Thoracic lymphangiomatosis

The prognostic value of p53 protein in tumor extracts as measured by ELISA was studied retrospectively in 228 non-small cell lung cancer (NSCLC) patients. The assay measures both wild-type and mutated p53. The specimens on which this study was performed have been used earlier to analyze the prognostic impact of components of the plasminogen activation system, which enabled an analysis of relationships between these components and p53 protein. The median of the p53 protein values in the 228 patients was 0.10 (range, 0-0.70) ng/mg protein. Survival analysis comparing patients with p53 levels below versus above the median showed no significant difference (P = 0.67). When analyzing the histological types, adenocarcinoma (n = 106), squamous cell carcinoma (n = 84), and large cell carcinoma of the lung (n = 38) separately, similarly, no significant differences in survival between patients having low versus high tumor p53 levels were found. When comparing levels of p53 protein in the three histological types, a significant difference (P < 0.0001) was found, with adenocarcinomas having the lowest levels. There was a weak positive correlation (r = 0.22) between p53 protein and plasminogen activator inhibitor type 1 (PAI-1). Multivariate analysis proved no impact of p53 on survival; tumor size, PAI-1, and lymph node involvement were the only variables with significant influence on survival. These data indicate that p53 protein quantitated with a sandwich ELISA in tumor extracts from NSCLC has no prognostic value, but the observed statistically significant difference of p53 protein content between histological subgroups may be related to differences in etiology and biology in different NSCLC subtypes. In addition, the weak association found between p53 protein and the independent prognostic marker PAI-1 could suggest yet undefined interactions in lung cancer.     

Hepatic lymphangiomatosis mimicking polycystic liver disease

Hepatic lymphangiomatosis is a rare disorder characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma. It can occur in the liver alone, in the liver and spleen, or in multiple organs. Clinically, diagnosis can be difficult because of the rarity and protean manifestations of this disorder. We describe a 53-year-old woman with hepatic lymphangiomatosis in whom polycystic liver disease had been previously diagnosed. In addition, we review 12 cases of hepatic, splenic, and hepatosplenic lymphangiomatosis with or without systemic lymphangiomatosis and discuss the differential diagnosis.     

Partial splenic embolization for treatment of disseminated intravascular coagulation in lymphangiomatosis

A 1-year-old boy presented with pericardial effusion, pulmonary infiltrates, and disseminated intravascular coagulation; lung biopsy indicated pulmonary lymphangiomatosis. He did not respond to medical therapy and was a poor surgical candidate; therefore, he underwent partial splenic embolization. The procedure resulted in a complete disappearance of the DIC and marked improvement in his cardiorespiratory status. He continues to thrive and is transfusion-independent 2 years after the procedure.     

Disseminated intra-abdominal cystic lymphangiomatosis with severe intestinal bleeding. A case report

We describe cystic lymphangiomatosis with intestinal bleeding developing multiple lymphangiomas in the small intestine, mesentery, mesocolon, omentum, retroperitoneum, and spleen. Small intestinal fluorography showed multiple polypoid lesions, mainly in the jejunum. Ultrasonography, computed tomography, and magnetic resonance imaging showed diffuse cystic tumors in the mesentery and spleen. Cystic lymphangiomatosis was proved by histologic findings of the biopsied specimen at laparotomy.     

Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect

Angiograms of 30 patients with congenital pulmonary valve atresia, ventricular septal defect, and large systemic-pulmonary collateral arteries (SPCAs) were evaluated. All had aortography, 28 had SPCA arteriography, and 26 had right ventriculography. Seventeen (65%) of 26 patients had a right ventricular infundibulum, 23 (77%) had a pulmonary artery confluence, and five of the nine patients without a right ventricular infundibulum had a confluence. Sixty-six SPCAs of aortic origin were seen; 28 (42%) had narrowing and 21 patients (70%) had one or more narrowed SPCAs. Five patients had collaterals from internal mammary, subclavian or innominate arteries. Fourteen (47%) had hilar pulmonary artery stenosis. Of these 14 patients mild peripheral stenosis was demonstrated in five. Right aortic arch was present in 15 patients (50%). Complete angiographic delineation of pulmonary vasculature is an essential procedure for preoperative detection of pulmonary and SPCA stenoses in these patients.     

Systemic calciphylaxis revisited

A syndrome characterized by rapidly progressive ischemic necrosis involving large areas of the skin and muscle, and by peripheral gangrene associated with extensive vascular calcifications was observed in a patient with end-stage renal failure on chronic hemodialysis. In an effort to control the disease, parathyroidectomy was performed which resulted in rapid improvement of tissue perfusion. However, the patient eventually died from sepsis within 2 months after admission. This case presents the typical features of the syndrome of systemic calciphylaxis. The literature is reviewed searching for similar cases of this poorly recognized, but life-threatening, clinical syndrome. The pathogenesis, clinical manifestations, and therapy of this unusual and rapidly progressive, but potentially reversible, condition are reviewed with emphasis on its prompt recognition and appropriate management.     

Systemic necrotizing vasculitis

The revival of interest in systemic necrotizing vasculitis was initiated by the discovery of its association with anti-neutrophil cytoplasmic antibodies (ANCA). The close association of certain ANCA subspecificities, for example, proteinase 3 (Pr3) and myeloperxoidase ANCA, with Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome has led to their designation as 'ANCA-associated vasculitides'. This article describes the common and divergent clinical and immunological features of the members of this 'new' family of systemic necrotizing vasculitis, which continues to grow with the widespread use of ANCA testing. In addition, the 'standard' treatment for systemic necrotizing vasculitis (daily 'low dose' cyclophosphamide plus glucocorticosteroids or 'Fauci's scheme') is compared with new stage and activity adapted therapeutic regimens.     

Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis

After we identified several novel cDNAs by screening a neonatal (P1) heterozygous weaver (wv/+) cerebellar cDNA expression library with a rabbit anti-mouse granule cell antiserum, we characterized and sequenced one cDNA, GCAP-8 (standing for granule cell antiserum positive, clone number 8). In this study we examined its expression and cellular distribution in adult cerebellar mutant mice as evidenced by in situ hybridization histochemistry. In wild-type (+/+) brain, strong hybridization signal is seen in cerebellum, hippocampus, substantia nigra (SN), and cerebral cortex; in the cerebellum, hybridization signal is seen in granule cells, Purkinje cells, and in cells of the deep cerebellar nuclei. In the granuloprival weaver (wv/wv) cerebellum, hybridization signal is seen mainly in Purkinje cells. GCAP-8 expression is reduced in wv/wv SN pars compacta, which is known to lose dopamine (DA) neurons. In Purkinje cell degeneration (pcd/pcd) mutants, granule cells show hybridization signal, but overall expression is decreased owing to the absence of Purkinje cells. In reeler (rl/rl) cerebellum, the strongest hybridization signal is found in a thin granule cell layer without the typical foliation pattern, while grain clusters representing ectopic Purkinje cells are observed in the subcortical white matter and the area of the deep cerebellar nuclei. GCAP-8 expression in the reeler hippocampus and cerebral cortex shows a mixing of layers, which is known to be an aspect of the histological phenotype of this mutant.(ABSTRACT TRUNCATED AT 250 WORDS)     

Systemic angioendotheliomatosis with metastases

The clinical and pathological findings of a case of systemic angioendotheliomatosis are presented. The previously published cases of this disease are analysed. The present case is only the third to show clear evidence of metastasis.     

Systemic inflammatory response syndrome

BACKGROUND: Localized inflammation is a physiological protective response which is generally tightly controlled by the body at the site of injury. Loss of this local control or an overly activated response results in an exaggerated systemic response which is clinically identified as systemic inflammatory response syndrome (SIRS). Compensatory mechanisms are initiated in concert with SIRS and outcome (resolution, multiple organ dysfunction syndrome or death) is dependent on the balance of SIRS and such compensatory mechanisms. No directed therapies have been successful to date in influencing outcome. METHOD: This review examines the current spectrum and pathophysiology of SIRS. RESULTS AND CONCLUSION: Further clinical and basic scientific research is required to develop the global picture of SIRS, its associated family of syndromes and their natural histories.     

Systemic mycoses in marine mammals

Thirty-four cases of systemic mycosis in marine mammals were represented by 9 genera of fungi. All cases were characterized by pulmonary involvement, with miliary spread to abdominal organs. Cases were geographically distributed among Hawaii, northern United States, Europe, New Zealand, and southern Asia.     

Systemic lupus erythematosus in India

The first case of systemic lupus erythematosus (SLE) was reported from India in 1995 followed by two more case reports and further, a series of eight cases, till 1969. Since the establishment of a clinical immunology laboratory at a major teaching institution in New Delhi in 1968, SLE was extensively studied and reported from that centre. From mid-1980 onwards several other centres in different regions in India including Chennai (old name Madras), Mumbai (old name Bombay), Calcutta and Hydrabad, also published their regional experience on SLE. Based on these data, the present report describes the clinical and laboratory characteristics of 1366 SLE patients seen in different regions of India. Arthritis, rash, photosensitivity, seizures and psychosis were seen in comparable proportions to other racial groups. Similarly, ANA and anti-DNA antibody positivity was also within the range seen in other racial groups. When compared with other series, however, alopecia, renal lupus, oral ulcers and neurological involvement was seen in higher proportions, reaching statistically significant figures in comparison to some racial groups. In contrast, haematological manifestations were seen in significantly less proportions in comparison to some of the racial groups. Serositis and discoid lesions were also seen in lower proportions than in most of other races. The proportion of those with anti-Sm antibodies was in between two extremes of highest among Africans and Israelis and lowest among Chinese and Europeans. Other manifestations were comparable to most other racial groups. Compared to North American and European reports, significantly low 5 and 10 year survival was observed among patients from India. This could be related to the general public health situation in the country including less than optimal management facilities in hospitals, delay in diagnosis due to lack of awareness of the disease, referral bias where only serious patients reach major city hospitals, or a truly severe disease among Indians, or a combination of these genetic, environmental and/or sociocultural factors. The Main causes of death were irreversible renal damage, infections and neurological involvement. Despite a comparable prevalence of anticardiolipin antibodies (aCL) and lupus anticoagulants (LAC), clinical antiphospholipid syndrome was significantly less common. Genetic studies showed appreciable increase of HLA DR4 (37.5%) among patients compared with controls (18%). Additionally the haplotype B8-DR3 was encountered frequently in the patient group.     

Systemic consequences of acid-base disorders

It is apparent that disturbances in acid-base equilibrium, particularly in the direction of acidosis, interfere with the normal physiologic function of a number of organs. While the effect on each organ system may not be severe, and while adequate compensatory mechanisms may come into play, cummulative involvement of a number of organs, especially when the changes are acute or left untreated, may prove fatal.     

Systemic hypertension and its management

The pathophysiology of hypertension in dogs and cats, the methods available to monitor blood pressure, and the signs and treatment of hypertension are reviewed. Clinical signs of hypertension are usually referable to target organ damage, most notably in ophthalmic, renal, and cardiovascular tissues, which have a rich arteriolar supply. Blood pressure should be measured in any animal with renal disease, hyperthyroidism, hyperadrenocorticism, retinal detachment or hemorrhage, hyphema, or echocardiographically determined cardiac hypertrophy. All cats with acquired cardiac murmur should also be evaluated for hypertension. Antihypertensive medication should be administered if the indirect blood pressure in cats is consistently over 170/100 mmHg, or if the indirect blood pressure in dogs is greater than 180/100 mmHg.     

Systemic amyloidosis in cystic fibrosis

We report two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis.