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Biomedical subjects

H Lang

Publications and source records attributed to H Lang.

At least 145 records · Page 8Linked to original sources

A chromogenic assay for activated protein C resistance.

Resistance to activated protein C (APC) diagnosed on the basis of prolongation of clotting time in an activated partial thromboplastin time (aPTT) assay is now considered a major cause of inherited thrombophilia. The majority of patients with APC resistance carry a factor V molecule with a point mutation at one APC cleavage site (Arg506Gln) which prevents the optimal inactivation of activated factor V by APC. To overcome the limitations of aPTT-based assays in the diagnosis of APC resistance, we have developed a chromogenic assay which is based on the capacity of APC to limit the generation of factor Xa by inactivating factor VIIIa in plasma. The ratio of the factor Xa amidolytic activity in a sample without APC to its factor Xa activity with the addition of APC reflects the response of the plasma coagulation system to APC. The normal range in 44 healthy individuals was 1.62-2.06. APC response ratios as measured by the chromogenic assay correlated with ratios measured by the aPTT assay and were below the normal range in 23/24 individuals with Arg506Gln mutant factor V from three different families with familial thrombosis and from 11 unrelated asymptomatic individuals. In reconstitution experiments, purified factor V corrected the decreased APC response in plasma samples from patients with the Arg506Gln mutation as well as with factor V deficiency, and increased the APC response in normal plasma, whereas the addition of activated factor V had no enhancing effect.

Anticoagulants↗

Hermeneutics and psychoanalytically oriented psychotherapy.

Psychic or psychosomatic disorders--symptoms as well as the structures on the basis of which symptoms usually develop--include substantial impairment of communication. Psychogenic symptoms often seem incomprehensible and strange. Psychotherapy then involves the figuring out of the meaning of a symptom and, at the same time, the effecting of change in the sense of an enhancement of communication. Here, philosophic hermeneutics can point the way for psychotherapeutic reflection. Hermeneutics, when applied to psychotherapy, is the art of understanding and of making understood when the means of understanding and agreement between persons is disturbed. The psychotherapeutic process itself is executed within a "hermeneutic circle." A short case presentation illustrates the hermeneutic technique.

Adult↗

Formation of differentiated tissues in vivo by periodontal cell populations cultured in vitro.

The periodontium contains heterogeneous mesenchymal cell populations with various differentiation potentials. The capacity of these cells for tissue formation as well as the origin of their precursors are still not entirely defined. In this study, cells originating from different periodontal tissues were cultured in vitro, and tissue formation in vivo following orthotopic re-implantation was investigated. Cells were recovered from the alveolar bone and periodontal ligament tissue of six minipigs, and cultured cells were then grown on extracted dental roots from the homologous animals by means of co-culture in vitro. Each minipig received 2 roots covered with alveolar bone cells, 2 roots covered with periodontal ligament cells, and 2 control roots (without cells) implanted into palatal bone defects. Intravital fluorochrome labeling was performed, and two minipigs were histologically examined after 2, 4, and 12 weeks in each case. Controls showed widespread resorption and ankylosis, whereas roots covered with cultured periodontal cells exhibited tissue formation in vivo. Alveolar bone cells synthesized a calcified cellular tissue resembling cellular cementum, suggesting that cells within this population might differentiate into cementoblasts when reimplanted with a dental substrate in vivo. Periodontal ligament cells exhibited no calcified tissue formation in vivo, but cells synthesized a connective tissue with orientated fiber bundles attached to both host bone and root, resembling periodontal ligament.

Alveolar Process↗

Pseudomyxoma peritonei of appendiceal origin--a report of seven cases and a review of published reports.

OBJECTIVE: To review our experience of different therapeutic regimens in the treatment of pseudomyxoma peritonei originating in the appendix. DESIGN: Retrospective study. SETTING: University hospital, Germany. SUBJECTS: 7 Patients with histologically confirmed pseudomyxoma peritonei. INTERVENTIONS: Debulking operations. MAIN OUTCOME MEASURES: Morbidity and mortality. RESULTS: Complete removal of the tumour was possible at the initial operation only in a patient with a benign tumour. Debulking operations helped the other six patients considerably, but all but one died eventually of complications of the disease (usually bowel obstruction). Two patients developed what were thought to be pulmonary metastases, and one liver metastases, but these were not confirmed histologically. The results of chemotherapy varied; only one patient was free of tumour for about four years and in another progress of the tumour was halted for about a year. Length of follow up ranged from 2-20 years. CONCLUSIONS: Operation is the treatment of choice for pseudomyxoma peritonei, although complete resection is rarely feasible. Because of the low morbidity and slow growth of the tumour, reoperation is always indicated for symptoms of recurrence.

Adult↗

Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients.

The optimal treatment of Budd-Chiari syndrome (BCS) remains an open question. It is still a matter of controversial discussion whether venous decompression or liver transplantation is superior. To elucidate the role and prognosis of both surgical options in our own experience, a consecutive series of 50 patients treated between 1981 and 1993 was retrospectively analyzed. Twelve patients had different types of portosystemic shunts or local decompressive procedures, and transplantation was performed in 43 cases, including five with previous conventional surgery. The overall mortality of 18 of 50 was conventional surgery. The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year. In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis. After transplantation, early complications were rejection, primary nonfunction, or graft necrosis, and contributed significantly to the risk of sepsis. Thirty of 43 liver recipients are currently alive, including four rescued after failed decompressive surgery, with 1- and 10-year survival of 69%, and excellent recurrence-free rehabilitation. These results clearly indicate that patient selection plays a dominant prognostic role in the treatment of BCS. Venous decompression and liver transplantation should both be integrated in a common therapeutic concept, and the individual decision for the preferred approach must be based on the leading clinical symptom: portal hypertension or liver failure, together with the assessment of reversibility of hepatic damage, and the potential of cure of the underlying disease.

Adolescent↗

[Social support and social stress in tumor patients and their partners].

Social support is widely believed to be an important buffer against stress in patients coping with cancer. Support efforts can, however, in turn prove to become a source of distress in themselves, both in patients and support providers. The present study was designed to explore the connections between support and emotional distress. N = 120 lung cancer patients and their relatives (n = 57) are interviewed at three intervals. Social support is assessed by multiple methods: Content analysis, a questionnaire and a confidant rating performed by experts. Cross-sectional and longitudinal analyses are combined. Content-analytical and self-report data show a correlation of support efforts and emotional distress. In the confidant rating, however, the expected differences between supportive and non-supportive spousal relationships emerge. The results are discussed from an interactional point of view.

Adaptation, Psychological↗

Liver segmentectomy as anatomically precise resections. An experimental study in sheep.

OBJECTIVE: To assess a new technique of anatomically precise hepatic segmental resection and to compare the degree of precision and biochemical profiles with results after traditional segmental resection and a sham operation. DESIGN: Experimental study. SETTING: University hospital, Germany. MATERIAL: 50 sheep (10 each had segments II, III, and IV removed by the new technique, and 10 each were studied in the traditional resection and sham operated groups). INTERVENTIONS: Operative ultrasonography and injection of methylene blue to identify segmental boundaries. In traditional operations boundaries were identified only from knowledge of the surface structure of the liver. MAIN OUTCOME MEASURES: Degree of precision, duration of operation, blood loss, mortality, transaminase activities and liver function tests. RESULTS: Anatomically precise segmentectomies were achieved in 6/9 (67%) for segment II, 6/9 (67%) for segment III, and 4/8 (50%) for segment IV. Using the traditional technique (segment III only) there was only 1/10 anatomically precise resections, together with 5 perisegmentectomies and 4 incomplete resections (p < 0.02). The operations for anatomically precise resection lasted significantly longer, but resection time was similar. Blood loss, survival, and transaminase activities were similar for the two groups, but the margin of necrosis at the cut edge was significantly less in anatomically precise resections. CONCLUSION: Anatomically precise hepatic resections are technically feasible with the use of intraoperative ultrasonography and selective staining of the segment(s) to be removed with methylene blue. Although it takes longer, there are no detrimental consequences compared with the considerably less accurate traditional technique.

Animals↗

[Psychosocial causal attributions by myasthenia gravis patients. A longitudinal study of the significance of subjective illness theories after diagnosis and in follow-up].

The present study evaluates the impact of subjective theories of illness in patients suffering from Myasthenia gravis. 46 inpatients were examined after the diagnosis had been made (T1) and 6 months later (T2). The following instruments have been used: a semistructured interview, the ¿Freiburg Personality Inventory¿ (FPI), the ¿Freiburg Questionnaire of Coping with Illness (FKV) and a questionnaire for the judgement of body experiences (FBK). Ratings concerning neurotic symptoms (PSKB), mechanisms of defence (KBAM) and the doctor-patient-relationship were made by the interviewers and the treating neurologist as well. 35% of the patients experienced a connection between psychosocial factors (stress) and the onset of the disease. As compared with those patients not showing a psychosocial causal attribution there are no differences in somatic (type and severity of the disease, thymus histology), but in psychological variables. Patients attributing their disease to psychosocial factors prove to be more depressive, insecure and excitable at T1. They are more likely to be women. These findings are in accordance with the results of previous studies. However, 6 months later (T2) there is some evidence of a psychological stabilization of those patients using psychosocial attributions, as different instruments show concurrently. Partially they appear now even less depressive. This result is understood as a hint for the significance of emotional factors for the process of coping with the disease: patients using a psychosocial explanation concept may appear more vulnerable at an early stage of the disease. But perhaps, by this more emotionally centered coping process, they can reach stability which is connected with the construction of a psychosocial theory of the disease.

Adaptation, Psychological↗

[IgG subclass distribution in intravenous immunoglobulin products: determination of reproducibility of a method of IgG subclass determination. Ring Study of the "Plasma Blood Components" Section of the German Society of Transfusion Medicine and Immunohematology].

BACKGROUND: The working group "Blood Plasma Constituents" of the DGTI has carried out a multicenter study with human immunoglobulins for intravenous administration (IVIG) and a NIBSC serum, with the designation 67/98C, since the European Pharmacopoeia IVIG monograph gives no information for the IgG subclass determination. Aim of the ring study was 1) the determination of the IgG subclass distribution with a standardized method and 2) the determination of the IgG subclass distribution in the NIBSC serum 67/98C and to test if the NIBSC serum coded 67/98C is suited as a reference serum for the IgG subclass determination. MATERIAL AND METHODS: Ten laboratories from Europe participated in the ring study. The IgG subclasses were determined by radial immunodiffusion (RID). RESULTS: The results showed good consistency between the participating laboratories and showed distinct differences in the IgG subclass composition of the tested IVIGs. The reproducibility of the method showed coefficients of variation of approximately 10%. CONCLUSION: The NIBSC serum 67/98C is suitable as reference serum for the IgG subclass determination in sera and IVIGs.

Germany↗

[Mesenchymal tumors of the liver: their frequency and histopathological diagnostic problems in surgical investigations].

BACKGROUND: Primary mesenchymal tumors of the liver are rarely observed. Histopathological diagnosis can be very difficult, in needle biopsies especially. This is a great difference to epithelial tumors of the liver. The frequency of their occurrence within surgical pathology will increase in the near future, since image analysis and improvement of liver surgery technics raise their recruiting. DESIGN: A total of 1102 primary liver tumors were evaluated from the files of this laboratory between 1981 and 1993, reclassified histologically and in cases of mesenchymal tumors compared with clinical data and course. RESULTS: 208/1102 (19%) mesenchymal tumors were revealed. When 177 cavernous hemangiomas were omitted for its choristomatous character and unproblematically diagnostic approach, a substantial group of 31/1102 (3%) mesenchymal tumors does remain. These were classified as: epithelioid hemangioendotheliomas (EHE) (8/31), angiosarcomas (7/31) infantile hemangioendotheliomas (5/31), mesenchymal hamartomas (5/31), angiomyolipomas (3/31), malignant mesenchymomas (2/31) and schwannoma (1/31). Among other misdiagnoses, 3 of hemangiopericytoma are remarkable, since they had to be reclassified as metastases from adult granulosa cell tumor of the ovary and meningeoma respectively. The histological distinction of EHE's is discussed versus angiosarcoma, since therapy and prognosis are different. CONCLUSIONS: These results reconfirm that mesenchymal neoplasms of the liver in adults are usually angiomatous differentiated. Diagnostic approach to probably mesenchymal derived masses of the liver does often request additional information from immunostaining and image analysis, especially when based on needle biopsies. Metastases are to be considered in this procedure. Primary pericytomas were not reconfirmed among this total of 1102 primary liver tumors.

Adenoma↗

Tissue specificity of rat mitochondrial dimethylglycine dehydrogenase expression.

Expression of mitochondrial dimethylglycine dehydrogenase (Me2GlyDH) was analysed in various tissues, liver cell types and developmental stages of the rat. Total RNA extracted from liver, spleen, brain, kidney, lung and heart was reverse-transcribed into cDNA and amplified with Me2GlyDH cDNA-specific oligonucleotides by PCR. Expression of the enzyme was observed mainly in liver and kidney. In addition, Me2GlyDH mRNA could be demonstrated in total RNA samples of lung, heart and brain but was barely detectable in spleen total RNA. In RNA prepared from 14-day rat embryos, Me2GlyDH-specific mRNA was clearly present. Among various liver cell types, besides hepatocytes, endothelial cells showed a high level of Me2GlyDH mRNA expression. There was no amplification product detectable in liver macrophages (Kupffer cells) and only a very faint one in fat-storing cells (Ito cells). Western blots confirmed at the protein level the predominant expression of the enzyme in liver and kidney, but Me2GlyDH protein was also present in the protein extract of lung, heart, spleen and brain. Immunohistochemical staining of liver slices with Me2GlyDH-specific antiserum revealed that expression of this enzyme is evenly distributed throughout the liver tissue. In the kidney, expression of the enzyme was located in the proximal tubule cells. Our results demonstrate that, contrary to the previously assumed liver-restricted expression, this enzyme is specifically expressed predominantly in the liver and kidney, but, in addition, it is detectable in many other tissues of the rat.

Aging↗

Liver transplantation for Budd-Chiari syndrome--palliation or cure?

This report documents two cases of Budd-Chiari syndrome (BCS) with essential thrombocytosis and antithrombin (AT) III deficiency as underlying etiological factors. Orthotopic liver transplantation was successfully performed in both patients but with different therapeutic intention. In the patient with essential thrombocytosis, hepatic transplantation only relieved the symptoms of the predisposing thrombogenic condition; it did not cure the underlying disorder. Prophylactic long-term anticoagulation, as well as adjuvant therapy for the causative disease, remained necessary. On the other hand, in the patient with AT III deficiency, liver transplantation was curative, resulting in complete reconstitution of serum AT III activity with resolution of the hypercoagulable state postoperatively. Thus, depending on the underlying etiology, liver transplantation for BCS can be considered as palliative, necessitating long-term adjuvant therapy, or as curative, with correction of a metabolic defect.

Adult↗

Northern epilepsy syndrome: an inherited childhood onset epilepsy with associated mental deterioration.

A new autosomal recessively inherited disease of the central nervous system involving childhood epilepsy and mental deterioration is described. Twenty three patients (11 males and 12 females) belonging to 11 families from northern Finland have been identified. A common ancestor has been found for nine families. The mean age of onset of epilepsy was 6.7 years (range 5-10 years) and the epilepsy was characterised by generalised tonic-clonic seizures increasing in frequency up to puberty. One third of the patients also had complex partial seizures during childhood. During young adulthood the epileptic activity began to decrease, but complete remission did not occur. Electroencephalography showed progressive slowing of the background activity with relatively scanty epileptiform activity. Out of four ictal recordings the paroxysmal activity was initiated focally in two cases. Clonazepam and sodium valproate had some antiepileptic effect, clonazepam being the more beneficial of the two. Mental development, which was originally normal, began to deteriorate two to five years after the onset of epilepsy, and the deterioration continued during adulthood in spite of good epilepsy control, leading to mental retardation by middle age. The pathogenesis of the disorder, called the Northern epilepsy syndrome, is unknown. Linkage analysis using DNA markers linked to the EPM1 gene for progressive myoclonus epilepsy of Unverricht-Lundborg type showed that the Northern epilepsy syndrome is not allelic to EPM1.

Adolescent↗

[The structure and psychodynamics of delusion].

Discussing classical psycho-analytic interpretations of delusion the authors show that neither the notion of "projection" nor the theory of regression to very early stages of ego-formation provide a satisfying explanation for the difficulty of those patients to discern inner world from external reality. An approach to this crucial question rather has to consider the underlying structural differences in order to differentiate between neurotic and psychotic symptom formation. The basis for psychotic symptoms is identified in a deficient mode of symbol-formation impeding certain structural patterns or spheres of conflict from being integrated into the whole of the subject's existence. If required under certain circumstances those "excluded" parts of the personality overwhelm the subjective experience arising as something radically strange and uncanny. A psychotic phenomenon like delusion bears witness of this break down, but at the same time is an attempt to cope with this threatening experience.

Adult↗

The Budd-Chiari syndrome: clinical presentation and diagnostic findings in 45 patients treated by surgery.

We report on the clinical presentation and diagnostic findings in 45 patients with Budd-Chiari syndrome. The diagnosis was confirmed by histology at the time of liver transplantation (n = 37) or shunt surgery (n = 8). An underlying disorder could be established only in half of the patients, oral contraceptives as predisposing factor were known in 18 cases. Clinically, abdominal pain and distension as well as hepatomegaly and ascites were most frequent findings, whereas changes in laboratory data were more or less unspecific. By use of repeated ultrasound, a definite diagnosis of a Budd-Chiari syndrome could be confirmed in all cases by obligatory demonstration of obstruction or thrombosis of at least one major liver vein. Hepatic venography revealed only one false-negative result. Celio-mesenteric arteriography plus portography, cavography and preoperative liver biopsy did not present additional diagnostic information. These techniques may contribute to treatment planning of portosystemic shunt surgery or hepatic transplantation. In conclusion, the presence of hepatosplenomegaly, ascites, abdominal pain and distension, especially in combination with a known hypercoagulable state, should alert to the possibility of a Budd-Chiari syndrome. Ultrasound is the diagnostic tool of choice. Hepatic venography should only be performed if even repeated ultrasound is not conclusive.

Adolescent↗