Evaluation of autoimmune scoring system in autoantibodies seropostive and seronegative children with autoimmune hepatitis

The scoring system of autoimmune hepatitis that was defined by the International Autoimmune Hepatitis Group [IAHG] in 1999 was applied on thirty seven children with autoimmune hepatitis [AIH] from the attendants of Pediatric Department of National Liver Institute [NLI], Menofiya University. Thirty three cases [89.2%] were seropositive to one or more of conventional autoimmune antibodies of AIH [antinuclear antibodies [ANA], smooth muscle antibodies [SMA], and antibodies to liver / kidney microsome type 1 [anti-LKM-1]] and 4 cases [10.8%] were seronegative. No statistically significant difference were found between the two groups as regards clinical presentations, the results of liver function tests, histopathological findings, hypergammopathy mean of scoring system before and after treatment as well as the mean of scoring system after response and / or relapse after treatment. These results point to the reliability of using the autoimmune scoring system to uncover cases of autoimmune hepatitis in patients seronegative for autoimmune markers in children

Billiary atresia cases among attendants of Menoufiya National Liver Institute: [a retrospective study]

This study included 126 cases of neonatal cholestasis syndrome [NCS] from the attendants of Pediatric Department of the National Liver Institute, Menoufiya University from 1994 up to 2004 . They were divided into two groups: First group[1stGr.] included 58 biliary atresia [BA] cases [46%] and the second group [2ndGr.] included 68 [54%] were due to other causes of NCS . The etiology of NCS due to causes other than BA were as follows: 28 cases [22.22%] neonatal hepatitis, 11 cases [8.73%] septicemia, 8 cases [6.35%] paucity of intrahepatic bile ducts, 5 cases [3.97%] inspessated bile syndrome, 4 cases [3.17%] choledochal cyst, 2 cases [1.59%] Byler's disease, 2 cases [1.59%] galactosemia, 2 cases ? 1 antitrypsin deficiency [1.59%], 1 case [0.79%] Alagille syndrome, and 5 cases [3.97%] due to unknown causes. Onset of jaundice whether early or late does not differentiate cholestatic cases due to BA from other causes of NCS.Clay coloured [acholic] stools were more frequently detected in BA cases [77.6%] than 2ndGr. [27.9%] [p<0.05]. Triangular cord sign [Tc sign] and absence of and/or gall bladder abnormality were detected in significantly higher proportion of BA group more than the 2ndGr.[p<0.05]. By histopathological examination portal tract fibrosis, bile duct proliferation, bile plugs in portal ductules and preservation of hepatic lobular architecture were detected more frequently among BA cases than the other group, while interface hepatitis and giant cell detection were observed more frequently among 2ndGr. than BA cases [p < 0.05]. The mean of alkaline phosphatase and gamma glutamyl transpeptidase was found to be statistically higher among BA group than that of the 2ndGr. [p<0.05] . Kasai operation was done for only 20 cases of BA cases [34.5%] and the mean of age of patients at time of operation was 75 days +/- 17.8. Complications encountered in BA cases were recurrent cholangitis, ascites, itching, coagulopathy, hematemesis and end-stage liver failure in 48%, 50%, 41.7%, 39.6, 18.8% and 54.2% of cases respectively. In conclution, the results of the present study indicate that clinical evaluation by an experienced pediatric hepatologist and liver biopsy together with careful ultrasonographic evaluation are considered as the most reliable methods for early differentiation of BA from other causes of neonatal cholestasis. Management of BA cases will be improved by public and professional education to encourage early referral of infants with neonatal cholestasis [>14 days] to specilised liver centers for early diagnosis to facilitate initial surgery before 8 weeks of age

Anti-cyclic citrullinated peptide antibodies in rheumatoid arthritis and their clinical significance

One of the characteristics of rheumatoid arthritis [RA] is the presence of several autoantibodies in the serum of the patient. However, most of these antibodies have failed to demonstrate adequate diagnostic and prognostic value so far. There is growing evidence that therapeutic intervention early in the course of RA leads to earlier disease control less joint damage, and a better prognosis. A new serological test, the anti-cyclic citrollinated peptide [anti-CCP] was developed. Anti-CCP was reported to have a high specificity for the diagnosis of RA, especially in patients with early disease and its presence before disease presentation is suggestive of its role in disease pathogenesis .The aim of this study is to estimate the level of anti-CCP antibodies in the serum of RA patients and to correlate them with RF isotypes [IgG and IgM] and clinical findings as disease activity and severity. This study comprised 68 RA patients [64 female and 4 males] diagnosed according to the revised criteria described by ACR [1987], in addition to 15 healthy control subjects. Clinical assessment of RA disease activity and severity, radiological investigations, and laboratory investigations [complete blood picture, ESR, CRP, determination of anti-CCP, IgG-RF and IgM-RF antibodies by ELISA technique] were done for all subjects.Highly significant increase in the levels of anti-CCP, IgG-RF and IgM-RF antibodies were found in RA patients compared to control group [P<0.001 for each]. Anti-CCP antibodies showed the highest diagnostic specificity [100%] than both RF IgM and IgG [93.33% for each].The anti-CCP and IgG-RF tests had excellent sensitivity [95.59% and 98.53% respectively] while IgM-RF had relatively lower sensitivity than both tests [86.76%]. Anti-CCP level was significant positively correlated with duration of the disease [P=0.024].Also,there was positive correlation between anti-CCP levels and all disease activity parameters which include the number of active joints [P=0.007], duration of morning stiffness [P<0.001], ESR [P<0.001] and CRP values [P=0.024]. Anti-CCP test had the best correlation with disease activity grades .disease severity, and radiological score [P=<0.001, P=<0.001 and P=0.000 respectively]. The level of anti-CCP in patients receiving methotrexate either alone or with lefwnomide is lower than other patients receiving methotrexate with corticosteroids [56.5 +/- 41.33,112 +/- 95.04 lU/ml respectively]. In conclusion, anti-CCP antibodies could be regarded as a new diagnostic marker for RA as they have 100% specificity and 95.8% sensitivity and it could predict erosive development early in the disease, and it could be used in evaluation of disease activity, severity and therapeutic response