Gestational gigantomastia complicated by pseudo-angiomatous stromal hyperplasia - a multidisciplinary management approach.

SUMMARY: Gestational gigantomastia is a rare condition typified by disproportionate bilateral breast enlargement in pregnant women, resulting in skin thinning, ulceration, and bleeding. Less than sixty cases have been documented worldwide, and only one other in South Africa. Pseudo-angiomatous stromal hyperplasia (PASH) is a rare benign proliferation of stromal tissue in a tumorous or diffuse pattern. This, to the best of our knowledge, is the first published case, a 27-year-old human immunodeficiency virus (HIV) positive woman, to present with both conditions concurrently. Medical management with cabergoline was initiated and, seven months post-delivery, a novel Goldilocks mastectomy was performed with acceptable outcomes.

Terminology and labeling of cellular products-2: Implementation plan.

The publication of new standards for terminology and labeling marks an important step in ensuring consistency and traceability of cellular therapies at the global level. However, it is only with the widespread implementation of the standard that the benefits can be truly realized. This paper provides guidance on the practical aspects of adopting these new standards for organizations with differing current levels of computerization. It discusses project management, equipment, licensing, and validation topics.

Terminology and labeling of cellular products: 1. Standards.

The International Cellular Therapy Coding and Labeling Advisory Group was established to address the growing need for standardization of terminology and labeling for cellular therapy products as a result of increasing international transfer of these products. This paper presents new standards for terminology and labeling. These standards have been developed through a consultative process and are supported by key professional and accreditation bodies. By using these standards, together with the unique donation identification numbers and international product reference tables provided by the International Society of Blood Transfusion (ISBT) 128 Standard, consistency and traceability can be assured at the global level. A companion paper provides guidance on the implementation of the ISBT 128 system.

Hirudin determination in plasma can be strongly influenced by the prothrombin level.

Recombinant hirudin is increasingly used for therapeutic and prophylactic anticoagulation. Several laboratory methods are available to measure r-hirudin, including clot-based and amidolytic methods. The snake venom ecarin converts prothrombin to meizothrombin. Hirudin inhibits meizothrombin, causing a prolongation of the ecarin clotting time (ECT). Because the ECT depends on prothrombin levels in plasma, it was compared with a chromogenic substrate assay (CSA) for the determination of r-hirudin levels in prothrombin deficient plasma samples. R-hirudin (0.0-2.0 microg/mL) was added to plasma samples with decreasing prothrombin concentrations (100-0%). Using the ECT, false high r-hirudin levels were observed even in r-hirudin-free plasma, when prothrombin levels were below 50%. This effect was more pronounced with increasing r-hirudin levels. Additionally, r-hirudin (0.5 microg/mL) was added to plasma of patients with acquired prothrombin deficiency due to oral anticoagulation. Hirudin levels were also overestimated in these plasma samples using ECT. In plasma samples of patients treated with r-hirudin, because of suspected heparin-induced thrombocytopenia (HIT), hirudin levels were already measured falsely high, when the prothrombin levels were below 70%. The chromogenic substrate assay (CSA) determined correct values in all prothrombin-deficient plasma samples. Therefore, the CSA should be used for hirudin level determination, if overestimation due to prothrombin deficiency should be avoided.

Report of general temporary anticoagulation in the treatment of acute cerebral and retinal ischaemia.

BACKGROUND: Recurrent thrombo-embolism represents by far the commonest cause of cerebral and retinal ischaemia. In an open clinical study, experience with a largely general anticoagulation in the acute phase of cerebral and retinal ischaemia is reported. METHODS: From October 1993 to October 1996, 781 patients with acute cerebral ischaemia were admitted to the medical emergency ward. Taking contra-indications into consideration and after CT exclusion of cerebral haemorrhage (2%). Immediate therapeutic heparinisation (1.5-2-fold prolongation of aPTT) was administered to 664 patients (85%) over 7-10 days. In a concurrent Doppler/duplex comparative study, 143 patients with retinal artery occlusion were investigated for the prevalence of potential sources of emboli in the region of the extracranial carotids and internal carotid circulation respectively. 108 patients served as a reference group whose acute loss of vision of vascular origin could be attributed to retinal vein thrombosis. RESULTS: The complication rate of anticoagulation in acute cerebral ischaemia was 0.8% (major complications), and the recurrence rate during the period of treatment was 2.4%. In 131 patients with acute retinal ischaemia, therapeutic heparinisation was performed without ocular cerebral or extracerebral complications. Potential ipsilateral sources of emboli (highgrade carotid stenosis or complex plaques with thrombotic deposits) were found in 55%, but in only 7% of the control group (p < 0.0001). CONCLUSION: Immediate anticoagulation in the form of aPTT-monitored therapeutic heparinisation represents a rational and low-risk concept for the treatment of acute cerebral and retinal ischaemia.

Differential roles of Mac-1+ cells, and CD4+ and CD8+ T lymphocytes in primary nonfunction and classic rejection of islet allografts.

The high rate of persistent hyperglycemia, termed primary nonfunction, after islet allotransplantation in C57BL/6 mice recipients of B10.BR strain islets, as compared with B10.BR recipients of C57BL/6 islets, led to a series of experiments to determine whether islet allograft primary nonfunction was attributable to technical aspects of the transplant procedure or whether it was a consequence of alloimmunity. Primary nonfunction was prevented by systemic pharmacologic immunosuppression of the host with cyclosporine. Selective immunodepletion of host CD4+ and CD8+ T lymphocytes significantly extended the time of classic rejection but did not significantly affect the rate of primary nonfunction. However, modulation of macrophages by administration to the host of silica completely abolished primary nonfunction. These observations, in conjunction with the immunohistological findings of intense macrophage infiltration in islet allografts from recipients exhibiting persistent post-transplant hyperglycemia, support the hypothesis that primary nonfunction results from a cell-mediated host-immune response of rapid onset that is dependent on macrophages or macrophage byproducts as the main effectors.

[Unilateral external ectopia of the male monoureter]. / Einseitige externe Ektopie des männlichen Monoharnleiters.

It is reported on the very rare clinical picture of a unilateral ectopy of the ureter in the male sex. Since about one third of all infantile malformations are of urological nature in the occurrence of a relapsing epididymitis/orchitis in childhood and adolescence, connected with the rectal palpation findings of a soft cell in the region of prostate gland and seminal vesicle which can be squeezed out, should be thought of such a clinical picture. An early recognition in good time prevents an atrophy of testicle and epididymitis or even their loss, connected with later infertility.

Correlation between in vitro autoradiographic results and histological WHO grades in human urothelial bladder tumours.

253 biopsy specimens of 106 urinary bladder tumours have been examined by means of in vitro autoradiography and the results compared with the WHO grading. An increase of the average labelling index occurred parallel to increasing WHO grades. However, great inter-tumorous differences in proliferation behaviour existed particularly in papillary G2 and G3 urothelium carcinomas. Possible causes of this heterogeneity are discussed. In vitro autoradiography is a suitable method for the objective characterization of the malignancy potential of urinary bladder tumours.

[Clinical significance of urinary tract infection caused by Providencia stuartii]. / Zur klinischen Bedeutung der Harnwegsinfektion durch Providencia stuartii.

Out of 75 patients in whom nosocomial infection of the urinary tract occurred following operation for disturbance of micturition 43 (57.3%) suffered clinically manifest inflammatory complications, however only in 26 cases (34.6%) was this germ the cause. In the remaining 17 cases there were superinfections. From these results it is concluded that the isolated Providencia stuartii strains were not very virulent. In cases of asymptomatic providencia-bacteriuria the authors consider specific antibacterial therapy to be indicated only if the excretion of germs does not cease spontaneously after micturition has been restored to normal.

Percutaneous transhepatic drainage for malignant biliary obstruction.

Percutaneous transhepatic drainage ( PTHD ) is an adjunct in the management of malignant biliary obstruction. It can be used for two purposes: as a palliative measure alone or as part of the preoperative preparation. This study assesses the efficacy of this technique. The charts of all patients undergoing PTHD were reviewed, and the data were collated according to the intent of catheterization. In the palliative group (18 patients), bilirubin levels fell from 20 +/- 1.8 to 10 +/- 1.9 mg/dl. PTHD complications developed in 14 (78%), the 1-month mortality was 56 per cent (10/18), and all of the patients have died (mean, 2.3 months). In the preoperative group (17 patients), bilirubin levels fell from 17 +/- 1.4 to 6 +/- 0.6 mg/dl, PTHD complications developed in three (18%), and postoperative complications occurred in five (30%). All patients in the preoperative group survived operation, 11 of 16 dying a mean of 4.5 months postoperatively. The five surviving patients have lived 2 to 25 months. The authors conclude that PTHD significantly lowers the serum bilirubin in both preoperative and palliative groups. In the preoperative group, its use is associated with low morbidity and may improve the patient's preoperative condition. In the palliative group, PTHD is associated with an appreciable morbidity that tempers the enthusiasm for its routine use in this circumstance.

Osseous metastases from renal cell carcinoma: embolization and surgery for restoration of function. Work in progress.

Five patients underwent preoperative embolization of osseous metastases from renal cell carcinoma. The group consisted of four men and one woman who ranged in age from 46 to 79 years. The lesions were located in the pubic ramus and acetabulum, proximal femur, femoral midshaft, proximal humerus, and proximal tibia. All embolizations were performed within 24 hours of surgery. The internal fixation and tumor curettage was accomplished with estimated perioperative blood loss ranging from 10 ml to 1,250 ml. All patients had significant restoration of function following surgery. We suggest that preoperative embolization is an important and efficacious adjunct in the management of hypervascular renal cell osseous metastases.

[Urothelial carcinoma of the bladder following cyclophosphamide therapy]. / Urothelkarzinom der Harnblase nach Cyclophosphamidtherapie.

A report is given on a 73-year-old patient in whom a urothelial carcinoma of the urinary bladder developed 11 years after a cyclophosphamide therapy. Taking the international literature into account, possible developmental mechanisms are discussed. Patients who have undergone cyclophosphamide therapy should be subject to constant urological observation as a high-risk group.

Angiographic assessment of renal artery pathology: how reliable?

The accuracy of the angiographic interpretation of the histologic type of renal artery stenosis was assessed using a renal pathologist's diagnosis as the "gold standard." The angiograms of 42 renal artery stenoses were interpreted without other information, except age and gender, independently by six angiographers. This assessment indicated that angiography is not an accurate means by which to distinguish between the individual types of fibromuscular disease of the renal artery. However, it is a fairly accurate means by which to distinguish fibromuscular disease in general from atherosclerosis of the renal artery, 207 (82%) correct interpretations of 252. In addition, in the presence of renal artery stenosis, the absence of abdominal aortic atherosclerosis on angiography is an excellent predictor of fibromuscular renal artery disease, 17 (94%) of 18 specimens. Likewise, in the presence of a renal artery stenosis, angiographically demonstrable abdominal aortic atherosclerosis is a fair predictor of atherosclerotic renal artery disease, 16 (76%) of 21 specimens.

Local thrombolytic therapy for subclavian and axillary vein thrombosis. Treatment of the thoracic inlet syndrome.

Four patients with primary subclavian-axillary vein thrombosis are described. Each patient received local thrombolytic therapy, resulting in reestablishment of antegrade flow via the axillary and subclavian veins. An intrinsic venous abnormality (perimural fibrosis and/or intimal dissection) identified at the first rib-clavicle junction (subclavian-axillary vein junction) was thought to be responsible for the thrombosis. One of the four patients subsequently underwent a surgical venous bypass of the abnormal segment, and a second underwent percutaneous transluminal angioplasty.