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Biomedical subjects

H Müller

Publications and source records attributed to H Müller.

At least 649 records · Page 36Linked to original sources

Genetic analysis of familial erythrophagocytic lymphohistiocytosis.

A family with two patients suffering from familial erythrophagocytic lymphohistiocytosis (FEL) is described. Reports on FEL found in the literature were collected and the family trees reconstructed. Genetic analysis of this material indicated that FEL is an autosomal recessive hereditary disease. This conclusion is supported by the following evidence: considerably increased consanguinity rate; complete concordance in monozygotic twins; more frequent occurrence in inbred populations and in cousins; limitation of cases to one generation, and results of segregation analysis.

Consanguinity↗

Palmar fibromatosis (Dupuytren's contracture). Ultrastructural and enzyme histochemical studies of 43 cases.

Forty three cases of palmar fibromatosis were studied by light and electron microscopy, enzyme histochemistry, and ultrastructural immunohistochemistry. By electron microscopy most of the cells composing the nodules in both the proliferative and the involutional stages were identical to myofibroblasts. The myofibroblasts in the involutional nodules often possessed microfilament aggregates probably representing contraction of micro(actin)filaments in the cytoplasm. The proliferative nodules revealed small perivascular haemorrhages and haemosiderin deposits accompanied by accumulation of macrophages and some lymphocytes; these inflammatory cells possibly secrete a certain growth factor inducing proliferation of genetically abnormal fibroblasts and myofibroblasts. Diaminopeptidase IV was detected in myofibroblasts and fibroblasts by enzyme histochemistry and ultrastructural immunohistochemistry; the enzyme may play a role in the metabolism of intercellular substances. Some perivascular mesenchymal cells, interpreted as variants of myofibroblasts, had moderate activity of alkaline phosphatase.

Acid Phosphatase↗

[Effect of guar flour on endogenous nitrogen excretion in rats determined by the 15N-tracer technic].

The metabolic fecal nitrogen was determined in 8 young rats (90-100 g body weight) using a single oral dose of 75 mg 15N-glycine. Four rats were fed a diet containing 8% guar and 4 rats received a control diet without guar. Compared to the control group, apparent protein digestibility and nitrogen balance were significantly lower in the guar group. After an initial steep rise the 15N-elimination in feces and urine (days 2-8 after 15N-glycine application) followed an exponential curve in at least 2 phases, with increasing half-time values. The proportion of metabolic fecal nitrogen to total fecal nitrogen was calculated from the 15N-atom-% excess, determined daily in urine and in feces. This calculation is based on the assumption that the specific 15N-activity (15N-atom-% excess) in urine represents the specific 15N-activity of the endogenous nitrogen pool. The addition of guar raised the proportion of metabolic fecal nitrogen from 51% (control) to 73%, corresponding to an increase in total fecal nitrogen loss (24 mg) observed in the guar-fed animals. This indicates that the rise in fecal nitrogen loss caused by guar is due almost entirely to enhanced secretion of endogenous nitrogen and not to undigested food protein. Based on the analysis of 2,6-diamino-pimelic acid in the feces of 4 rats the proportion of bacterial nitrogen in the feces was calculated. It amounted to 19.8% of total fecal N in the control group, and to 30.1% in the guar group, or to 39.4% and 44.4% of metabolic fecal nitrogen respectively. Obviously the increased dietary fiber intake also caused a rise in the amount of metabolic fecal nitrogen, which is derived from gastro-intestinal secretions and epithelial tissue.

Animals↗

[Determination of fecal bacterial nitrogen in rats].

A simple method is described by which the fecal flora of rats has been isolated with high recovery and a high purity grade. Total nitrogen and 2.6-diamino-pimelic acid were determined in the isolated bacteria. From both values a factor was calculated, which permits the estimation of fecal bacterial nitrogen from fecal DAP content. In the feces of rats on a semisynthetic control diet (4% cellulose) this factor was 15.7. Addition of 8% guar (by substitution for starch) resulted in a drastic increase in fecal DAP content thus lowering the factor to 11.5. When the proportion of cellulose in the control diet was raised from 4 to 12%, no change in the factor was observed. This indicates a pronounced effect on the bacterial activity by guar but not by cellulose. It is concluded that the factor used to calculate fecal microbial nitrogen from fecal DAP content is dependent on the kind of dietary fiber ingested.

Amino Acids, Diamino↗

Cohort and period effects in peptic ulcer mortality from Japan.

Japan's death rate from gastric ulcer is far higher than that in any other country. The time pattern of peptic ulcer mortality between 1937 and 1980 was analysed in order to find out whether gastric ulcer in Japan, compared with Europe, shows other epidemiologic features which may indicate a different type of gastric ulcer disease. The age-adjusted death rates of both ulcer types and sexes have continuously decreased. In spite of the much higher death rates in the years 1937-80, the mortality of gastric ulcer shows a similar cohort phenomenon both in Japan and in Europe. The Japanese generations born in the last quarter of the 19th century ran the highest risk of dying from peptic ulcer and carried it throughout their lives. The cohorts with a high risk for duodenal ulcer lag 10 yr behind those with a high risk for gastric ulcer. Male and female cohorts with the highest risk were born at the same time. The cohort effects of both ulcer types are superimposed by period effects. The most obvious period effect during 1946-50 is seen in the short-timed increase of the death rates in all age groups, both sexes, and both ulcer types. The similarities between Japan and Europe suggest that worldwide, at the same time, the same environmental factors have precipitated the risk of dying from gastric and duodenal ulcer.

Adolescent↗

Malignant ameloblastoma or ameloblastic carcinoma.

The World Health Organization defines malignant ameloblastoma as a lesion exhibiting features of an ameloblastoma in primary and metastatic growths. To cases collected from the literature we have added two of our own cases in which features of an ameloblastoma were coupled with malignant behavior. It was noted that the diagnosis of "malignant ameloblastoma" is at present used in a rather indiscriminate way, resulting in the grouping of lesions that exhibit considerable differences in biological behavior and histomorphology. This might be due to the fact that the WHO classification emphasizes metastasis as a diagnostic criterion but is rather vague in defining histopathologic aspects. It is advocated that the term malignant ameloblastoma be reserved for those lesions that, in spite of a seemingly innocuous histology, have given origin to metastatic growths, while the WHO classification should be modified to include ameloblastic carcinoma as a diagnostic term for lesions that combine features of an ameloblastoma with a less-differentiated histomorphology.

Aged↗

Neuroradiologic and neuropathologic findings with growing giant intracranial aneurysm. Review of the literature.

The neuroradiologic observations on a case of growing giant intracranial aneurysm originating from a small saccular aneurysm are described in detail. The reports in the literature of growing giant aneurysms are reviewed. The computed tomographic and angiographic findings are described and special diagnostic difficulties analyzed. A correlation between the computed tomographic and neuropathologic findings is made, and the pathogenetic mechanisms of enlargement of aneurysms are discussed.

Cerebral Angiography↗

Localized infantile myofibromatosis. Report of a case originating in the mandible.

A case of congenital solitary myofibromatosis occurring in the mandible is presented. Recognition of this entity is of clinical importance as the lesion can be easily confused with juvenile fibromatosis. In contrast to this latter disease which exhibits a locally aggressive growth pattern requiring radical surgery, myofibromatosis consists of well-circumscribed nodules amenable to simple excision. Moreover, myofibromatosis may show spontaneous regression. Histological features permitting the differentiation of myofibromatosis from juvenile fibromatosis are discussed.

Diagnosis, Differential↗

Fibrosarcoma of the jaws. A study of 7 cases.

Seven cases of fibrosarcoma of the jaws are presented. Five cases were in the mandible and 2 involved the maxilla. There were 3 males and 4 females and ages varied from 11 to 59 years. Four patients were in their third decade. All patients died of their tumour. Reason for death was local extension (2 patients); metastatic spread (2 patients); both local extension and metastatic spread (2 patients); and post-operative complications (1 patient). Factors causing this unfavourable clinical course are discussed. Chemotherapy and irradiation were of no therapeutic benefit. Some notes on histogenesis are made with respect to an odontogenic origin of jaw fibrosarcoma. This possibility exists but contributory evidence is equivocal unless a secondary fibrosarcoma has arisen in a benign odontogenic tumour.

Adolescent↗

The influence of the age at which the palate is closed on speech in the adult cleft patient.

A follow-up study was done on adult cleft patients with a total unilateral cleft with regard to the influence of the time of closure of the palate on speech. It was established that postponement of surgery of the palate from 3 to 6 years did not influence the ultimate result in respect of speech. One of the interesting findings was that in those patients who were operated on at the age of 3 there were more compensations than in those patients who were operated on at the age of 6. Pharyngoplasty did produce a satisfactory obturation between nasopharynx and the oral cavity in both age groups, but the ultimate quality of speech was clearly inferior to those patients who were not subjected to pharyngoplasty.

Adolescent↗

The influence of the age at which the palate is closed on the rhinological and otological condition in the adult cleft patient.

A comparative study was made of the otological and rhinological conditions at adult age of two groups of patients with unilateral total cheilognathopalatoschisis. In group A (51 patients), the palate was closed around the 3rd year of life, in group B (54 patients), around 6th year. All patients were operated on with an identical technique, by the same surgeon. There was no difference between the two groups in regard to permanent loss of auditory acuity; however, nasal patency appeared to be better in patients in group A than in those of group B.

Adolescent↗

Angioglomoid tumour of the mandible. A case report.

A case of an angioglomoid tumour of bone occurring in the mandible is presented. The lesion is characterized by epithelioid and endothelioid cells that exhibit single and multilayered rings with a central signet ring-like cell suggesting vascular differentiation. These cells are arranged in strands and nests that are dispersed in a myxoid stroma. Radical resection of the involved mandibular bone was followed by a recurrence of the tumour in the interposed graft after 7 years. A second radical operation provided permanent cure as is demonstrated by a disease-free interval of 10 years. Histological features permitting the differentiation from other central jaw tumours are discussed.

Adult↗

Beta-adrenergic blocking agents: substituted phenylalkanolamines. Effect of side-chain length on beta-blocking potency in vitro.

The synthesis of a group of potential beta-blockers bearing a new 5-ethoxysalicylamide substituent on nitrogen is described. These compounds were tested for beta-adrenergic blocking potency in vitro and compared with analogous compounds bearing a tert-butyl group on nitrogen. The new N-substituent increased the beta-blocking potency substantially. In a series of five homologous compounds of the type Ar(CH2)nCHOHCH2NHR (R = 5-ethoxysalicylamide; n = 0-4), two maxima of beta-blocking potency were found for n = 0 and 2. Moreover, the carbon isostere of the corresponding (aryloxy)propanolamine still proved to be a very potent beta-blocker. The ether oxygen in the side chain is therefore not an absolute requirement for activity. Structure-activity relationships are discussed.

Adrenergic beta-Antagonists↗

Bilateral occlusion of the extracranial internal carotid artery secondary to closed neck injury. Case report and review of the literature.

The authors report a case of traumatic bilateral internal carotid artery occlusion following blunt neck trauma in order to point out the significance of repeated angiography at a time when CT scanning has become the primary method for cervico-cranial traumas. Here angiography yields 1. a precise diagnosis and 2. evidence of a possible spontaneous recanalisation.

Adult↗

The influence of anticoagulation on acid-base status and blood-gas analysis.

The results of acid-base status and blood-gas analysis can be influenced massively by incorrect sampling. We found that the addition of heparin has no great effect on blood-gas measurement. Citric acid and fluoride can also be admixed to blood samples in the usual concentrations without severe side-effects. The major error in the determination of blood gases and acid-base status is a consequence of even minimal dilution: filling the deadspace of a syringe with any diluent leads to unacceptable results. Hence, we conclude that blood specimens for determination of blood gases and acid-base status should be taken in specially prepared syringes containing the anticoagulant in the form of a dry substance. We think that citric acid is also acceptable for anticoagulation. Fluoride should be added in order to minimize alterations of pH due to anaerobic glycolysis.

Acid-Base Equilibrium↗

Reversible end-of-life indicator leading to erroneous pacemaker replacement-pitfalls of telemetry.

In several modern pacemakers, end-of-life (EOL) is indicated by a single-step rate drop, which is initiated by a voltage-sensitive electronic switch. This switch may also be activated by other causes, such as very short voltage drops, low temperatures, and electrocautery. We report a case in which a Cordis 233 F Sequicor II pulse generator was operating at 51.6 bpm in VOO mode at the time of implantation. As telemetry seemed to indicate that back-up pacing (the EOL indicator in this model) was "off," it was erroneously assumed that the pacemaker was not working properly. The unit was replaced by another impulse generator. At laboratory testing, the pacemaker was within specifications, when back-up pacing was separately programmed off. In conclusion, physicians should be aware of the new phenomenon of reversible EOL indicators, and that they may not rely on telemetry in this respect. Future pacemakers should incorporate a telemetry message which prevents similar misunderstandings.

Equipment Design↗

Immunocytochemical demonstration of intermediate filaments in a granular cell ameloblastoma.

The nature and location of intermediate filament proteins (IFP) may provide new insights into the origin and differentiation of neoplastic cells. An immunofluorescent study of these IFP in a case of a granular cell ameloblastoma revealed that all tumor cells contained the IFP keratin. Some granular cells, however, also contained the IFP vimentin, which is considered specific for mesenchymal tissues only. The implications of these observations are discussed. Study with monoclonal antibodies indicated the origin of the ameloblastoma from non-keratinized squamous epithelium. A comparison of the anti-keratin immunofluorescence pattern of the ameloblast-like cells in the present tumor with ameloblasts in the tooth germ revealed no similarities, indicating that despite some resemblance of the peripheral columnar cells to ameloblasts, these cells differ in other aspects.

Aged↗

[Isolated hyperthermic extremity perfusion with vindesine, dacarbazine and cisplatin in the treatment of malignant melanomas].

A three drug combination consisting of dacarbazine, vindesine and CDDP (DVP) was used for isolated extremity perfusion in 26 patients with stage I, II and III melanomas of the limbs. Dosages of 100 mg/l dacarbazine, 0.4 mg/l vindesine and 20 mg Cis-Platinum/l extremity volume have been tolerated without major side effects. All perfusions of bulky tumors or metastases resulted in more than 50% tumor-size reduction (8 patients stage III A, 6 patients stage III AB). In five stage III patients relapse occurred 2, 3, 5, 6 months after isolated DVP perfusion.

Antineoplastic Combined Chemotherapy Protocols↗