Myelolipoma: A New Adrenal Finding in Carney's Complex?

Pigmented nodular cortical hyperplasia, a rare cause of Cushing’s syndrome, is characterized by resistance to inhibition with dexamethasone and normal sized adrenal glands with multiple, small pigmented nodules. The disorder may be a component of a syndrome inherited as an autosomal dominant pattern that includes intra- and extracardiac myxomas, lentiginous lesions, blue nevi, other functional endocrine tumors, and peripheral nerve tumors (Carney’s complex). We report a patient in whom bilateral myelolipomas were found, in addition to the usual features of this complex. A 29-yr-old man was admitted to the hospital for Cushing’s syndrome of probably more than 15 yr duration. Physical examination showed diffuse facial hyperchromatic macules, 0.2–0.5 cm, predominantly around the lips and on the palmar surfaces of the fingers. Results with dexamethasone suppression nocturnal testing (1 and 8 mg) were compatible with an adrenal adenoma. The computed tomography (CT) of the sella turcica was normal. Adrenal CT showed a tumor in the left gland with a double component: one solid and another suggestive of fat, consistent with an angiomyelolipoma. Following 5 wk treatment with ketoconazole, 800 mg per day po, serum cortisol decreased to 5.9 μg/dL, morning and evening, respectively. Bilateral adrenalectomy was performed. Pathologic examination revealed pigmented nodular cortical hypersplasia and a dominant myelolipoma in the left adrenal. A microscopic myelolipoma was identified in the right adrenal. An echocardiogram showed a mass on the posterior wall of the left ventricle which was a myxoma. Study of the patient's family disclosed two sisters with facial lentigines. Echocardiograms were performed on all available first degree relatives: all were normal. Nocturnal inhibition with dexamethasone revealed that one of the patient’s sisters with lentigines also had hypercortisolism. Myelolipoma has been reported in association to Cushing syndrome in humans and experimentally after pituitary extracts in animals. The relationship between this finding and the Carney’s complex remain elusive.     

Cytogenetic and molecular studies of a familial renal cell carcinoma.

In a previously studied family with inherited renal cell carcinoma (RCC), RCC was shown to segregate with a constitutional balanced t(3;8)(p14.2;q24.1). In addition, we recently showed that in a RCC tumor from this family the constitutional translocation became unbalanced, suggesting a genetic mechanism that may be associated with the primary genetic events of tumorigenesis. We now report that the RCC tumor cells from this case showed additional cytogenetic alterations, possibly related to tumor progression, which include an additional tumor-specific translocation involving band 14 of chromosome 13. Because this band contains the retinoblastoma (RB) gene, we examined the tumor for aberrations in the RB gene using DNA sequence polymorphism analysis and pulsed-field gel electrophoresis (PFGE), but did not detect alterations in the RB gene.     

The influence of age on neurotransmitter turnover in the rat's superior colliculus.

Measurements of the turnover of dopamine, noradrenaline and serotonin and their metabolites have been performed in the superior colliculus of adult and aged rats. The turnover of dopamine, noradrenaline and their metabolites after pargyline treatment was significantly lower in aged rats than in adults. On the contrary, the synthesis rate of serotonin (measured by accumulation of 5-hydroxytryptophan after decarboxylase blockade) and the turnover rate of serotonin (after pargyline treatment) did not change during aging. These findings suggest that aging has a different effect on catecholamines and serotonin turnover in the superior colliculus of the aged rats.     

Electron microscopy renders the diagnostic capabilities of cytopathology more precise: an approach to everyday practice

Cytology is a powerful diagnostic tool but to make definitive diagnoses, the use of ancillary techniques is imperative. By combining immunohistochemistry (IHC) and electron microscopy (EM), cytologic diagnoses can be as precise as those of surgical pathology. In the authors' daily practice of cytopathology they use all ancillary techniques available to them: histochemistry, IHC, EM, flow cytometry, and molecular pathology. IHC is frequently used as an ancillary technique in their daily practice but EM is many times their technique of choice. By the use of EM the authors can make specific final diagnoses, make the diagnosis more definitive, narrow the differential diagnosis, or determine the origin of a neoplasm with unknown primary site. Specimens obtained by fine-needle aspiration as well as all body fluids are suitable for EM. The limiting factor is to obtain the appropriate material with the diagnostic cells for ultrastructural examination. The common diagnostic dilemmas in the everyday practice of cytology are the following: mesothelioma vs. adenocarcinoma, neuroendocrine differentiation or not, the distinction of melanoma from adenocarcinoma and sarcoma, hepatocellular carcinoma vs. adenocarcinoma, and the origin of adenocarcinomas of unknown primary. The authors discuss how they approach these diagnostic problems in their everyday practice and how they incorporate EM in solving them.     

The use and effects of vital fluorescent dyes: observation of motor nerve terminals and satellite cells in living frog muscles

Summary Several different fluorescent mitochondrial dyes were tested as vital stains for motor nerve terminals and other cells in frog skeletal muscles. It was found that 3,3diethyloxadicarbocyanine iodide and 4-(4-diethylaminostyryl)-N-methylpyridinium iodide were most useful. Both dyes labelled motor nerve terminals with high reliability. Electrophysiological and morphological control experiments showed that these dyes could be used to repeatedly observe neuromuscular junctions in living animals without affecting synaptic growth or remodelling. The importance of appropriate controls was emphasized by the finding that illumination, if excessively intense or prolonged, can cause physiological and structural damage to nerve terminals. Additional observations indicated that these dyes may be useful for determining the mitochondrial content, and therefore oxidative capacity, of living muscle fibres. It was also found that the fluorescent dyes labelled cells identified as muscle satellite cells, and that these myoblast precursors could be visualized in fixed whole mounts with a nitroblue tetrazolium stain. Both methods were used to study reactive cells that were closely associated with muscle fibres in lesioned muscles. Mitochondrial dyes also labelled the microvasculature, associated axons and other cells.     

The prevalence of serological markers for the human immunodeficiency virus and the hepatitis B virus in a psychiatric hospital

OBJECTIVES: To estimate the prevalence of antibodies to human immunodeficiency virus (HIV), antibodies to hepatitis B core antigen (anti-HBc), hepatitis B surface antigen (HBsAg), and antibodies to delta virus (anti-DV). DESIGN: Cross-sectional (prevalence) study. A non-proportional (stratified by length of stay) random sample of 20% of admitted patients was selected. CLINICAL SETTING: The Hospital Psiquiátrico Nuestra Se?ora de Montserrat (Sant Boi de Llobregat, Barcelona, Spain) admits about 670 patients in its short-term, rehabilitation (mid-term) and long-term wards. SUBJECTS: 139 patients were selected: 91% were males, mean age was 55 years, and 10% belonged to an HIV risk group; mean length of hospitalization since last discharge was 13 years. MEASUREMENTS: Blood samples were drawn in October, 1988. Anti-HBc, HBsAg and anti-DV were determined by competitive enzyme immunoassay (EIA). Anti-HIV-1 were determined by sandwich EIA; negativity of results with high absorbency but below the cut-off point was confirmed by Western blot. RESULTS: None of the sampled patients had circulating anti-HIV-1 antibodies. Four cases showing high absorbency (below the "cut-off" point) were ruled out by Western-blot. Overall, the prevalence of anti-HBc was 52.8% (54.4% in long-term wards, 35.0% in mid-term wards, and 13.8% in short-term wards). The prevalence of HBsAg was 2.3%, of anti-DV 1.5%, and of HDAg 0%. CONCLUSIONS: Prevalence of anti-HIV is null in the studied institution. Current efforts to prevent HIV infection must continue; a hepatitis B vaccination program is highly warranted among patients and professionals of the studied hospital.     

Utilisation of organs proceeding from donors with heart failure: review of the pathogenesis of organic damage and the possible mechanisms of prevention

There is an evident imbalance between the number of patients awaiting a kidney transplant and the availability of organs proceeding from donors with brain death. A high number of patients die each day from heart failure, whose organs could be used for transplants if specific care is employed. Although centres do exist where these methods of extraction are established, the problems of organic damage have yet to be resolved, since one third of the organs are still lost, besides the increase in the need for early dialysis, and the number of dysfunctioning grafts two years after the transplant, when this type or organ is employed. There is increasingly detailed knowledge of the pathogenesis of organic damage following heart failure and reanimation, as well as of the damage following the conservation and reimplantation of the kidney. Knowledge of the maximum time of hot ischemic that an organ can withstand is of crucial importance if organs are not to be unduly discarded. Besides, the increasing understanding of the physiopathology of oxidative stress could make it possible for us, through the use of antioxidants, to attempt to improve the utilisation of the organs and diminish the incidence of dysfunctions and rejections.     

‘Hybrid’ Bariatric Surgery: Bilio-pancreatic Diversion and Duodenal Switch - Preliminary Experience

Background: Hybrid, combined or mixed bariatric surgery is the combination of a degree of ‘malabsorption’ (as achieved by the intestinal bypass) with a ‘rrestriction’ (as achieved by gastric bypass or gastroplasty), thereby simultaneously reducing the absorption of fats in the small bowel and decreasing the intake of food. Methods: A modification of the bilio-pancreatic diversion (BPD) with a duodenal switch procedure, vertical lineal gastrectomy and preservation of the pylorus, has been used in 23 patients. The antropyloric pump and 4 cm of the duodenum are left intact to preserve physiologic gastric emptying and to prevent anastomotic ulcer. The use of staplers and continuous running sutures reduces surgical risks and operative time. Results: One patient, converted from a vertical gastroplasty, had an intrathoracic esophageal perforation and died of multisystemic organ failure, a mortality rate of 4.5%. One patient had a partial dehiscence of the laparotomy wound. Three patients developed subcutaneous seromas. Mean weight losses during the first 4 months were 13, 11, 6 and 5 kg, with a loss of 70% of excess weight in patients approaching 1 year. No patient needs treatment for diarrhea. No serious secondary side-effects have been detected. Conclusion: This operation appears to result in very satisfactory weight loss, improved quality of life, and a low incidence of complications.     

Adult-derived neural precursors transplanted into multiple regions in the adult brain

Neural stem cells persist in the adult brain subventricular zone (SVZ). These cells generate a large number of new neurons that migrate to the olfactory bulb, where they complete their differentiation. Here, we transplanted cells carrying beta-galactosidase under the control of neuron-specific enolase promoter (NSE::LacZ) from the SVZ of adult mice into the striatum cortex and olfactory bulb, with or without an excitotoxin lesion. Between 2 and 8 weeks after transplantation, grafted cells were present in the recipient regions, but extensive migration and differentiation into mature neurons of grafted cells were only observed in the olfactory bulb. Clusters of graft-derived neuroblasts forming chain-like structures were observed within or close to the grated sites in the cortex and striatum; electron microscopy confirmed that graft-derived cells in the olfactory bulb and a small number in the striatum were neurons. Surprisingly, most of the cells expressing NSE::LacZ outside the olfactory bulb were astrocytes. We conclude that primary precursors from the SVZ migrate and differentiate effectively only within the environment of the olfactory bulb. Only limited survival and differentiation were observed in other brain regions studied.