Home About us Contact
|
Home About us Contact | ||
|
|
||
Postmortem Examination (postmortem + examination)
Selected AbstractsThe fatal outcome of an individual with anorexia nervosa and Sheehan's syndrome as a result of acute enterocolitis: A case reportINTERNATIONAL JOURNAL OF EATING DISORDERS, Issue 1 2010Werner Köpp MD Abstract Objectives: To illustrate the close association between a disturbed psychosocial up-bringing, frequent physical illness, and medical interventions. Method: We report a case of a 44-year-old woman with anorexia nervosa (AN) and Sheehan's syndrome who died as a result of a toxic cardiac arrest. Results: The patient presented with a BMI of 13.6 kg/m2. She refused any intensive-care treatment and died from toxic cardiac arrest. Postmortem examination revealed an acute gastroenterocolitis. Discussion: The history of this patient illustrates how psychological deprivation led to eating disturbances, early pregnancy, and the life-threatening delivery of twins. This resulted in a diagnosis of Sheehan's syndrome, hepatitis C, and a ventricular ulcer. A psychosocial event triggered a late exacerbation of her AN. A helpful alliance between patient and staff did not occur as she rejected it. © 2009 by Wiley Periodicals, Inc. Int J Eat Disord 2010. [source] Cardiac amyloidosis in a patient with multiple myeloma: A case report and review of literatureJOURNAL OF CLINICAL ULTRASOUND, Issue 3 2009David Sedaghat MD Abstract We report a case of a 52-year-old man with multiple myeloma and rapidly progressive heart failure who died unexpectedly from a probable arrhythmia. Postmortem examination revealed infiltrative amyloid cardiomyopathy, a rare cause of predominantly diastolic myocardial disease. Cardiac amyloidosis should be considered in any patient presenting with congestive heart failure, preserved systolic function, and a discrepancy between a low QRS voltage on electrocardiography and an apparent left ventricular hypertrophy on sonogram. The pattern of left ventricular diastolic dysfunction changes during the course of amyloidosis and the classically described restrictive physiology occurs only in advanced stages of the disease. © 2009 Wiley Periodicals, Inc. J Clin Ultrasound, 2009 [source] A patient with TSC1 germline mutation whose clinical phenotype was limited to lymphangioleiomyomatosisJOURNAL OF INTERNAL MEDICINE, Issue 2 2004T. Sato Abstract. Background:, Lymphangioleiomyomatosis (LAM) can occur as in isolated form (sporadic LAM) or as a pulmonary manifestation of tuberous sclerosis complex (TSC) (TSC-associated LAM). Recent studies, however, revealed that both forms of LAM are genetically related but that sporadic LAM is a distinct clinical entity caused by somatic mutations of TSC2 (not TSC1) rather than a forme fruste of TSC carrying either of the TSC1 or TSC2 germline mutations. Method:, Case presentation and in-depth molecular and histopathological examinations. A 34-year-old Japanese woman was diagnosed as having pulmonary lymphangioleiomyomatosis (LAM) when bilateral pneumothoraces were surgically treated in 1992. Although slowly progressive renal disfunction was observed due to bilateral multiple renal cysts during the past 4 years, she had no other clinical features of TSC and was diagnosed as having sporadic LAM with multiple renal cysts of undetermined aetiology. Her subsequent clinical course was complicated by an endobrochial carcinoid tumour, which eventually resulted in her death in June 1999 due to massive haemoptysis. Results:, Postmortem examination revealed the presence of LAM lesions in the lungs, mediastinal lymph nodes, kidneys and uterus. Diffuse renal LAM lesions are presumed to generate multiple renal cysts by constricting the nephron rather than epithelial hyperplasia obstructing lumina, which is analysis of the TSC genes demonstrated that she did not have TSC2/PKD1 contiguous gene syndrome but had a TSC1 germline mutation (Sato T et al. J Hum Genet 2002; 47: 20,8) that had occured de novo. Conclusion:, This patient therefore illustrates that clinical manifestations of TSC are sufficiently diverse as to allow a forme fruste of TSC that mimics sporadic LAM and that TSC1 mutation can cause multiple renal cysts resulting in renal failure. [source] Prospective Evaluation of the Balloon-to-Annulus Ratio for Valvuloplasty in the Treatment of Pulmonic Stenosis in the DogJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 4 2006Amara Estrada Background: In dogs, treatment of pulmonic valve stenosis (PS) with pulmonary balloon valvuloplasty (PBV) is a viable method to decrease the pressure gradient across the valve. However, to the authors' knowledge, the variables that influence the selection of the correct balloon size for the procedure have not been explored. Moreover, the lesions caused by the procedure have not been detailed. Hypothesis: Variables that influence the measurement of the annulus could affect selection of the balloon size. We sought to determine the effects of treatment when the balloon-to-annulus ratio (BAR) was or > 1.3, but within the recommended range of 1.2,1.5, regardless of whether dilation was performed with single or double balloon technique. Animals: Twenty-five Beagles with PS were studied. Methods: Inter-and intra-observer variability, echocardiography versus angiocardiography, and systolic versus diastolic timing were evaluated for the BAR. Assessment of right ventricular (RV) pressure, Doppler gradient, stenotic valve area, and RV wall thickness were compared before and 1, 90, and 180 days after treatment. Postmortem examination of the heart was done. Results: Significant correlations existed in measurement of the annulus; however, variation existed that would change balloon size. Improvement in the degree of PS was significant regardless of the BAR or single or double ballooning. In the most severely affected dogs, continued improvement was noted on day 90. Postmortem examination revealed tears in the commissures and the valve leaflets. Conclusions: Multiple factors influenced determination of the BAR and a range of 1.2,1.5 was effective without detrimental consequences. Dogs with severe PS had continued decrease in RV pressure 3 months after treatment. [source] Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complexNEUROPATHOLOGY, Issue 2 2009Hidehiro Takei Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date. The patient had a history of radical vulvectomy for malignant melanoma (MM), and died of autopsy-confirmed widespread systemic metastasis. Postmortem examination of the brain revealed a single 2.1 × 1.0 × 0.8 cm intraventricular nodule in the lateral ventricle. Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA. Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells. Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis). Autopsy showed no manifestations of TSC systemically or intracranially. The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma. This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC. Tumor metastasis to an occult SEGA is extremely rare. [source] Postpacemaker Implant Pericarditis: Incidence and Outcomes with Active-Fixation LeadsPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 5 2002SOORI SIVAKUMARAN SIVAKUMARAN, S., et al.: Postpacemaker Implant Pericarditis: Incidence and Outcomes with Active Fixation Leads. Pericarditis has been noted as a potential complication of pacemaker implantation. This study evaluated the risk of developing pericarditis following pacemaker implantation with active-fixation atrial leads. Included were 1,021 consecutive patients (mean age 73.4 ± 0.4 years, range 16,101 years; 45.2% women) undergoing new pacemaker system implantation between 1991 and 1999 who were reviewed for the complication of pericarditis. The incidence and outcomes of postimplantation pericarditis in patients receiving active-fixation atrial leads were compared to those not receiving these leads. Of 79 patients who received active-fixation atrial leads, 4 (5%) developed pericarditis postpacemaker implantation. Of 942 patients with passive-fixation atrial leads or no atrial lead (i.e., a ventricular lead only), none developed pericarditis postoperatively (P < 0.001). Of patients receiving active-fixation ventricular leads only (n = 97), none developed pericarditis. No complications were apparent at the time of implantation in patients who developed pericarditis. Pleuritic chest pain developed between 1 and 28 hours postoperatively. Three patients had pericardial rubs without clinical or echocardiographic evidence of tamponade. They were treated conservatively with acetylsalicylic acid or ibuprofen and their symptoms resolved without sequelae in 1,8 days. One patient (without pericardial rub) died due to cardiac tamponade on postoperative day 6. Postmortem examination revealed hemorrhagic pericarditis with no gross evidence of lead perforation. Pericarditis complicates pacemaker implantation in significantly more patients who receive active-fixation atrial leads. It may be precipitated by perforation of the atrial lead screw through the thin atrial wall. Patients developing postoperative pericarditis should be followed closely due to the risk of cardiac tamponade. [source] Polyangitis overlap syndrome: A fatal case combined with adult Henoch-Schönlein purpura and polyarteritis nodosaPATHOLOGY INTERNATIONAL, Issue 8 2003Kazuo Watanabe Henoch-Schönlein purpura (HSP) is a rather common disease characterized by systemic hypersensitivity vasculitis in the skin and other visceral organs. It has a favorable prognosis unless it is complicated by severe glomerular disease. We report a distinctive fatal case of systemic vasculitis combined with HSP and polyarteritis nodosa (PN) in a 56-year-old man who died of progressive renal failure one month after the onset of the disease. He complained of arthralgia, purpura of both lower extremities, nasal bleeding and tarry stool, and acute renal failure was noted at the time of admission to hospital. A skin biopsy from the purpura lesion exhibited leucocytoclastic vasculitis with IgA deposition, and HSP was considered. However, renal failure progressed rapidly and subsequently was complicated by acute myocardial infarction. Postmortem examination revealed PN type necrotizing vasculitis in the kidneys, heart and mesentery resulting in acute multiple infarctions of these organs. We think the current case was a polyangitis overlap syndrome. It is important to suspect the polyangitis overlap syndrome positively when progressive acute renal failure is seen in a patient with HSP and to begin appropriate therapy immediately. [source] Miliary Neonatal Hemangiomatosis with Fulminant Heart Failure and Cardiac Septal Hypertrophy in Two InfantsPEDIATRIC DERMATOLOGY, Issue 4 2004A. H. O'Hagan M.B., M.R.C.P. We report two infants with this condition who had fulminant cardiac failure and cardiac septal hypertrophy. The first was a 5-day-old boy who presented with increasing numbers of cutaneous hemangiomata associated with worsening cardiac failure. Magnetic resonance imaging (MRI) showed extensive hepatic hemangioma. Despite treatment with systemic corticosteroids and subcutaneous interferon alfa-2b his disease progressed. Hepatic artery embolization was unsuccessful. The infant died of congestive cardiac failure at 6 weeks of age. Postmortem examination showed a massively enlarged cardiac interventricular septum and biventricular hypertrophy. The second patient was a 1-week-old girl who also had cutaneous hemangioma and cardiac decompensation. MRI showed extensive intrahepatic involvement. She was treated early with corticosteroids and interferon ,, which resulted in involution of the cutaneous and hepatic lesions. Cardiac septal hypertrophy did not persist at late follow-up, and the association of miliary neonatal hemangiomatosis and cardiac septal hypertrophy has not yet been established. [source] Fulminant Liver Failure After Vancomycin in a Sulfasalazine-Induced DRESS Syndrome: Fatal Recurrence After Liver TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 9 2009M. Mennicke DRESS syndrome (drug rash with eosinophilia and systemic symptoms) is a rare drug hypersensitivity reaction with a significant mortality. We describe a 60-year-old man with polyarthritis treated with sulfasalazine who developed DRESS and fulminant liver failure after additional vancomycin treatment. Liver histology revealed infiltration of granzymeB+ CD3+ lymphocytes in close proximity to apoptotic hepatocytes. After a superurgent liver transplantation and initial recovery, the patient developed recurrent generalized exanthema and eosinophilia, but only moderate hepatitis. Histology showed infiltration of FasL+ lymphocytes and eosinophils in the transplanted liver. Treatment with high-dose methylprednisolone was unsuccessful. Postmortem examination revealed extensive necrosis of the liver transplant. This case report illustrates that patients with DRESS may develop fulminant liver failure and that DRESS recurrence can recur in the transplanted liver. Histological and immunological investigations suggest an important role of granzymeB and FasL mediated cell death in DRESS associated hepatitis. [source] A mitochondrial ATPase 6 mutation is associated with Leigh syndrome in a family and affects proton flow and adenosine triphosphate output when modeled in Escherichia coliACTA PAEDIATRICA, Issue 2004R Carrozzo A multidisciplinary strategy was used to identify the molecular defect in a family with Leigh syndrome (LS). The propositus presented severe developmental delay, an ataxic-spastic gait and seizures. She died at 3.5 y of age from cardiorespiratory arrest. Postmortem examination disclosed pathological features typical of LS. A 12-y-old sister is affected with the same disease. Respiratory chain enzyme complex activities in skeletal muscle biopsy were normal. Adenosine triphosphate (ATP) synthesis during oxidative phosphorylation in skin fibroblasts mitochondria showed a severely hampered ATP production. Mitochondrial DNA sequencing revealed a new mutation in the ATPase 6 gene (T9176G). Site-directed mutagenesis in Escherichia coli strains was used to measure H+ pumping and ATP synthesis. Results were comparable to findings obtained in human cells. These data corroborate the use of E. coli strains as a feasible "animal" model for functional studies in pathogenic mutations of the ATPase 6 gene. [source] Gliomatosis cerebri in a 10,year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumourDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 2 2001Sandeep Jayawant Gliomatosis cerebri is the unifying term used when diffuse glial infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiological and pathological findings. Adult cases may simulate an acute diffuse encephalomyelitis and show postmortem evidence of a marked swelling of the spinal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10,year-old girl who presented with a history and physical signs suggestive of benign intracranial hypertension. A diffuse encephalomyelopathy occurred, which was complicated by spinal cord swelling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination. [source] Cerebral cortical laminar necrosis on diffusion-weighted MRI in hypoglycaemic encephalopathyDIABETIC MEDICINE, Issue 8 2005Y. Yoneda Abstract Background Laminar necrosis of the cerebral cortex characterized neuropathologically by delayed selective neuronal necrosis occurs in hypoglycaemic encephalopathy and other brain diseases. Case report A 37-year-old male with insulin-treated Type 1 diabetes mellitus developed hypoglycaemic encephalopathy associated with respiratory failure. Brain diffusion-weighted MRI during the subacute period demonstrated high signals along the cerebral cortex. Brain single-photon emission computed tomography showed diffuse, severe cerebral hypoperfusion. The patient remained comatose and died 1 month later. Conclusions High signals along the cortical bands on diffusion-weighted MRI suggest cortical laminar necrosis, although a postmortem examination was unavailable. Sustained hypoglycaemic brain injury, possibly associated with respiratory hypoxia, may be the underlying mechanism. [source] Otological manifestations of acute leukaemia: report of two cases and review of literatureINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 1 2001E. Andrès Otological manifestations exceptionally reveal acute leukaemia, whereas leukaemic infiltration of any tissue is frequent at postmortem examination. We present two cases of acute myeloblastic leukaemia revealed by a loss of hearing due to a middle-ear leukaemic infiltration. The characteristics of such a clinical and radiological finding are emphasized. It is suggested that middle and inner ear can be a ,sanctuary' localization, which might sometimes require radiation therapy to achieve durable and complete remission. [source] Prenatal and early postnatal morphogenesis and growth of human laryngotracheal structuresJOURNAL OF ANATOMY, Issue 2 2008Pierre Fayoux Abstract Advances in neonatal medicine have resulted in increased care of fetal and neonatal airways. These advances have required an exhaustive knowledge of fetal airway anatomy and development. The aim of this study was to determine the anatomical development of laryngotracheal structures during the fetal and immediate postnatal period and to correlate these observations with other fetal biometric parameters to estimate developmental particularities of the fetal airway. An anatomical prospective study was based on examination of larynx and trachea from 300 routine autopsies of fetuses and infants, free of malformation and never intubated. Anatomical measurements of cricoid cartilage, thyroid cartilage, glottis, arytenoid cartilage and trachea were performed using a precision calliper and precision divider. Statistical analysis was performed to represent the growth of anatomical structures and to evaluate the correlation with biometric data. Raw data and 10th and 90th percentile curves were fitted satisfactorily with a linear model for gestational age. A linear relationship between laryngotracheal measurement and body weight and height was observed except for glottis length, interarytenoid distance and anterior cricoid height. The diameter of the cricoid lumen was significantly less than that of the trachea and glottis lumen. A sexual dysmorphism was noted for thyroid cartilage measurements and interarytenoid distance, with measurements significantly smaller in females. This study reports the anatomical development of normal laryngotracheal structures during the fetal period. Despite the fact that this study was performed during postmortem examination, these observations can be useful to develop criteria, materials and surgical procedures adapted to fetal and neonatal airways as well as for the purposes of early diagnosis and management of laryngotracheal malformations. [source] Characterization of the Triacylglycerol Crystal Formation in Adipose Tissue During a Vehicle CollisionJOURNAL OF FORENSIC SCIENCES, Issue 4 2007Barbara H. Stuart Ph.D. Abstract:, The unusual appearance of crystalline fat structures was observed during the postmortem examination of a motor vehicle accident victim. The crystal structures were characterized using Fourier transform infrared spectroscopy and x-ray diffractometry. The structures were found to be made of triacylglycerols, a dominant lipid structure found in human adipose tissue, capable of forming various polymorphic structures. The morphology of the crystalline material was found using both techniques to be predominantly the ,, form of triacylglycerols. The accelerated growth of such triacylglycerol morphology has been observed as a result of shear stresses in other studies involving edible fats. As a result of the findings of this study, it is proposed that increased shear forces may be responsible for the formation of the unusual fat structure found in the victim. An understanding of the effect of forces on the structure of body fat in high-impact collisions can potentially assist in verifying a high-velocity impact. [source] Intermandibular malignant mesenchymoma in a crossbreed dogJOURNAL OF SMALL ANIMAL PRACTICE, Issue 9 2006S. Murphy A 12-year-old German shepherd crossbreed dog was presented with a submandibular mass that was initially diagnosed as myxosarcoma on incisional biopsy. Chest radiographs were taken for staging, and magnetic resonance imaging was performed to assess the feasibility of cytoreductive surgery before adjuvant radiotherapy. The dog underwent debulking surgery, and histology permitted reclassification of the tumour as a malignant mesenchymoma (with myxosarcomatous and osteosarcomatous differentiation). The dog was subsequently treated with four fractions of radiotherapy given at seven-day intervals and three doses of carboplatin. The dog remained stable following therapy until its condition acutely deteriorated, and it was euthanased 153 days after surgery. On postmortem examination, there were no signs of local tumour recurrence, but metastases were observed both in the thorax and in the abdomen. [source] Pulmonary oedema in Swedish hunting dogsJOURNAL OF SMALL ANIMAL PRACTICE, Issue 5 2003A. Egenvall A syndrome of acute dyspnoea after hunting in 16 Swedish hunting dogs is characterised. Radiographic pulmonary infiltrates interpreted as pulmonary oedema were found in the acute stage. In 12 dogs, the infiltrates regressed after five to 14 days. Subendocardial necrosis and pulmonary oedema were found at postmortem examination in four other dogs with acute and recurrent dyspnoea after hunting, and myocardial fibrosis in a further three dogs with a history of recurrent dyspnoea after hunting; none of these pathological changes was seen in dogs which had no previous history of dyspnoea after hunting. A pathogenetic mechanism is proposed whereby high catecholamine levels, present during hunting due to the stress of excitement and exercise, cause acute cardiac and pulmonary lesions in some susceptible dogs, similar to neurogenic or postictal pulmonary oedema. [source] Testing Genetic Susceptibility Loci for Alcoholic Heart Muscle DiseaseALCOHOLISM, Issue 10 2001Olli A. Kajander Background: Although many heavy alcohol users have subclinical alcoholic heart muscle disease, only a very few develop severe dilated cardiomyopathy. Therefore, and because cardiac abnormalities correlate only weakly with the duration or quantity of drinking, individual susceptibility differences may exist. In this work we examined whether common gene variants previously associated with cardiac hypertrophy or altered alcohol metabolism could modify the effects of alcohol on the heart. Methods: We studied 700 middle-aged male victims of sudden death who underwent a medicolegal autopsy. In addition to routine postmortem examination, the weights and the cavity and wall dimensions of the left and right ventricle were measured. Coronary artery stenoses were determined from a silicone rubber cast of the arteries. Alcohol consumption and cardiovascular risk factors were assessed by a structured interview of the spouse. The following gene polymorphisms were determined by using polymerase chain reaction restriction fragment length polymorphism and solid-phase minisequencing techniques: angiotensin converting enzyme I/D, angiotensin II type 1 receptor 1166A/C, aldosterone synthase ,344C/T, alcohol dehydrogenases 2 and 3, acetaldehyde dehydrogenase 2, and cytochrome P-450 2E1 Dra I, Pst I, Rsa I, and Msp I. Results: The most consistent effects of alcohol (p < 0.05) were a higher total heart weight and a larger right ventricle size with increasing daily drinking. However, these and other effects of alcohol were statistically fully independent of the studied genotypes. Conclusions: The gene polymorphisms selected for and analyzed in our study are unlikely to modify the effects of alcohol on the heart. Other unknown factors determine the individual susceptibility to alcoholic heart muscle disease. [source] Molecular Evidence for Persistence of Anaplasma phagocytophilum in the Absence of Clinical Abnormalities in Horses after Recovery from Acute Experimental InfectionJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 3 2009P. Franzén Background: Anaplasma phagocytophilum infects several mammalian species, and can persist in sheep, dogs, and calves. However, whether this organism persists in horses or induces long-term clinical abnormalities is not known. Objectives: To evaluate whether A. phagocytophilum can persist in horses and to document clinical findings for 3 months after complete recovery from acute disease. Animals: Five clinically normal adult horses that had recovered spontaneously from experimentally induced acute disease caused by a Swedish equine isolate of A. phagocytophilum. Methods: Horses were monitored for up to 129 days post inoculation (PI) by daily clinical examination and at least alternate day blood sampling for evidence of A. phagocytophilum on polymerase chain reaction (PCR) and blood smears. All horses were euthanized and underwent postmortem examination. Results: All horses were periodically PCR positive after recovery from acute infection. Before day 66 PI 2 horses were persistently PCR negative whereas 3 horses were intermittently PCR positive. Subsequently, 4 of 5 horses were intermittently PCR positive, particularly after stress mimicking interventions. One animal was positive immediately before postmortem examination. Clinical abnormalities related to persistence of anaplasma were not observed. No specific changes were found at postmortem examination, and all sampled tissues from all horses were negative on PCR for A. phagocytophilum. Conclusions and Clinical Importance: Infection with A. phagocytophilum can persist in the horse for at least 129 days. However, the continued presence of the organism is not associated with detectable clinical or pathological abnormalities. [source] Prognostic Usefulness of Blood Leukocyte Changes in Canine Parvoviral EnteritisJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 2 2008A. Goddard Background: Despite treatment, many dogs still die of complications related to canine parvoviral (CPV) enteritis. Effective prognostication would be beneficial in managing this disease. Hypothesis: We hypothesize that the occurrence of leukocytopenias at admission and at 24 and 48 hours after admission, and changes in absolute leukocyte counts over time, could be used to predict outcome. Animals: Sixty-two puppies with confirmed CPV. Methods: A prospective study was performed. CBC was performed daily until discharge or death (in which case a postmortem examination was performed). Results: Of the nonsurvivors (10/62; 16%), 9 died because of complications of the disease and 1 was euthanized because of a poor prognosis. There was a statistical significant difference in the occurrence of leukocytopenias between groups at 24 and 48 hours postadmission. The survivors showed a significant increase over time in certain leukocyte types (specifically lymphocytes) compared with values at admission. The positive predictive value for survivors was high. Nonsurvivors had marked thymic and lymphoid atrophy and marked bone marrow hypocellularity. Conclusion: An accurate prognosis could be obtained at 24 hours after admission by evaluating the change in total leukocyte, band neutrophil, lymphocyte, monocyte, and eosinophil counts. [source] Fatal encephalitis in a patient with refractory celiac disease presenting with myorhythmia and carpal spasmMOVEMENT DISORDERS, Issue 3 2007Elliot L. Dimberg MD Abstract We report the case of a woman with refractory celiac disease who developed abnormal spontaneous movements of the extremities and face consistent with myorhythmia. Investigation led to a diagnosis of encephalitis, confirmed by postmortem examination. The movements were likely caused by nonparaneoplastic encephalitis associated with refractory celiac disease. Etiologic and diagnostic considerations and treatment options are discussed. © 2006 Movement Disorder Society [source] Autopsy case of neuro-Behçet's disease with multifocal neutrophilic perivascular inflammationNEUROPATHOLOGY, Issue 6 2006Yoshifumi Arai We report here an autopsy case of neuro-Behçet's disease. The patient was a 28-year-old man, who developed a slight fever, right uveitis, and right sensory neural hearing loss at the age of 25. These symptoms relapsed repeatedly despite treatment. Eventually he was admitted to hospital because of progressing neurological deficits such as pyramidal symptoms, somatic sensorial and autonomic disorders, and bulbar palsy. The patient's condition deteriorated and he died of heart failure. Total clinical course was about three years. In postmortem examination, various-sized necrotic foci, often accompanied by gliosis and foamy macrophage infiltration, were scattered in the diencephalic region and brain stem. Meningitis was observed on the ventral side of the brain stem as well as inferior cerebral surface. Non-bacterial or non-fungal acute perivascular inflammatory foci were also present in the brain stem and cerebellar parenchyma. These histopathological findings suggest that a destructive multifocal neutrophilic inflammation might have caused the neurological deficits. Perivascular inflammation might be important to understanding the pathogenesis of neuro-Behçet's disease. [source] Wernicke's encephalopathy in nonalcoholic patients: Clinical and pathologic features of three cases and literature reviewedNEUROPATHOLOGY, Issue 3 2006Qiu-Ping Gui Three cases of Wernicke's encephalopathy in nonalcoholic patients diagnosed by postmortem examination were reported to improve the recognition of this disease. All three cases were male, ages ranged from 33 to 73 years old. All the cases had a clinical history of malnutrition but no history of chronic alcoholism. Routine autopsy and neuropathologic investigations examining the histological changes of the brain were performed. Pathological findings included recent petechial and local hemorrhages in the mamillary bodies, periventricular regions around the third and fourth ventricles and aqueduct. Under light microscopy the proliferation and dilatation of the capillaries was particularly prominent in the mamillary bodies and pericapillary hemorrhages were present in the periventricular regions. Neuronal losses were found only in the medial nucleus of the thalamus and inferior olive, myelin staining demonstrated demyelination and gliosis in those areas. The diagnosis of Wernicke's encephalopathy was made. In combination with the reviewed literature, our cases suggest that Wernicke's encephalopathy can occur not only in patients with alcohol abuse, but also in those who have suffered thiamine deficiency due to metabolic and nutritional disorders. [source] Peculiar venous lesions in fatal hyponatremic brain edemaNEUROPATHOLOGY, Issue 1 2005Makoto Nishie A 19-year-old woman with a 3-year history of schizophrenia suddenly began to vomit, and rapidly developed a coma an hour after the onset of vomiting. A brain CT scan showed diffuse brain edema with compression of the ventricles. Laboratory tests showed a low serum sodium concentration of 117 mmol/L. She died 67 h after the onset of the first symptom. A postmortem examination showed diffuse swelling of the brain with bilateral uncal and tonsillar herniations. Histologically, no necrotic, hemorrhagic or encephalitic changes were seen. However, microvacuolar changes with lymphocytic infiltration were found in the venous walls (media and adventitia) mainly in the basal ganglia, thalamus and brainstem. To our knowledge, this is the first demonstration of venous alterations in fatal hyponatremic brain edema. These changes may have participated in the exacerbation of the brain edema due to functional disturbance of venous drainage. [source] Atrial Linear Lesions: Feasibility Using CryoablationPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 3 2006KLAUS KETTERING Background: Long linear lesions are created in the left atrium to modify the atrial substrate, thereby curing atrial fibrillation. The creation of long linear left atrial lesions using radiofrequency (RF) ablation is time consuming and difficult. Furthermore, it might result in significant complications. Cryoablation might overcome some of the disadvantages of RF ablation. Therefore, the aim of our study was to assess whether the creation of a long linear lesion is possible using cryotherapy. Methods: A right atrial septal linear lesion was created in six pigs (median weight: 50 kg; range: 40,60 kg). The ablation procedure was performed with a 7-F Freezor cryocatheter. The nonfluoroscopic mapping system LocaLisa was used as a navigation tool. At each point, freezing was maintained at the lowest attainable temperature (,75°C) for 4 minutes. The CARTO system was used for the evaluation of the linear lesions. Furthermore, all animals were sacrificed immediately after the ablation procedure and a postmortem examination of the lesions was performed. Additionally, an analysis of the amplitudes of the intracardiac electrograms registered via the ablation catheter was performed before and after the ablation procedure. Results: A right atrial septal linear lesion could be created successfully in all six pigs. For the performance of this ablation line, a median number of 16 cryoapplications (range, 11,26) was necessary. The amplitudes of the intracardiac electrograms registered via the ablation catheter decreased significantly after ablation. The CARTO bipolar voltage map revealed very low potentials along the ablation line and showed a sharply demarcated ablation area at the septum in all pigs. Further analysis of the CARTO map revealed an incomplete conduction block in all cases. Most of the pigs had a small gap close to the fossa ovalis. The postmortem examination of 2,3,5-triphenyl-tetrazolium chloride-stained specimens showed sharply demarcated lesions without any ulcerations. There were no major complications during the procedure. Conclusions: The creation of long linear lesions using cryoablation is feasible and safe. Lesion characteristics are different and more favorable than those created by RF. However, the aim of creating a transmural lesion and a complete conduction block remains an unsolved problem even with current cryoablation techniques. Nevertheless, growing experience and technical improvements might overcome some of the current limitations of this new technique. [source] Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantationPEDIATRIC TRANSPLANTATION, Issue 2 2006Andrew C. Glatz Abstract:, Idiopathic infantile arterial calcification (IIAC) is a rare, but important, cause of rapidly progressive ischemic heart disease in children. In this paper, we report two recent cases of IIAC seen at tertiary referral hospitals. Both cases presented in infancy with signs of heart failure and, ultimately, died with the diagnosis of IIAC confirmed at postmortem examination. A thorough review of the literature reveals approximately 160 reported cases of IIAC. The clinical outcomes, radiographic findings and pathologic details are summarized. Proposed etiologic mechanisms are reviewed, including promising research into the role of inorganic pyrophosphate as a regulatory factor in the development of IIAC. Because of the typically fatal outcome of IIAC and the lack of proven therapies, the potential role for cardiac transplantation is discussed. [source] Minimally invasive fetal postmortem examination using magnetic resonance imaging and computerised tomography: current evidence and practical issuesPRENATAL DIAGNOSIS, Issue 8 2010S. Thayyil Abstract For a variety of reasons, acceptance of traditional postmortem examination following foetal or neonatal death has declined significantly in recent years in the UK. Here, we review the case for the development of less invasive autopsy using combined investigations including imaging techniques, in particular, magnetic resonance imaging and computerised tomography. Copyright © 2010 John Wiley & Sons, Ltd. [source] Structural heart defects associated with an increased nuchal translucency: 9 years experience in a referral centrePRENATAL DIAGNOSIS, Issue 4 2008S. A. Clur Abstract Objective To investigate the congenital heart disease (CHD) found in association with an increased nuchal translucency (NT) at 11,14 weeks of gestation in chromosomally normal and abnormal fetuses. Methods Patients referred from January 1998 until May 2007 with an increased NT (,95th percentile) where CHD was diagnosed were included. Chromosome analysis, fetal and postnatal echocardiography were performed. A postmortem examination followed pregnancy termination when possible. Results Major CHD was identified in 68 of 967 fetuses with an increased NT (median NT 4.8 mm, range 2.5,22 mm). Major CHD was found in 34 of 693 fetuses (4.9%) with a normal karyotype and increased NT (median 5.2 mm, range 2.5,9.6 mm). CHD frequency increased from 1.9%, with NT between 2.5 and 3.5 mm, to 27.7% when NT was , 6.5 mm. Septal defects predominated (20%) when NT was ,3.5 mm. With NT > 3.5 mm an equal distribution of CHD types was seen. Major CHD was identified in 34 of the 274 fetuses with an abnormal karyotype and increased NT (median 4.2 mm, range 2.5,22 mm). Conclusions A variety of CHD is associated with an increased NT in the first trimester of pregnancy. Conotruncal defects, branchial arch derivative defects, left and right obstructive lesions (inflow and outflow) and shunts were seen. Copyright © 2008 John Wiley & Sons, Ltd. [source] First-trimester diagnosis of hydrolethalus syndrome in a Chinese familyPRENATAL DIAGNOSIS, Issue 8 2004Ben Chong Pun Chan Abstract We report a case resembling hydrolethalus syndrome in a Chinese family. Fetal polydactyly, syndactyly, encephalocele and cardiac malformation were detected on ultrasound examination at 12 weeks' gestation. Termination of pregnancy was performed, and postmortem examination confirmed the findings. This is the first report of a first-trimester prenatal diagnosis of hydrolethalus syndrome in the Chinese population. Copyright © 2004 John Wiley & Sons, Ltd. [source] Prenatal findings on ultrasound and X-ray in a case of overgrowth syndrome associated with increased nuchal translucencyPRENATAL DIAGNOSIS, Issue 5 2001P. Schwärzler Abstract A case of prenatal diagnosis of an overgrowth syndrome at 30 weeks of gestation is reported. The diagnosis was suggested on the basis of increased fetal growth from 16 weeks onwards, advanced bone age, and characteristic facial features such as hypertelorism, broad forehead and small chin. The fetus presented at 12 weeks with a markedly increased nuchal translucency thickness and generalized skin edema, but normal karyotype. Serial ultrasound scans revealed brain abnormalities including mild unilateral ventriculomegaly and a cyst in the cavum septi pellucidi. The pregnancy was terminated at the parents' request at 32 weeks of gestation and postmortem examination confirmed the prenatal findings. This case demonstrates the possibility of prenatal diagnosis of early overgrowth syndromes and highlights the dilemma arising from the prenatal diagnosis of a non-lethal condition associated with an uncertain prognosis and poorly documented in utero. Copyright © 2001 John Wiley & Sons, Ltd. [source] | ||||||||||||||||||||||||||||||||||