Síndrome de Nelson: una causa infrecuente de hiperpigmentación cutánea generalizada / Nelson syndrome: a rare cause of generalized hyperpigmentation of the skin

El síndrome de Nelson (SN) supone una causa infrecuente de hiperpigmentación mucocutánea generalizada, cuyas manifestaciones clínicas se derivan de la secreción excesiva de corticotropina por un adenoma hipofisario secundario a la realización de una suprarrenalectomía bilateral terapéutica. Debido a que esta intervención quirúrgica ha caído en desuso en la actualidad, su presentación es hoy sumamente rara y poco reconocible. Presentamos un caso muy grave de hiperpigmentación generalizada por SN en una paciente de 37 años (AU)

Nelson syndrome is a rare cause of generalized mucocutaneous hyperpigmentation. Its clinical manifestations are due to excessive secretion of adrenocorticotropic hormone from a pituitary adenoma, which develops after bilateral therapeutic adrenal ectomy. As this operation has fallen into disuse, Nelson syndrome is now extremely rare and difficult to recognize. We present a very severe case of generalized hyperpigmentation due to Nelson syndrome in a 37-year-old woman (AU)

[Nelson syndrome: a rare cause of generalized hyperpigmentation of the skin]. / Síndrome de Nelson: una causa infrecuente de hiperpigmentación cutánea generalizada.

Nelson syndrome is a rare cause of generalized mucocutaneous hyperpigmentation. Its clinical manifestations are due to excessive secretion of adrenocorticotropic hormone from a pituitary adenoma, which develops after bilateral therapeutic adrenalectomy. As this operation has fallen into disuse, Nelson syndrome is now extremely rare and difficult to recognize. We present a very severe case of generalized hyperpigmentation due to Nelson syndrome in a 37-year-old woman.

Polychromatic X-ray microdiffraction studies of mesoscale structure and dynamics.

Polychromatic X-ray microdiffraction is an emerging tool for studying mesoscale structure and dynamics. Crystalline phase, orientation (texture), elastic and plastic strain can be nondestructively mapped in three dimensions with good spatial and angular resolution. Local crystallographic orientation can be determined to approximately 0.01 degree and elastic strain tensor elements can be measured with a resolution of approximately 10(-4) or better. Complete strain tensor information can be obtained by augmenting polychromatic microdiffraction with a monochromatic measurement of one Laue-reflection energy. With differential-aperture depth profiling, volumes tens to hundreds of micrometers below the surface are accessible so that three-dimensional distributions of crystalline morphology including grain boundaries, triple points, second phases and inclusions can all be mapped. Volume elements below 0.25 microm3 are routinely resolved so that the grain boundary structure of most materials can be characterized. Here the theory, instrumentation and application of polychromatic microdiffraction are described.

Gold vapor laser versus tunable argon-dye laser for endobronchial photodynamic therapy.

BACKGROUND AND OBJECTIVE: To compare the effectiveness of a gold vapor pulsed laser versus an argon dye continuous wave laser system in decreasing the amount of obstruction caused by endobronchial tumors when they are treated with photodynamic therapy (PDT). STUDY DESIGN/MATERIALS AND METHODS: The percentage of endobronchial obstruction from tumors before and at the end of PDT, and before and at the end of toilet bronchoscopy of 96 sites treated with light generated by a Spectra Physics tunable argon dye system were compared to those of 17 sites treated with light generated by a gold vapor laser. All patients were injected intravenously with 60 mg of dihematoporphyrin ethers per square meter of body surface. All treatments were done with a power density of 500 mW/CF and a light dose of 400 J/CF delivered from cylinder diffusing fibers. RESULTS: Paired Student's t-tests and Wilcoxon signed ranks tests showed significant decreases in the percentage of endobronchial obstruction due to PDT regardless of the laser used. Unpaired Student's t-tests and Mann-Whitney U statistical comparisons showed no significant difference between the two lasers in the percentage decrease at PDT, percentage increase from exudate seen at toilet bronchoscopy, nor the percentage decrease at the end of the toilet bronchoscopy from that before PDT. CONCLUSION: We found no statistically significant difference in the decrease in the amount of endobronchial tumor obstruction obtained when the light for treatment was generated by a pulsed gold vapor or a continuous wave argon dye laser system.

[The dynamics of intravascular thrombocyte aggregation in photodynamic exposure]. / Dinamika vnutrisosudistoi agregatsii trombotsitov pri fotodinamicheskom vozdeistvii.

The alterations taking place in the microvessels of the rat mesentery and liver under a photodynamic effect, were studied. Excitation of a photosensitizer injected preliminarily in the organism led to aggregation of thrombocytes in the lumen of hepatic and mesenteric microvessels until a complete obturation of the lumen. The dependence of the thrombocyte aggregate size on the duration of the photodynamic effect had a linear character whereas the photodynamic effect depended on the dosage of the photosensitizer. The level of fluorescence from the surface of the liver was found to decrease from the beginning of the photodynamic effect and become stable in hemostasis.

Photodynamic therapy to treat tumors of the extrahepatic biliary ducts. A case report.

The poor survival rate of patients with extrahepatic bile duct tumors is well documented. Over the course of 4 years, we treated a white woman with diabetes diagnosed with histologically proven adenocarcinoma of the common bile duct with six injections of dihematoporphyrin ether followed by seven photodynamic therapy treatments to the biliary duct. As of July 1989, the patient was still alive, was not jaundiced, and had a Karnofsky performance status of 70. No changes occurred in any blood chemistry value from the time of injection to the time of photodynamic therapy. Of the transient elevations of some blood chemistry values and the white blood cell count, which occurred within 24 to 48 hours after photodynamic therapy, only those of alanine aminotransferase, aspartate aminotransferase, and amylase were significant.

Nd:YAG laser and photodynamic therapy for esophageal and endobronchial tumors under general and local anesthesia. Effects on arterial blood gas levels.

STUDY OBJECTIVE: Our objective was to determine the respiratory and acid-base metabolism response to endoscopic laser surgery for obstructive tumors, as related to the duration and different types of endoscopy, anesthesia, and laser treatment. DESIGN: The design was a case-control, cohort analytic, nonrandomized controlled survey of case series before and after endoscopic procedures. SETTING: A referral-based surgery and oncology practice at one hospital's laser center. PATIENTS: We studied a sequential sample of 82 patients in the age range from 35 to 92 years, with malignant and benign, primary and metastatic, partially and completely obstructing esophageal (15 patients) and endobronchial (67 patients) tumors. INTERVENTIONS: A total of 229 diagnostic, laser treatment, and follow-up endoscopic procedures was performed under general or local anesthesia (46 esophagoscopies and 183 bronchoscopies). The latter group consisted of 29 cases of general and 154 cases of local topical anesthesia. The last group involved 37 diagnostic and toilet bronchoscopies, 86 cases of YAG-laser tumor ablation, and 31 cases of PDT. MEASUREMENTS AND MAIN RESULTS: Direct-reading electrode measurements of arterial blood, sampled before and immediately after the endoscopic procedure, revealed statistically significant (p less than 0.001) increases in PaCO2 (200 of 229 cases) and decreases in pH (195 of 229 cases) and PaO2 (215 of 229 cases). These findings were similar after bronchoscopy and esophagoscopy, general and local anesthesia (only the decrease in pH was less pronounced in the latter case), and explorative endoscopies and different laser treatments and did not correlate with the total duration of the procedure within the wide time range of 7 to 210 minutes. The initial preoperative level of PaCO2 was considerably higher and the level of PaO2 was significantly lower in patients with endobronchial tumors, as compared to patients with esophageal cancer. A strong, inverse linear relationship was found between the perioperative changes in PaO2 and its initial level and between PaCO2 and pH changes. CONCLUSIONS: The PDT for esophageal and endobronchial malignancies is no more harmful for acid-base metabolism and respiratory functions than YAG-laser tumor ablation or any other common, nonlaser endoscopic procedure.

[The pharmacokinetics of fluorescein, hematoporphyrin and its derivative during the development of granuloma]. / Farmakokinetika fliuorestseina, gematoporfirina i ego proizvodnogo pri razvitii granulemy.

In experiments on rats with granuloma reproduced by subcutaneous implantation of foreign material there was shown a slowing of excretion from the organism as well as a considerable redistribution between the organs of fluorescein, hematoporphyrin and its derivative as compared with intact animals. The noted changes practically did not depend on the time of granuloma development.

[The pharmacokinetics of hematoporphyrin, its derivative and fluorescein during the development of carcinosarcoma]. / Farmakokinetika gematoporfirina, ego proizvodnogo i fliuorestseina pri razvitii kartsinosarkomy.

The pharmacokinetics of fluorescein, hematoporphyrin and a derivative of hematoporphyrin was studied microspectrofluorometrically on rats with transplanted carcinosarcoma. It was shown that in the rats in contrast to intact animals the tissue concentration of fluorescein exhibits a general tendency towards an increase, that of hematoporphyrin towards a decrease and that of hematoporphyrin derivative towards an increase in all parenchymatous organs and a decrease in the skin and mucosae of the hollow organs. At the equally short period of the maximal accumulation of luminophores, the tumour tissue differs from the normal one by a pronounced delay of excretion only of hematoporphyrin derivative.

[Pathogenesis of porphyrin-sensitized lethal and sub-lethal photo-injury]. / Patogenez sensibilizirovannogo porfirinami letal'nogo i subletal'nogo fotopovrezhdeniia.

By means of morphological methods the mechanisms of thanatogenesis were studied in 57 white mice after the hematoporphyrin derivative administration and xenon lamp radiation imitating the sun spectrum. In dependence of integrative doses of sensitized photoradiation and individual resistance of the organism there was observed immediate (hours), retained (days) and remote (weeks) death of animals. In the first two groups death came as a result of photocoagulation of plasma proteins and toxemic shock with liver, lung and brain injury, and in the third group--as a result of suppurative resorptive intoxication against the background of ulcerous skin necrosis.

[Various features of peroxidase in human saliva]. / Nekotorye svoìstva peroksidazy sliuny cheloveka.

Optimal conditions are determined for isolation and purification of peroxidase (EC 1.11.1.7) from the mixed human saliva by gel-filtration and ion chromatography. Such properties as pH and temperature optima, molecular weight, Km, amino acid composition, the effect of activators and inhibitors (purified saliva peroxidases and commercial preparation of horse-radish peroxidase "Reanal", Hungary) are studied with the use of different substrates. The data obtained are necessary to optimize production processes of isolation, storage and methods of the experimental and clinical application of peroxidases of different origin.

[The role of lysozyme and peroxidase in the regulation of the mitogenic activity of lymphocytes]. / O roli lizotsima i peroksidazy v reguliatsii mitogennoi aktivnosti limfotsitov.

The effect of the commercial preparations, egg white lysozyme and horse-radish peroxidase, as well as that of purified enzymes isolated from human saliva on lymphocyte biosynthesis of nucleic acids and protein was studied. Mitogenic activity of lymphocytes was found to be stimulated by crude preparations of egg white lysozyme and peroxidases of plant and animal origin. Lysozymes of the saliva and egg white purified by ion exchange chromatography and gel filtration had no such properties. As a result of egg white gel filtration, a new mitogen of the non-protein nature with a molecular weight several times higher than that of lysozyme was obtained.

[Source of enzymes of mixed human saliva]. / Proiskhozhdenie fermentov smeshannoi sliuny cheloveka.

Production of enzymes was studied in parotid, submandibular and mucosal salivary glands as well as in exfoliated epithelial cells, emigrating leukocytes and in dental bacterial plaque. alpha-Amylase and iodide peroxidase were mainly secreted into mixed saliva by parotid and submandibular glands. Alkaline phosphatase was produced mainly by mucosal salivary glands. These glands secreted also about one half of alkaline and acid proteases, acid phosphatase, both ribonucleases, lysozyme, alkaline peroxidase and lactate dehydrogenase. Role of leukocytes and epithelial cells as well as bacterial plaque was relatively negligible in formation of mixed saliva enzymes.