Clinicl Significance of Mesangial IgM Deposits in Minimal Change Nephrotic Syndrome

The clinicopathologic features and the response to corticosteroid therapy were compared in 9 patients with minimal change nephrotic syndrome (MCNS) and diffuse mesangial IgM deposits (Group I) and in 32 of those without IgM deposits (Group II). However, serum IgM levels in Group I in both relapse and remission were significantly higher than those of Group II and controls (p<0.001). In Group I mesangial IgM deposits were diffuse in 9 (100%), mesangial C_1q was present in 4, IgA and fibrinogen were each observed in 1, respectively. Electron dense deposits in the mesangium were also present in 2 to 5 patients in Group I. No significant differences were found between the two groups in age of onset, sex ratio, laboratory data except for serum IgM level, duration before biopsy, follow-up periods, outcome, and response to steroid therapy. Our data suggest that a more severe degree of either impairment of mesangial clearance of IgM or overproduction of IgM may be involved in patients with MCNS and mesangial IgM deposits but that these patients could not be considered a distinct group of patients.     

Nephrotic syndrome in mesangial proliferative lupus nephritis

Renal involvement is a major complication of systemic lupus erythematosus (SLE) and occurs in 30-70% of patients with SLE. Lupus nephritis is classified into six classes (I-VI) by the International Society of Nephrology and Renal Pathology Society (ISN/RPS). Although nephrotic syndrome is commonly associated with diffuse (ISN/RPS class IV) or membranous (ISN/RPS class V) lupus nephritis, several reports have described nephrotic syndrome in adult patients with minimal mesangial lupus nephritis (ISN/RPS class I) or mesangial proliferative lupus nephritis (ISN/RPS class II). However, nephrotic syndrome in mesangial proliferative lupus nephritis has rarely been reported in children. Although the pathogenesis of nephrotic syndrome with mesangial lupus nephritis is incompletely understood, three potential mechanisms have been postulated including lupus nephritis itself, non-steroidal anti inflammatory drug (NSAID)-induced minimal change nephrotic syndrome (MCNS) and coincidental occurrence of MCNS. We describe here a child with mesangial proliferative lupus nephritis who developed MCNS.     

The Nature and Molecular Weight of the Circulating Immune Complex in Glomerulonephropathy Associated With Hepatitis B Virus Infection

Circulating immune complex (CIC) in patients with hepatitis B virus associated glomerulonephropathy was fractionated into two parts by sucrose gradient ultracentrifugation. A CIC of higher molecular weight (more than 900,000 daltons) consisted of hepatitis B virus surface antigen (HBsAg), and one of lower molecular weight (between 900,000 and 66,000 daltons) consisted of hepatitis B virus e antigen (HBeAg). The patients with membranous glomerulonephropathy (MGN) had much more CIC of higher than of lower molecular weight, while deposits in the glomerular basement membrane were stained with anti-HBeAg but not with anti-HBsAg. A patient with minimal changes had only higher molecular weight CIC and no deposits in the kidney. Our results suggest that lower molecular weight CIC containing HBeAg may play an important role in the development of MGN.     

Elevated polymorphonuclear phagocytic function in thalassemia patients by chemiluminescence

Twenty five patients with β thalassemia major, with no evidence of infection were evaluated for their polymorphonuclear cell (PMN) metabolic function and serum opsonic activity by chemiluminescence assay. These were divided into Group I of normal adults (n=21), Group II thalassemia major < 5 years (n=9) and Group III thalassemia major > 5 years (n=16). The ability of the chemiluminescence assay (CL) to reflect opsonic and phagocytic dysfunction suggested its potential application in the evaluation of phagocytic function. The peak count of Group I was (1.07 ±0.24×10⁻⁵), Group II (1.60±0.83×10⁻⁵) and Group III was (2.71±0.98×10⁻⁵) respectively in the presence of autologous sera. The peak count compared between Group I and III was found to be statistically significant (p<0.05). The peak count of Group I and II when compared showed a trend in the increase activity not statistically significant. The polymorph function of all the groups were compared with autologous serum as well as normal serum. There was no increase in polymorph function of Group III in the presence of thalassemia serum, nor any decrease in the polymorph function of thalassemia patients of Group II and III. This concluded, that polymorphs of thalassemia patients are active in the presence of autologous as well as normal serum. The increased activity of thalassemia polymorphs may be due to antigenic stimulation which may be due to multiple transfusion and not due to circulating iron load.     

Characterization of a Monoclonal Antibody Recognizing the Blastemal Element of Wilms' Tumors and Fetal Kidneys

A blastema-associated antigen (BLA.-1) was detected using a monoclonal antibody against malignant blastema from a Wilms' tumor. The localization of BLA-I was investigated in a series of nine Wilms' cases, five fetal, one childhood, and two adult kidneys. In this series, BLA-1 antibody consistently stained cell surfaces of all Wilms' tumors containing blastemal components. The same staining pattern was maintained in tumors grown as heterotransplants in nude mice. The expression of BLA-1 antigen was examined in normal blastema of fetal kidneys. BLA-1 was immunolocalized to condensed blastemal cells in the nephrogenic zone throughout gestation. In addition, kidney samples from a young child or adults contained no blastemal cells and therefore showed no blastemal cell surface staining. Glomerular mesangial cell staining was demonstrated in kidneys from 12 weeks of gestation through adulthood. This staining in developing and mature glomeruli implies that mesangial cells may be derived from condensed blastemal cells. The finding of a cell surface antigen common to Wilms' blastema, fetal blastema, and mesangial cells has not been previously demonstrated.     

八例C1q肾病的诊断与鉴别诊断

目的：探讨C1q肾病的诊断和鉴别诊断。方法；分析8例C1q肾病患儿肾活检组织中C1q沉积的发生率、强度和分布特点，并与同期352例原发性和42例继发性肾小球疾病作比较。结果：8例C1q肾病患儿系膜区均有显著的C1q沉积，阳性率为100％，平均沉积强度达2．1＋；20例狼疮性肾炎患儿中，13例系膜区有显著的C1q沉积，阳性率为65％，平均沉积强度达1．7＋；22例乙肝病毒相关性肾炎中，5例系膜区有显著的C1q沉积，阳性率为23％，平均沉积强度达0．5＋。在原发性肾小球疾病中，5例膜增殖性肾小球肾炎患儿中的3例系膜区有显著的C1q沉积，阳性率为60％，平均沉积强度达1．6＋；其他病理类型的患儿均缺乏。结论：儿童C1q肾病的诊断除依赖于免疫荧光发现系膜区有显著的C1q沉积外，尚需与狼疮性肾炎、乙肝病毒相关性肾炎和膜增殖性肾小球肾炎相鉴别。     

Immunological Profile in Children with Minimal Change Nephrotic Syndrome

ABSTRACT. Peripheral blood from patients with active stage of minimal change nephrotic syndrome (MCNS) was examined for concanavalin A (ConA)-inducible suppressor T cell activity, proliferative response to phytohemagglutinin (PHA) and in the autologous (AMLR) and allogeneic (MLR) mixed lymphocyte reaction, proportions of T cells with receptors for IgM (Tu) or IgG (Tγ) and the levels of serum immunoglobulin M, G and A. Six of 9 patients with MCNS studies showed deficiency of ConA-induced suppressor cell activity. In the AMLR, only one of 9 patients with MCNS demonstrated depressed proliferative response (p<0.05). In the allogeneic MLR, T cells from 5 of 9 patients with MCNS demonstrated poor proliferative response when stimulated with normal control non-T cells. Five of 9 patients with MCNS had depressed proliferative response to PHA. The proportion of total T cells, Tu cells and Ty cells in the patient group were comparable to healthy control group. Serum IgG was significantly decreased in 7 of 11 patients. This study demonstrates multiple immunological abnormalities in patients with MCNS that might play a role in its pathogenesis.     

Shunt nephritis: the nature of the serum cryoglobulins and their relation to the complement profile.

The serum complement profiles of four patients with shunt nephritis indicated classical pathway activation of the complement system. The presence of mixed cryoglobulins was correlated with disease activity and the cryoglobulins were shown to be complement reactive. Antisera to two of the cryoglobulins recognized antigens of the infecting organism, and a specific bacterial antibody was identified in one cryoglobulin, giveing evidence that the cryoglobulins contained immune complexes. Bacterial antibody without detectable antigen was demonstrable in the sera indicating antibody excess. Renal morphology demonstrated mesangial proliferation and interposition with subendothelial and mesangial deposits. Parallels are drawn with active lupus nephritis.     

Chronic partial ureteral obstruction in the neonatal guinea pig. I. Influence of uninephrectomy on growth and hemodynamics

Although obstructive nephropathy is a frequent cause of renal insufficiency in infancy, factors influencing adaptation to chronic partial ureteral obstruction (CPUO) are poorly understood. Guinea pigs were subjected to unilateral CPUO within the first 2 days of life (Group I) and microsphere studies were performed at 23 +/- 3 days of age. To also investigate the role of functional renal mass on growth and hemodynamics, contralateral nephrectomy was performed in Group II at the time of ureteral constriction. Compared to sham-operated controls (ureteral diameter = 1 mm) CPUO caused impaired somatic growth in both groups. Resulting hydroureteronephrosis was variable and most severe (ureteral diameter greater than 3 mm) in azotemic animals of Group II, which demonstrated reduced compensatory renal hypertrophy and poorest somatic growth. Mean arterial blood pressure was not affected by CPUO or uninephrectomy. Renal blood flow (RBF) was higher in Group II than Group I, and did not fall significantly with severity of CPUO in Group II, in which number of perfused glomeruli was similar to controls. In contrast, renal vascular resistance (RVR) increased by 172% in animals of Group I with severe CPUO, and was associated with 34% fewer perfused glomeruli than in the hypertrophied contralateral kidney. With increasing severity of CPUO, RBF tended to be distributed to outer cortical regions in the obstructed kidney of Group I, while the shift in RBF distribution was from outer to inner cortex in the remaining kidney of Group II. Filtration fraction fell as a result of CPUO in both groups, such that reduction in the glomerular filtration rate was due in large part to factors other than RBF.(ABSTRACT TRUNCATED AT 250 WORDS)     

Immunological profile in children with minimal change nephrotic syndrome

Peripheral blood from patients with active stage of minimal change nephrotic syndrome (MCNS) was examined for concanavalin A (ConA)-inducible suppressor T cell activity, proliferative response to phytohemagglutinin (PHA) and in the autologous (AMLR) and allogeneic (MLR) mixed lymphocyte reaction, proportions of T cells with receptors for IgM (Tu) or IgG (T gamma) and the levels of serum immunoglobulin M, G and A. Six of 9 patients with MCNS studies showed deficiency of ConA-induced suppressor cell activity. In the AMLR, only one of 9 patients with MCNS demonstrated depressed proliferative response (p less than 0.05). In the allogeneic MLR, T cells from 5 of 9 patients with MCNS demonstrated poor proliferative response when stimulated with normal control non-T cells. Five of 9 patients with MCNS had depressed proliferative response to PHA. The proportion of total T cells, Tu cells and T gamma cells in the patient group were comparable to healthy control group. Serum IgG was significantly decreased in 7 of 11 patients. This study demonstrates multiple immunological abnormalities in patients with MCNS that might play a role in its pathogenesis.     

Positive skin test and age

OBJECTIVE: To evaluate positive responses to skin tests for immediate hypersensitivity to allergens in children with asthma and rhinitis at different ages. METHOD: We observed positive skin test reactivity in prick tests using fifteen allergens of same origin (total dust and Dermatophagoides sp.; Dermatophagoides pteronyssinus; Dermatophagoides farinae; Blomia tropicalis; Penicillium sp; Alternaria alternata; Cladosporium herbarium; Aspergillus fumigatus; Bermuda grass; forage grass; dog and cat epithelia; feathers; Blatella germanica and wool). We placed 713 selected patients into different age groups - Group I: 6 to 11 months; Group II: 1 to 3 years and 11 months; Group III: 4 to 8 years and 11 months; and Group IV: 9 to 15 years. We used the chi-square test for statistical analysis. RESULTS: The total significant differences between these groups were: I to II = 5; II to III = 5; II to IV = 5; III to IV = 6; I to III = 10; and I to IV = 10. CONCLUSION: Skin test reactivity is acquired progressively with age, and can be observed as early as at 12 months. Reactivity is significantly more positive from the age of 4 on.     

Anti-Streptococcus mutans antibodies in saliva of children with different degrees of dental caries

The aim of this study was to evaluate the relationship between the secretory immune system and dental caries. Forty-nine 3–5-year-old children with primary dentition were classified into three groups according to their caries indices: no caries (group I), one or two surfaces with caries lesions (group II) and rampant caries (group III). Lower numbers of mutans streptococci were found in group I in relation to groups II and III. Secretory IgA and anti-S. mutans IgA, IgM and IgG antibody levels were not significantly different among the groups. Western blotting analysis showed that some S. mutans proteins, including the 39, 59, 97 and 150 kDa molecular mass bands, were recognized by almost all the saliva samples. Antibodies against the 185 kDa band, known as antigen I/II, were present in all adults’ saliva and in only one child in group III. The absence of antibodies to the 185 kDa band in children’s saliva suggest a specific immunologic immaturity. Further prospective studies will be necessary to establish the possible effect of reactivity to this antigen on the S. mutans colonization in this age group.     

Characterization of ovomucoid-specific T-cell lines and clones from egg-allergic subjects

In the pathogenesis of allergic reactions, T cells and cytokines play a major role. However, characterizations of food allergen-specific T cells are very limited. In this study, we screened the peripheral blood mononuclear cells ( PBMC ) of 14 patients for reactivity to ovomucoid ( Gal d I ), the major hen's egg allergen, and ovalbumin ( Gal d II ). Cell lines and clones specific to ovomucoid were generated from PBMC of four egg-allergic subjects, in order to study antigen domain specificity and cell cytokine production profiles. The results demonstrated, firstly, that egg-allergic patients respond to ovomucoid rather than to ovalbumin, and secondly, that antigen specificity is predominantly directed toward the second and third domains of ovomucoid. The T-cell cytokine message was characterized by reverse transcrip-tase polymerase chain reaction ( RT-PCR ). Cell lines and clones from all four patients consistently expressed interleukin ( IL )-5. IL-4, IL-13. and in-terferon-gamma were found to be expressed only by certain lines or clones. This observation suggests a central pathogenic role for IL-5 in food allergy-related symptoms.     

Diagnostic value of Elisa serological tests in childhood tuberculosis

Two separate studies (study I and study II) were conducted to evaluate the efficacy of ELISA serological test for the detection of IgG antibodies against specific glycolipid antigen (PGLTb1) and ESAT 6 antigen of Mycobacterium tuberculosis, respectively. These results were compared with bacteriological tests [Ziehl Neelson (ZN) staining for acid-fast bacilli and culture on Lowenstein Jensen (LJ) medium] and polymerase chain reaction (PCR) targeting IS6110 sequence. Both studies were carried out on children with pulmonary, central nervous system, lymph node, and gastrointestinal tuberculosis along with matching controls (65 cases and 27 controls for study I and 83 cases and 22 controls for study II). Informed consents of their parents or guardians were taken. They were subjected to clinical examination, relevant laboratory investigations, tuberculin test and chest radiograph. Relevant body fluids were subjected to bacteriological tests and PCR. Sera samples were analyzed for antibodies against PGLTbl and ESAT 6 antigen in study I and study II, respectively. ELISA tests showed a significantly higher sensitivity (49% study I; 53%, study II) as compared with LJ medium culture method (15.4%, study I; 28.9% study II) and ZN staining (27.7%, study I; 20.5%, study II) in all patients (p < 0.05). The results were comparable with PCR (40%, study I; 42.2% study II). Specificity of ELISA test was 100% in all the patients except in those with pulmonary disease (92.8%, study I; 84.8%, study II). In view of the convenience, low cost and comparable sensitivity with PCR, these ELISA tests have a promising future in the diagnosis of childhood tuberculosis.     

Hepatitis B-associated nephrotic syndrome in Jamaican children

Between December 1984 and November 1996, 171 children under 12 years old presented to the University Hospital of the West Indies with nephrotic syndrome. Hepatitis B surface antigen (HBsAg) was found in ten (6%) of these children, eight of whom had membranous nephropathy (MN), and one each had mesangial proliferative glomerulonephritis (MesN) and minimal change nephrotic syndrome (MCNS). Only those children with MesN and MCNS were steroid-sensitive. The HBsAg-positive status was identified incidentally on screening. At a mean follow-up of 34 months, seven of ten children had experienced complete or partial remission and three had persistent nephrotic syndrome, although none was in renal failure. Six of the ten had biochemical hepatitis. All the children were still HBsAg-positive. Hepatitis B virus (HBV) is a factor contributory to nephrotic syndrome in Jamaican children. As diagnostic clinical markers for HBV-associated nephropathy are usually absent, all children presenting with nephrotic syndrome should be screened for HBsAg. A policy should be implemented in Jamaica for screening pregnant women and at-risk groups for HBsAg, as well as for immunising susceptible neonates, in order to reduce the incidence of HBV-associated pathology.     

Clinicopathological features of childhood nephrotic syndrome in Saudi Arabia.

The clinicopathological features are described in 119 Arab children in Saudi Arabia with the nephrotic syndrome. The clinical and laboratory data are similar to those described in other parts of the world. However, mesangial proliferative glomerulonephritis (MesPGN) was found in 21 of 66 biopsies (31.8%), giving a frequency of 17.6% of all children with the nephrotic syndrome. Minimal-change nephrotic syndrome (MCNS) was diagnosed in 17 biopsies (25.8%) and in 58 patients (48.7%). Onset of the nephrotic syndrome was at less than 1 year of age in 17 patients (14.3%). Seven children had 11 episodes of peritonitis. Seven children had positive hepatitis B surface antigen (HBsAg) in their serum: renal biopsy carried out on four of them showed membranous glomerulonephritis (MGN) in three, and four of the seven patients developed end-stage renal disease (ESRD). There were nine deaths, all in patients with end-stage renal disease: six of the deaths occurred in infants. The pattern of childhood nephrotic syndrome in Saudi Arabia is different from the pattern in tropical countries.     

Ovarian function after successful bone marrow transplantation in postmenarcheal females

Ovarian function was followed serially in a group of six postmenarcheal females after successful bone marrow transplantation (BMT). The patients were between 13 9/12 and 22 6/12 (median 16 5/12) years of age at the time of BMT and were followed a median of 20 months (range 17-45 months) posttransplantation. Two subjects received short-term high-dose cyclophosphamide combined with single-dose total lymphoid irradiation (Group I), whereas the remaining four were treated with short-term, high-dose chemotherapy plus single-dose total body irradiation (Group II). Group II subjects also received combination chemotherapy prior to BMT. One subject from Group I continues to have regular menses and normal gonadotropin levels, 36 months post-BMT. The remaining five patients have demonstrated persistently elevated plasma concentrations of LH and FSH over a 17- to 45-month period of time. None of the four patients in Group II has menstruated since undergoing BMT. We conclude that single-dose radiation combined with short-term, high-dose chemotherapy results in profound ovarian damage in the majority of young women undergoing BMT.     

The use of an intra vesical silicon inflatable baloon device improves results of bladder autoaugmentation technique. Long term follow up

PurposeTo present the long-term RESULTS of bladder autoaugmentation in children with low compliant neurogenic bladders comparing the usual technique with a modified technique using an intra vesical silicon inflatable balloon device.Material and MethodsTwenty-two patients with low-compliant neurogenic bladders and incontinence confirmed urodynamically were divided in two groups. Group I (12 patients) underwent autoaugmentation using the classical detrusorectomy approach. Group II (10 patients) underwent autoaugmentation associated to an intra vesical silicon inflatable device filled with saline solution customized to allow urine elimination and sustain partial bladder distention.ResultsFollow up ranged from 2 to 11 years (mean 4 years). Patients in Group I showed unsatisfactory RESULTS, with only one patient achieving continence using clean intermittent catheterization (CIC). Four patients showed mild improve on urodynamic parameters. Patients in group II showed encouraging RESULTS. Six patients achieved continence with CIC. Two patients achieved continence using CIC and anticholinergic agents. Bladder capacity had a mean increase of approximately 71% and bladder compliance mean increased from 15.6 ± 16.8 ml/cmH2O to 34.3 ± 22.8 ml/cmH2O (p = 0.02). Two patients with previously dilated upper urinary tract showed a decrease in the grade of dilatation.ConclusionsBladder autoaugmentation using an intra vesical silicon inflatable balloon device showed better RESULTS compared to the usual autoaugmentation technique. The presented device didn't add morbidity to the surgical procedure. This technique can be used in selected patients in order to improve urodynamic parameters and continence without the potential disadvantages of using bowel segments for bladder augmentation.     

The value of postoperative radiotherapy in childhood nonrhabdomyosarcoma soft tissue sarcoma

OBJECTIVE: To determine the value of postoperative radiotherapy (RT) in the management of nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) of childhood. PROCEDURE: From 1964 to 2000, 62 children with a median age of 14 years were seen at the University of Iowa and underwent a wide local excision for non-metastatic NRSTS. Tumors were high grade in 36 (58%) and >5 cm in 24 (39%). Margins of resection were negative (Group I) in 37 (60%) and positive (Group II) in 25 (40%). Postoperative RT was delivered to 20 patients (32%); eight of 37 (22%) Group I and 12 of 25 (48%) Group II children received postoperative RT. Chemotherapy was employed in 19 patients (31%). Median follow-up was 9.6 years. RESULTS: The 5- and 10-year overall survival rates for Group I were 69 and 63% and for Group II were 66 and 60%. The 5- and 10-year local control rate was 66%. On multivariate analysis, size of tumor (P < 0.001) and postoperative RT (P = 0.017) were prognostic factors for local control. All 13 Group I children with low grade, 相似文献   

AMPHETAMINE ADDICTION AND PREGNANCY

Abstract. Billing, L., Eriksson, M., Larsson, G. and Zetterström, R. (Department of Paediatrics, Karolinska Institute, St. Göran's Children's Hospital, Stockholm, Sweden). Amphetamine addition and pregnancy. Acta Paediatr Scand, 69: 675, 1980.—Sixty-six infants born to amphetamine-addicted mothers were followed during their first year of life. The children were divided into three groups, according to whether or not the mother stopped her abuse in early pregnancy (Group I) or continued (Group II) and whether or not the latter children were placed in foster homes immediately after birth (Group III). All but 2 of 16 mothers in Group I stayed off drugs and mostly met non-addicted friends. In Group II, on the contrary, all but 2 of 36 mothers continued their abuse one year after delivery. At the age of one year, all but one child in Group I were in their mothers' custody and all children in Group III had remained in foster care. In Group II one-third of the children lived in foster homes after revocation of the maternal custody. Several infants in Group II had experienced multiple transfers between the biological home and different foster homes. There were indicators that maternal amphetamine abuse causes temporary drowsiness in the infants during the first months after birth. However, all children at the age of 12 months, regardless of group, had a somatic and psychomotor development in accordance with the normal Swedish standard. In all groups there was an increased rate of medical care mainly because of infections. Some infants in Group II compared to none in Groups I and III were hospitalized be-cause of failure to thrive or suspected physical abuse. Symptoms indicating emotional disturbance were more common in infants of Group II than in infants of Groups I and III.