Subepithelial collagen in intestinal malabsorption.

Intestinal biopsies from 146 patients with adult coeliac disease and 13 patients with intestinal villous atrophy of different aetiology were assessed for the presence of subepithelial collagen and compared with a group of 20 control subjects. Subepithelial collagen was a common and non-specific finding observed in intestinal biopsies from patients suffering from adult coeliac disease (36%) and tropical sprue. In adult coeliac disease the described subepithelial changes usually regress following treatment, though marked subepithelial collagen deposition may indicate a poor prognosis. The study showed that the presence of marked subepithelial collagen in a flat jejunal biopsy does not define a separate clinical entity.     

Histopathological study on myocardial hypertrophy associated with ischemic heart disease

The mode and causes of myocardial hypertrophy occurring in association with ischemic heart disease were studied. The investigation involved autopsied hearts (15 cases of subendocardial infarction, 27 of transmural infarction, 20 of non-infarcted three vessel disease and 17 controls) and biopsied materials obtained during coronary-aorta bypass graft surgery (23 patients with angina pectoris and 46 with myocardial infarction). The subendocardial infarction group showed most marked myocardial hypertrophy that reflected extensive infarction and fibrosis, dilatation of the left ventricular cavity and the loss of myocytes. Despite a marked decrease in the number of myocyte layers, the residual myocardium of the left ventricle was uniformly hypertrophic, accompanied by an increase in the heart weight. The larger the area of fibrosis, the more marked was myocardial hypertrophy irrespective of the luminal diameter of the responsible coronary artery. These findings indicate that myocardial hypertrophy associated with ischemic heart disease is enhanced by the compensatory mechanisms for a decrease in the contractile myocardium due to fibrosis.     

Immunoglobulin containing cells in inflammatory bowel disease of the colon: a morphometric and immunohistochemical study.

Immunoglobulin containing cells in rectal and sigmoid colonic mucosa in endoscopically obtained biopsies from 10 patients with ulcerative colitis and 10 patients with Crohn's disease were studied, using an indirect immunoperoxidase technique. These findings were compared with the immunoglobulin containing cell number in colonic biopsies from 10 control patients with no evidence of colitis. In biopsies from the 20 patients with inflammatory bowel disease a marked increase in area of the lamina propria per millimetre mucosa length was found. In ulcerative colitis a marked increase in number of IgG containing cells was observed. In Crohn's disease the increase in IgG containing cell number is dependent on the degree of activity of inflammation. In quiescent of active Crohn's disease of the colon we found a significant increase of the IgM containing cells. The number of IgM containing cells per millimetre mucosa length will differentiate the pathology of Crohn's disease from ulcerative colitis.     

Lymphadenopathy in coeliac disease

We present two cases of adult coeliac disease whose major clinical feature was marked lymphadenopathy. One patient also displayed a peripheral lymphocytosis which varied with treatment of the underlying disease.     

Electrocardiographic loading tests in persons with various ECG changes (findings of epidemiological studies)

The results from a complex of functional electrocardiographic tests (exercise test, transesophageal pacing of the left atrium, dipyridamole test and cold test) were compared with resting ECG data by using epidemiological findings from a study conducted in Novosibirsk. In the absence of other signs of the disease, coronary heart disease was verified in 11.1% of the persons with prominent Q-QS waves, in 10.0% of those with small Q-QS, in 16% of those with slightly altered ST segment and T wave, in 35.3% with marked altered ST-T interval, and in 50% of those with marked altered ST-T interval in the presence of left ventricular hypertrophy. The total number of cases with coronary heart disease amounted to 20.8% in the presence of ischemic ECG changes.     

An autopsy case of Alzheimer's disease associated with Parkinson's disease, compared to 2 autopsy cases of diffuse Lewy body disease

A 68 year old male, diagnosed as Alzheimer's disease clinically, pathologically showed both findings of Alzheimer's disease and Parkinson's disease. The brain weight was 940 g. Macroscopically, severe cortical brain atrophy and depigmentation of the substantia nigra was noted. Microscopic examination showed marked appearance of senile plaque and a large number of neurofibrillary tangle with sever neuronal loss of the cerebral cortex. Additionally, the loss of neuron with many Lewy bodies was found in the substantia nigra. Lewy bodies were also found in the locus ceruleus and the dorsal vagal nucleus, but few in the cerebral cortical neurons. We compared this case neuropathologically with two autopsy cases of diffuse Lewy body disease (DLBD). There was no distinction concerning the lesions of the brain stem between this case and the cases of DLBD. In all three cases, the nucleus of basalis of Meynert showed marked neuronal loss. However, the brain was lighter than those of the cases of DLBD. Senile changes such as senile plaque and neurofibrillary tangles were more marked in this cases than in the cases of DLBD. Furthermore a large number of cortical Lewy bodies were found in the cases of DLBD, but few in this case. The distribution and number of Lewy bodies did not correspond with those of senile changes in the cases of DLBD. Also the cerebral cortical structure was better preserved in the cases of DLBD than in this case. In conclusion, from the clinicopathological findings, we considered that this case is Alzheimer's disease associated with Parkinson's disease. According to Kosaka's study, this case seemed to correspond with a transitional type of the Lewy body disease.     

Phospholipid transmethylation and choline phosphotransferase in microsomal fraction of human diseased liver

The activities of three enzymes catalyzing the production or degradation of phosphatidylcholine, a major structural phospholipid of cell membranes, were assessed in hepatocyte membrane microsomal preparation from patients with various types of liver disease. Choline phosphotransferase activity of preparation from patients with chronic aggressive, chronic active, chronic persistent, alcoholic hepatitis and cirrhosis accompanied by marked necrosis and relatively slight fibrosis was markedly decreased, compared with normal liver; the activity from patients with fatty liver and chronic inactive hepatitis was slightly decreased. Specimens from patients with acute transient hepatitis were not significantly different from normal. Methyltransferase and phospholipase A2 activities tended to parallel that of choline phosphotransferase, although the degree of changes was generally less marked. Our studies indicate that enzyme activities that are critical for hepatic cell membrane integrity and activity are attenuated in liver specimens from patients with disease in which there is marked hepatic cell necrosis.     

Improved morbidity with the use of nasal continuous positive airway pressure in I-cell disease

Patients with I-cell disease (mucolipidosis II) present with progressive morbidity failure to thrive, cardiomegaly, and recurrent respiratory tract infections leading to progressive deterioration and early death. We evaluated use of nasal continuous positive airway pressure (NCPAP) for 6 months in a 2-year-old girl with I-cell disease, obstructive sleep apnea (OSA), and craniofacial anomalies. We observed a marked decrease in hospitalizations for respiratory problems and a marked improvement in arterial blood gases with the use of NCPAP. In patients with I-cell disease, anatomical defects with superimposed upper respiratory tract infections cause worsening of OSA, and OSA contributes significantly to morbidity. In such patients NCPAP can lessen morbidity and can improve the quality of life. Pediatr. Pulmonol. 1998; 25:128–129. © 1998 Wiley-Liss, Inc.     

Photodynamic therapy: Palliation and endoscopic technique in cholangiocarcinoma

Cholangiocarcinoma is the primary malignancy arising from the biliary epithelium. The disease is marked by jaundice, cholestasis, and cholangitis. Over 50 percent of patients present with advanced stage disease, precluding curative surgical resection as an option of treatment. Prognosis is poor, and survival has been limited even after biliary decompression. Palliative management has become the standard of care for unresectable disease and has evolved to include an endoscopic approach. Photodynamic therapy (PDT) consists of administration of a photosensitizer followed by local irradiation with laser therapy. Several studies conducted in Europe and the United States have shown a marked improvement in the symptoms of cholestasis, survival, and quality of life. This article summarizes the published experience regarding PDT for cholangiocarcinoma and the steps required to administer this therapy safely.     

Limb-threatening lower extremity ischemia successfully treated with intra-arterial infusion--case reports

The authors present two patients with acute arterial vasospasm of the lower extremities causing marked ischemia. One patient had a history of Raynaud's disease, the second had been taking Cafergot for migraine headaches. Both patients's were given a test dose of intra-arterial tolazoline (50 mg). The patient with Raynaud's disease demonstrated marked improvement diffusely and was successfully treated with overnight infusion of papaverine. The second patient, taking Cafergot, demonstrated no angiographic response to tolazoline. It was speculated that the arteries of this patient were thrombosed. The patient was successfully treated with urokinase and remained free of pain at the 15-month follow-up.     

Biclonal lymphoplasmacytic immunocytoma associated with Crohn's disease.

A 33-year-old man with a 4-year history of Crohn's disease presented with marked ascites and an abdominal tumor. Two M-protein peaks, immunoglobulin (Ig) G-kappa and IgA-kappa, were detected in the serum. Neoplastic lymphoplasmacytic cells were infiltrated in the bone marrow and ascites. Histological examination of the abdominal tumor showed marked proliferation of lymphoplasmacytic cells that were positive for either IgG or IgA. Moreover, DNA sequences of the expressed IgG and IgA genes were different in the complementarity-determining region 3. These results suggest that chronic inflammation in Crohn's disease contributes to the simultaneous development of biclonal lymphoplasmacytic immunocytoma of the small intestine.     

胱抑素C与冠心病相关性的研究进展

既往研究已明确胱抑素C是评价肾功能损伤的一项敏感指标,然而近年的研究提示胱抑素C跟动脉粥样硬化包括冠心病以及外周动脉疾病等的发生和发展及预后密切相关,并且这种相关性反映了胱抑素C生物学的非肾性方面,其中可能通过参与炎症过程而发挥作用.     

胱抑素C与心血管疾病相关性的研究进展

既往研究已明确胱抑素C是评价肾功能损伤的一项敏感指标,然而近年的研究提示胱抑素C与组织蛋白酶等相互作用,参与了动脉粥样硬化心肌梗死等心血管疾病的病理过程,这种相关性反映了胱抑素C生物学的非肾陛方面,其中可能通过参与炎症过程而发挥作用。     

Quantitation of fibrosis of the heart in chronic obstructive pulmonary disease with and without cor pulmonale

This study examined the hearts of 55 patients dying of chronic obstructive pulmonary disease, with and without cor pulmonale, quantitated histologically the degree of myocardial fibrosis in the left and right ventricle, and determined the relationship to associated disease states. Comparison has been made to a control group of 17 patients free of cardiopulmonary disease. Patients with associated and advanced ischemic heart disease, as proved by marked atherosclerosis and myocardial infarction, have significantly increased myocardial fibrosis throughout all layers of the left ventricular wall in comparison to control patients or patients with chronic obstructive pulmonary disease free of associated cardiac disease. Right ventricular fibrosis was not significantly increased; however, one case showed a marked degree of fibrosis related to myocardial infarction. Subdivision of patients with chronic obstructive pulmonary disease into groups with definite anatomic right ventricular hypertrophy, a clinical diagnosis of cor pulmonale, or with chronic hypoxemia failed to show any difference in the percentage of myocardial fibrosis of the ventricles among these groups. Increased fibrosis of the right or left ventricle in patients with chronic obstructive pulmonary disease, therefore, is not related to the degree of myocardial hypertrophy pathologically, the hypoxemic state, or clinical heart failure, but to ischemic heart disease with myocardial infarction.     

Invasive aspergillosis of the lung and pericardium in a nonimmunocompromised 33 year old man

In a 33 year old man with no discernible immunologic defect, invasive aspergillosis developed in both the pericardium and lung with marked granulomatous reaction. The patient received 2 g of intravenous amphotericin B over eight weeks, with partial regression of the pulmonary infiltrate and disappearance of symptoms. However, five months later, he returned with marked progression of his disease. Evaluation of host defense, including granulocyte and lymphocyte function, was normal. The patient was given an additional 3 g of amphotericin B over nine weeks with marked improvement in symptoms and chest roentgenogram. At six-month follow-up, he was asymptomatic with a stable radiographic appearance. A recurrence in symptoms and the pulmonary infiltrate was noted two months later. He was treated with an additional course of amphotericin and currently is receiving ketoconazole in hopes of suppressing the infection. We could find no immune impairment to explain the severe pulmonary and pericardial disease due to Aspergillus flavus in this young man.     

Inflammatory Demyelinating Polyradiculoneuropathy Associated with Interstitial Lung Disease

A 58-year-old woman presented with inflammatory demyelinating polyradiculoneuropathy accompanied by sensory and motor disturbance and interstitial lung disease. Corticosteroid therapy led to a marked amelioration of both the neuropathy and the lung disease. We suggest that a demyelinating neuropathy is associated with an interstitial lung disease. Received: 20 April 1999 / Accepted: 1 December 1999     

Cryptosporidium infection in acquired immunodeficiency syndrome: not always a poor prognosis

Chronic diarrhea and malabsorption accompanied by simultaneous infection with the protozoa Giardia lamblia and Cryptosporidium occurred in a 22-year-old homosexual man with antibody to human immunodeficiency virus (HIV). Small bowel biopsy demonstrated total villous atrophy and marked mononuclear infiltration in the lamina propria simulating celiac disease. Treatment with metronidazole resulted in resolution of diarrhea, clearance of parasites, and marked improvement in small bowel histology. Although diarrhea and malabsorption in immunocompromised patients with cryptosporidiosis are regarded as ominous, our patient remained disease free for the next 3 years. Thus, infection with Cryptosporidium in patients with HIV does not always lead to intractable diarrhea or death.     

Chronic rheumatic heart disease in India: a reappraisal of pathologic changes.

Rheumatic heart disease contributes to significant cardiac morbidity and mortality in India. The disease predominantly affects the valvular endocardium culminating in crippling valvular deformities, preferentially involving the mitral valve which may be severely affected in children and young adults. This appears to be unique to India and has been termed juvenile mitral stenosis. It is characterized by cardiomegaly, refractory congestive heart failure, and marked by elevated pulmonary vascular pressures and a progressive, fulminant clinical course. Autopsies of patients dying of rheumatic heart disease revealed that the mitral valve was most commonly afflicted either alone or in combination with the aortic and tricuspid valves in 31.6% and 52.8%, respectively. Organic involvement of the tricuspid valve was documented in 38.4% of cases. The extent and severity of the disease process was most marked in the mitral valve, followed by the aortic and tricuspid valves. Mitral valves showed various degrees of calcification, moderate or severe calcification being observed in 36.4%. Chronic inflammatory cell infiltration was observed in both calcified and non-calcified valves. The phenotypic profile of the inflammatory cells by immunohistochemical staining revealed a significant number to be T-helper/inducer lymphocytes. Lungs from cases of mitral stenosis exhibited prominent vascular and parenchymal changes. Pulmonary vessels revealed moderate to marked medial hypertrophy of the medium sized branches of the pulmonary artery. Dilatation lesions were also seen in a few cases. The most striking parenchymal change was the prominent smooth muscle in the bronchoalveolar walls. The extent and severity of the vascular and parenchymal changes were more marked in juvenile patients. The presence of inflammatory cells in cases of chronic heart disease reflects a possible ongoing insult/injury to some persistent antigenic stimulus by beta hemolytic streptococcal antigens that have primed the various target tissues. Further study of surface characteristics of various mesenchymal cells may help in understanding the nature and pathogenesis of this serious cardiac problem.     

Adaptive immune responses during acute uncomplicated and fulminant hepatitis E

Background and Aim: Hepatitis E virus (HEV) infection is endemic in several developing countries. Clinical manifestations of this infection vary widely from asymptomatic infection to uncomplicated acute viral hepatitis and fulminant hepatic failure. The pathogenesis of this disease and the reason of varying disease severity remain unknown. In viral infections, tissue injury can be caused either by virus itself or by host immune responses directed against infected cells. We therefore studied adaptive immune responses to HEV antigens in patients with hepatitis E of varying disease severity and healthy controls. Methods: Cytokine secreting CD4+ T cells and antibody‐producing B cells specific for HEV were enumerated through intracellular cytokine staining and enzyme‐linked immunosorbent spot assay, respectively. Results: Patients with fulminant hepatitis E had a less marked expansion of HEV‐specific interferon‐γ or tumor necrosis factor‐α secreting CD4+ T cells than patients with uncomplicated hepatitis E and healthy controls. These patients also had fewer CD4+ T cells that produce γ‐interferon or tumor necrosis factor‐α upon in vitro polyclonal stimulation. In addition, patients with fulminant disease had a more marked expansion of B cells that can secrete immunoglobulin G anti‐HEV than patients with uncomplicated infection and control patients. Conclusion: These findings suggest that less‐marked antiviral cellular immune responses and heightened antiviral humoral responses are associated with a more severe disease during HEV infection.     

The relationship of marked left axis deviation to the risk of ischemic heart disease

The purpose of this study was to examine the value of marked left axis deviation in men without apparent heart disease in the assessment of ischemic heart disease risk. In the Manitoba Study, a cohort of 3983 men who were predominantly between 25 to 34 yr of age at entry in 1948, 247 cases of marked left axis deviation (mean frontal plane QRS vector of −45° to −90°) were identified at a mean age of 46.1 ± 0.7 (±1 SEM) yr with a mean follow-up of 12.1 ± 0.6 yr. The results were that the development of left axis deviation in men 40 to 59 yr of age, independent of blood pressure is a significant predictor of ischemic heart disease events that are usually manifest 5 to 10 yr after the onset of this electrocardiographic abnormality. Subsets of QRS variables examined to identify cases with different degrees of ischemic heart disease risk revealed that neither Q waves in leads I or aVL, or QRS duration or actual frontal plane QRS vector identified groups with different ischemic heart disease risk. However, subsets of age (less than 40 and 60 yr or greater) perhaps reflecting different etiologies of marked left axis deviation and previous electrocardiographic findings (S₁S₂S₃ pattern) identify a low risk group.