Pathognomonic Alterations Research Articles

ZNS-Infektionen bei immunsupprimierten Patienten

CNS infections caused by infective agents are rare in immunocompetent hosts, but more frequent in immunocompromised patients. In addition, the spectrum of causative agents is completely different. There are no pathognomonic alterations in radiologic imaging, even in clinically severely ill patients imaging is often non-specific or inconspicious. This article gives a review of the most frequent infective agents and image alterations. Modern radiology is not yet able to replace the gold standard of pathogen detection.

Electron microscopy in the diagnosis of lysosomal storage diseases.

Lysosomal storage diseases (LSD) affect cells of the conjunctiva in the absence of clinical eye findings. Consequently conjunctiva was added to skin, rectal mucosa, and peripheral blood lymphocytes as a biopsy site for electron microscopic examination in suspected LSD. The abnormally stored substrates in LSD are the result of diminished lysosomal enzyme activity. Although all cells lack the deficient lysosomal enzyme, the quantity of stored material varies with the extent of breakdown of substrate in the cell under normal circumstances. The stored material does not accumulate in all cells and may have a different cellular morphology at different sites. Although no one tissue provides universally diagnostic material for electron microscopy, skin and conjunctiva are optimal for most LSD. Rectal mucosa and peripheral blood lymphocytes are also useful in many cases. Biochemical assays have replaced brain biopsy for diagnosis but these techniques may give equivocal or non-diagnostic results, and in some diseases the enzyme defect remains undefined. Of the 359 biopsies of conjunctiva, skin, rectal mucosa, and lymphocytes we evaluated for LSD and other neurodegenerative diseases, 65 showed abnormal lysosomal storage; in 41 a specific diagnosis was made by biochemical assay or morphology. Ultrastructural examination of tissue from patients with clinically suspected storage disease may disclose pathognomonic alterations or suggest a differential diagnosis even in the absence of clinically evident involvement of the biopsied tissue. Biopsy has particular value in those diseases with incompletely characterized biochemical abnormalities.

Kinetics of 64Copper in primary biliary cirrhosis.

To assess the pathogenetic mechanisms involved in the accumulation of copper in primary biliary cirrhosis (PBC), the kinetics of 64Copper (64Cu) were studied in 6 healthy volunteers, 3 patients with PBC (Stages I to III) and a normal liver copper, and 7 patients with PBC (Stages II to IV) and a high liver copper. The kinetics of oral and i.v. administered 64Cu in patients with a normal liver copper were similar to those in controls. In patients with a high liver copper, the initial disappearance rate from the plasma of i.v. administered 64Cu was more rapid, the disappearance rate from the liver of 64Cu taken up by this organ was slower, and the fecal excretion of 64Cu less as compared with the two other groups of subjects. In addition, the fecal excretion of orally administered 64Cu was less in these patients. Computation of the rate of absorption of orally administered 64Cu revealed no differences between the three groups. Two of the patients with a high liver copper were decopperized by treatment with D–penicillamine. In these patients, the disappearance rate of i.v. administered 64Cu from the liver and the secondary reappearance rate of 64Cu into plasma became normal; however, the fecal excretion of 64Cu remained low. It is concluded that there is no pathognomonic alteration of copper metabolism in PBC, but that in some patients, copper accumulates in the liver due to decreased biliary excretion and increased clearance from the plasma, and that dilution of the tracer dose a large amount of stable copper is responsible for all abnormalities of 64Cu kinetics encountered in PBC, except low fecal excretion, which is caused by impairment of biliary excretion.

Sialoangiography: x-ray visualization of major salivary glands.

AbstractSialoangiography is demonstrated to be useful in the diagnosis and evaluation of inflammatory processes, and tumors of the major salivary glands and their areas. Parenchymal changes as well as space occupying lesions within the glands, and tumors exerting pressure from outside the gland are shown to manifest their specific changes by their effect upon the ducts, with pathognomonic alterations and distortion of the ducts or normal architecture of the ductal system.Intrinsic duct changes are all due to ductal obstruction and a wide variety of causes is shown.The normal findings, including minor anatomical variations is contrasted with abnormal sialoangiographic findings. These abnormalities are evidenced by changes in the duct morphology and the course of the ducts altered by the presence of intrinsic, extrinsic, or deep lobed tumors.The technique of sialoangiography is discussed. The author's method is outlined, including specific views and special methodology.

Xanthomatous Biliary Cirrhosis in the Male

Xanthomatous biliary cirrhosis or biliary cirrhosis with xanthomatosis is a rare form of cirrhosis which attracts unusual interest because of the marked and pathognomonic alterations of the plasma lipids and the conspicuous and grotesque skin lesions. This report is prompted by the rarity of this disease in the male and the rapid improvement in one of these patients following exploratory laparotomy.

SUPPURATIVE ANTERIOR MEDIASTINITIS IN AN INFANT FOLLOWING INTRASTERNAL BLOOD TRANSFUSION

HISTORY OF INTRAMEDULLARY THERAPY At least as far back as 1903 Wolff 1 practiced marrow puncture on laboratory animals, and in 1910 Ghedini 2 reported an experience of several years in obtaining specimens of bone marrow from 24 living human patients through puncture holes in the tibia. He recommended the method for the diagnosis of blood dyscrasias without pathognomonic alterations in the circulating blood and for the diagnosis of kala-azar. In 1923 Seyfarth, 3 who had been seeking a substitute for splenic puncture in the diagnosis of kala-azar, described his use of a small hand trephine and a platinum wire loop for obtaining specimens of sternal marrow with the patient under local anesthesia. His discovery foreshadowed the observations of Debre and his associates 4 that sternal puncture in man gives more frequent positive cultures for the diagnosis of septicemia than does venipuncture. In 1928 Sonnenfeld 5 described a very small

Adrenal cortical hyperfunction

1. 1. Five cases of adrenal cortical hyperfunction treated surgically by unilateral adrenalectomy are described. Four of the patients were benefited and one died. 2. 2. Histologic examination revealed a pathognomonic alteration of the reticular zone. 3. 3. The symptomatology is reviewed, and the pathogenic relationship of the pituitary, ovary and adrenal to the disorder is discussed. 4. 4. A differential diagnosis between this cortical hyperfunction, basophilic adenoma, arrhenoblastoma and related endocrinopathies is presented. 5. 5. The operative procedure is described.