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PubMedDate not supplied

Fatal outcome in a patient with metastatic prostate cancer and acquired severe hypogammaglobulinemia with complete absence of mature peripheral blood B-cells.

Common variable immunodeficiency (CVID) is the second most common immunodeficiency and may be classified according to the presence or absence of mature B-cells, the latter subgroup being exceedingly rare. We describe a 66-year-old patient who over six months developed profound hypogammaglobulinemia associated with a complete absence of circulating B-lymphocytes and had a fatal outcome. Immunophenotypic analysis of a bone marrow aspirate demonstrated a strongly reduced number of B-cell precursors (CD34+/CD19+) but normal numbers of CD34+/CD45+ hematopoietic progenitor cells. This case is remarkable with respect to its documented rapid evolution. Possible triggers are discussed, a combination of drug toxicities being the most likely.

Adenocarcinoma↗
PubMedDate not supplied

[Cerebrovascular disease with a fatal outcome and the recent consumption of alcohol].

The authors have investigated 19 cases of nontraumatic cerebrovascular accidents with a fatal outcome from a total of 1,627 consecutive nonselected forensic autopsies. Alcohol concentration in the blood was determined in 15 cases of which 60% (9 subjects) tested positive. The median age of the group with positive alcohol blood tests was 50.4 +/- 6.2 years. The median age of the group with negative results was 51.7 +/- 5.4 years. In the group with positive tests 4 were chronic consumers of alcohol. As compared with 1,773 determinations of blood alcohol done in the general casuistics of forensic medicine it appears that the incidence of alcohol concentrations under 1 g/thousand is greater in subjects that died with cerebrovascular accidents. In the group of subjects with alcohol concentrations in the blood between 1 and 2 g/thousand, and over, reflecting an advanced degree of inebriation, there are no significant differences between the two lots. In the authors cases the haemorrhagic forms of nontraumatic cerebrovascular accidents were predominant. Ischaemic lesions were found in only two cases.

Adult↗
PubMed2002

Expression of pulmonary lactoferrin in sudden-onset and slow-onset asthma with fatal outcome.

The objective of this forensic autopsy study was to determine the immunohistochemical expression pattern of lactoferrin (LF) in pulmonary tissue sections deriving from fatal slow-onset asthma (time interval between onset of asthma attack and death >2.5 h) and fatal sudden-onset asthma (cases in which death occurred within 1 h of the onset of an asphyxic asthma attack) relative to controls (sudden death due to diseases other than respiratory disorders). LF was applied to paraffin sections using a standard peroxidase-labelled streptavidin-biotin technique. LF immunoreactivity was graded semi-quantitatively in relation to different histoanatomic distribution sites of LF on a five category ordinal scale (maximum score of 15). We found a statistically significant difference between an enhanced expression of LF in both asthma groups relative to the controls (P<0.004 and P<0.001, respectively). When comparing both asthma groups, there was a statistically significant difference in LF immunoreactivity between the slow-onset and sudden-onset asthma group (P<0.001). Since LF immunoreactivity was far less intense in the sudden-onset asthma group (mean expression +/-SD: 7.3+/-1.3) than in the slow-onset asthma group (12.5+/-1), and an absent or weak LF expression pattern was observed in the control group (1.4+/-1.3), we assume that our results permit the following cautious estimations: (1) pulmonary LF expression is enhanced in asthma attacks with fatal outcome relative to controls and (2) a different expression pattern of LF can be observed in fatal sudden-onset asthma compared to slow-onset asthma in so far as the pulmonary expression of LF seems to be positively correlated with the preceding period of time between the asphyxic asthma attack and death. Further clinicopathologic studies including in-patient asthma fatalities with a well-known medical history are required to scrutinize if the pulmonary expression of LF is in fact positively associated with the time span of the asthma attack, thus possibly providing further therapeutic opportunities to intervene in severe asphyxic asthma.

Acute Disease↗
PubMed2000

[Chronic inflammation of the petrous bone with fatal outcome].

We report the case of a 44-year-old woman, suffering from lethal cerebritis of the temporal lobe caused by an unusual chronic otitis media with granulations. A chronic inflammatory process of the left temporal bone had affected the patient over a period of 4 years. This had started with mastoiditis accompanied by a lesion of the dura mater, leading to labyrinthitis, encephalitis and cerebellar abscess. The patient's symptoms persisted, especially vertigo and uncertainty, and then deteriorated acutely. MRI showed otogenic cerebritis. Intracranial bleeding from vasculitis had worsened the patient's condition, finally resulting in the patient's death. This case impressively demonstrates that a cerebral abscess can cause acute worsening of general complaints associated with ear disease and results in a fatal outcome at any stage.

Adult↗
PubMed1999

Malakoplakia of the caecum in a kidney-transplant recipient: presentation as acute tumoral perforation and fatal outcome.

Malakoplakia is a rare pseudotumoral inflammatory disease known to affect immunocompromised subjects, mainly with a history of recurrent Escherichia coli infection. The urinary tract is the most frequent site of the disease, although all organs can be involved. In the present article, we report a case of malakoplakia of the caecum, that developed in a 52-year-old man, who had received a kidney transplant 9 years before and had a history of recurrent E. coli urinary tract infections. Malakoplakia presented as acute intestinal perforation, and, despite aggressive surgical and medical management, disease progressed toward a fatal outcome due to sepsis and multiple organ failure 9 months later. A defect in the macrophagic activity was demonstrated.

Bacteremia↗
PubMed2000

Fatal outcome of Erysipelothrix rhusiopathiae bacteremia in a patient with oropharyngeal cancer.

Bacteremia due to Erysipelothrix rhusiopathiae is rare; the most common presentation reported in the literature is endocarditis. We report a 32-year-old man with oropharyngeal cancer who developed aspiration pneumonia and E. rhusiopathiae bacteremia, and presented with fever, chills, dyspnea, and productive cough with purulent sputum. Despite treatment with amoxicillin/clavulanate and nutritional support for 9 days, he died of respiratory failure. He had no clinical evidence of endocarditis. He had no history of animal or occupational exposure, and might have been colonized with E. rhusiopathiae in the oral cavity, followed by aspiration pneumonia and bacteremia. A fatal outcome in a patient with bacteremia due to E. rhusiopathiae without endocarditis is rare.

Adult↗
PubMed1998

Systemic infantile complex I deficiency with fatal outcome in two brothers.

A male infant presented at 5 months of age with vomiting, developmental stagnation and convulsions. Complex I activity was in skeletal muscle 0.025 mU/mU CS (N 0.044-0.265) and in fibroblasts 0.046 mU/mU CS (N 0.100-0.307). Despite riboflavine supplementation progressive neurological deterioration occurred and he died at 14 months of age. During the mother's following pregnancy complex I activity was measured in chorionic villi and found mildly reduced, pregnancy was continued. A male infant was born who presented at 7 months of age with vomiting, developmental stagnation and hypotonia. Complex I activity was in skeletal muscle 0.031 mU/mU CS and in fibroblasts 0.100 mU/mU CS. There was progressive neurological deterioration and he died at 17 months of age. Complex I activity in autopsy liver of both patients was normal. Apparently, complex I deficiency presenting in infancy can have a fatal outcome despite only mild reduction of enzyme activity in skeletal muscle and/or fibroblasts, and chorionic villi and normal activity in liver.

Chorionic Villi↗
PubMed2004

Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome.

UNLABELLED: We report the unusual case of a 2-month-old boy with systemic fibromuscular dysplasia (FMD). He presented with congenital renovascular hypertension due to stenosis of the right renal artery, and later developed renal infarction on the contralateral side resulting in renal failure. The boy subsequently died of intracranial haemorrhage at the age of 14 months. During the course, hemiconvulsion caused by a Moyamoya disease-like vascular lesion was noted. Stenotic lesions of both the abdominal aorta and its branches were also revealed by angiography. Post-mortem examination confirmed that the coronary, splenic and mesenteric arteries were also affected and their histological findings were compatible with FMD. To our knowledge, this is the first congenital case of FMD demonstrating a rapidly progressive course resulting in a fatal outcome. In this case, multivessels in both intracranial and extracranial arteries were involved. CONCLUSION: Our case suggests that the nature of fibromuscular dysplasia is congenital in origin and its aetiology, at least in some cases, is a systemic abnormality of vascular development.

Angiography↗
PubMed2005

Epidemiology of international terrorism involving fatal outcomes in developed countries (1994-2003).

We aimed to describe the public health burden and epidemiology of international terrorism (i.e. involving foreign nationals) with fatal outcomes in developed countries. Data was abstracted from a United States Department of State database for 21 'established market economy' countries and 18 'former socialist economies of Europe' for 1994-2003. To put the findings in a wider context, comparisons were made with WHO data on all homicides for each country. A total of 32 international terrorist attacks causing fatalities were identified over the 10-year period. These resulted in 3299 deaths, giving a crude annual mortality rate of 0.3 per million population. The mortality burden attributable to international terrorism in these countries was 208 times less than that attributable to other homicide. Even for the country with the highest mortality burden from international terrorism (the United States), this ratio was 60. There was no statistically significant trend in the number of attacks over time, but the attack severity (in terms of deaths per attack) was higher in the latter part of the 10-year period. A number of limitations with this data set were identified. If a more rigorous definition of 'international terrorism' was used, then this would substantially reduce the total number of such attacks defined in this way. In conclusion, there is a need for better quality data and improved classification systems for describing international terrorism. Nevertheless, the available data indicates that the mortality burden from international terrorism in developed countries is small compared to that from other homicide.

Developed Countries↗
PubMed2001

[Fatal outcome of a multisystemic sarcoidosis in a 54-year-old patient].

BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown etiology in which the immune system is overstimulated. The intrathoracic lymph glands and the lungs are the most common site of involvement but all other organs and systems can be affected. Although the prognosis is mainly favourable there is a small group of patients who are resistant to therapy and will eventually succumb to their disease. There is no causative treatment but corticosteroids are the most effective therapeutic agents for sarcoidosis. If there is resistance to corticosteroids an immunsuppressive combination treatment is available. The immunsuppressive drugs (Azathioprin, Methotrexat, Ciclosporin, Pentoxifyllin, Thaliodomid) and the clinical effectiveness of the combination treatment are still under investigation. CASE REPORT: We describe a 54-year-old patient admitted to our hospital because of abnormal liver function tests. The cause was a sarcoidosis of the liver but we found also an involvement of the lung. The chest x-ray did not show any pathological findings, the lung function tests - VC and FEV1 - were normal but the diffusion capacity (DLCO) was reduced to 60 %. The course of the disease was characterized by a progressive deterioration of the lung function and of the general condition (loss of weight, fatigue, decrease in vitality). In the further course colon, spleen, bone marrow and kidneys were involved and hypercalcemia, pancytopenia and hyperspleenism occur. The treatment consisted in the application of corticosteroids. We started with 30 mg prednison daily and reduced it over a period of nine weeks to 7.5 mg. In the periods of deterioration we increased the doses up to 40 mg daily and achieved a temporary improvement of the general condition and the function tests. But eventually the fatal outcome could not be prevented not even after the removal of the massive enlarged spleen. The patient died four years after the diagnosis was established. The cause of death was an advanced hepatic encephalopathy as a result of extensive granulomatous involvement of the liver by a multisystemic sarcoidosis. CONCLUSION: We conclude that a steroid resistant multisystemic sarcoidosis should be treated early in the course with a combination of two immunsuppressive agents. We recommend Azathioprin in addition to corticosteroids. We refer to the corresponding literature.

Fatal Outcome↗
PubMed1976

Cells containing Langerhans cell granules in human lymph nodes of "atypical hyperplasia" with fatal outcome and leukemic reticuloendotheliosis.

Langerhans cell granules could be found in atypical histiocytes in lymph nodes of three patients with "atypical hyperplasia" with fatal outcome and one patient with leukemic reticuloendotheliosis. These atypical histiocytes might be derived from immature mesenchymal cells in lymph nodes and the Langerhans cell granules might be induced in these cells by a particular condition. Only one of the Langerhans cell granules seemingly associated with the plasma membrane could be observed in these atypical histiocytes and all of the granules were seen within the cytoplasm. Quite a number of Langerhans cell granules were located near the Golgi apparatus. Several atypical granules very similar to the Langerhans cell granules could also be observed in these atypical histiocytes. These Langerhans cell granules were assumed to be directly derived from the Golgi apparatus and/or derived from the atypical granules which were secreted from the Golgi apparatus. The relationship between the Langerhans cell granules and the microtubules must also be considered, because Langerhans cell granules were found near the centrioles and microtubules.

Adult↗
PubMed2003

[Fatal outcome after multiple trauma. The thoracic injury as the decisive factor].

In patients suffering from multiple injury, chest trauma is often the main cause of fatality. A case report is given and the literature reviewed.A 49 years old motorcyclist hit a car frontally in a road accident. After primary stabilization and first clinical care, he was transferred to our trauma centre because of severe chest injury, suspected pericardial effusion and lesion of the thoracic aorta. Initial diagnostics (plain radiographs, CT scan of thorax, abdominal ultrasound, echocardiography) showed left-sided serial rib fractures, a fracture of the left scapula, a hematopneumothorax left-sided, bilateral lung contusion, a small pneumothorax of the right side, a minimal pericardial effusion and a small splenic hematoma. The patient was treated in the intensive care unit, and the situation was initially stable. After 12 h, respiration deteriorated and a bronchoscopy showed filling of the airways with mucous fluid. The CT scan showed a worsening of the pulmonary damage and increasing pericardial fluid compression. A pericardiotomy was carried out, but the situation remained unstable. The patient was treated with invasive ventilation (PEEP>10, FiO2>0,5). Sudden severe bleeding out of left lower lobe was managed by thoracotomy and lobectomy. The patient remained unstable and died 95 h after the accident. This case shows that the severity of chest trauma does not necessarily correlate with the initial clinical and radiological findings. Even with all diagnostic and therapeutic procedures, a fatal outcome could not be prevented. This demonstrates the role of chest injury as a major and unforeseeable cause of death in multiple trauma patients.

Accidents, Traffic↗
PubMed1997

Predictors of a fatal outcome following childhood cerebral malaria.

Despite prompt treatment with an effective anti-malarial drug, cerebral malaria still has a mortality of 20-30%. To identify factors that may contribute to this high fatality rate, we have studied the relationship between clinical and laboratory features and a fatal outcome in 624 Gambian children with strictly defined cerebral malaria. One hundred twenty-four children (21.5%) died. Three-quarters of the deaths occurred within 24 hr of admission. Multiple logistic regression analysis showed that a cold periphery (odds ratio [OR] = 2.7), a deep coma (OR = 2.0), and hypoglycemia (OR = 4.1) were the clinical signs and laboratory parameters that predicted death most strongly. More than 90% of the children who died had at least one of these conditions. Also, children with elevated urea levels on admission or those who experienced multiple episodes of hypoglycemia or multiple convulsions subsequently were more likely to die. A combination of clinical and laboratory abnormalities can identify a group of children with cerebral malaria who are most at risk of dying, who require intensive care and who are candidates for new forms of therapy.

Age Factors↗