27 Ann Thorac Surg 2009;88: Solitary (localized) fibrous tumors of the pleura (SFTP) are rare slowgrowing neoplasms that generally have a favorable prognosis. In a series of 110 patients, the visceral pleura was the site of origin in 95 tumors, the parietal pleura in 13, the mediastinal pleura in 2 cases. Tumors were pathologically benign in 95 cases (86.4%), and malignant in 15 (13.6%)
28 Magnetic resonance imaging allows for sagittal and coronal views and is often able to provide a topographic and differential diagnosis, particularly for mediastinal masses. It is also useful in differentiating the tumor from surrounding structures. High negative predictive value of PET scan in assessing the malignancy of such lesion The reported diagnostic accuracy of FNAB is almost invariably low. Surgery is the gold standard of treatment as neither radiotherapy nor chemotherapy proved to be effective. Nevertheless, all SFTP patients need long term follow-up of 15 to 20 years due to the possibility of late recurrences.
81 6 MWT: Γsat: 1% (από 99% 98%), 609 m Λεηηνπξγηθόο έιεγρνο ηεο αλαπλνήο: DLCO: 62%
85 Hypersensitivity pneumonia is a form of diffuse interstitial lung disease resulting from sensitization to an inhaled antigen. Clinical and radiological features are relatively nonspecific, overlapping significantly with other forms of diffuse interstitial lung disease. Establishing the diagnosis in the absence of lung biopsy is challenging and is heavily dependent on being able to identify a specific antigenic exposure. Lung biopsy is especially important in diagnosing hypersensitivity pneumonia in patients for whom no incriminating exposure has been elucidated.
87 The incidence of HP is low if we consider that the offending antigens are numerous and widely distributed around the world
88 Strong evidence indicates that cigarette smoking protects from HP
100 Criteria for diagnosis of chronic hypersensitivity pneumonia generally include an appropriate exposure history, subjective and objective evidence of lung disease temporally linked to antigen exposure, and the presence of serum antibodies directed against the suspected antigen. Confirming the diagnosis is often difficult, however, because an incriminating exposure history is frequently elusive, and precipitating serum antibodies are unreliable. Given the absence of a diagnostic gold-standard, recognition of characteristic histological findings in lung biopsies often has a pivotal role in diagnosis.
101 In general, acute HP is applied to patients suffering from a first attack, with symptom duration of less than 1 month. Subacute HP refers to patients with periodic symptoms for less than 1 year. Chronic HP refers to patients with progressive respiratory complaints for at least 1 year. Chronic hypersensitivity pneumonia need not be preceded by acute disease, and only a small number of patients with acute disease develop chronic hypersensitivity pneumonia.
102 Chronic presentation of HP may result from two different clinical scenarios: (1) from unrecognized acute/subacute episodes (recurrent chronic HP) and (2) from a slowly progressive disease in patients exposed to low levels of antigen and without history of acute episodes (insidious chronic HP)
103 Acute Exacerbations in Chronic HP
112 Although for a long time it was considered that CD8+ T cell subset was predominant, recent evidence demonstrates that the CD4+:CD8+ ratio varied according to several variables, including, the type of causative antigen, the clinical status (ie, acute/subacute vs chronic), smoking habit, and others
121 Lung biopsy can have a key role in recognizing patients with chronic HP and hinges on recognition of a characteristic combination of interstitial pneumonia, bronchiolitis, and granulomatous inflammation. Late-stage disease is associated with fibrosis that may mimic other forms of fibrotic lung disease, including most importantly UIP.
135 A 1 AT : 335.0mg/L (Φ.Σ.: ) Γενεηικός έλεγτος για ανεπάρκεια A 1 AT: νκνδπγώηεο Pi ZZ
137 A 1 -Antitrypsin (A1AT) deficiency is an underrecognized genetic condition that affects approximately 1 in 2,000 to 1 in 5,000 individuals and predisposes to liver disease (cirrhosis and hepatocellular carcinoma) and early-onset emphysema. Panniculitis and vasculitis are more rare associations. A 1 AT is mainly produced in the liver and functions to protect the lung against proteolytic damage (e.g., from neutrophil elastase). Among the approximately 120 variant alleles described to date, the Z allele is most commonly responsible for severe deficiency and disease.
138 Z-type A 1 AT molecules polymerize within the hepatocyte, precluding secretion into the blood and causing low serum A 1 AT levels (3 7κM with normal serum levels of mm, mg/dl). A serum A 1 AT level of 11 κm represents the protective threshold value below which the risk of emphysema is believed to increase.
139 Alpha-1-antitrypsin deficiency: History Laurell und Eriksson 1963: Absence of alpha-1 band in electrophoresis in patients suffering from panlobular emphysema a 1 -band absent + - Serum: AATD patient Serum: Control Modified from Eriksson, Chest 1989 (95)
140 A second clinical landmark was the recognition of A 1 ATD associated cirrhosis by Sharp and colleagues in They described cirrhosis in 10 children from six families, all with marked decreases in the a-1 globulin on SPEP patterns and tryptic inhibitory capacity.
141 A 1 ATD ranks among the most common fatal congenital diseases in Caucasians in Europe and the US Compared with other genetic disorders, the incidence of A 1 ATD is similar to that of cystic fibrosis Most patients with A 1 ATD carry the PiZZ genotype An estimated 80,000 to 100,000 individuals are affected by A 1 ATD in the US (0.03 % prevalence)
142 10-15% 85-90% No signs or symptoms Symptoms, but misdiagnosed
143 Frequency of PiZZ in Europe: approx. 1:5,000 Estimated prevalence in Germany: approx. 8,000 PiZZ-carriers So far, only 10 to 15 % of all homozygous carriers have been diagnosed Affected individuals who, despite of a high genetic risk factor (e.g. homozygous genotype PiZZ), develop no symptoms, even at an advanced age (estimated % of PiZZ carriers)
145 alpha-1-antitrypsin is synthesized and secreted by: hepatocytes of the liver at a rate of 34 mg/kg body weight serum half-life of 4-5 days serum levels range from mg/dl and, to a lesser extend: alveolar makrophages, respiratory epithelium circulating monocytes kidneys small intestine
146 A 1 AT is secreted by the endoplasmatic reticulum of the hepatocytes is released into the blood plasma protein fraction diffuses passively into the lung tissue concentration of AAT in the interstitium is approximately 80% of the serum level
147 The main function of the alpha- 1-antitrypsin molecule is protection of the lower respiratory tract against proteolytic enzymes: the primary substrate is neutrophil elastase (NE) neutrophil elastase is released by neutrophil granulocytes: these are core factors of the innate immune response
148 Asn 95 Alpha-1-antitrypsin Asn 46 ILe 356 Pro 357 Met 358 Ser 173 His 41 Asp 88 Asn 144 Ser 359 ILe360 Pro 361 Asn 247 Neutrophil elastase Asn 83
149 A 1 ATD is inherited as an autosomal codominant condition for which more than 120 alleles have been identified Protease inhibitor gene (Pi-gene): codes for alpha-1- antitrypsin Pi-gene location: chromosome 14 (12.2 kb) SERPINA1 Over 100 Pi-alleles are known: M-variant (normal) S-, Z- (95%), Null-variants et al. varying risk of pulmonary und gastroenterological manifestations
150 Alpha-1-antitrypsin variants M variants Most common normal alpha-1-antitrypsin protein variants S variant A1AT plasma levels slightly reduced Minimal clinical relevance Protein misfolding leads to A1AT polymerization Z variant A1AT plasma levels greatly reduced Most common deficiency variant Null variants Results in no circulating alpha-1-antitrypsin (complete lack of synthesis)
151 Family trees of AAT genotypes M Z M Z M S M Z M M M Z M Z Z Z M M M Z M S S Z Parents Children
160 Negative effects of cigarette smoke on AAT activity Oxidants from cigarette smoke 46 Asn 247 Asn Alpha-1- antitrypsin 356 Ile 361 Pro 357 Pro 358 Met Ser 360 Ile 359 Met 358 AAT- methionine sulfoxide 358 Met 358 : 83 Asn Active AAT binding site (Ser 173 ) for interaction with neutrophil elastase Reduced binding affinity of AAT for neutrophil elastase (Ser 173 ) 1/2000
161 Imbalance between proteases and antiproteases Bronchopulmonary infections Pneumonia Chronic bronchitis Antiproteases Inactivation by cigarette smoke Proteases Congenital alpha-1- antitrypsin deficiency
162 Liver disease, including hepatitis, cirrhosis, and hepatoma, represents another clinical manifestation of A 1 ATD, at least for individuals with phenotypes characterized by intrahepatocyte polymerization (e.g., with Z, Mmalton, and Siiyama alleles). Liver disease in neonates: % develop neonatal hepatic syndrome* Approx. 50 % of symptomatic children show abnormal liver enzymes* 1-5 % develop cirrhosis The polymerized Z-protein: cannot be secreted by the hepatocytes cannot be degregated by intracellular proteosomes remains trapped in the hepatocytes causes damage to the liver
164 Panniculitis occurs at the site of trauma in one third of individuals and may accompany several phenotypes, including PI*ZZ, PI*SZ, PI*SS, and PI*MS. Diagnosis often requires deep excisional biopsy, which shows areas of fat necrosis interspersed among normal-appearing areas. That the cause of panniculitis is unopposed proteolysis is suggested by the finding of Z-type polymers in the skin of a PI*ZZ patient with panniculitis and by the dramatic clinical response to intravenous augmentation therapy.
166 Prolastin is licensed*: For long-term augmentation therapy In A 1 ATD patients with genotypes PiZZ, PiZ/Null, PiNull/Null and PiSZ Within the limits of moderate airflow obstruction (FEV 1 35 % to 60 %) And the evaluation of the patient s clinical conditions (disability)
167 Lung disease: Panacinar progressive emphysema Early onset of COPD Occurrence of respiratory symptoms between 35 and 50 years of age - Shortness of breath - Wheezing - Cough - Frequent infections of the upper/lower respiratory tract Cirrhosis or other liver diseases of unknown origin Panniculitis (rare) Rare cases in c-anca positive vasculitis (Wegener s Disease)
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