[Ticlopidine-associated thrombotic thrombocytopenic purpura (Morbus Moschcowitz)]. / Ticlopidin-assoziierte thrombotisch-thrombozytopenische Purpura (Morbus Moschcowitz).

ANAMNESIS AND CLINICAL FINDINGS: A 75-year-old woman with a history of recurrent ischemic cerebral events was admitted with acute unspecific neurological symptoms and fever. EXAMINATION: Intracerebral hemorrhage due to hypertension and antithrombotic therapy with ticlopidine was ruled out with cranial computed tomography. Laboratory findings on admission included thrombocytopenia (12/nl), renal insufficiency (serum creatinine 1.6 mg/dl) and LDH elevation (1,218 U/l). The hemoglobin on admission was normal. THERAPY AND CLINICAL COURSE: In the presence of rapidly declining hemoglobin values and fragmentation of red cells thrombotic-thrombocytopenic purpura (TTP) was diagnosed and the patient received fresh frozen plasma. Shortly after the plasma infusion the patient's condition deteriorated rapidly showing clinical signs of an allergic shock. In the sequel of 24 to 48 hours the patient developed renal failure, severe anemia and the thrombocyte count fell to 5/nl. The patient was mechanically ventilated during the next 48 hours and needed intravenous catecholamines. Even after restoration of spontaneous respiration and cessation of pharmacological sedation the patient remained comatose. Cranial computed tomography on the fourth day after admission showed multiple infarction syndrome. The patient died on the ninth day after admission in status epilepticus which could not be stopped with pharmacological means. CONCLUSIONS: The combination of neurological symptoms, thrombocytopenia, fever, renal failure and hemolytic anemia in a patient taking ticlopidine points to a diagnosis of TTP. The high mortality of TTP can probably only be reduced by early plasmapheresis.

[Primary cutaneous plasmacytoma. A case report and review of the literature]. / Primär kutanes Plasmozytom. Fallbericht und Literaturübersicht.

Primary extramedullary plasmacytoma of the skin is a very rare disease; to our knowledge, only a few cases have been published in the world literature. In this paper, a patient who had a primary cutaneous plasmacytoma of the lower lip for 20 years is described. Serological, radiological and histological examinations excluded the possibility of an underlying multiple myeloma. The position of cutaneous plasmacytomas within the group of malignant plasma cell neoplasias is discussed and the relevant literature is reviewed.

[Actinomycosis of the bladder]. / Aktinomykose der Harnblase.

A palpable tumour was discovered in the left lower abdomen of a 66-year-old woman with uncharacteristic lower-abdominal pain and treatment-resistant pollakisuria and stress incontinence. On ultrasound examination the tumour was about 6.0 x 2.5 x 2.5 cm in size and was located between bladder roof and anterior abdominal wall. Ultrasound-guided fine-needle biopsy failed to produce any cytologically interpretable material, and there was no bacterial growth from the aspirate. All clinical and biochemical findings were normal, except for a raised blood-sedimentation rate (15/43 mm). The tumour, completely removed at laparotomy, was diagnosed to be actinomycosis of the bladder. No long-term postoperative antibiotic treatment was undertaken. Nine months after the operation the patient was without symptoms and there were no abnormal clinical findings.

Effect of FOY-305 (camostate) on severe acute pancreatitis in two experimental animal models.

Two models of severe acute pancreatitis were chosen and pancreatitis induced by sodium taurocholate and by a choline-deficient ethionine-supplemented diet, to evaluate the effectiveness of FOY-305 (camostate), a new synthetic trypsin inhibitor. Prophylactic administration of FOY-305 had a significantly favorable effect on the course of the sodium taurocholate-induced disease and on the survival rate of the treated group. A beneficial effect on the amylase and lipase content in serum and ascites was found, but no effect was observed on enzyme concentration in pancreatic tissue or on the degree of histologically detectable organ destruction. Therapeutic administration of FOY-305 had a significantly positive influence when infused directly, 5 and 30 min after the operation, whereas enzyme increase and organ destruction remained unaffected. FOY-305 showed a beneficial effect when given prophylactically or therapeutically at the beginning of the pancreatitis induced by a CDE diet, with no significant change in enzyme increase and degree of organ destruction. The favorable effect on survival time and rate in the early phase of these two severe experimental forms of pancreatitis may justify an evaluation of FOY-305 in a clinically controlled study.

Emery-Dreifuss muscular dystrophy: disease spectrum and differential diagnosis.

We report six patients with Emery-Dreifuss muscular dystrophy (EDMD) and four patients including one female with EDMD phenotype (EDMDP). This series includes one sporadic case who had previously been reported in this journal under the diagnosis of "rigid spine syndrome" in 1977. Time of observation ranged from three to ten years. Detailed cardiological assessment was performed in all patients, skeletal muscle biopsies were obtained from 9 out of 10 and cardiac muscle biopsies from 2 out of 10 patients. One patient showed evidence of cardiomyopathy in the absence of clinically apparent neuromuscular disease and one sibling of another EDMD patient reportedly had a similar combination of symptoms which, to our knowledge, has not yet been reported. Cardiac involvement was found to consist of four independent, albeit often combined features: 1) impairment of impulse generating cells; 2) conduction defects with atrial preponderance; 3) increased atrial and ventricular heterotopia; and 4) functional impairment of ventricular myocardium. Ventricular involvement as apparent from ventricular heterotopia, abnormal enddiastolic diameter, decrease of contractility and/or morphological evidence of ventricular myocardial disease was found in 7 out of 10 patients and confirmed by myocardial histopathology in two EDMD patients. In one myocardial biopsy extensive accumulations of intermediate filaments were observed, a rare finding, which has not been linked to EDMD before. Skeletal muscle biopsies showed evidence of myopathy throughout but several equivocal features such as fibre type grouping in EDMD and fibre type disproportion in EDMDP were also observed. The variability of clinical manifestation of both cardiac and neuromuscular disease encompassed a broader spectrum than apparent from the literature. The consequences for the inherent differential diagnosis are discussed.

When should treatment of acute experimental pancreatitis be started? The early phase of bile-induced acute pancreatitis.

Sodium taurocholate pancreatitis in the rat is a frequently used experimental model for evaluating therapeutical regimes in this disease. It is, however, uncertain when treatment should be started, as the early phase of this experimental model and thus the time when the pancreatitis really develops is unknown. Serum and pancreatic enzymes, as well as pancreatic morphology, were therefore studied 5, 30, and 60 min after induction of sodium taurocholate pancreatitis. It was found that increase in serum enzymes and decrease in pancreatic enzymes and morphological changes characteristic for acute pancreatitis develop as early as 5 and 30 min after induction of pancreatitis. Thus, therapy in this model may be started shortly after induction of acute pancreatitis.

Myo-, neuro-, gastrointestinal encephalopathy (MNGIE syndrome) due to partial deficiency of cytochrome-c-oxidase. A new mitochondrial multisystem disorder.

A 42-year-old woman had a 10-year history of external ophthalmoplegia, malabsorption resulting in chronic malnutrition, muscle atrophy and polyneuropathy. Computer tomography revealed hypodensity of her cerebral white matter. A metabolic disturbance consisted of lactic acidosis after moderate glucose loads with increased excretion of hydroxybutyric and fumaric acids. Post-mortem studies revealed gastrointestinal scleroderma as the morphological manifestation of her malabsorption syndrome, ocular and skeletal myopathy with ragged red fibers, peripheral neuropathy, vascular abnormalities of meningeal and peripheral nerve vessels. Biochemical examination of the liver and muscle tissues revealed a partial defect of cytochrome-c-oxidase (complex IV of the respiratory chain). This mitochondrial multisystem disorder may represent a separate entity to be classified between the spectrum of myoencephalopathies and oculo-gastrointestinal muscular dystrophy.

Fatal outcome of aortocoronary bypass grafting in a 62-year-old man with unsuspected coronary arteritis.

A 62-year-old man was admitted to our hospital with unstable angina. The coronary arteriography showed the occlusion of the left anterior descending artery and other stenotic lesions. The patient underwent an aortocoronary bypass operation, but unfortunately he did not survive. Surprisingly, the necropsy revealed him to be affected by an inflammatory process, involving the aorta and the coronary arteries. According to the clinical history and examination, the findings at necropsy and the histologic picture, the diagnosis of Takayasu's arteritis appears the most probable. Coronary arteritis has to be considered as a possible etiology of ischemic symptoms also in subjects who appear affected by typical atherosclerotic ischemic heart disease.

Continuous intravenous infusion of elastase in normal and agranulocytic minipigs--effects on the lungs and the blood coagulation system.

In order to investigate the acute effects on lung morphology, lung function, hemodynamic and blood coagulation system, elastase (330 units (U) kg-1 h-1) was continuously infused into 16 anesthetized and mechanically ventilated minipigs. Elastase infusion induced a disturbance of blood coagulation leading to hypocoagulability, a pulmonary leukostasis, interstitial edema, a progressive respiratory failure with prompt increase in pulmonary vascular resistance, decrease in systemic vascular resistance, increased venous admixture, and increased dead space ventilation. Agranulocytosis prevented interstitial edema but not disturbances in pulmonary or hemodynamic function or hypocoagulability. The results clearly indicate that elastase may be involved in the pathophysiology of acute lung failure and defects in the blood coagulation system.

Percutaneous intracardiac direct-current shocks in dogs: arrhythmogenic potential and pathological changes.

In 12 dogs, a total of 65 direct-current (DC) shocks of 100-300 J were delivered through a standard USCI6F tripolar electrode catheter to selected sites in the heart. Severe arrhythmias were more frequent after electric shocks of high energy to the ventricles and AV-nodal or His-bundle region than after comparable shocks to the left or right atria. There was a direct relationship between the strength of the electric shocks, and the extent and severity of the injury. Application of 300 J shocks led to massive necrosis and damage to all components of the myocardium including the walls of small blood vessels. However, perforation of the atrial and ventricular walls or septum did not occur in any of the animals (mean follow-up period 97 days: range 8 to 167 days). Percutaneous DC shocks up to 250 J proved to be a safe technique for closed-chest ablation of conduction tissue in dogs. These might be of value for patients with arrhythmias requiring electrical ablation of accessory pathways or foci situated in various sites in the heart.

Morphology of healing in vascular prostheses.

Vascular grafts differ in shape and size, material, mode of construction, and porosity. The latter was found to be of great importance with respect to tissue response of the host to the graft. The first reaction of the host after implantation represents the phase of exsudation. An aggregate of platelets and fibrin will cover most of the luminal and external surface of the graft, which is also seen between the interstices. The following phase of resorption develops within 2 weeks. It is characterized by replacement of the fibrinous material on the outer surface and between the interstices by capillaries, histiocytes, and myofibroblasts. Organisation of the outer capsule will then occur, and is also seen inside the transprosthetic bridges within one month. However, organization of the luminal lining remains very slow and is almost never completed. Healing depends on blood flow and local hemodynamic factors, and a mismatch in mechanical properties between the graft and host vessel may be important. Sources of endothelium in graft healing are anastomotic sites, pluripotent cells growing through the graft wall or deposition of pluripotent cells from the blood. Early failure of a prosthetic vascular graft occurs mainly as a result of separation at its sites of attachment. Late complications may be the result of mechanical failure (anastomotic sites or within the graft), kinking, inadequate or incomplete healing, and infection. The incidence of infectious complications varies from 0.25% to 6.0%, usually associated with high mortality rates.

Spontaneous hemodynamic improvement or stabilization and associated biopsy findings in patients with congestive cardiomyopathy.

The hemodynamic courses of 56 patients with congestive cardiomyopathy (CCM) were investigated. Fourteen patients died within 24 months after diagnosis. The hemodynamic courses of the remaining 42 patients were investigated in subsequent examinations by determination of left ventricular ejection fraction (LVEF), mean pulmonary arterial pressure at maximal workload, and peak systolic pressure/end-systolic volume index. During the study interval of 32.2 +/- 20.0 months the conditions of 20 patients (48%) deteriorated, according to their hemodynamic status, and at least five of these died of terminal heart failure. Surprisingly, the conditions of 22 patients (52%) improved or stabilized. One of these died of leukemia. Seven patients in the latter group with initial LVEFs of 0.30 or less experienced an average increase from 0.22 to 0.51. Retrospectively consideration of age, alcohol intake, exercise capacity, and hemodynamic status were not helpful in predicting the course of the disease. In 38 patients endomyocardial biopsy samples could be obtained at the time of diagnosis. Reduced myofibril volume fraction (less than 60%) had prognostic significance for both hemodynamic deterioration and death (sensitivity 23/24 = 96%), while 14 of 15 patients whose conditions improved or stabilized had a myofibril volume fraction of 60% or more (specificity 14/15 or 93%, p less than .002). A relationship between hemodynamic status and the myofibril volume fraction could not be found. Individual patients with CCM differ significantly with respect to course of the disease. A distinct separation of the patients by means of morphologic criteria is possible. This makes it more likely that the pathogenesis of the disease is not unique.

[Electrophysiologic findings in patients with Kearns-Sayre syndrome--report on 2 cases]. / Elektrophysiologische Befunde bei Patienten mit Kearns-Sayre-Syndrom--Bericht über zwei Fälle.

The Kearns-Sayre syndrome is identified by the triad of progressive external ophthalmoplegia, atypical pigmentary retinopathy, and conduction disturbances. In addition, clinical manifestations may include mental retardation, sensorineural deafness, cerebellar ataxia, and facial and peripheral muscle weakness. Morphologic alterations in skeletal muscle may be characterized by ragged-red fibers. Two patients with Kearns-Sayre syndrome underwent electrophysiological examination. The first patient had a first and second degree AV block (Mobitz type II), right bundle branch block, and left axis deviation. The His-bundle electrogram showed a prolonged HV interval as a hint at an intraventricular conduction delay. The signal-averaging technique and endocardial mapping revealed ventricular after-depolarizations. The second patient had an unsuspicious ECG, recurrent atrial tachycardias, normal atrial and ventricular conduction, and after-depolarizations in endocardial mapping. Two months later he showed a second degree AV block combined with clinical symptoms. Pacemakers were implanted in both patients. Beside disturbances of the conduction system in both patients signs of electrical instability of the myocardium were found. In this way the Kearns-Sayre syndrome may be seen as a form of cardiomyopathy.

[Right ventricular inflow tract obstruction in primary non-Hodgkin lymphoma of the heart]. / Rechtsventrikuläre Einflusstraktobstruktion bei primärem Non-Hodgkin-Lymphom des Herzens.

An unusual case of non-Hodgkin lymphoma (centroblastic B-cell type) is described presenting with severe tricuspid stenosis due to a large right atrial intracavitary tumor mass obstructing the tricuspid orifice. The clinical picture of cardiogenic shock was relieved by surgical removal of the greater part of the tumor mass from the right atrium.

Rupture of a thoracic aortic aneurysm after prosthetic bypass from the ascending aorta to the descending aorta in a case of congenital aortic arch hypoplasia.

An unusual case of a successfully operated ruptured thoracic aneurysm of the aorta with bleeding into the atelectatic pulmonary left lower lobe and acute occlusion of the prosthesis in a 28-year-old woman is reported. The underlying disease was congenital aortic arch hypoplasia still present following implantation of a prosthetic bypass from the ascending aorta to the descending aorta performed in 1963.

[The heart in the elderly. Pathology: macroscopic and light microscopy findings in the heart]. / Das Herz des älteren Menschen. Pathologie: Makroskopische und lichtmikroskopische Befunde am Herzen.

The average heart weight increases up to the 9th decade. An existence of senile atrophy is not confirmed. Various morphological abnormalities of the heart in the elderly can be found during the course of life--polypathy of the heart. The number of these changes increases on average in each heart linearly with age. In polypathy of the heart the abnormalities which have functional consequences, such as stenosing coronary sclerosis, small vessel disease, heart hypertrophy, calcification of mitral ring and/or the aortic valves, as well as a cardiac amyloidosis are of the utmost importance. The polypathy of the heart in the elderly increases its vulnerability and may lead to a latent or manifest heart insufficiency. The law of polypathy is not only valid for the heart, but also for the whole organism.

Meningiomas in a male fetus from a hormonally dysequilibrated mother.

Congenital multiple meningiomas in a male fetus of 37 cm length are reported. The tumor was histologically a highly vascular meningioma. An unbalanced level of hormones before and at the beginning of the mother's pregnancy could be ascertained, which resulted in gestational abnormality.