Successful liver, kidney, and pancreas transplantation from a donor with cerebral emboli from a left atrial myxoma.

Although transmission and engraftment of donor-derived malignancies is rare in recipients of solid organ transplants, it is associated with unfavorable allograft and patient survival. Therefore, a recent history of malignancy is considered a contraindication to organ donation. Although atrial myxomas are benign cardiac tumors of stromal origin, they can lead to systemic embolization with ectopic myxoma formation. We report successful liver, kidney, and pancreas transplantation into 3 recipients from a donor with cerebral emboli from a left atrial myxoma. Eighteen months after transplantation, all 3 patients enjoy good allograft function and are free of donor-derived atrial myxoma. Although the duration of follow-up in this report is limited, we suggest that the presence of atrial myxoma should not be viewed as an absolute contraindication to organ recovery, particularly in view of the shortage of organ donors and the attendant morbidity and mortality for patients on waiting lists.

[Interactions of phytotherapeutic drugs, foods and drinks with medicines]. / Interaktionen von Phytopharmaka, Nahrungs- und Genussmitteln mit Medikamenten.

Phytotherapeutic preparations contain a large number of pharmacologically active components. Protective systems have evolved to detoxify and eliminate these xenobiotics. Among them is the cytochrome P450 system and the transporter p-glycoprotein in intestine and liver that control the absorption, biotransformation and elimination of drugs. Components of phytotherapeutic preparations can interfere with the function of these systems and lead to interactions with drugs. St John's wort, for example, induces the expression of p-glycoprotein and CYP3A4 in liver and intestine and thereby decreases the activity of other drugs. Garlic extracts as well may decrease the activity of drugs that are substrates for CYP3A4. In contrast, grapefruit juice inhibits intestinal CYP3A4. This results in a higher bioavailability of some drugs and possibly more adverse effects. Some relevant interactions were only detected after many years of widespread use, indicating that the treating physician should not only inquire about a change in co-medication but also about the use of alternative medicines or a change in dietary habits when a patient presents with unexpected and unusual adverse effects or a sudden loss of drug efficacy. It would be desirable if more information regarding the potential for interactions with commonly used drugs was available prior to registration of new phytotherapeutic preparations in order to document their safety for patients who require continuous treatment with a drug because of a chronic disease.

A bile acid protects against motor and cognitive deficits and reduces striatal degeneration in the 3-nitropropionic acid model of Huntington's disease.

There is currently no effective treatment for Huntington's disease (HD), a progressive, fatal, neurodegenerative disorder characterized by motor and cognitive deterioration. It is well established that HD is associated with perturbation of mitochondrial energy metabolism. Tauroursodeoxycholic acid (TUDCA), a naturally occurring bile acid, can stabilize the mitochondrial membrane, inhibit the mitochondrial permeability transition, decrease free radical formation, and derail apoptotic pathways. Here we report that TUDCA significantly reduced 3-nitropropionic acid (3-NP)-mediated striatal neuronal cell death in cell culture. In addition, rats treated with TUDCA exhibited an 80% reduction in apoptosis and in lesion volumes associated with 3-NP administration. Moreover, rats which received a combination of TUDCA + 3-NP exhibited sensorimotor and cognitive task performance that was indistinguishable from that of controls, and this effect persisted at least 6 months. Bile acids have traditionally been used as therapeutic agents for certain liver diseases. This is the first demonstration, however, that a bile acid can be delivered to the brain and function as a neuroprotectant and thus may offer potential therapeutic benefit in the treatment of certain neurodegenerative diseases.

Thin basement membrane disease and acute renal failure secondary to gross hematuria and tubular necrosis.

A patient with thin basement membrane disease (TBMD), macroscopic hematuria, and acute renal failure is described. A renal biopsy showed massive occlusion of renal tubules by red blood cells and casts. This was accompanied by tubular cell damage consistent with acute tubular necrosis. The patient was receiving warfarin because of a history of deep venous thrombosis at the time he developed the acute renal failure. The possible relationship of the warfarin therapy to the TBMD, intratubular hemorrhage, and acute renal failure are discussed.

[Suggestions for the health care system of the future: family-oriented primary health care]. / Vorschläge für das Gesundheitssystem der Zukunft: Familienorientierte Primary Health Care.

This paper will describe one of the major trends in health care delivery: family oriented primary care. Implications and future directions for practice, education and research in the United States and Germany will be described and contrasted. The paper is a result of an ongoing dialogue between the first author, a Fulbright Professor at the University of Witten/Herdecke, and the second and third authors. The authors believe that, contextualizing emerging trends in health care delivery in this way, helps to clarify both what is unique to each country and what is actually or potentially shared in common.

Hamartoma of mature cardiac myocytes: a case report.

A 24-year-old man presented with hypertension, palpitations, and premature atrial and ventricular contractions. A mass was discovered in the distal interventricular septum that was composed of dense collagenous tissue, fat, and disorganized, hypertrophic, mature cardiac myocytes indicative of a cardiac hamartoma. This entity has only rarely been reported and must be distinguished from the much more common rhabdomyoma and from oncocytic cardiomyopathy, which is also referred to as "cardiac hamartoma."

Newer glomerular diseases.

Three recently described primary, non-immune-mediated glomerular diseases are reviewed. These three distinct forms of glomerulopathy include collagen type III glomerulopathy, fibronectin glomerulopathy, and lipoprotein glomerulopathy. The three entities are characterized pathologically by the glomerular deposition of substances either produced or trapped in the renal glomerulus. All three diseases usually present with proteinuria or nephrotic syndrome, and are slowly progressive. Collagen type II and fibronectin glomerulopathy are familial diseases, and lipoprotein glomerulopathy may be familial or sporadic.

Transvascular catheter infiltration of the ductus arteriosus with formalin in the newborn lamb: a novel method of establishing and maintaining ductal patency.

OBJECTIVES: This study sought to establish and maintain patency of the ductus arteriosus by a new method of transvascular formalin infiltration of the ductus arteriosus wall. BACKGROUND: Maintaining patency of the ductus arteriosus postnatally is necessary with many forms of congenital heart disease to ensure survival until definitive surgical repair. A variety of approaches have to date met with variable success. METHODS: Seven newborn lambs underwent catheterization at 1 to 5 days of age. A functionally closed ductus arteriosus was traversed with a specially designed porous balloon catheter, and the wall was infiltrated with 10% formalin. The ductus was then further dilated with either a 7- or 8-mm diameter balloon. RESULTS: The ductus arteriosus wall was successfully infiltrated and dilated in all animals. Two lambs were euthanized within 24 h with congestive heart failure from a large ductus, and one lamb was electively euthanized 5 days after transvascular infiltration. Four lambs underwent serial follow-up catheterizations, one of which required repeat balloon dilation 47 days after infiltration. At latest follow-up (mean age [+/- 1 SD] 83 +/- 34 days, range 33 to 108), the pulmonary/systemic flow ratio (2.7 +/- 1.2) was unchanged from immediately after infiltration (1.9 +/- 0.5, p > 0.1). The narrowest diameter of the ductus arteriosus (4.3 +/- 0.4 mm vs. 6.9 +/- 2.6 mm, p > 0.1) and its ratio to that of the adjacent descending aorta (0.5 +/- 0.1 vs 0.4 +/- 0.1, p > 0.1) were also unchanged at latest follow-up. No systemic toxicity was observed. At postmortem study, the ductus was patent, and histologic analysis showed variable intimal and medial destruction, endothelial regeneration, loss of elastic tissue and calcification. Adjacent pulmonary artery and aorta were normal. Based on the small sample size and the observed maximal effect size of 70%, the power of the study is at most 40% to detect significant differences. CONCLUSIONS: To our knowledge, this is the first time that transvascular formalin infiltration of the ductus arteriosus wall has been applied successfully to maintain ductal patency in the newborn lamb. This method may provide a means of palliation for neonates with certain forms of congenital heart disease.

Water-clear cell adenoma of the parathyroid. A case report with immunohistochemistry and electron microscopy.

We report a water-clear cell adenoma of the parathyroid gland, a lesion which to our knowledge has not been described previously. Like its rare but well-described hyperplastic counterpart, water-clear cell hyperplasia, this adenoma is composed of cells with abundant foamy-to-granular cytoplasm and mild nuclear pleomorphism. The cells form glandular structures and cell nests separated by fine fibrovascular septae. The tumor cells stain positively with anti-parathyroid hormone and show characteristic glassy and flocculate material by electron microscopy. Unlike water-clear cell hyperplasia, water-clear cell adenoma is a solitary lesion that compresses the residual nonneoplastic parathyroid gland.

Glomerulopathy associated with predominant fibronectin deposits: a newly recognized hereditary disease.

A newly recognized type of familial glomerulopathy observed in patients of both sexes in six families is reported. Proteinuria, often within the nephrotic range, microscopic hematuria, hypertension and a slowly decreasing renal function over several years were common. No underlying systemic diseases were identified. Generally, light microscopy showed enlarged glomeruli with minimal hypercellularity and with extensive deposits in the mesangium and subendothelial space. By electron microscopy, granular deposits with some admixture of fibrils were most common. In one family, the deposits were predominantly fibrillary. Immunoglobulins and complement factors were inconstant or lacking. A main finding was a strong immune reactivity to fibronectin, corresponding to the distribution of the deposits. In one patient, the deposits recurred in a renal transplant. There was no indication of systemic deposition. Abnormalities in the metabolism of circulating fibronectin may play a pathogenetic role in this disease of probably autosomal dominant inheritance.

The effect of interinstitution anatomic pathology consultation on patient care.

OBJECTIVE: To determine the effect of routine interinstitution anatomic pathology consultation on patient evaluation and treatment. DESIGN: All interinstitution anatomic pathology consultation diagnoses made during a 1-year period were compared with the original pathologic diagnoses. Patients with discrepant diagnoses were evaluated after an interval of 1 year to determine the correct clinical diagnosis. The relevance of the pathologic consultation to furthering medical evaluation and treatment was determined from a review of the medical record and when necessary from consultation with the patient's physician. SETTING: Patients referred to a university hospital. MAIN OUTCOME MEASURES: We determined the number of patients with discrepant pathologic diagnoses and whether these diagnoses changed the planned surgical procedure, chemotherapy, radiation therapy, or medical evaluation. RESULTS: Seventy-one (9.1%) discrepant diagnoses were identified among the 777 patients. Of these 71 patients, 45 (63%) demonstrated a change in therapy or clinical evaluation as a result of the interinstitution anatomic pathology consultation. In five of these patients the consultation diagnosis was in error. There was a significantly greater percentage of discordant diagnoses among the cytology and fine-needle aspiration biopsies (21%) as compared with the surgical pathology specimens (7.8%; P < .001). CONCLUSIONS: Routine interinstitution anatomic pathology consultation resulted in a change in patient evaluation or treatment in 45 (5.8%) of the 777 cases reviewed. Our interinstitution anatomic pathology consultation policy appears to provide useful diagnostic information, which should contribute to improved patient care. However, when a discrepancy is identified, additional consultation or evaluation should be considered.

Chinese herbal medicine induced acute renal failure.

A patient with acute interstitial nephritis secondary to ingestion of a Chinese herbal medicine adulterated with mefenamic acid is presented. Following hemodialysis and cessation of the medication the patient's renal function returned to normal.

Epidermal growth factor enhances intestinal mitotic activity and DNA content after acute abdominal radiation.

BACKGROUND: Mediators of radiation-induced enteritis and colitis remain undefined. Epidermal growth factor (EGF) is an endogenous peptide that is trophic to the gastrointestinal tract. We tested the hypothesis that EGF enhances DNA synthesis and mitotic activity and prevents acute radiation enteritis after total abdominal radiation. METHODS: Four equal groups (n = 6) of Sprague-Dawley rats were studied: I (control), II (radiation), III (EGF), and IV (radiation + EGF). Animals in groups III and IV received EGF (10 micrograms/kg) every 8 hours for 48 hours before radiation exposure and for 72 hours after radiation, and the remaining animals were given an equal volume of vehicle. Animals in groups II and IV were administered a single dose of abdominal radiation (1000 cGy) 48 hours after the start of either vehicle or EGF. Distal ileum and colon were harvested 72 hours after radiation, examined histologically, and assayed for total DNA content. RESULTS: Group II or radiated animals had diarrhea, significant weight loss (p < 0.05), and decreased food consumption consistent with acute clinical radiation enteritis. Mitotic activity and total DNA content were significantly reduced (p < 0.05) when compared with group I (nonradiated controls). Group IV animals treated with EGF and exposed to radiation did not suffer the acute clinical manifestations of radiation enteritis. In addition, total DNA content and mitotic activity of the terminal ileum increased significantly (p < 0.05), and a significant increase in mitotic activity occurred in the distal colon when compared with radiated controls. CONCLUSIONS: The results of this study suggest that (1) a decrease in mitotic activity and total DNA content occurs early and persists for at least 72 hours after acute radiation, (2) EGF treatment significantly increases small and large bowel mitogenicity in acutely radiated animals, and (3) EGF significantly decrease the acute clinical manifestations of radiation enteritis.

Fetal rhabdomyoma and nevoid basal cell carcinoma syndrome.

A 6-year-old white female presented with a fetal rhabdomyoma of the posterior mediastinum and retroperitoneum. Radiologic evaluation and family history revealed features of the nevoid basal cell carcinoma syndrome (NBS). Literature review disclosed two other children with NBS and fetal rhabdomyoma, which should be regarded as one of the soft tissue tumors associated with NBS.

Familial lobular glomerulopathy.

A family with an unusual lobular glomerulopathy is described. Renal tissue from three males and one female in two successive generations was available for review. The glomerulopathy was characterized by a marked lobular accentuation with only a modest increase in mesangial cellularity. Immunofluorescence in two patients showed focal or diffuse staining with immunoglobulins G, A, M, and C3 in the mesangium and along the glomerular capillary basement membranes. Ultrastructural study showed amorphous granular subendothelial material distending capillary loops and mesangial regions. This material accounted for the pronounced lobular accentuation. The patients in this family presented with proteinuria, hematuria, and hypertension. Three of the four patients have sustained cerebral vascular accidents and two have died. This family is compared with a previously reported family that showed similar glomerular pathology.

Results of aortic anastomoses made under tension using polydioxanone suture.

After early repair of congenital cardiovascular defects, such as coarctation of the aorta, late stenosis may become a problem. Use of absorbable sutures has been shown to be superior to use of nonabsorbable sutures in allowing growth of an anastomotic site along with the individual. Some concern has been raised, however, about the potential for aneurysm formation at the site of anastomosis when absorbable sutures are used. This study was undertaken to observe the effects of longitudinal tension on anastomoses made with absorbable polydioxanone suture in growing animals. Six piglets (aged 3 to 4 weeks) underwent a 1-cm resection of the infrarenal aorta and reanastomosis with polydioxanone suture. One animal died prematurely of respiratory illness. The 5 remaining animals were killed after 6 months. The excised aortas demonstrated no stenosis, no dilatation, and no burst-test failure to 250 mm Hg. Histological examination showed disrupted elastic laminae without thinning of aortic wall in all samples of aorta. We conclude that polydioxanone suture is a suitable suture material for vascular anastomoses made under tension where growth of the anastomotic site is expected.

A patient with multiple, malignant melanomas of the lower genital tract.

Malignant melanomas of the female genital tract are uncommon lesions. A patient with multiple cutaneous, vulvar melanomas and a subsequent malignant melanoma of the cervix is described.

An improved method for mounting frozen-section specimens.

We describe an efficient method for embedding frozen-section specimens. Transparent tape is used to produce a well on the surface of the tissue holder ("chuck"). This well contains a quantity of nonmotile mounting media in which a tissue specimen can be easily oriented. After freezing, the tape is removed, leaving a relatively smooth surface.