Hepatitis B infection in pediatric dialysis and transplant patients: significance of e antigen

We examined the clinical significance of hepatitis Be antigenemia in 36 HBsAg positive pediatric dialysis and renal transplant patients. One hundred twenty-seven sera were tested for HBeAg and anti-HBe. Seventy-three sera (57%) from 29 patients (81%) contained HBeAg. The presence of HBeAg was associated with an increased titer of HBsAg (P < 0.005) and with the presence of the HBsAg carrier state (P < 0.001). HBeAg was found in 40% of specimens taken from dialysis patients, and in 70% of specimens from transplant patients (P < 0.001). No serum contained anti-HBe, although 28 of 29 sera (97%) tested had antibody to HBcAg. No association was found between the presence of HBeAg and serum aminoleucine transferase levels or the histologic evidence of chronic active hepatitis. Fifteen HBeAg negative sera from patients persistently positive for HBsAg were tested for HBV-specific DNA polymerase activity; 7 (47%) had significant activity. Since both HBeAg and DNA p are indicators of infectivity, many HBeAg negative sera from immunosuppressed HBsAg carriers may be infectious.     

Abnormal growth of the thyroid cartilage in the digeorge syndrome

Larynges from 17 patients with DiGeorge syndrome (DGS) and from 14 patients with tetralogy of Fallot (TOF) (11 non-DGS and 3 possible but unproven DGS) were dissected, measured, and compared to a control population of comparable body length. The patients with DGS and the 3 patients with TOF suspected of having DGS showed the following: small thyroid cartilages with increased anterior angle, abnormally short superior cornua, low ratio of mean superior cornual length to distance between superior cornual tips, and delayed time of maximal rate of increase in superior cornual length relative to increase in body length.     

73例慢性咳嗽的病因分析

目的 常规查体和理化方法不能明确诊断病因的慢性咳嗽，应用慢性咳嗽解剖学诊断程序和痰细胞分类学方法对其进行病因分析、诊断和治疗。方法 选择呼吸内科门诊的73例慢性咳嗽患者，采用Irwin的慢性咳嗽解剖学诊断流程和痰细胞分类的检查方法，对其病因进行分析，并根据病因给予特异性治疗。结果 73例慢性咳嗽患者经以上方法检查后，明确了病因，并采用特异性治疗，提高了临床疗效。结论 慢性咳嗽的解剖学诊断流程和痰细胞分类学的方法能够帮助医生明确慢性咳嗽的病因，做出正确的诊断，采用特异性治疗效果良好。     

Ethnic differences in parathyroid hormone secretion and mineral metabolism in response to oral phosphate administration

Ethnic differences in bone metabolism have been reported and it has been suggested that these may be partly due to prolonged exposure to an elevated plasma parathyroid hormone (PTH) concentration or a decreased sensitivity to PTH. We explored ethnic differences in bone and mineral metabolism by 5 days of oral phosphate (P) loading to stimulate PTH secretion. Healthy older people from UK (B), The Gambia (G) and China (C), 15 individuals from each sex and ethnic group, were studied. Blood and urine samples were collected before and 2 h after P dose on days 1, 4 and 5 and on a control day. The induced changes (%) in PTH and markers of mineral and bone metabolism after 2 h and over 5 days were examined.At baseline, PTH, 1,25(OH)₂D and bone turnover markers were higher in Gambian subjects than in British and Chinese subjects (P ≤ 0.01).2 h after P loading, ionized calcium (iCa) decreased and PTH and plasma P (P) increased in all groups (P ≤ 0.01, n.s. between groups). Urinary P to creatinine ratio (uP/Cr) increased, the increase being greater in Chinese subjects than in British and Gambian subjects on days 4 and 5 (P ≤ 0.01). By day 5, fasting iCa was decreased and P increased in British and Gambian (P ≤ 0.01) but not in Chinese subjects. Fasting PTH and uP/Cr increased in all groups. There were ethnic differences in changes in bone markers, but the relationship with changes in PTH was comparable between groups.In conclusion, ethnic differences in mineral metabolism in response to 5-day P loading were found. Chinese subjects showed a more rapid renal clearance of P than British and Gambian counterparts and there were differences between the groups in the skeletal response to P loading, but no evidence was found for resistance to the resorbing effects of PTH.     

Morphometric studies of cystic and tubulointerstitial kidney diseases with hepatic fibrosis in children

Portal tract fibrosis with biliary ductular enlargement or proliferation occurs in a number of genetic diseases that have cystic or tubulointerstitial renal lesions. These include some with renal cystic disease such as autosomal recessive diseases (e.g., infantile polycystic disease, juvenile polycystic disease, and Meckel's syndrome), autosomal dominant diseases (e.g., adult polycystic disease) and, rarely, tuberose sclerosis and dominant glomerulocystic disease. Portal tract fibrosis with biliary enlargement and proliferation occurs also with tubulointerstitial kidney diseases. These probably include at least three disorders in the category nephronophthisis-congenital hepatic fibrosis (one autosomal recessive disease and two either autosomal or X-linked recessive diseases) plus Jeune's syndrome (the tubulointerstitial diseases Fanconi's familial nephronophthisis and anti-tubular membrane antibody disease do not regularly cause hepatic fibrosis). Morphometric data on ratios of bile ductules to connective tissue in hepatic portal tracts show high values for infantile polycystic disease (mean, 0.616) compared to lower values for juvenile polycystic disease (mean, 0.286). That the cystic renal lesions of the first two diseases differ in type and time course is known. Similar data on ratios of glomeruli plus tubules to connective tissue in renal cortices and of tubules to connective tissue in outer medullary zones of kidneys, respectively, are as follows: for Fanconi's nephronophthisis, 0.445 and 0.197; for anti-tubular basement membrane antibody disease, 0.585 and 0.164; and for the three types of nephronophthisis-congenital hepatic fibrosis studied, 0.668 and 0.446, 1.39 and 0.921, and 1.18 and 0.12. These data support clinical impressions that the category nephrophthisis-congenital hepatic fibrosis includes more than one disease entity.     

Multicentric osteolysis: report of the second successful renal transplant

A patient with end stage renal disease due to sporadic idiopathic multicentric osteolysis (type 3 multicentric osteolysis) is described. His pre-transplant course was similar to those of previously described patients, while his post-transplant course has been uncomplicated. The pathology and pathogenesis of the nephropathy of sporadic idiopathic multicentric osteolysis is not well characterized. The short term outcome of renal transplantation is excellent in our patient and in the other similar case known to have been transplanted.     

Iron status and fibroblast growth factor-23 in Gambian children

A relationship between iron and fibroblast growth factor-23 (FGF23) metabolic pathways has been proposed. Iron deficiency anaemia is prevalent in The Gambia and concentrations of fibroblast growth factor-23 FGF23 are elevated in a large percentage of Gambian children with rickets-like bone deformity. We speculate that low iron status may be involved in the aetiology of Gambian rickets. The aim of this study was to determine if there was a relationship between haemoglobin, as a marker of iron status, and FGF23 in samples from children with and without a history of rickets-like bone deformities in The Gambia. We conducted a retrospective analysis of studies carried out from 2006 to 2008 in children from a rural community in The Gambia where iron deficiency anaemia is endemic and where elevated circulating concentrations of FGF23 have been found. To investigate the relationship between circulating FGF23 and haemoglobin concentrations we used an age-adjusted linear regression model on data from children <18y of age with a family or personal history of rickets-like bone deformity (BD) (n=108) and from the local community (LC) (n=382). We found that circulating concentration of FGF23 was inversely correlated with haemoglobin concentration. This effect was more pronounced in BD children compared with LC children (interaction: P≤0.0001). Anaemia and elevated FGF23 were more prevalent in BD children compared to LC children (P=0.0003 and P=0.0001 respectively). In conclusion, there is a stronger relationship between FGF23 and haemoglobin in Gambian children with a history of rickets compared to local community children. This study provides support for the contention that iron may be involved in FGF23 metabolic pathways.     

A Possibly Distinctive Vacuolar Change of the Amniotic Epithelium Associated with Gastroschisis

Histological examination of the placental membranes of 3 of 4 infants with gastoschisis revealed peculiar vacuolar change of the cytoplasm of the amnitic epithelial cells. No storage material was demonstrated by special stains or electron microscopic study. This vacuolar change is different from other amniotic cell abnormalities, such as those related to meconium staining.