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M. Jaffer - One of the best experts on this subject based on the ideXlab platform.
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Alpha!-Antitrypsin Deficiency with Severe Panniculitis Report of Two Cases
2016Co-Authors: Herbert M. Rubinstein, F. A. C. P. Adrian, M. JafferAbstract:Two patients with profound decrease of Alpha^Antitrypsin (PiZZ) presented with severe panniculitis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and Alphai-Antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease. THE CAUSE of idiopathic panniculitis (Weber-Christian disease) is unknown (1, 2). Its severity is variable, rang-ing from restricted, transient inflammation of subcutaneous adipose tissue to a generalized and sometimes fatal process (3-5). We present here two cases of severe panniculitis asso-ciated with marked Deficiency of Alpha^Antitrypsin. We believe that this association is unlikely to be accidental and that marked Alphaj-Antitrypsin Deficiency may predis-pose to or worsen the course of panniculitis. Methods The trypsin inhibitory capacity of serum was measured by the enzymatic method of Dietz and colleagues (6, 7). In our laboratory healthy male adults (PiMM) * have values between 2.1 and 3.5 /xmol/min • ml (mean, 2.73 ± 0.29 (SD)) and healthy female adults between 2.4 and 3.8 (mean, 2.97 ± 0.32). Twenty-eight PiZZ * homozygotes had values between 0.4 and 0.8. PiMZ heterozygotes have values of 1.05 to 2.1 fimol/ min • ml, a definite overlap with normal values. Alphai-anti-trypsin was also measured by immunodiffusion using plates from Helena Laboratories. Values for the different classes of sera by immunodiffusion are MM, 210 to 500; ZZ, 10 to 90; MZ, 100 to 300 mg/dl. Antitrypsin phenotyping was done b
Hengcheng Chu - One of the best experts on this subject based on the ideXlab platform.
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increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant sjogren syndrome
Medicine, 2016Co-Authors: Chunting Chen, Yuchen Tseng, Chihwei Yang, Hsuanhwai Lin, Pengjen Chen, Tienyu Huang, Yulueng Shih, Weikuo Chang, Tsaiyuan Hsieh, Hengcheng ChuAbstract:The incidence of Sjogren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, Alpha-Antitrypsin Deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6%) were PBC-only and 48 (38.4%) were PBC-SS patients.The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant.Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients.
Herbert M. Rubinstein - One of the best experts on this subject based on the ideXlab platform.
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Alpha!-Antitrypsin Deficiency with Severe Panniculitis Report of Two Cases
2016Co-Authors: Herbert M. Rubinstein, F. A. C. P. Adrian, M. JafferAbstract:Two patients with profound decrease of Alpha^Antitrypsin (PiZZ) presented with severe panniculitis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and Alphai-Antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease. THE CAUSE of idiopathic panniculitis (Weber-Christian disease) is unknown (1, 2). Its severity is variable, rang-ing from restricted, transient inflammation of subcutaneous adipose tissue to a generalized and sometimes fatal process (3-5). We present here two cases of severe panniculitis asso-ciated with marked Deficiency of Alpha^Antitrypsin. We believe that this association is unlikely to be accidental and that marked Alphaj-Antitrypsin Deficiency may predis-pose to or worsen the course of panniculitis. Methods The trypsin inhibitory capacity of serum was measured by the enzymatic method of Dietz and colleagues (6, 7). In our laboratory healthy male adults (PiMM) * have values between 2.1 and 3.5 /xmol/min • ml (mean, 2.73 ± 0.29 (SD)) and healthy female adults between 2.4 and 3.8 (mean, 2.97 ± 0.32). Twenty-eight PiZZ * homozygotes had values between 0.4 and 0.8. PiMZ heterozygotes have values of 1.05 to 2.1 fimol/ min • ml, a definite overlap with normal values. Alphai-anti-trypsin was also measured by immunodiffusion using plates from Helena Laboratories. Values for the different classes of sera by immunodiffusion are MM, 210 to 500; ZZ, 10 to 90; MZ, 100 to 300 mg/dl. Antitrypsin phenotyping was done b
F. A. C. P. Adrian - One of the best experts on this subject based on the ideXlab platform.
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Alpha!-Antitrypsin Deficiency with Severe Panniculitis Report of Two Cases
2016Co-Authors: Herbert M. Rubinstein, F. A. C. P. Adrian, M. JafferAbstract:Two patients with profound decrease of Alpha^Antitrypsin (PiZZ) presented with severe panniculitis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and Alphai-Antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease. THE CAUSE of idiopathic panniculitis (Weber-Christian disease) is unknown (1, 2). Its severity is variable, rang-ing from restricted, transient inflammation of subcutaneous adipose tissue to a generalized and sometimes fatal process (3-5). We present here two cases of severe panniculitis asso-ciated with marked Deficiency of Alpha^Antitrypsin. We believe that this association is unlikely to be accidental and that marked Alphaj-Antitrypsin Deficiency may predis-pose to or worsen the course of panniculitis. Methods The trypsin inhibitory capacity of serum was measured by the enzymatic method of Dietz and colleagues (6, 7). In our laboratory healthy male adults (PiMM) * have values between 2.1 and 3.5 /xmol/min • ml (mean, 2.73 ± 0.29 (SD)) and healthy female adults between 2.4 and 3.8 (mean, 2.97 ± 0.32). Twenty-eight PiZZ * homozygotes had values between 0.4 and 0.8. PiMZ heterozygotes have values of 1.05 to 2.1 fimol/ min • ml, a definite overlap with normal values. Alphai-anti-trypsin was also measured by immunodiffusion using plates from Helena Laboratories. Values for the different classes of sera by immunodiffusion are MM, 210 to 500; ZZ, 10 to 90; MZ, 100 to 300 mg/dl. Antitrypsin phenotyping was done b
Chunting Chen - One of the best experts on this subject based on the ideXlab platform.
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increased risks of spontaneous bacterial peritonitis and interstitial lung disease in primary biliary cirrhosis patients with concomitant sjogren syndrome
Medicine, 2016Co-Authors: Chunting Chen, Yuchen Tseng, Chihwei Yang, Hsuanhwai Lin, Pengjen Chen, Tienyu Huang, Yulueng Shih, Weikuo Chang, Tsaiyuan Hsieh, Hengcheng ChuAbstract:The incidence of Sjogren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, Alpha-Antitrypsin Deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the remaining 125 patients, 77 (61.6%) were PBC-only and 48 (38.4%) were PBC-SS patients.The mean follow-up duration was 8.76 years. During the observation period, the incidence of interstitial lung disease was higher in the PBC-SS group than in the PBC-only group (P = 0.005). The occurrence of spontaneous bacterial peritonitis was significantly different in PBC-SS patients than in PBC-only patients (P = 0.002). The overall survival was lower in PBC-SS patients than in PBC-only patients (P = 0.033). Although the incidence of hepatocellular carcinoma, end-stage renal disease, variceal bleeding, and hypothyroidism were all higher in the PBC-SS group than in the PBC-only group, the differences were not significant.Our study suggests that PBC-SS patients have a higher risk of developing interstitial lung disease and spontaneous bacterial peritonitis and have a poor prognosis. Aggressive surveillance of thyroid and pulmonary functions should therefore be performed in these patients.