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Biomedical subjects

R Kleinert

Publications and source records attributed to R Kleinert.

At least 37 records · Page 2Linked to original sources

The spectrum of age-associated brain abnormalities: their measurement and histopathological correlates.

Magnetic resonance imaging (MRI) has dramatically increased our ability to detect morphological abnormalities in relation to aging of the brain. Among those changes are alterations of the white matter which display high signal intensity on both proton density and T2-weighted images. They may be seen in the deep and subcortical white matter or in a periventricular location. In clinically asymptomatic individuals the reported prevalence ranges from 20% to 60% for deep and subcortical white matter hyperintensities and from 15% to 94% for periventricular changes. Besides different characteristics of the populations examined these wide ranges are a consequence of quite diverse rating schemes and measurement approaches. Inadequate grading of MRI hyperintensities may also explain some of the inconsistencies in the reported associations of white matter damage with cerebrovascular risk factors or cognitive functions. Therefore development of a commonly accepted rating scheme would be desirable. Histopathologic observations could lay the basis. Hyperintense periventricular capping of the frontal horns and a smooth halo of periventricular hyperintensity have been linked to disruption of the ependymal lining, subependymal gliosis and concomitant loss of myelin. Punctate lesions in the deep and subcortical white matter corresponded to minor perivascular reduction in myelin content possibly because of a lower permeability of thickened arteriolar walls. Larger patchy and confluent hyperintensities, however, appear to indicate more extensive ischemic damage consistent with advanced microangiopathy. In parallel, newer MRI techniques may also contribute to the delineation and separation of these various types of tissue alteration.

Aging↗

Primary paraganglioma simulating pituitary macroadenoma: a case report and review of the literature.

A 76-year-old woman presented with an intrasellar and suprasellar mass which caused deterioration of visual acuity and bitemporal visual field defects. Transsphenoidal and transcranial partial resection revealed a primary chemodectoma. This tumour is very rare in the sellar area, where there are normally no paraganglionic cells. We review the literature and discuss possible mechanisms for the development of this tumour.

Adenoma↗

[Immunohistologic studies of the vitreous humor].

BACKGROUND: Immunohistological staining of the vitreous is difficult because of its high water content. We present a method for immunohistological staining of celloidin-embedded eyes. Results from diabetic and non-diabetic eyes are demonstrated. METHOD: Diabetic and non-diabetic eyes were firstly immersed in formol and then slowly dehydrated using rising concentrations of glycerine (sink method). Subsequently, the whole globe was embedded in celloidin and cut into 200-micron sections. Control areas of interest were dissected from the 200 microns sections under a lightmicroscope. These specimens were then embedded in paraffin and cut into 7-micron sections. The 7-micron sections were immunohistochemically stained for type I-collagen, type IV-collagen, fibronectin and laminin. RESULTS: This method makes immunohistochemical staining of the vitreous possible. Type IV collagen, laminin and fibronection were found at higher concentrations in diabetic eyes than in normal eyes. Type I collagen was detected in neither diabetic nor in normal eyes. CONCLUSIONS: Our method of examination allows immunohistological staining of the vitreous in its place of origin. Although our method is time consuming, it has some advantages over biochemical analysis: Even minimal changes and their exact distribution can be detected. Our first results show that the vitreous is built up inhomogeneously and that pathological influence can cause structural changes.

Aged↗

[Epidemiology of transmissible spongiform encephalopathies (prion diseases) in Austria].

Between 1969 and 1996, transmissible spongiform encephalopathy was definitely diagnosed by autopsy and/or biopsy in 98 Austrian patients. The yearly incidence increased significantly in past years (1996: 1.41 cases per million inhabitants). This increase likely results from increased awareness in the medical community and effectuation of the diagnostic autopsy. The new variant of Creutzfeldt-Jakob disease (CJD), probably transmitted from bovine spongiform encephalopathy (BSE), has not occurred in Austria. The percentage of patients older than 70 years increased until 1989 and declined slightly thereafter. One patient received a dura mater graft 11 years before death. Another patient had familial CJD with a glutamatelysin mutation on codon 200 of the prion protein (PrP) gene PRNP. One more patient died from Gerstmann-Sträussler-Scheinker disease (GSS), three patients from fatal familial insomnia (FFI). Another patient received intramuscular injections of a purified RNA preparation (Regeneresen) produced from various organs including brain. The age at death symmetrically distributes around a median of 64 years. Two CJD patients were unusually young (27 and 30 years). Most patients (72.7%) died within 6 months of disease. Retrospectively, 81% of patients had clinical diagnostic criteria of probable or possible CJD (52% probably and 29% possible). In 19%, clinical criteria for CJD were not fully met. There is no case clustering with specific professional groups or geographic areas. However, residents of Vienna, or Vienna and Lower Austria, respectively, had CJD diagnosed twice or three times more frequently than the rest of the country, indicating regionally differing qualities of case retrieval.

Adolescent↗

Cerebral primitive neuro-ectodermal tumour following treatment of a unilateral retinoblastoma.

Cerebral primitive neuro-ectodermal tumour (PNET) occurring as a second primary malignancy in childhood is exceedingly rare. We present a 7-year-old boy who developed a proven supratentorial PNET five years after enucleation and radio-/chemotherapy for a sporadic, unilateral retinoblastoma with optic nerve invasion. The association with this malignant eye disease as well as the effect of irradiation and multi-agent chemotherapy on second tumour induction are evaluated.

Chemotherapy, Adjuvant↗

[Intestinal schistosomiasis, a facultative precancerous condition? Review of the literature with reference to Schistosoma japonicum associated rectum carcinoma].

After a latency period of 20 years, in a 39-year-old Austrian citizen of Chinese origin, a surgically removed rectal carcinoma, as well as the neighboring chronic inflammatory rectal mucosa with various degrees of dysplasia and one positive neighboring lymph node, showed helminthiasis in the histopathological examination, convincing us of a link between carcinoma and chronic helminthiasis. Whereas the etiological context between chronic infection by Schistosoma haematobium and endemic frequent urinary bladder carcinoma is considered a matter of fact, whether of not the incidence of intestinal carcinoma is increased in connection with chronic intestinal schistosomiasis is controversial. The etiological and pathogenetic link between helminthiasis and carcinoma should be considered in the same way as for other related inflammatory large-bowel diseases. In the sequence chronic inflammation-severe dysplasia, the formation of carcinoma could possibly occur. Besides a survey of trematodes parasitology and pathology, the link between rectal carcinoma and Schistosomiasis japonicum is pointed out by means of appropriate literature investigations.

Adult↗

Brain-stem auditory evoked potential monitoring. The increase of the stimulus artifact in the development of brain death: a biological phenomenon?

Brain-stem auditory evoked potentials (BAEPs) were recorded in 12 dead subjects (mean age, 72.6 +/- 14.8 years), 30.6 +/- 19.5 hours (range 9-70) after abolished systemic circulation. Death was due to cardiac failure (n = 10), intracerebral hemorrhage (n = 1) and larynx cancer (n = 1). The presence and amplitude of the stimulus artifact were evaluated. The mean (+/- SD) amplitudes of the stimulus artifact was 0.03 +/- 0.02 microV on the left side and 0.01 +/- 0.02 microV on the right side. These findings in accordance with previous studies on comatose patients and brain dead subjects confirm that the increase of the stimulus artifact in the development of brain death, in spite of stimulation with alternating polarity, seems to reflect a biological phenomenon which is not found in dead subjects after complete cessation of systemic circulation.

Aged↗

[Creutzfeldt-Jakob disease in Austria].

Between 1969 and 30th June 1996, Creutzfeldt-Jakob disease (CJD) was definitively diagnosed in 88 Austrian patients by autopsy and/or biopsy. The number of diagnosed cases has steadily increased in recent years (average incidence in 1969-1985: 0.18 per million; 1986-1994: 0.67 per million; 1995: 1.25 per million; estimate for 1996: 1.7 per million). The percentage of patients older than 70 years increased until 1989 and has decreased slightly since then. One patient received a lyophilised dura transplant 11 years before death. Another patient gave a history of intramuscular injections of bovine RNA (Regeneresen) extracted from various organs including brain administered over a ten-year period. One female patient had familial CJD with a glutamate-->lysine mutation at codon 200 of the prion protein (PrP) gene PRNP. The ages at death are distributed symmetrically around the median of 64 years. Two female patients died at the unusually young ages of 27 and 30 years. Median duration of disease was 4.5 months; 77% of the patients died within 6 months after onset of the disease. Retrospectively, 83% of the patients fulfilled the clinical criteria of probable or possible CJD. Neuropathologically, each patient showed accumulation of immunocytochemically detectable pathological PrP in the central nervous system. No patient had the neuropathological profile of the new CJD variant recently described in the U.K. The recent rise in diagnosed CJD in Austria probably reflects increased awareness and recognition rather than a real increase. Since bovine spongiform encephalopathy (BSE) has not been observed in Austria, our data argue against an association between the rise of incidence of CJD and BSE.

Adult↗

Cerebral oximetry in dead subjects.

Near-infrared spectroscopy is a technique used to monitor cerebral oxygenation. To validate the method, we measured regional oxygen saturation (rSo2) in the brains of 18 dead subjects (mean age, 74.4 +/- 14.6 years) 19.8 +/- 18.2 h (range, 1-73) after cessation of systemic circulation, and in 15 healthy probands (mean age, 34.2 +/- 8.7 years) with an INVOS 3100 cerebral oximeter. The mean (+/-SD) rSo2 in the dead subjects was 51.0 +/- 26.8% [range, 6-88%; left, 48.4 +/- 28.0% (n = 21); right, 54.4 +/- 25.7% (n = 16)]. The mean rSo2 in the control group was 68.4 +/- 5.2% (range, 60-76%; left, 68.1 +/- 5.0%; right, 68.7 +/- 5.6%). After removal of the brain at autopsy in five of the dead subjects, the rSo2 was 73.4 +/- 13.3% (15 measurements). Six of 18 of the dead subjects had values above the lowest values found in the healthy adults (> or = 60%). These findings raise concerns about the validity of cerebral rSo2 data in adults obtained by the INVOS 3100 system.

Adult↗

Creutzfeldt-Jakob disease in Austria.

Between 1969 and 30 September 1995, 79 Austrian patients had Creutzfeldt-Jakob disease (CJD) diagnosed neuropathologically by necropsy or biopsy. The annual incidence has significantly increased in recent years (average 0.18 per million in 1969-85, and 0.67 per million in 1986-94; estimate for 1995: 1.5 per million). Also, the percentage of patients with CJD over 70 years at death increased significantly until 1989 but is since in decline. There is no regional clustering, familial occurrence, or recognised iatrogenic risk. One patient had a 10 year history of intramuscular injection of purified bovine RNA preparation (Regeneresen) from various organs including the brian. The ages at death are symmetrically distributed around the median of 64 years. The median duration of disease is four months. Most patients (76%) died within six months of onset. Retrospectively, 86% of patients fulfilled clinical criteria of probable or possible CJD. Neuropathology showed the classic triad of spongiform change, astrogliosis, and neuronal loss in most cases. Two cases did not show unequivocal tissue alterations, but anti-PrP immunocytochemistry detected PrP deposits also in these cases. It is concluded that the recent rise in incidence of CJD in Austria most likely reflects increased awareness and diagnosis of CJD rather than a real increase. As bovine spongiform encephalopathy (BSE) has not been reported in Austria, the data do not support a link between a rise in incidence of sporadic CJD and BSE.

Adolescent↗

Paced epimyocardial electrograms for noninvasive rejection monitoring after heart transplantation.

BACKGROUND: Diagnosis of acute heart rejection after transplantation with the help of epimyocardial electrograms has been reported as a sensitive and practicable method. Long-term follow-up has been limited, however, by variations o signal, which can be avoided by using pacemaker-induced signals. METHODS: For stimulation and detection of the ventricular evoked response, a new type of electrode with fractal surface structure was used. Seventeen patients undergoing heart transplantation were included in the study. Amplitudes of the depolarization and repolarization parts of ventricular evoked response signals were analyzed and related to the degree of acute rejection according to histologic findings from endomyocardial biopsy. RESULTS: In cases of focal moderate rejection (grade 2, International Society for Heart and Lung Transplantation grading) and higher degrees of rejection, significant amplitude decreases were found. CONCLUSION: This sensitive noninvasive method for rejection monitoring with a high level of reliability provides the possibility of reducing the number of endomyocardial biopsies.

Biopsy↗

[Possible diagnostic error of "Bechterew disease"].

A 41-year old caucasian male presented with limited range of motion of the entire spine and myogenous intermittent claudication. He was referred with the diagnosis "ankylosing spondylitis". The clinical picture mimicked ankylosing spondylitis, but there were no corresponding radiological abnormalities despite a 13-year history of his disease. Laboratory values showed markedly elevated levels of muscle enzymes. The diagnosis of rigid spine syndrome, a subtype of muscular dystrophy, was made from the clinical picture a normal EMG and degenerative changes on a biopsy from the muscle trapezius. We would like to draw attention to this rare cause of back pain.

Adult↗

Angiotropic large cell lymphoma presenting as thrombotic microangiopathy (thrombotic thrombocytopenic purpura).

BACKGROUND: Angiotropic large cell lymphoma is a rare lymphoproliferative disorder that affects vessels of almost all organs. Therefore, many different signs and symptoms can be observed and may delay a rapid diagnosis in these patients. However, no association between angiotropic large-cell lymphoma and thrombotic microangiopathy (TMA) has been reported so far. METHODS: The case report describes a 69-year-old female Caucasian who presented with fever, neurologic symptoms, microangiopathic hemolytic anemia, thrombocytopenia, and hyaline thrombi within small vessels. TMA was diagnosed and intense treatment, including plasma exchange and corticosteroid therapy, was initiated. Nevertheless, the patient died 3 days after admission. A postmortem examination including immunohistochemistry and molecular studies was performed. RESULTS: Autopsy revealed angiotropic large cell lymphoma with tumor cell aggregates in small vessels of the brain, myocardium, lungs, liver, small and large intestines, mesenterium, kidneys, and lymph nodes. Immunohistochemical analysis of the tumor cells showed positive reactions with B-cell markers, but negative T-cell and epithelial cell markers. Molecular studies using polymerase chain reaction with primers for the rearranged immunoglobulin heavy chain and the T-cell receptor beta chain gene confirmed the diagnosis of a monoclonal B-cell disorder. CONCLUSION: TMA can occur in association with angiotropic large cell lymphoma and, furthermore, can be its sole clinical manifestation.

Aged↗

Plasma levels of parathyroid hormone-related peptide are elevated in hyperprolactinemia and correlated to bone density status.

Osteopenia is an important clinical manifestation of hyperprolactinemia. Bone loss in these patients has mainly been attributed to concomitant deficiency of gonadal hormones rather than to hyperprolactinemia per se. Parathyroid hormone-related peptide (PTHrP) is expressed in human mammary tissue, and elevated circulating PTHrP levels as well as concomitant hypercalcemia have been described during lactation. We sought to determine circulating PTHrP levels in patients with long-standing hyperprolactinemia and whether PTHrP may exert possible systemic effects on bone and mineral metabolism. We studied 45 patients (30 women and 15 men) with persisting hyperprolactinemia 6 +/- 4 years (mean +/- SD) after trans-sphenoidal surgery for prolactin-producing pituitary adenomas. PTHrP levels in 117 healthy controls were 10.6 +/- 7.3 pmol-eq/l (mean +/- SD). In hyperprolactinemic patients, plasma PTHrP was elevated to 30.3 +/- 13.4 pmol-eq/l (p < 0.001, n = 45), and in patients with humoral hypercalcemia of malignancy PTHrP levels were 52.9 +/- 29.6 (p < 0.001 to controls and hyperprolactinemic patients). Fifty-three percent of hyperprolactinemic patients (n = 24) had clearly elevated PTHrP levels (> 2 SD). Retrospective immunocytochemical studies of the removed pituitary adenomas from 19 patients generally showed a higher degree of immunoreactivity for PTHrP (1-34) in all but one case when compared with normal pituitary tissue. Patients with elevated circulating PTHrP levels showed in most instances strong immunoreactivity to PTHrP in 70-100% of tumor cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Absorptiometry, Photon↗

Brain-stem auditory evoked potential monitoring. Variations of stimulus artifact in brain death.

Brain-stem auditory evoked potentials (BAEPs) were recorded in 20 subjects with brain death (mean age, 33.2 +/- 15.1 years) and 20 healthy volunteers (mean age, 29.8 +/- 6.8 years). Brain death was due to head injury (n = 14), encephalitis (n = 3), brain-stem hemorrhage (n = 1), cerebellar hemorrhage (n = 1) or cerebral infarction (n = 1). The presence, latency and amplitude of the individual BAEP components and variations of the stimulus artifact were evaluated. The mean (+/- S.D.) amplitude of the stimulus artifact was 0.26 +/- 0.12 microV in the brain-dead subjects and 0.09 +/- 0.05 microV in the control group (P < 0.001, t test). The causes of the phenomenon of increasing stimulus artifacts in the evolution of brain death remain unclear.

Acoustic Stimulation↗

[Miliary tuberculosis of the liver as a cause of septic shock with multi-organ failure].

A woman aged 63 presented with septic fever, followed by hepatocellular jaundice. Viral hepatitis was ruled out by serologic tests, but no definite diagnosis could be made. Due to severe disturbance of the plasmatic coagulatory system and a serum bilirubin level above 4 mg/dl, a liver biopsy was not performed. The patient had a persistent septicemia refractory to Imipenem. In spite of intensive care measures, the patient died of disseminated intravascular coagulation and multiorgan failure caused by septic shock. The correct diagnosis of miliary tuberculosis was made only post mortem by histopathological examination of liver specimens and confirmed by detection of Mycobacterium tuberculosis DNA in the patient's liver by polymerase chain reaction.

DNA, Bacterial↗