Clinical And Prognostic Analysis Of 40 Infants With Cholestasis And Cholestatic Liver Disease

Objective:To explore the clinical characteristics and prognosis of infantile cholestasis and cholestatic liver disease, and analyze the pathogenesis, etiology, prognosis and changes of lymphocyte subsets in order to providing clues for diagnosis and treatment.Methods:The clinical data of 40 infants with cholestasis and cholestatic liver disease hospitalized in our department were collected from September 2012 to January 2015. The prognosis were followed up by the phones. The clinical data were statistically analyzed.Result:1. Among the 40 cases, Including 17 cases with cholestasis and 23 cases with cholestatic liver disease,22 males and 18 females, aged 1-4 months. The youngest patient was 36 days and the maximum was 4 months. Including 17 cases with cholestasis and 23 cases with cholestatic liver disease. The main symptom was jaundice and jaundice reclaim delay. The stool color was lighter in 32 cases.2. The tested cases were 25 (62.5%) for positive pathogen, of which 18 cases (45%) with positive CMV,9 cases (22.5%) with positive urine culture,2 cases (5%) with blood culture positive and 2 cases with positive EB virus. There were a variety of mixed pathogenic infections in some cases.11 cases might have biliary malformations, but eight of them were undiagnosed.3 cases were accepted the surgical exploration, the results proved that 1 case was biliary atresia and choledochal cyst,2 cases were biliary atresia.Genetic metabolic screening:There were elevated blood ammonia in 26 cases,14 cases was normal, blood lactate were higher with variant degrees. The tandem mass spectrometry among 16 cases showed that a variety of amino acids and carnitine were obvious abnormalities.2 cases were diagnosed as Citrin protein deficiency. There were significantly increased Citrulline in 5 cases.16 cases were of Tandem mass spectrometry, all of them were with elevated blood ammonia. One case was with hypothyroidism and one case with subclinical hypothyroidism by thyroid function test. One case was with hypoglycemia, the repeatedly fasting glucose was with 1.7-2.2mmol/L.3. Infantile cholestasis and cholestatic liver disease had an cellular immune dysfunction with variation and without rulers. Cytomegalovirus infection mihgt be one cause of cellular immune dysfunction. Cellular immune dysfunction might be a risk factor among cases with death.4.26 cases recovered after conservative treatment,2 patients after surgical treatment currently alive, and one cases healed.11 of 40 cases were death.Conclusion:The early clinical manifestations of Infantile cholestasis and cholestatic liver disease are jaundice reclaim delay, while change with lighter stool color. Infantile cholestasis and cholestatic liver disease has an cellular immune dysfunction with variation and without rulers. Causes of infantile cholestasis and cholestatic liver disease are complex, clinical diagnosis and treatment is limited, and it is difficult for doctors to clear. Clinical outcomes are very differences. The mortality is high. The cause is a key factor for affecting the prognosis.