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PubMed2006

Autoimmune pancreatocholangitis: a series of ten patients.

BACKGROUND: During a 10-year period we observed 10 patients who suffered from an inflammatory-fibrosing disease mimicking pancreatic carcinoma and primary sclerosing cholangitis (PSC). METHODS: A review of the presenting features, the clinical course and the relevant literature. RESULTS: Ten male patients (mean age 55 years) presented with weight loss, jaundice and pruritus. Pancreatic cancer was suggested by imaging studies, which showed focal or generalized pancreatic enlargement and compression of the distal common bile duct. Cholangiography also demonstrated intrahepatic biliary stenoses consistent with sclerosing cholangitis. None had evidence of IBD. Exocrine pancreatic insufficiency was found in six cases and diabetes in four. Pancreatic histology (n=3) showed fibrosis and extensive inflammatory infiltrates. Immunosuppressive treatment was instituted in five patients. Clinical and biochemical remission occurred in three; in one other patient, previously documented intrahepatic biliary strictures had disappeared after 3 months. One patient had concomitant Sjögren's disease. The clinical features, pancreatic involvement, age at presentation, absence of IBD and response to steroids all plead against a diagnosis of "classical" PSC. The natural course of the disease was highly variable. Thirty-five comparable cases, with a largest series of three, have been reported in the literature. The disease has been associated with Sjögren's disease, retroperitoneal fibrosis and other fibrosing conditions, and may be a manifestation of a systemic fibro-inflammatory disorder. CONCLUSION: Autoimmune pancreatocholangitis is a distinct inflammatory disorder involving the pancreas and biliary tree. The disease may mimick pancreatic carcinoma and PSC and responds to immunosuppressives.

Adult↗

PubMed1991

Measurement of lung tissue mass in interstitial lung disease.

A gamma camera and 99m Technetium were used to obtain transmission and emission scans of the thorax in order to estimate transthoracic tissue thickness, and volumes of blood and interstitial tissue in each pixel of the gamma camera image. This technique has been applied to six patients with stage 2/3 pulmonary sarcoidosis and eight with cryptogenic fibrosing alveolitis and age and sex-matched control groups without lung disease. In both conditions transthoracic tissue thickness was significantly increased: mean values (SD) were 14.6 (1.8) cm in cryptogenic fibrosing alveolitis and 12.3 (2.2) cm in stage 2/3 sarcoidosis. This was partially accounted for by an increase in the interstitial fluid compartment. Blood volume was unaffected. Measurement of transthoracic tissue thickness involves minimal radiation exposure and may be of value in monitoring these diseases; measurement of interstitial fluid volume may give information on disease activity.

Adult↗

PubMed1992

Growth and phenology of seedlings of four contrasting slash pine families in ten nitrogen regimes.

Seedlings of two fast- and two slow-growing families of slash pine, Pinus elliottii Englm. var. elliottii, were grown in a greenhouse for one growing season in one of 10 nitrogen (N) regimes. Increasing the N concentration in the nutrient solution resulted in both increased growth rates during the exponential growth phase and extended duration of the growing season. The two components of total height, free growth (epicotyl length to the first bud) and summer growth (height growth after the first bud), both increased significantly with increasing N concentrations up to 40-60 mg l(-1) but decreased at N concentrations above 180 mg l(-1). Compared to seedlings grown in the presence of an optimum N concentration, seedlings grown in the presence of trace amounts of N were smaller and had less summer growth as a percentage of total growth, earlier cessation of height growth, fewer flushes, lower shoot/root ratio, higher root fibrosity, and lower N concentrations in all seedling tissues. Compared to slow-growing families, fast-growing families had more summer height growth, more flushes and later growth cessation, higher shoot/root ratios and higher root fibrosity at all N concentrations. In the presence of an optimum or higher concentration of N, the fast-growing families also had higher needle and total N concentrations than the slow-growing families. Strong family by N-treatment interactions occurred for height, phenology and biomass traits because of the extra responsiveness of one family to increasing N concentration. Several seedling traits were identified that appear promising for predicting field performance in slash pine. The results indicated that the nutrient environment greatly influences genetic expression (e.g., family patterns of summer growth were most closely related to field rankings for seedlings in the trace-N treatment).

Journal Article↗

PubMed1993

Hepatitis B virus infection in liver allografts.

Liver transplantation of hepatitis B surface antigen (HBsAg)-positive patients has been associated with high morbidity and mortality secondary to hepatitis B (HB) recurrence in the graft. Eight patients of the Queensland Liver Transplant Service were HBsAg positive pretransplant. Six acquired HB infection of the graft, one developed serological recurrence of HB before early death from sepsis, and one HB e antigen-negative patient permanently cleared the virus. HB-infected grafts showed early expression of viral antigen, acute hepatitis, fibrosing cholestatic hepatitis, chronic active hepatitis, cirrhosis, or minimal changes associated with a carrier state. Only in the latter case was HB mild and nonprogressive. Cases of fibrosing cholestatic hepatitis progressed rapidly to liver failure; they showed fibrosis and plates of ductular epithelium extending from portal tracts into lobules, cholestasis, ballooning of hepatocytes, and prominent hepatocyte expression of viral antigens. Perioperative HB immunoglobulin proved ineffective in preventing HB recurrence. One other patient became HBsAg positive for the first time after retransplantation; he developed severe acute hepatitis, then chronic active hepatitis. Our biopsy findings support the view that, in liver allografts, the HB virus may be directly cytopathic.

Acute Disease↗

PubMed1989

Uptake and kinetics of Tc-99m hexakis 2-methoxy isobutyl isonitrile in benign and malignant lesions in the lungs.

Kinetics of Tc-99m MIBI uptake was studied in 19 patients with lung lesions (6 benign, 13 malignant). Two dynamic studies were acquired after the I.V. injection of 10-15mCi (370-550 MBq); the first was every second for 60 seconds, followed by a second one every minute for 30 minutes. Delayed images were acquired at two and three hours. By assigning regions of interest (ROI) over the lung lesions (TR), contralateral normal lung (NL) and the heart (Ht), time activity curves (TAC) were generated and time to peak activity was calculated as well as the ratios of TR/NL, Ht/TR and Ht/NL. The Ht/NL ratio was also assessed in five patients referred with diagnosis of ischaemic heart disease (IHD) for myocardial imaging. There was a localized increase of Tc-99m MIBI uptake in ten patients with untreated malignant tumours of the lung. No localized uptake was found in one patient with untreated poorly differentiated squamous cell carcinoma, two treated lung cancer and four patients with non-malignant lesions of the lungs. Two patients with fibrosing alveolitis showed diffuse increase lung uptake. In all positive studies peak tumour was reached within the first minute. There was no statistical difference in the ratio of Ht/NL between all groups except in patients with fibrosing alveolitis. There was no significant difference between the ratios TR/NL, Ht/TR and Ht/NL at 5-10 minutes and 25-30 minutes. The ratio of TR/NL at 25-30 minutes was 1.58 +/- 0.36 in the positive cases versus 1 +/- 0.22 in the negative ones.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1998

Abnormal uptake of Tc-99m MIBI, a novel myocardial imaging agent, in the lungs of patients with systemic sclerosis.

OBJECTIVE: Fibrosing alveolitis is a prominent feature of systemic sclerosis (SSc), and accounts for much of the morbidity and mortality of this disease. Sensitive techniques for the detection and monitoring of fibrosing alveolitis could target patients for early therapeutic intervention. The objective of this small pilot study was to assess the frequency and clinical significance of abnormal lung uptake of Tc-99m MIBI, a novel radionuclide imaging agent that selectively accumulates in cells rich in mitochondria. METHODS: Sixteen patients with SSc and evidence of pulmonary involvement were studied. The uptake of radionuclide in the lungs, and the ratio of pulmonary to cardiac uptake were evaluated after intravenous injection of Tc-99m MIBI. Results were correlated with clinical and laboratory parameters. RESULTS: Lung uptake of Tc-99m MIBI was increased in all 16 SSc patients compared to control patients with coronary heart disease but no evidence of pulmonary abnormality. The degree of isotope uptake in the lungs was correlated with the extent of maximal skin induration and with radiologic evidence of interstitial lung disease, but not with other clinical or laboratory parameters of disease activity or extent of pulmonary involvement. The ratio of pulmonary to cardiac uptake of isotope was also increased in patients with SSc compared to controls. CONCLUSION: Accumulation of Tc-99m MIBI is abnormally elevated in the lungs of SSc patients with pulmonary involvement. Isotope accumulation in the lungs may be related to activation of fibroblasts or endothelial cells. The specificity and sensitivity of Tc-99m MIBI scanning in the detection and monitoring of pulmonary involvement, and its potential role in the management of SSc, deserve further investigation.

Coronary Disease↗

PubMed2003

Sonographic evaluation of bowel wall thickness in patients with cystic fibrosis.

GOALS AND BACKGROUND: Fibrosing colonopathy causing severe thickening of the colon wall was recently described in cystic fibrosis (CF). Since it has been suspected that subclinical colon-wall thickening is a common feature in CF patients, bowel-wall thickness was measured in a series of patients and compared with controls. Additionally, possible clinical factors influencing wall thickness were investigated. STUDY: In 83 CF patients and 31 controls transabdominal ultrasound was performed in the terminal ileum, cecum, ascending and descending colon. The relation of the measured wall thickness to age, sex, pancreatic enzyme intake, and intestinal diseases was analyzed. RESULTS: In controls, mean wall thickness depending on gut region was 1.1 to 1.3 mm (SD 0.19-0.23). In CF patients, no evidence of severe thickening or stricture was found, but wall thickness was significantly higher than in controls in all measured regions (1.5-1.6 mm; SD 0.31-0.41). Patients with meconium ileus and distal intestinal obstruction syndrome had significantly increased thickness of the terminal ileum compared with patients without these complications. Neither the intake of high-strength enzymes nor enzyme dosage, age or sex were associated with wall thickness. CONCLUSIONS: We found no evidence that a subclinical stage of fibrosing colonopathy is prevalent among the CF patients. Slightly thickened gut walls in CF can be interpreted as an expression of glandular dysfunction in the CF intestine.

Adolescent↗

PubMed1996

Augmentation cystoplasty using pedicled and de-epithelialized gastric patches in the mini-pig model.

PURPOSE: The most common methods of bladder augmentation are gastrocystoplasty and enterocystoplasty. Gastrocystoplasty is advantageous due to minimal mucous secretion and a well developed muscular wall as well as good urodynamic properties of the patch. However, the permanent contact of urine with the gastric mucosa is not free of complications. We report the urodynamic, macroscopic and histological outcomes of a pedicled de-epithelialized gastric patch incorporated in the bladder. We compared the results to those of our previous study, which sought to analyze these techniques of patch coverage using sigmoid patches. MATERIALS AND METHODS: We performed 20 augmentation cystoplasties in the mini-pig model using a pedicled de-epithelialized gastric patch and 5 techniques of patch coverage. RESULTS: Three months after surgery all bladders had an increase in volume except those in which the auto-augmentation technique was used. However, all gastric patches were smaller compared to preoperative size. Many had irregular fibrosed inner surfaces and histological evaluation revealed a fibrosed newly formed submucosal layer with a complete urothelial coverage in every patch. No gastric mucosal remnant was found. CONCLUSIONS: De-epithelialized gastrocystoplasty is an attractive procedure that can increase bladder capacity as well as provide a complete urothelial lining without mucosal remnants. However, the success of this procedure seems to be limited by increased morbidity and fibrotic changes, and decreased surface of the patch.

Animals↗

PubMed2003

Animal and in vitro models of alcoholic pancreatitis: role of cholecystokinin.

Although ethanol abuse is the major etiologic factor in the development of acute and chronic pancreatitis, the mechanisms of ethanol effects to cause pancreatitis are poorly understood. The major reason for the lack of progress is the relative lack of animal models that reproduce the deleterious effects of ethanol on the pancreas that are observed in human disease. We propose that the effect of ethanol on the pancreas is due to its ability to sensitize animals and humans to the potentially injurious effects of other stimuli. We have developed models of ethanol-induced acute and chronic pancreatitis in rats as well as pancreatic acinar cells in primary culture demonstrating that ethanol sensitizes the pancreas to the inflammatory, cell death, and fibrosing responses caused by cholecystokinin (CCK). Our results indicate that the ethanol-sensitized inflammatory response is the key or trigger event for the development of the other pathologic responses in both acute and chronic pancreatitis, such as cell death, intracellular digestive enzyme activation, and fibrosis. These findings suggest that experimental strategies designed to reveal the modulating effects of ethanol on the mechanisms underlying the inflammatory, cell death, and fibrosing responses stimulated by CCK will provide the key information needed to understand how ethanol abuse causes pancreatitis.

Animals↗

PubMed1985

Acinar structure and function in canine pancreatic autografts with duct drainage into the urinary bladder.

Autotransplants of pancreas in 8 dogs, with exocrine drainage into the urinary bladder, were stimulated in vivo with cholecystokinin-pancreozymin (CCK-PZ). Transplant biopsies, when compared with 6 normal pancreases, showed normal acinar structure by light and electron microscopy 13-18 months after initial surgery; 2 transplants with sutures unintentionally transecting ducts were fibrosed and had duct obstruction. After in vivo stimulation, the normal-appearing transplants produced a 7-fold increase in urinary amylase, and quantitative electron microscopy showed a 50% reduction in mature zymogen granules; there were no intracellular organelle abnormalities prior or subsequent to stimulation. Fibrosed transplants produced lesser urinary amylase both prior to and after stimulation. In vitro stimulation of grafts with normal structure increased amylase secretion from 1.5-2.1-fold. In vitro dose-response showed a maximum at 10(-9)M cholecystokinin-octopeptide (CCK-OP) in transplant and control. The in vivo stimulation is more responsive and may be useful for clinical monitoring of graft survival. In vivo stimulation occurred after induced urinary tract infection; because no pancreatitis ensued, a regimen of trophic stimulation by CCK-PZ was not contraindicated. The bladder tolerated exocrine drainage with no significant change, and bladder infection did not adversely affect the transplant. The islets appeared normal in the transplants by light and qualitative electron microscopic observation; fasting blood glucose and insulin values were normal during the 12-18-month follow-up. Bladder drainage of segmental grafts of pancreas provides a preparation with intact acinar-islet relationships; the present observations suggest that this may permit longer islet survival in the absence of acinar destruction and subsequent fibrosis.

Amylases↗

PubMed2004

Nonspecific interstitial pneumonia: a provisional category of idiopathic interstitial pneumonia.

PURPOSE OF REVIEW: Idiopathic interstitial pneumonias (IIP) represent a complex group of relatively rare entities with similar clinical, vaguely similar radiographic and differing histologic features. The recent international multidisciplinary consensus statement produced by the American Thoracic Society/European Respiratory Society aiming to standardize the classification of IIP recognizes nonspecific interstitial pneumonia (NSIP) as a provisional category. While not representing a single disease, but rather a collection of pathologic processes with similar histomorphology, NSIP has been a great source of confusion for pulmonologists, radiologists, and pathologists. RECENT FINDINGS: Lacking diagnostic clinical or radiographic features, NSIP is an IIP with recognizable and reproducible morphologic patterns different from usual interstitial pneumonia (UIP)-pattern as well as other disease patterns. And while overlap with UIP-pattern can be seen in individuals with multiple biopsy samples, those with either cellular or fibrosing variants of NSIP have a better prognosis than UIP-pattern patients. SUMMARY: A morphologic diagnosis of NSIP-pattern alerts the clinician to a wide spectrum of potential clinical possibilities and enables researchers to study both this fibrosing interstitial pneumonia pattern and the more common and deadly UIP-pattern separately. Thus, this provisional category is useful to both clinicians and researchers.

Humans↗

PubMed2005

Cutaneous desmoplastic melanoma.

Desmoplastic melanoma (DM) is a fibrosing variant of spindle cell melanoma. It most often presents as an indurated lesion in chronically sun-damaged skin. Due to the lack of characteristic clinical features, early detection is uncommon. At the time of excision, the tumors usually extend into the reticular dermis or deeper. DM is prone to misdiagnosis. It may simulate histologically sclerosing melanocytic nevi as well as various benign and malignant nonmelanocytic lesions. There is significant morphologic variability among tumors classified as DM. Desmoplasia may be prominent throughout the entire tumor ("pure" DM) or represent a portion of an otherwise nondesmoplastic melanoma ("combined" DM). Some tumors show neuroma-like features with prominent nerve involvement, in which case the term "desmoplastic neurotropic melanoma" is used. Immunophenotypically, DMs are usually strongly and homogeneously positive for S-100 protein but are often negative or only focally positive for melanocyte differentiation antigens such as tyrosinase, gp100, Melan-A, and microphthalmia transcription factor. DM differs from conventional melanoma in its clinical course. It is associated with a higher tendency for local recurrence, but metastases to regional lymph nodes are less common. Evidence is also emerging that for patients with thick melanomas, the presence of a paucicellular fibrosing tumor histology (pure DM) is a favorable prognostic factor for survival.

Biomarkers, Tumor↗

PubMed2026

Histoplasmosis in children: emerging insights and evolving guidelines.

PURPOSE OF REVIEW: This review provides an update on the epidemiology, risk factors, clinical presentation, diagnosis, and management recommendations incorporating recommendations from recent publications including the Infectious Disease Society of America guidelines for the management of pulmonary and disseminated histoplasmosis. RECENT FINDINGS: Updates to the epidemiology of histoplasmosis indicate a broader geographic range than historically defined. Updated guidelines do not recommend routine treatment for asymptomatic, mild and moderate pulmonary histoplasmosis although itraconazole can be offered for immunocompromised children or for prolonged or worsening symptoms. Liposomal amphotericin B is recommended as initial treatment for severe histoplasmosis syndromes (severe pulmonary and disseminated histoplasmosis). Fibrosing mediastinitis, a late complication of histoplasmosis is treated with stenting of vessels and bronchi. Recent studies demonstrate that rituximab (anti-CD20 monoclonal antibody) may stop progression or lead to regression of progressive fibrosis. SUMMARY: Histoplasmosis has manifold manifestations, many of which are self-limited and do not require treatment. Severe histoplasmosis and its complications should be treated with liposomal amphotericin B followed by itraconazole. Clinical trials are needed to assess the efficacy of rituximab for the treatment of fibrosing mediastinitis.

Humans↗

PubMed2005

Female pattern hair loss and its relationship to permanent/cicatricial alopecia: a new perspective.

Female pattern hair loss (FPHL) is a common hair disorder of the central scalp. The clinical change in hair density, related to a change in the hair cycle and miniaturization of the hair follicle, is generally considered to be potentially reversible. However, there is now evidence of a permanent hair loss that develops in a subset of women with FPHL. The presence of a perifollicular lymphohistiocytic infiltrate and fibrosis is seen without follicular drop-out in biopsies of women with FPHL and with a notable follicular drop-out in a cicatricial form of this condition (heretofore called cicatricial pattern hair loss) as well as in fibrosing alopecia in a pattern distribution, currently classified as a subset of lichen planopilaris. The potential relationship of these conditions as well as frontal fibrosing alopecia and central centrifugal cicatricial alopecia, two other conditions of permanent hair loss seen primary in women, is discussed.

Black or African American↗

PubMed2003

Serum nitric oxide (NO) levels in systemic sclerosis patients: correlation between NO levels and clinical features.

Vascular damage in systemic sclerosis (SSc) may be a factor in the abnormal regulation of several vasoactive agents. It has been well confirmed that plasma endothelin-1, as a vasoconstrictive factor, is elevated in patients with SSc. However, it is still controversial whether the levels of serum nitric oxide (NO), a strong vasodilator, are increased or decreased in SSc patients compared to healthy donors. In this study, we measured the levels of serum NO metabolites in SSc patients and determined the contribution of the excessive production of NO synthase (NOS)-2 by skin fibroblasts to NO synthesis. Serum NO levels of 45 patients with SSc were significantly higher than those of 20 healthy volunteers. In addition, some clinical features of SSc (the extent of skin fibrosis, short disease duration, and the complication of active fibrosing alveolitis) were all correlated positively with the levels of NO metabolites in SSc patients. To evaluate the levels of NOS-2 produced by skin fibroblasts, skin fibroblast cultures were established from SSc patients and healthy volunteers. Reverse transcription-polymerase chain reaction indicated that NOS-2 mRNA was spontaneously expressed in cultured fibroblasts derived from SSc patients, but not in those derived from healthy normal controls. Immunohistochemical staining also showed that NOS-2 proteins were detected in SSc fibroblasts but not in normal fibroblasts. The production of NO by cultured fibroblasts was visualized directly by a reagent (DAF-2 DA) used for the fluorescent detection of NO. Cultured SSc fibroblasts were capable of NO synthesis in culture media containing L-arginine, whereas normal fibroblasts (with no expression of NOS-2) did not synthesize detectable NO. These observations indicate that NO production is increased markedly in early-stage diffuse cutaneous SSc patients with active fibrosing alveolitis, and that constitutive NOS-2 expression in SSc fibroblasts may contribute to increased NO production.

Adolescent↗

PubMed1978

Chondroectodermal dysplasia (Ellis--van Creveld syndrome) with dysplasia of renal medulla and bile ducts.

A case of a 28-month-old boy with chondroectodermal dysplasia (Ellis-van Creveld syndrome) is reported. Besides polydactyly, ectodermal dysplasia, acromelic dwarfism and congenital heart defect, which are characteristic morphologic features of the syndrome, additional dysplastic developmental defects were discovered in the kidneys, liver, and lungs. Detailed histopathologic studies of the growth plates of tibia, femur and ribs disclosed an irregular, partly hyperplastic, partly dystrophic appearance of the epiphyseal cartilage, which was not resorbed properly by the invading blood vessels. Focal areas of necrosis occurred and barriers of tongue-shaped cartilaginous peninsulas persisted. Regular enchondral ossification was hindered and compensatory membrane ossification was found in the fibrosing metaphyseal bone marrow adjacent to the cartilage. Dysplasia and fibrosis of the renal medulla plus interstitial fibrosing nephritis in the cortex resulted in kidney contraction and renal failure. Hepatomegaly, portal fibrosis and bile duct hyperplasia and dysplasia were detected at autopsy, but did not have any clinical significance. These findings once again emphasize that derivatives of all three germ layers are involved in the Ellis-van Creveld syndrome. The possibility that a single metabolic or structural abnormality of the mesenchymal tissues could be responsible for the various organ dysplasias is discussed.

Bile Ducts↗

PubMed1990

Mast cells in normal and pathological specimens of the equine lung.

In lungs from 22 horses, the number and type of mast cells in histologically normal specimens were compared with those in specimens with various histological lesions. Tissues fixed in Carnoy's solution were superior to those fixed in formalin for the identification of mast cells. Fixation of lung specimens from eight horses in Carnoy's solution and formalin allowed the identification of two subpopulations of mast cells: formalin-sensitive, which predominated and formalin-resistant. Specimens with mild chronic tracheitis and mild and moderate chronic bronchitis generally had slight increases in mast cell numbers in comparison to histologically normal specimens. An increase in mast cell numbers was also observed around small and large bronchioles with mild, moderate and severe bronchiolitis and in fibrosed pulmonary pleurae in comparison to specimens with no histological lesions. The most striking increase in mast cell numbers was seen in fibrosed alveolar septa; there were approximately four times as many mast cells in these specimens as in specimens with normal histology.

Animals↗

PubMed1986

Morphogenesis of septa in hepatic fibrosis induced by choline deficiency in rats. Correlation between ito cells, pericellular fibrosis, and septa.

Examination of hepatic fibrosis induced by choline deficient diet showed the histogenesis of early fibrosis and septa formation. Male rats were fed a choline deficient diet. Groups of three were sacrificed after 1, 2, 3, 6, 8, 12, and 13 weeks. Control groups of three rats were sacrificed after 6, 8, and 12 weeks. All choline deficient rats showed initial steatosis around the portal tracts. The fat droplets were small at first and gave a microvesicular appearance. As they grew, they gathered around the terminal hepatic venule by the 3rd week. Then, reticular fibers developed around the hepatocytes in the centrolobular area and fibroses connected the terminal hepatic venules. The fat droplets became larger and made nodular masses around the portal tract. These compressed the fibroses to the periphery where they formed septa. After 12 weeks, regenerating nodules developed. Electron microscopy showed that fat storing cells helped cause pericellular fibrosis.

Animals↗