| Objective:To raise awareness of hereditary spherocytosis (HS), improve the level of diagnosis and treatment, and investigate the mothod to improve the cure rate and the quality of life, by retrospecting the clinical characteristics and therapeutic process of HS.Methods:Clinical data of35cases of HS hospitalized during2001to2012in Children’s Hospital of Chongqing Medical University. The age of onset, gender, region, season of on set, clinical manisfestations, laboratory examination,pathology, process of diagnosis and treatment and prognosis.Results:1.35cases were diagnosed according to 《Practical pediatrics》 by Zhufutang.2. There were16male,19female, and the ratio male to female was0.84:1. The age of patients ranged from12hours to13year and7month’s old, more than1year’s old on average.25.71%patientd were less than1month,14.29%were less than1years’ old,34.29%were between1to5years’old,20.00%were between5to10years’old, and5.71%were more than10years’ old. Infants were more common in the group. 3.35cases of children from rural areas in26cases,9cases from the city.15patients from Chongqing,12patients from Sichuan,7patients from Guizhou,1patients from Gansu.4.35patients in7cases of spring,11cases of summer,13cases of autumn, and4cases of winter.5.35patients in18cases with respiratory infections,2cases with the digestive tract infection,5cases with anemic heart disease,2cases with cholelithiasis (8years and8months’ old,7years and11months’ old),1cases with free from autoimmune hemolytic anemia,1case with kawasaki disease,1case with ascariasis,1case with hepatitis B.6.35cases have a positive family history in13cases,6cases of father,4cases of mother,3cases of sisters.7.35patients in11patients (31.43%) had been diagnosed "pathological jaundice newborn "(1case with clear cause for HS), and2cases had bilirubin encephalopathy.8.35cases in34cases (97.14%) with anemia,34cases (97.14%) with jaundice,35cases (100.00%) with splenomegaly,27cases (77.14%) with hepatomegaly.19patients (54.29%). with cardiac souffle.25patients (71.43%) become sicker because of infection factors.9.35cases were performed blood analysis, no anemia in1case, mild anemia in13cases (37.14%), moderate anemia in11patients (31.43%), severe anemia and extremely severe anemia in0case.10cases were unknown because of infection or blood transfusions during be in hospital, and they were anemia in peacetime without the definite number of Hb.27cases were performed reticulocyte count, the results were all rise.30cases were performed red blood cell distribution width, and finally29cases(96.67%) were all rise. Increased more than360g/l of MCHC were2cases (5.71%).10.35cases underwent peripheral blood spherical RBC count, the results of spherical red blood cells showed:<10%1cases (2.86%),10%-20%6cases (17.14%),>20%28cases (80.00%).11.35cases,32cases underwent bone marrow aspiration, the results of spherical red blood cells showed:<10%1cases (3.13%),10%-20%7cases (21.87%),>20%24cases (75.00%).12.35cases,34cases underwent osmotic fragility test,33cases were all positive and1case was negative.13.35cases,34cases underwent the test of bilirubin, the results showed that1case was normal,33cases were on the rise and they indirect bilirubin elevated to give priority to.14.35cases,16cases underwent splenectomy, and15cases underwent the pathological examination, the results showed according with HS.15.35cases,25cases (71.43%) underwent blood transfusion treatment,3cases (8.57%) had oral folic acid treatment outside the hospital, and16cases (45.71%) had splenectomy, and the postoperative recovery were good.Conclusion:1. The clinical manifestations of HS are differents, but most of HS are mild to moderate anemia, the severe patient can appear nuclear jaundice, and someone can be trait.2. HS can seen in all age groups of children, but see more at infant. No obvious difference of men and women.3. The major clinical manifestations of HS are anemia, jaundice,splenomegaly,hepatomegaly, and it can have acute hemolysis attack with the factors such as infection, family history is helpful. Osmotic fragility test positive is an important index to diagnose HS.4. Spherical red blood cells in peripheral blood and/or bone marrow increased significantly is important for diagnosis of HS.5. We should consider HS when newborn has vigilant bilirubin encephalopathy.6. Splenectomy is the most effective mean for HS at present.7. Splenectomy should be carried out between5to10years’ old in general case (According to the specific situation of children, the timing of surgey and management of HS are different)... |
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