdominal aortic aneurysm has been reported infrequently. However, the clinical presentation of patients with this entity and the histopathologic findings at surgery are similar to those in patients with idiopathic retroperitoneal fibrosis. We describe a patient with perianeurysmal fibrosis and bilateral ureteral obstruction who presented with severe renal failure. The diagnosis of an abdominal aortic aneurysm with perianeurysmal fibrosis was made only at the time of surgery to repair bilateral ureteral obstruction. Previous case reports of perianeurysmal fibrosis are reviewed, and possible pathogenetic mechanisms are discussed. It is important to consider the presence of an occult abdominal aortic aneurysm in patients suspected of having retroperitoneal fibrosis because of the serious prognostic and therapeutic implications.     

Renal vein thrombosis as the major cause of renal failure in familial Mediterranean fever

Eighteen out of 57 patients (31-6 per cent) suffering from Familial Mediterranean Fever (FMF) were found to have the nephrotic syndrome, histologically proven amyloidosis and progressive renal failure. In 14 cases renal function deteriorated rapidly after the first appearance of significant proteinuria, and 12 cases (66-7 per cent) required regular haemodialysis. Seven of these patients, seen in the early stages of renal impairment, were subsequently diagnosed clinically as probably having developed renal vein thrombosis. There was radiological proof of intrarenal or major renal vein occlusion in five which in one patient progressed to inferior vena cave obstruction. Treatment with heparin, plasminogen activators and fibrinogenolytic agents was disappointing although renal function has stabilized in one patient on long term oral anticoagulant therapy. It is suggested that renal vein thrombosis is common in FMF with renal amyloidosis and usually causes rapid deterioration of function and irreversible renal failure requiring dialysis. Renal phlebography may delineate clot in the main renal veins or indicate areas of reduced blood flow due to thromboses in intrarenal venules. Treatment is only partially satisfactory but there is some evidence to suggest that renal phlebography should be undertaken promptly when renal function begins to fall followed by anticoagulant therapy to prevent further thromboembolic complications.     

Intraocular haemorrhage as a complication of pneumoencephalography

The ocular fundi of 20 patients were examined before and after pneumoencephalography. In four of these, fresh venous retinal haemorrhages were seen, and a further patient had developed an exudate. Possible reasons for a rise in retinal venous pressure include bodily inverting the patient, compression of the thorax, the use of positive pressure respiration, and the air injection itself. It may be advisable to take steps to limit the effects of such possible causative factors.     

Renal angiomyolipoma with extrarenal involvement--in vascular, lymph-node and perirenal tissue. Reports of four cases

Extrarenal involvement was found in four cases of renal angiomyolipoma. In one case the wall of the inferior right bronchial artery was infiltrated, lymph nodes were involved in two cases and the perirenal fat in one case. Extrarenal extension does not seem to alter the prognosis of renal angiomyolipoma. Expectant treatment of this rare condition therefore appears to be warranted.     

Jejunal schwannoma in neurofibromatosis: an unusual cause of massive gastrointestinal bleeding

A patient who had the "fairy ring" finding shows another new radiographic presentation of pulmonary sarcoidosis that clinicians can add to the list of signs of the disease.     

CT of Erdheim-Chester disease presenting as retroperitoneal xanthogranulomatosis

Using mouse probes specific to cytochrome cs and to cytochrome cT, the single-copy genes encoding these two proteins have been mapped to paralogous chromosomal regions by analysis of restriction fragment length variants in interspecific crosses. The gene for cytochrome cs, Cycs, maps to a position between Tcrb and Cbl-1 on proximal mouse Chromosome 6, and the gene for cytochrome cT, Cyct, maps between Gad-1 and Sfpi-1 on mouse Chromosome 2.