Tumor suppressor cylindromatosis: expressed in IgA nephropathy and negatively associated with renal tubulo-interstitial lesion

Background IgA nephropathy is the major cause of end-stage renal failure in patients with primary glomerular diseases. Tumor suppressor cylindromatosis （CYLD）, the recently identified member of the deubiquitinating enzymes, has been actively involved in regulation of inflammation. This study was undertaken to investigate the CYLD expression profile in IgA nephropathy and identify factors associated with CYLD expression. Methods Forty-one cases of IgA nephropathy were selected. CYLD expression in the kidney biopsy tissue was measured by immunohistochemical staining. Relevant clinical and pathological data were analyzed, and Logistic regression analysis was carried out to identify factors associated with CYLD expression. Results CYLD was specifically expressed in renal tubular epithelial cells in 70% of the studied patients with IgA nephropathy. All patients with positive CYLD staining had proteinuria, while only 72.7% of patients with negative CYLD had proteinuria （P=0.003）. Among studied proteinuric patients, those with positive CYLD had significantly less tubulo-interstitial lesions and higher estimated glomerular filtration rate （eGFR） levels when compared with those patients showed negative CYLD results. Logistic regression analysis indicated that the urinary protein excretion and eGFR were identified as predictors for the CYLD expression. Conclusion CYLD is expressed in renal tubular epithelial cells and appears to be associated negatively with tubulointerstitial lesions, however, its exact functional role remains to be clarified in further experiments.     

Clinicopathological Analysis of 155 Patients with Persistent Isolated Hematuria

Objectives To reveal etiologies of persistent isolated hematuria （PIH） through ultrastructural pathological examination, to disclose clinicopathological correlation in cases with PIH, and to summarize appropriate management of patients with PIH. Methods we retrospectively studied 155 P1H patients receiving renal biopsy between January, 2003 and December, 2008 in Peking Union Medical College Hospital. All the clinical data and follow-up result were analyzed. Results All subjects included 38 children and 117 adults, with mean age of 11.38±3.25 years for children and 35.17±8.44 years for adults. Thin basement membrane nephropathy （TBMN） was the most common pathology （55.3% of children and 49.6% of adults）, followed by IgA nephropathy （18.4% of children and 32.5% of adults, mainly grade 2-3） and mesangial proliferative glomerulonephritis （MsPGN） without IgA deposition （13.2% of children and 12.8% of adults）. Besides, A1port syndrome （2.6% of children） and membrane nephropathy （2.6% of children and 0.9% of adults） were demonstrated as other causes of PIH. Elevated mean arteral pressure or protein excretion rate, as well as episodic macrohematuria, indicated higher risk for MsPGN rather than TBMN. On the other hand, severity of microhematuria was irrelevant to pathological types of PIH. Totally, 86 patients were followed up and 37 cases therein stayed on track for long term （mean duration 41.11±28.92 months, range 8-113 months）. Most cases had benign clinical course except 3 cases with TBMN, 5 cases with IgA nephropathy, 1 case with MsPGN （without IgA deposition）, and 1 case with Alport syndrome, who developed hypertension or proteinuria. All of them were administered timely intervention. Conclusions Close follow-up should be required as the primary management for PIH. Equally important is careful monitoring for early identification of undesirable predictors; while renal biopsy and other timely intervention are warranted if there is hypertension, significant proteinuria or     

Potential association of hyperhomocysteinemia with the progression of IgA nephropathy: a retrospective study

Background The high blood homocysteine （Hcy） levels found in patients with hyperhomocysteinemia （HHcy） have been implicated in an increased risk of cardiovascular disease morbidity and mortality in end-stage renal disease （ESRD）.This study investigated the association of HHcy with progression of IgA nephropathy.Methods We analyzed 108 participants newly diagnosed with IgA nephropathy between August 2005 and August 2007 in the Department of Nephrology,Chinese People＇s Liberation Army General Hospital.The association between clinicopathological factors and the Hcy levels were analyzed by Logistic regression and those with ESRD risk were analyzed by Cox regression.Results Patients were aged （35.71±10.73） years and included 45.71％ women and 12.04％ patients with HHcy.In multivariate Logistic regression analysis,HHcy was associated with arterial lesions （OR 2.60; 95％ CI 1.55-4.34; P＜0.001） even when age,body mass index,estimated glomerular filtration rate,mean arterial pressure,and initial proteinuria were taken into account.Mean follow-up was （67.37±16.21） months.HHcy was also associated with worse ESRD-free survival （HR 4.71; 95％ CI 1.45 to 15.31; P=0.010）.Conclusion HHcy is associated with the risk of intrarenal arterial lesions and may be useful for estimating the prognosis of IgA nephropathy.     

NON-IgA MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS CLINICAL AND PATHOLOGICAL ANALYSIS OF 77 CASES

Primary mesangial proliferative glomerulonephritis without IgA deposition (non-IgA MsPGN)is one of the most common types of glomerular disease in China .In an attempt to investigate its clinical and pathological features ,we reviewed 77 such cases from 380 patients with primary glomerulonephritis taken renal biopsies during 1980-1987. Prodromal upper respiratoty tract infection occurred in 31 cases (40%) .In immunofluorescence Microscopy .prominent IgG granular deposits in mesangium were observed in 45 cases (58%) .These features are quite different from those in western countries ,indicating it might have different pathogenetic ptocesses. According to the severity of mesangial lesions , the 77cases were divided into 3 groups: mild (55 cases ) ,moderate (14) and severe (8) .In the patients with mild mesangial lesion and massive proteinuria , the therapeutic response to prednisone was similar to that in adult minimal change disease . In the moderate and severe groups ,there was a significantly higher incidence of superimposed tubulo – interstitial lesions associated with hypertension, persistant renal insufficiency and a poor response to prednisone . This work showed non-IgA MsPGN covered about 20% of our primary glomerulopathy ,which may be related to a higher incidence of infection. It was suggested that minimal change nephritic syndrome ,inspite of the variety of immunoglobulin mesangium deposits, could be treated as a single disease entity, and light microscopy is most important in offering prognostic information.     

A Clinicopathological Study on Aortic Valves in Children

In order to investigate the clinicopathological characteristics of aortic valve disease in children, all the native surgically excised aortic valves obtained between January 2003 and December 2005 were studied macroscopically and microscopically. The patients’ medical records were reviewed and the clinical information was extracted. According to preoperative echocardiography, intraopera- tive assessment, and postoperative pathology, combined with clinical symptoms and signs, aortic valve diseases were divided into three categories: aortic stenosis (AS), aortic insufficiency (AI), and aortic stenosis with insufficiency (AS-AI). The etiology was determined according to the macro- scopic, microscopic and clinical findings. The results showed that among 70 aortic valves, patient age ranged from 6 to 18 years, with a mean of 15.4 years, and there were 56 boys and 14 girls (male: fe- male=4:1). Forty-four children only had pure aortic valve disease, and the other 26 children had aor- tic valve disease associated with other heart valve diseases. There were 5 cases of AS (7.14%), 60 cases of AI (85.71%) and 5 cases of AS-AI (7.14%). The causes were congenital aortic valve mal- formation (32 cases, 45.71%), rheumatic disease (28 cases, 40%), infective endocarditis (7 cases, 10%), Marfan syndrome (2 cases, 2.86%), and undetermined (1 case, 1.43%). It was concluded that the common causes of aortic valve disease in order of frequency in children were congenital aortic valve malformation, rheumatic disease, infective endocarditis, and Marfan syndrome. AI was more common in children with aortic valve disease. Compared with adult patients, congenital bicuspid aor- tic valve in children was often AI. Histologically, the leaflets of congenital bicuspid aortic valve were mainly myxomatous, fibrosis and calcification less seen. AI was frequently found in rheumatic dis- ease, mostly associated with other heart valve diseases. Macroscopic and microscopic examinations together with clinical information, echocardiographic findings and operative details were important in evaluating the etiology of aortic valve disease.     

The clinical and pathological features of idiopathic membranous nephropathy in 246 Chinese adults

Objective: To investigate the clinical and pathological features of idiopathic membranous nephropathy (IMN) in Chinese adults. Methods: From 1986 to 1997, 264 patients with biopsy proven membranous nephropathy were selected in this study. Clinical and pathological features were compared between patients at different ages by t test. Results: (1) Patients from 21- 40 years old were inclined to membranous nephropathy. (2) One hundred and six of the patients had heavy proteinuria at presentation. Hypertension was found in 35 patients. Renal insuffeiency occurred in 7.7% of the patients in renal biopsy. Microscopic hematuria was found in 40.2% of the patients. Seventy-four patients presented nephrotic syndrome. (3) Eight of the 57 patients had deterioration of renal function during an average 49-month follow-up. (4) Patients of stage Ⅰ, Ⅱ, Ⅲ and Ⅳ accounted for 42.3%, 48.7%, 6.0% and 3.0% respectively. Glomeruli IgG, C3 and C1qdeposition was found in 93.2%, 98.8% and 58.3% of the patients. Conclusion: Younger patients are inclined to membranous nephropathy. The incidence of hypertension, microscopic hematuria and renal insuffcieney is similar to that of other countries, while nephrotic syndrome is uncommon.     

EARLY CHANGES OF KIDNEY MORPHOLOGY AND FUNCTION IN STREPTOZOTOCIN-INDUCED DIABETIC RATS

In order to study the nephropathy associated with experimental streptozotocin diabetes, serial functional and ultrastructural studies were done in insulin-treated (Group T), untreated (Group D) diabetic Wistar rats and normal controls on d 3, d 8, d 14 and d 28 of diabetes. The ratio of kidney weight to the body weight (KW/BW) and mean glomerular diameter (MGD) in diabetes rats at varying durations were increased as compared with those in control animals, but an increase of KW/BW and MGD in Group D was more marked than in Group T. The creatinine clearance (Ccr) and total urinary protein excretion rate (TUPER) were increased in Group D end T, but the increase in TUPER appeared later in Group T than in Group D. Enlargement of epithelial cells, disappearance of folds on their surface and widening of foot processes were observed after 3 d of diabetes. After 14 d of diabetes, increased basement membrane-like material in the mesangium was found. Morphologically an increase in glomerular size, expanded foot processes with ball-like terminal expansions, thickened basement membrane were observed.     

Comparison of T-SPOT TB and Tuberculin Skin Test in Children with Tuberculosis of Intrathoracic Lymph Nodes

TB （Tuberculosis） ofintrathoracic lymph nodes is the most common form of tuberculosis disease in childhood. In Bulgaria, about 67%-70% of all newly diagnosed children patients are with this diagnosis. The purpose of this study was to determine the diagnostic capabilities of the T-SPOT TB in this form of TB disease. In a period of one year, 50 children aged 0-12 years treated in our hospital with tuberculosis of lung lymph nodes were examined. The diagnosis was set based on history, clinical, microbiological and radiological check. T-SPOT TB （ELISPOT TB test） and the TST （tuberculin skin test） were performed simultaneously on the studied children. The relationship between immunological samples TST and T-SPOT TB （presence of BCG vaccination and contact with adult TB patient）. One child had TST negative samples, 32% had normergic reactions to 15mm. 66% had hyperergic above 15ram. In a study with T-SPOT TB, 28% showed negative results and 72% showed positive.     

Waist Circumference Distribution of Chinese School-age Children and Adolescents

Background Waist circumference （WC）, a proxy measure of central body fat, is a better predictor than overall body fat for cardiovascular risk factors in both adults and children. WC cutoffs have been established in many countries, but the national WC reference has not been developed in China. Objective To study the distribution of WC in a representative group of Chinese children for establishing Chinese WC cutoff points. Methods Cross-sectional data obtained from 15 mainland provinces and Hong Kong were collected. There were 160 225 children and adolescents aged 7-18 years old in total. The subjects were divided into five regional groups namely costal city, other city, noah rural, south rural, and Hong Kong, for analyzing the distribution of WC. Results A large variation in WC distribution was found among the five groups for both sexes. Coastal city group had the highest P85 WC values consistently from the early school ages to 18-year-old compared with other groups. In contrast, south rural group had P85 values consistently lagged behind not only the city groups but also the north rural group. Hong Kong group had a high P85 WC at early ages, but because of the smaller increments of WC during adolescence, the P85 curve was gradually exceeded by the north rural groups. Catch-up trend was found in the two rural groups since the peak of puberty. These disparities were caused by not only socioeconomic and urban-rural factors but also north-south differences. Because of the regional variation of WC, a ＂gradient＂ prevalence of central obesity was prospected among these groups. Comparisons of WC distributions between the Chinese and the U.S.A or Netherlands demonstrated the necessary of setting up China＇s own WC cutoffs. Sex and age-specific percentiles were obtained and smoothed by using LMS method. Conclusion The data obtained from this study could be used to develop national WC cutoff points for Chinese children and adolescents.     

The effect of platyspondyly and pubertal growth spurt on the stature of patients with beta-thalassaemia major

OBJECTIVE To study the effect of the body proportion and pubertal growth spurt on the stature of children with beta-thalassaemia major.

METHODS The height, sitting height, upper to lower segment (U:L) ratio and pubertal development were determined in 71 Chinese children (38 girls and 33 boys) with beta-thalassaemia. The growth patterns of 20 patients with complete growth data between 3 years and final height, were analyzed according to whether they underwent a pubertal growth spurt or not.

RESULTS 27% of the boys and 32% of the girls had a height below the 3rd percentile. About 60% of all the children had a U:L ratio below the 10th percentile for age. Abnormal body proportion was found in patients with or without growth retardation. 34% of the 41 children over the age of 14 years underwent spontaneous puberty. In 28 patients over the age of 16 years, a growth spurt was observed in 46% of the children during spontaneous or induced puberty. The retrospective analysis showed that the height deviation from the mean in adulthood was significantly higher in patients without pubertal growth acceleration than in those with a growth spurt (x = -11.8 cm, s = 7.6 cm vs x = -4.4 cm, s = 4.4 cm; P = 0.02).

CONCLUSIONS An abnormal U:L ratio was commonly observed in patients with beta-thalassaemia major and may be one factor contributing to the short stature of these patients. Abnormal puberty was present in a significant proportion of children and the lack of a pubertal growth spurt was found to be detrimental to adult height.

     

C509T and T869C polymorphisms of transforming growth factor β1 and the risk of IgA nephropathy: a meta-analysis

Background IgA nephropathy （IgAN） is the most common primary glomerular disease.Transforming growth factor β1 （TGFβ1） plays an important role in pathogenesis of IgAN.Associations between the polymorphisms of TGFβ1 gene and the risk of IgAN remained inconsistent.A meta-analysis was conducted to investigate the association between polymorphisms in the TGFβ1 gene and IgAN susceptibility.Methods Databases including Pubmed,EMBASE,ISI,et al.were searched to find relevant studies.Odds ratios （ORs）with 95％ confidence intervals （CIs） were used to evaluate the strength of associations.Results Ten studies involving 1770 cases and 1953 controls were included.Significant association between C509T polymorphism and IgAN risk was observed （OR 1.42,95％ CI 1.12-1.81,P=0.0004; I2=0％） in Caucasians by the overdominant model （CT vs.CC ＋ TT）,but no significant association was found （P=0.200） in Asians by the dominant model （CC ＋ CT vs.TT）.Significant association between T869C polymorphism and IgAN susceptibility was found （OR 1.21,95％ CI 1.02-1.44,P=0.030） in overall populations by the dominant model （TT ＋ TC vs.CC）.Subgroup analysis found T allele of T869C polymorphism was associated with IgAN susceptibility in Caucasians （P=0.030）,but not in Asians （P=0.290）.Conclusion Both heterozygotes of C509T polymorphism and T allele of T869C polymorphism in TGFβ1 were associated with the risk of IgAN in Caucasians,but not in Asians.     

Recent changes in the prevalence of Helicobacter pylori infection among children and adults in high- or low-incidence regions of gastric cancer in China

Background The prevalence of Helicobacterpylori （H. pylon） infection varies by geographic locations. Studies indicate that the infection rate of H. pylori was previously high in China but that rates had been declining worldwide over recent decades. The aims of our study were： （1） to determine the current prevalence of H. pylori infection among children and adults residing in areas with high （Muping County, Shandong） and low （Yanqing County, Beijing） incidences of gastric cancer in China, and （2） to compare the prevalence for 2006 with the prevalence for the early 1990s. Methods Using Warthin-Starry silver staining of gastric mucosal biopsy specimens and H. pylori stool antigen tests （HpSA）, we tested a total of 2065 asymptomatic children aged 8-15 years and adults aged 40-79 years in the above two regions from May to July 2006. We evaluated 520 children and 526 adults from Muping, and 516 children and 503 adults from Yanqing. Subjects were selected randomly and H. pylori status was determined by HpSA in children and either HpSA or histology of gastric biopsies in adults. Data obtained in the early 1990s in the same two areas of China were also collected and studied. Results For children, the prevalence of H. pylori infection was significantly higher in Muping （37.69%） than it was in Yanqing （25.58%, P〈0.001）. In both regions, the prevalence of H. pyloriincreased with age but was not related to gender A significant difference was observed between 8-9-years old and 10-11-years old （P 〈0.05）, but not between other adjoining age groups （P 〉0.05）. From 1991 to 2006 H. pylori prevalence among 8-10-year-old children decreased in Muping （60.00% vs 32.07%, P 〈0.001）, but not Yanqing （24.06% vs 19.10%, P 〉0.05）. In the adult group, H. pylori prevalence was 50.95% in Muping, which was significantly higher than the 41.35% positive rate in Yanqing （P 〈0.01）. But there were no statistically significant differences between different age groups of 40-49, 50-59, and 60-79 years, or between males and females. A significant decrease in H. pylori prevalence in both regions was observed when the results of 2006 were compared with the data obtained in 1990 in Muping （50.95% vs 73.78%, P 〈0.001） and in 1992 in Yanqing （41.35% vs 55.35%, P〈0.01）. Conclusions After fifteen years, the prevalence of H. pylori infection among both children and adults remained significantly higher in areas with a high incidence of gastric cancer in China compared with that in areas with a low incidence of gastric cancer. H. pylori infection rates have decreased in the general Chinese population during recent years.     

Pathological demography of native patients in a nephrology center in China

Objective To analysis the pathological demography in Chinese patients undergoing renal biopsy from our niphrology center. Methods Between January 1979 and October 2000 in Jinling Hospital, Nanjing, China, 10002 attempts of percutaneous renal were performed in patients with renal disease from 33 provinces of China. The pathological classifications were made according to the WHO criteria of 1982 for renal pathology or the modified WHO criteria of 1995 by a panel of pathologists and nephrologists during routine clinical-pathological rounds. The pathological demography between those specimens collected from 1979-1989 and those from 1990-1999 was compared.Results The mean age of the 10 002 subjects undergoing renal biopsy was 31.4±13.0 years (ranging from 1 to 78 years), with a male to female ratio of 1.3∶1; for the 592 renal transplant recipients, the mean age was 37.5±9.1 years (ranging from 16 to 66 years), with a male to female ratio of 2.36∶1. Primary glomerular diseases (PGD) accounted for 71% of the total patients undergoing renal biopsies, secondary glomerular nephritis (SGN) 23%, tubular-interstitial diseases 3.2%, unclassified renal diseases 1.3%, hereditary and congenital renal diseases 1.0%, end stage renal diseases 0.96%, and recently realized or rare renal diseases 0.15%. IgA nephropathy (IgAN) was the most frequent pathological pattern (40%) of PGD, followed by mesangial proliferative lesion (MsPL) (30%), membranous nephropathy (MN) (10%), and focal segmental glomerulosclerosis (FSGS) (6%). Lupus nephritis (LN) was the most pathology common seen (74%) in SGN. During the 22 years of the study period, there was a steady increase in patients with SGN discovered during pathological evaluation of renal disorders. A rise in prevalence was found in IgA nephropathy, MN (both P<0.001), crescentic glomerulonephritis (P<0.0001), anti-GBM disease, and hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura related renal damages (both P<0.001). There was a decrease in endocapillary proliferative glomerulonephritis (P<0.001) and IgM nephropathy (IgMN) (P<0.01) from 1990-1999 as compared to 1979-1989. Infrequent renal pathological entities were also diagnosed in this group, including Niemann Pick disease, Fabry’s disease, POEMS syndrome, and lipoprotein glomerulonephropathy. Conclusions This is the largest series of renal biopsy data in China, and therefore may reflect the demographic picture of renal diseases in this country. Changes in prevalence of renal pathological entities were reflected in this group of patients over the last 22 years. In primary glomerular diseases, IgA nephropathy is still the most frequently observed pathological pattern. In SGN, LN appeared the most often. Increased prevalence was found in anti-GBM nephritis and HUS/TTP.     

Angiotensin-converting Enzyme Gene Insertion／Deletion Polymorphism in Children with Henoch-Schonlein Purpua Nephritis

This study investigated the relationship between angiotensin-converting enzyme (ACE) gene insertion/deletion polymorphism and the occurrence, severity, prognosis of HSPN. The polymorphism of ACE gene in 103 HSPN cases and 100 healthy children was studied by using the poly-merase chain reactions (PCR). Its relation to the clinical manifestation, pathological classification and prognosis of HSPN was analyzed accordingly. The results showed that: (1) there was a significantly higher frequency for DD genotype in HSPN children (P＜0.01) ; (2) DD genotype was more frequently seen in HSPN children with gross hematuria and massive proteinuria (P＜0.05), while DI genotype was more common in HSPN children group with renal insufficiency (P＜0.05); (3)although mesangial proliferative lesion was most frequently observed in 21 biopsied HSPN children,and DD genotype frequency was still higher in children with severe pathology (Class Ⅲ Ⅳ) ; (4)Ⅱ genotype was significantly frequent in HSPN children with complete remission in the follow-up of 32 HSPN children. It was concluded that the deletion allele of ACE gene might play a role, at least to some extent,in the occurrence,deterioration and progression in juvenile HSPN.     

Long-term visual outcome of dense bilateral congenital cataract

Background Dense congenital cataracts often cause severe visual impairment. The results of long-term follow-up of dense bilateral congenital cataract in China have not been well documented. The purpose of this study was to evaluate the long-term visual function in children who underwent cataract extraction for dense bilateral congenital cataract in southern part of China. Methods Medical records of children who underwent surgery of dense bilateral congenital cataract between January 1992 and December 2000 at Zhongshan Ophthalmic Center of Sun Yat-sen University were retroactively reviewed. In 38 children available for current follow-up, best corrected visual acuity （BCVA） and stereoscopic vision, as well as nystagmus, strabismus, and other complications, were evaluated. The mean follow-up period was 107.6 months （range 60 to 167 months）. Results The mean age of cataract extraction and secondary intraocular lens implantation were 5.6 months （range 3 to 12 months） and 4.2 years （range 2.4 to 15 years）, respectively. The mean BCVA was 0.25 in the better eye and 0.16 in the fellow eye. Stereoscopic vision was absent in all patients, and 3 children had simultaneous perception. Nystagmus was detected in all cases and strabismus in 35 cases. A high correlation was found between timing of cataract extraction and final BCVA of the better eye （r=-0.55, P=0.00）. A statistically significant difference was found in BCVA between post- and pre-treatment of amblyopia （t=5.65, P=0.00）. Conclusions Long-term visual function in children with dense bilateral congenital cataract was poor when cataract surgery was performed at age of 3 months or later. Earlier cataract surgery with adequate optical rehabilitation contributed to better visual outcome.     

Brain abscess in children, experience Of Mashad University

Objective： Brain abscesses continue to pose a problem in neurosurgery, and it is a serious life-threatening complication for several diseases. The objective of this research was to lock at the clinical profile of children suffering from brain abscess, predisposing conditions, microbiology and outcome of these patients. Methods： Ninety children aged less than 15 years were reviewed. The clinical presentation, predisposing factors, diagnosis, management and outcome were noted. There were 56（ 62.2% ） males and 34 （ 37.7% ） females. The mean age of presentation was 5.7 ± 4.2 years. Results： The mean duration of illness at the time of presentation was 10.6 ± 12.4 days. Typically patients presented with fever, vomiting, headache, neurological deficit, and seizures. The predisposing conditions found were cyanotic congenital heart disease in 18 （20 % ） of children, meningitis in 6 （6.7 % ）, Otitis or mastoiditis in 32 （35.5 % ）, Head trauma in 6 （ 6.7 % ）, previous intraeranial surgery in 8 （8.8 % ） and no underlying cause was found in 22 （24.4 % ） children. The most common microbe in children with cyanotic congenital heart disease was of the Streptococcus group （54 % ）. Computerized tomography confirmed the diagnosis and the most common location of the abscess was the parietal lobe of the cerebral hemisphere. That abscesses were larger than 2 cm in diameter where aspirated surgically. Excision was performed for eight children. Six children expired, two due to intracranial bleeding and the others due to severe cerebral edema and tentorial herniation. Complications were observed in 11 children out of which six had squeal and five had bemiparesis. Conclusion： Attention to hygiene of mouth and teeth in infant and child patients with cyanotic heart disease is very important in prevention of infectious brain diseases. We must also pay attention to ear-nose-throat diseases of children. That is very important the patients be examined again after treatment because of finding and trea     

WILSON'S DISEASE (HEPATOLENTICULAR DEGENERATION CLINICAL ANALYSIS OF 80 CASES

80 typical cases of Wilson's disease were observed over 54 years, 58 0f whom were hos- pitalized, an incidence of 19.7 per 100,0'00 general hospital inpatients and 522 per 100,0001 neurologic inpatients. The average age of onset was 23.5 years. Average age of onset before 15 years was 50To, between 20-30 26.91To and before 30 76.5.9To. 51.25'Io of our 80 patients had first involvement of the nervous system commencing at an average age of 25.2 years and 95To exhihited neurologic symptoms and signs. 39.99To had primary onset of hepatic disorder at an average age of 14.2 years and 80% showed evidence of hepatic dysfunction. 3.99To had renal involvement as first manifestation of disease and 40V/o showed evidence of renal damage. Aminoaciduria was demonstrated in 6.25%, 0.5'/o had affection of bones and joints, 2.5'/o had mental sympt.oms as first manifestation of the disease and 46.25% showed mental symptoms. 3 showed evidence of heart involvement. EEG findings and study of copper content of brain and liver wiU be pre- sented in other papers. Autopsy was performed on 5 with typical findings of Wilson's disease. Copper oxidase activity was lowered in 95%. Kayser-Fleischer ring was found in 73.75%. Urinary copper content was high in 96.97To (32/33 cases). Serum copper content was low in 65.22T。 (15;23 cases). 62 0f our 80 patients (77.50To) had well documented family histories. It was posi- tive in 10 patients of 9 families and highly suspected in another 4 in agreement with auto- somal recessive heredity. With regard to pro- gnosis 11 patients remain apparently healthy, 38 continue working or studying while taking treat- ment, 24 are invalids and 7 died. It appears that the longer the interval between onset, of disease and diagnosis, the worse the prognosis, indicat- ing the importance of early diagnosis and treat- ment.     

A multi-center study of hemodynamic characteristics exhibited by children with unexplained syncope

Background Syncope is common in children and adolescents, with 15% estimated to have had at least one syncopal episode by age 18. In recent years, an increasing number of children, especially girls at their school age, have developed unexplained syncope. The mechanism of an unexplained syncope exhibited by children is incompletely studied; the association between different hemodynamic patterns and clinical features is also not clear. The aim of the study was to investigate the hemodynamic patterns of children with unexplained syncope and to examine the clinical relevance.Methods Two hundred and eight children [87 boys, 121 girls, aged 3－19 years, mean (11.66±2.72) years] were selectively recruited from May 2000 to April 2006 when they presented syncope as their main complaint at the Multi-center Network for Childhood Syncope in Beijing, Hunan Province, Hubei Province, and Shanghai of China. All of the patients underwent head-up tilt tests; data were analyzed using SPSS version 10.0 for Windows. Continuous variables were expressed as the mean±standard deviation. Dichotomous variables were compared through a χ(2) test. A value of P<0.05 (two sided) was regarded as statistically significant.Results The age distribution of children with syncope was approximately normal. Head-up tilt tests was positive in 155 children, and the incidence of positive response of the baseline head-up tilt test for diagnosing unexplained syncope was 50.48%. The sensitivity value and diagnostic value of sublingual nitroglycerin head-up tilt test were both 74.52%. The hemodynamic pattern was normal in 53 children. The 155 children, who were positive in head-up tilt tests, showed signs of postural orthostatic tachycardia syndrome (60, 28.8%), the vasoinhibitory pattern (72, 34.6%), the cardioinhibitory pattern (5, 2.4%), and the mixed pattern (18, 8.7%). The gender distribution between the two age groups (age < 12 years vs age ≥ 12 years) was not different (P>0.05). The distribution of hemodynamic patterns between the children of the two age groups (age < 12 years vs age ≥ 12 years), and the children with different complaints (dizziness vs syncope) was significantly different (P<0.05), while the distribution between the children of different sexes and different lasting time of syncope (≤ 5 minutes vs> 5 minutes) was not significantly different (P>0.05). Different hemodynamic patterns were differentiated by differing syncope inducements, presymptoms, and complicated symptoms during and after syncope.Conclusion The tested girls were more prone when compared with the boys to have unexplained syncope, and the peak age was around twelve years old. The incidence of positive response of head-up tilt tests was also relatively higher for the girls. The distribution of hemodynamic patterns for different ages was different. For children with unexplained syncope, we should use head-up tilttests to distinguish the hemodynamic patterns in order to adopt rational therapeutic measures.     

Enzyme-linked immunosorbent assay for detection of immunoglobulin G antibody against Helicobacter pylori.

Eight strains of sonicated Helicobacter pylori (H. pylori) were pooled as group antigen to detect the serum IgG antibody to H. pylori in patients with various gastroduodenal diseases and subjects at health check-up. The enzyme-linked immunosorbent assay method used had a sensitivity of 94%, a specificity of 87%, and a positive predictive value of 96%. The prevalence of serum IgG antibody to H. pylori was 19.0% in patients with Type A gastritis, 76.0% in patients with Type B gastritis, 91.5% in duodenal ulcer, and 92.9% in gastric ulcer. Moreover, the prevalence of serum IgG antibody to H. Pylori in newborns was 53% (probably acquired from mother), in children below 10 years old was 25%. The detection rate gradually rose to 70% with the increase of age, but decreased to 40% in those over 70 years of age.