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Biomedical subjects

V Hofmann

Publications and source records attributed to V Hofmann.

At least 19 recordsLinked to original sources

[Pheochromocytoma as dominant manifestation of v. Hippel-Lindau syndrome].

A diagnosis of von Hippel-Lindau syndrome was made in two families originating from the same part of the Black Forest but apparently unrelated. Nine affected persons (seven males and two females) had a total of 17 tumours: retinal angioma (4), haemangioblastoma of the CNS (1), and phaeochromocytoma (12). Three of the affected persons and eight of the tumours (six phaeochromocytomas, two retinal angiomas) were diagnosed by family screening. Phaeochromocytoma was diagnosed in eight persons; in four it was the only symptomatic lesion. After extensive diagnostic tests the phaeochromocytoma was the sole tumour in four. Despite severe symptoms the diagnosis of von Hippel-Lindau syndrome had not been made prior to the screening examinations because either the common aetiology of the tumour was not known or there was insufficient exchange of information between the two families.--It is recommended that in each case of phaeochromocytoma von Hippel-Lindau syndrome should be excluded so that lesions can be discovered early in other organs and in other affected family members. If the syndrome is present, annual examinations are indicated because of asynchronous and multi-focal tumour growth.

Adolescent

["Bolus fatalities" in an autopsy sample 1981 to 1989].

Resulting from a historical study of the so-called bolus death (choking) an 8-years analysis of 11 bolus cases is presented, the autopsies of which were carried out at the Institute of Forensic Medicine Chemnitz. External conditions, autopsy findings, and accompanying investigations carried out are considered. In five cases a bolus was found in the pharynx, just in front of the larynx entrance, and three times each in the larynx, causing the blockade of the larynx entrance, and in the trachea. A correlation between the localisation of the foreign substance and the presence respectively expression degree of the macromorphological signs of asphyxia was not evident. In conclusion a discussion to differentiate reflex mechanisms from asphyxia mechanisms following foreign substance aspiration is given.

Adult

[Autopsy of accident victims--incidence and criteria for selection].

In a first analysis the frequencies of autopsies in the area district of Karl-Marx-Stadt (Chemnitz) are investigated in respect to types of accident, sex and age groups. Victims of traffic- and working accidents are dissected in far more than 50% of all cases (predominantly with forensic medicine) because of the obvious legal consequences; accident occurring at home or during leisure are paid less attention to. Possible criteria for selection are discussed.

Accidents

[Changes in the diagnosis and therapy of invagination].

The alterations in diagnosis and therapy of intussusception in infants is described in 155 cases from 1978-1989. The primary diagnostic procedure is the abdominal sonography. It is followed to 1988 the attempt of hydrostatic reduction by barium enema and now the pneumatic reduction. The rate of success with conservative treatment was finally over 80%. Primary operative treatment is only necessary in the rare cases with signs of perforation of intestine or of peritonitis.

Air

Value of ultrasound in the treatment of solitary kidneys in infancy and childhood.

Every second patient with a solitary kidney suffers from renal disease. This accumulation of renal diseases of varying origin makes special care for these children necessary. The quality of diagnostic methods is decisive for the choice of therapy. The advantages of primary ultrasound diagnosis and sonographical function tests are described. Sonographical differentiation of disturbances of the urinary transport is possible by means of forced-diuresis ultrasonography, whereas a vesico-ureteral reflux can be detected by means of voiding sonocystography. The value of ultrasound for primary diagnosis, assessment of therapeutic course and postoperative long-term follow-up is discussed.

Adolescent

[Ultrasonic studies of craniocerebral injuries in infants].

This is a report on the sonographic diagnostic possibilities in 46 closed-head injured infants. Cerebral examination via the anterior fontanelle is completed by additional sections of the surface of the brain. The traumatic oedema is limited in most cases and - besides the increase in echogenicity - an increase of the arterial pulsation at the border of the oedema is seen in real-time scanning. The sonographic signs of the epidural and subdural haematomas are described and also the differentiation between both. Surgical treatment was necessary in three cases of subdural haematoma because of increasing ICP. Treatment of extradural and chronical subdural haematomas or effusions is discussed in connection with the sonographic follow-up. Alterations in symmetry and compression of the ventricular system is often caused by intracerebral haemorrhages and-or traumatic local oedema. Parenchymal cysts may occur after liquefaction of cerebral necrosis or clots. The advantage of the cerebral sonographic examination is the uncomplicated follow-up. In connection with the clinical state and with continuous registration of ICP, sonographic findings facilitate the decision if, where and when trepanation must be performed.

Brain

[Efficacy of recombinant interferon alpha-2 in 34 patients with hairy cell leukemia. Effect of splenectomy and dosage reduction results of therapy].

In a retrospective study of 34 patients with hairy cell leukemia treated with recombinant interferon-alpha 2, we have observed a hematological remission rate of 97%. The true complete remission rate based on bone marrow findings was 17%. Although complete remissions can be obtained only in a few cases, treatment with interferon is justified in cytopenic patients since long-standing clinically meaningful improvement of the peripheral blood values can be attained. If interferon is stopped in the remission phase, the blood values deteriorate in 40% of the patients within 5 months. In contrast, it appears that the remission can be maintained with intermittent administration of interferon weekly or even every other week. As compared to splenectomized patients, granulocyte recovery is delayed in nonsplenectomized patients. However, after several months of treatment there is no difference in peripheral blood values between splenectomized and non-splenectomized patients. In the light of the present results, primary splenectomy remains justified in a selected group of patients for whom the risk of surgery (due to low granulocytes and/or platelets) is low and for whom careful evaluation predicts a high potential for long-standing remission after splenectomy. In the other cases, initial interferon therapy seems justified.

Adult

[The significance of screening studies for the early detection of metastases in curatively operated breast carcinoma].

A retrospective analysis of 80 women with breast cancer operated on with curative intent showed that metastases developed within 8 years. The value of laboratory tests and radiological surveys for the early detection of metastases is considered. In fewer than 50% of the patients was the finding of a single abnormal laboratory result associated with tumor recurrence. However, when several findings were abnormal concomitantly this suggested tumor dissemination in about 90% of women. In 80% of the patients clinical symptoms were present at the time of tumor recurrence. In only 20% of the patients were metastases clinically silent. Since metastatic breast cancer cannot be cured by present treatment modalities, the relevance of detecting early recurrence is limited.

Adult

Prognostic significance of agar and liquid cultures in AML patients before treatment, early postinduction and in remission.

In the present study, the growth and differentiation capacity of myeloid leukemic cells in agar and liquid cultures have been investigated in relation to their prognostic significance for treatment outcome and early detection of relapse. Prior to induction therapy, leukemic cells failed to differentiate and the colony or cluster number did not correlate with response to treatment. Seventeen to 42 days after induction, patients with BM cells producing greater than 10 colonies or greater than 30 clusters resp. had a high likelihood of achieving a complete remission. Cells from refractory patients had a significantly impaired differentiation capacity. During remission, a colony number greater than 50 was significantly associated with a high probability to remain in further remission for greater than 3 months. An impaired differentiation was significantly associated with the likelihood of relapsing within 3 months. In the light of these results, agar and liquid cultures appear to be useful for monitoring the effect of induction chemotherapy and detecting patients likely to relapse.

Agar

[Mycoplasma etiology of acute pancreatitis].

In a 10 year-old girl the clinical and laboratory findings led to the diagnosis of pancreatitis. At the same time an infection by mycoplasma pneumoniae was serologically stated. Since there could not be found another cause for pancreatitis, a relation between pancreatitis and infection by mycoplasms is very probable.

Child

[Effectiveness and toxicity of high-dosage Ara-C. Clinical observations in 10 cases and review of the literature].

Ten patients with acute myeloid leukemia were treated with a high-dose ara-c regimen (3 g twice daily for 6 days). 5 patients (50%) achieved a complete remission lasting for a median duration of 5 months. These patients had all achieved a complete remission following standard treatment with ara-c. High-dose ara-c was ineffective in 2 patients resistant to standard ara-c protocols. The most important side effects of high-dose ara-c were conjunctivitis and cerebellar symptomatology. In one case a severe diffuse encephalopathy with cerebellar predominance set in at a remarkably late stage (45 days after starting treatment). Our results and those of other authors suggest that treatment with high-dose ara-c should be used with caution.

Adolescent

[Multiple myeloma].

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Antineoplastic Combined Chemotherapy Protocols

Leukemic samples characteristics associated with clonogenic growth.

In this study, we attempted to delineate readily assessable characteristics from 100 leukemic samples associated with in-vitro growth. Successful growth defined as production of greater than 30 clusters and/or colonies per culture dish was obtained in 68% of samples. More than 10 colonies were found in 59% and greater than 30 colonies in 51% of cultures, respectively. Leukemic cells from patients previously treated with aggressive cytotoxic chemotherapy grew significantly better than cells from untreated patients, independently of the above definitions of cloning success. Cells from peripheral blood had a weak, albeit significant growth advantage over bone marrow cells (p = 0.032) when cluster growth was taken into account for growth success. When colony growth alone was used as criterium, no growth advantage was found. The morphological subtype and the proliferation kinetics prior to cell plating did not affect cloning success. A high labeling index had predictive value for subsequent growth, but only in bone marrow cells. By multivariate analysis, we found that treatment status was the most important factor correlated with in-vitro growth.

Analysis of Variance

Limitations of the salmonella/mammalian microsome assay (Ames test) to determine occupational exposure to cytostatic drugs.

Urine samples of nursing personnel working in medical oncology divisions of several Swiss hospitals were examined for mutagenic activity. Urine samples were concentrated 100 times following XAD-2 chromatography and mutagenicity was determined using the Salmonella/mammalian microsome assay (Ames test). Apart from the urine samples of patients treated with cytostatic drugs and urine samples of nurses who are cigarette smokers, no mutagenic activity could be found. Also following exposure to an increased and defined quantity of cytostatic drugs no mutagenicity could be recovered from the urine. Four different nurses worked with cyclophosphamide, methotrexate, 5-fluorouracil, adriamycin and cis-platinum for 3-4 hr without using any protection such as gloves, masks or a vertical laminar airflow hood. Aqueous extracts of filters, through which air was pumped during the whole experiment (a personal air-sampler was fixed near the face of the test persons), were non-mutagenic. Parallel to the mutagenicity test chemical analyses were also done. The methotrexate content was determined in serum samples and the aqueous filter extracts and urine samples were examined for cis-platinum. All chemical determinations were negative. With the aid of urine concentrates of a patient treated with sub-therapeutic doses of cyclophosphamide as well as with normal urine to which single small amounts of different cytostatics (adriamycin, cyclophosphamide, cis-platinum) were added, the detection limits for the corresponding cytostatic drugs were determined and found to be in the range of 2-10 mg for cyclophosphamide and approx. 10 micrograms for adriamycin. Cis-platinum was lost during the passage through the XAD-2 columns. With these results at hand the sensitivity of the hitherto preferably used method (Ames test) for the monitoring of exposure to cytostatic drugs must be seriously questioned.

Antineoplastic Agents