Autoantibodies in minimal change nephrotic syndrome

The presence of low level antibody (AAb) activity and circulating immune complexes (CIC) were studied in 17 patients with minimal change nephrotic syndrome (MCNS). Study groups include 12 MCNS without mesangial deposits (Group I) and 5 MCNS with mesangial deposits (Group II). In Group I reactivity against normal tissue antigens was demonstrated (kidney tubular microsomal antigen - 8, smooth muscle - 5, gastric cell - 5). In Group II reactivity against kidney basement membrane was demonstrated in all five patients. CIC were detected in eight (Group I - 6, Group II - 2). Dissociation of the CIC showed that they all contained antibodies with a corresponding autoantibody activity which could be removed by prior incubation with their complexed antigen. The presence of these AAb's and their formation of CIC may indicate their role in initiating a primary immunological insult to the kidney.     

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.     

肾小球系膜区IgM沉积在儿童原发性肾小球疾病中的意义

目的：探讨肾小球系膜区IgM沉积在儿童原发性肾小球疾病中的意义。方法：选取2005年6月至2011年6月于北京大学第一医院儿科住院并行肾穿刺活检的原发性肾小球疾病患儿作为研究对象。根据免疫荧光下肾小球系膜区有无IgM沉积进行分组,将肾小球系膜区IgM沉积≥+且IgM免疫荧光强度≥其他免疫球蛋白荧光强度的患儿作为IgM沉积组,其余患儿为对照组。回顾性分析两组的临床、病理特点和随访等情况。结果：125例原发性肾小球疾病患儿进入分析,其中IgM沉积组76例,对照组49例。①两组在发病年龄、血总蛋白、血白蛋白、血总胆固醇、肌酐清除率、肾早期损伤指标、高血压和肾功能不全发生率等方面差异无统计学意义。IgM沉积组24 h尿蛋白定量及血IgM水平显著高于对照组（P分别为0.025和0.038）。②IgM沉积组肾小球硬化及小动脉病变发生率显著高于对照组(P分别为0.002和0.039),基底膜增厚发生率显著低于对照组（P=0.000）。③对激素治疗反应两组差异无统计学意义（P=0.364）。④随访2~78个月,两组肾功能不全及ESRD发生率差异无统计学意义。⑤IgM沉积组3/76例男性患儿行重复肾活检。除1例首次肾活检已诊断为局灶节段性肾小球硬化外,余2例在第2次肾活检时均由系膜增生性肾小球肾炎转化为局灶节段性肾小球硬化。结论：伴系膜区IgM沉积的原发性肾小球疾病患儿蛋白尿程度更重,肾脏病理改变亦更突出。对于伴系膜区IgM沉积的原发性肾小球疾病患儿应格外关注并密切随访,警惕其进展为局灶节段性肾小球硬化的可能。     

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.     

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.     

Glomerulopathy with mesangial IgM deposits: Long-term follow up of 64 children

BACKGROUND: The aim of the present study was to investigate to what extent IgM nephropathy in children with minimal change nephrotic syndrome (MCNS) and diffuse mesangial hypercellularity (DMH) evolves to focal segmental glomerulosclerosis (FSGS). METHODS: Tissues from renal biopsies were examined by light microscopy (LM), immunofluorescence (IF) and, in four cases, by electron microscopy (EM). From a total of 352 nephrotic children, 121 had renal biopsy results as steroid dependent or resistant. A diagnostic renal biopsy was also performed in 331 children with non-nephrotic proteinuria and/or hematuria. A second renal biopsy was performed in 16 children whose renal function was impaired during the follow up. The clinical course of IgM-positive children was compared with that of IgM-negative children. RESULTS: Of the 121 nephrotic children with renal biopsy, 85 were MCNS. Twenty were IF positive mainly for IgM, six of whom (30%) presented evolution to FSGS, while of the remaining 65 IF-negative children, only three (4.6%) presented evolution to FSGS. Of the total 331 children with non-nephrotic proteinuria and/or hematuria, 139 were diagnosed as IgA--IgG nephropathy, 44 had positive IF for IgM and 148 were IF negative. Of the 44 children IF positive for IgM, seven (15.9%) presented evolution to FSGS, while none of the 148 IF-negative children presented evolution to FSGS. The follow-up time for all children ranged from 1 to 14 years. CONCLUSIONS: Of IgM nephropathy patients with MCNS and DMH, a significant percentage develop impaired renal function, due to the evolution of FSGS, as revealed by repeat biopsy during long-term follow up.     

Enhanced suppressor T cell activity resulting in increased IgM and decreased IgG productions in children with minimal change nephrotic syndrome

In order to clarify the cellular basis accounting for the decreased serum IgG and increased serum IgM in children with minimal change nephrotic syndrome (MCNS), peripheral blood mononulcear cells (MNC) were obtained from 15 children with biopsy-proved MCNS and 15 age- and sex-matched normals. MNC were then separated into helper (OKT4) T cells, suppressor (OKT8) T cells, B cells and monocytes. Different cell populations from patients and normals were then recombined and pokeweed mitogen-stimulated in vitro immunoglobulin biosynthesis was studied. The results strongly suggest the presence of isotype-specific suppressor T cells which may affect the switch of IgM B cells to IgG B cells in patients with MCNS and may be used to explain partly the clinical findings of lowered serum IgG and increased IgM in those individuals.     

伴有新月体形成的原发性IgA肾病的临床与病理分析

目的了解儿童伴有新月体形成的原发性IgA肾病的临床与病理特点.方法对29例伴新月体形成的原发性IgA肾病患儿的临床及病理资料进行分析,并依受新月体累及的肾小球比例分组比较,≥50%(A组),9例;<50%(B组),20例.结果 (1)临床方面29例均有血尿+蛋白尿,尿蛋白≥1 g/24 h 者22例(76%)和肉眼血尿86%,水肿、高血压、肾功能异常者均不及半数.A组以肾病综合征和急进性肾炎为主,持续性肉眼血尿、大量蛋白尿、高血压、肾功能衰竭均较B组明显(P<0.05).B组无症状性血尿+蛋白尿者65% .(2)病理方面新月体形成累及肾小球5%～85%, A组为52%～85%(其中新月体型IgA 肾病10%),B组5%～40%,以细胞性为主.均有系膜增生和小管-间质病变,球囊粘连易见. 两组比较A组系膜增生严重、小球硬化和小管灶状萎缩明显(P<0.05),B组球囊粘连多见(P<0.05).(3)免疫荧光均有IgA+IgM+C3沉积,合并IgG沉积者18例(62%),其中5例(17%)为"满堂亮"(A组占4例).未见一例单纯IgA沉积.结论伴有新月体形成的原发性IgA肾病临床均有血尿合并蛋白尿,以持续性肉眼血尿和大量蛋白尿为主;以弥漫性系膜增生为主要病理改变,易见球囊粘连和小管-间质病变;沉积物以IgA+IgM型或IgA+IgM+IgG型多见,部分呈"满堂亮";较一般型IgA肾病临床、病理明显加重.     

Comparison of standard and high dosage recombinant interferon alpha 2b for treatment of children with chronic hepatitis B infection

BACKGROUND: Interferon is currently the most useful therapeutic agent for chronic viral hepatitis. The aim of this study was to compare the efficacy of standard and high dosages of interferon in children with chronic hepatitis B virus (HBV) infection. METHODS: Thirty children with chronic hepatitis B infection were randomly assigned to receive 5 million units/m2 body surface area (Group I) or 10 million units/m2 body surface area (Group II) recombinant interferon alpha 2b three times weekly for 6 months. Patients were followed for at least 6 months (range, 6 to 18; median, 9 months) after the end of therapy, by physical and serologic examination every 3 months. RESULTS: Clearance of HBV DNA occurred in 4 (27%) patients from Group I and 9 (60%) patients from Group II at the end of therapy. Hepatitis B e antigen (HbeAg) clearance was 7% (1 patient) and 53% (8 patients) in the two groups, respectively (P < 0.05). HBV DNA was undetectable in 40 and 60% of the children at the 12th month of randomization in Groups I and II, respectively. HBeAg/antibody to HBeAg seroconversion was found in 33% (5 patients) who received standard dosage and 60% (9 patients) in the high dosage group. Sustained complete response (normal alanine aminotransferase, negative HBeAg and HBV DNA at 12th month) was obtained in 5 and 9 patients respectively from groups I and II (P > 0.05). Only mean baseline serum alanine amino-transferase concentrations were predictive of response to interferon. CONCLUSIONS: A 6-month course of interferon alpha 2b in children with chronic HBV disease was well-tolerated by most patients. Sustained suppression of HBV was obtained in 60% of patients with high dosage interferon and in 33% of the patients receiving standard dosage. Although these results were not statistically significant, studies with more patients are needed to ascertain whether high dosage improves the response rate.     

Plasma separation as an effective treatment of glomerulonephritis in lupus erythematosus

Positive anti-DNA-antibodies and lowered C3 and C4 levels were found in serum of a 13 year old girl presenting with edema, microscopic hematuria and proteinuria. Renal biopsy revealed diffuse endo- and extracapillary glomerulonephritis with mesangial deposits of IgA, IgG, IgM, C1q, C3 and fibrinogen. In spite of treatment with prednisone (60 mg/m2/d) severe nephrotic syndrome (proteinuria greater than 20/d) developed with excessive hypertension and deterioration of renal function (GFR: 20 ml/min./1,73m2 BSA). Plasma exchange therapy (3 sessions) led to recovery of renal function, blood pressure and complement activity and to a reduction of anti-DNA-antibodies and protein excretion.     

Failure of clinical and laboratory characteristics to differentiate mesangial proliferative from minimal-change nephrotic syndrome

Twelve clinical and laboratory characteristics of nephrotic syndrome were compared in 24 children with biopsy-proven mesangial proliferative glomerulonephritis (MesPGN) and 17 children with biopsy-proven minimal-change nephropathy (MCNS). The objective of the study was to determine if these characteristics alone, without renal biopsy, could be used to differentiate the two histopathologic entities. Sex, urinary protein level and IgM immunofluorescence were found to be significantly different in the two groups. Discriminant analysis produced two formulae which gave a discriminant rate of 79% for MesPGN and 76% for MCNS. We conclude that the clinical and laboratory characteristics studied could not differentiate MesPGN from MCNS.     

Sialic acid in childhood renal diseases: Correlation with clinical and laboratory indices

There are many kinds of glycoproteins that have sialic acid residues and it has been reported that these are elevated in some renal diseases and their significance in the pathogenesis of several renal diseases has been investigated. In the present study the serum and urine levels of sialic acid were measured in healthy controls and in children with either poststreptococcal acute glomerulonephritis (PSAGN) or minimal change nephrotic syndrome (MCNS) to test if there is any correlation with clinical and laboratory indices. In PSAGN and MCNS patients the serum and urine sialic acid concentrations at onset and relapse were significantly different from healthy controls (Mann-Whitney U-test P < 0.005). There was not a significant correlation between the clinical severity, serum creatinine and complement C₃ levels and serum sialic acid concentrations in PSAGN patients. Also there was not a significant correlation between edema, serum albumin, IgG, transferrin, α-1-antitrypsin and serum sialic acid concentrations in MCNS patients. Although high serum and urine sialic acid levels were found in both PSAGN and MCNS patients, it does not have any clinical significance nor is it important as a diagnostic or prognostic marker.     

Role of irradiation in management of synovial sarcoma: St. Jude Children's Research Hospital experience

The role of irradiation in the management of synovial sarcoma (SS) in pediatric patients is evaluated. The review covers all children seen at St. Jude Children's Research Hospital between May 1969 and December 1992 with the diagnosis of soft tissue sarcoma, of the 37 patients with the subtype SS, 16 received irradiation for the management of primary site disease. There were four IRS Group I, six Group II, four Group III, and two Group IV patients receiving irradiation. Tumor grade included seven Grade II and nine Grade III lesions. TMN staging identified eight T1 and eight T2 lesions. Follow-up has ranged from 14 to 117 months (med = 33 months). All IRS Group I patients had documented local control. Five of six IRS Group II and 4/4 Group III patients have had documented local control at last follow-up. IRS Group IV patients had either local control tumor stabilization (n = 1) or evidence of tumor regression (n = 1) at autopsy. Complications following irradiation include wound dehiscence (n = 1), surgery to revise a painful scar (n = 1) extremity length discrepancy (n = 2), and femoral head avascular necrosis (n = 1). At last follow-up, 10 of 14 patients receiving curative intent irradiation remain alive. This review indicates questionable benefit to the addition of irradiation for patients with adequate surgical resection and having “good” tumor characteristics (Grade I, II; IRS Group I, TMN T_1A, T_1B). For lesions that have had incomplete resection or partial response to chemotherapy, there is evidence that irradiation may provide durable local control. The role of irradiation in those patients with IRS Group IV disease is at present confined to palliative roles until the time when more effective chemotherapy will mandate the decision to treat primary disease for curative measures. © 1996 Wiley-Liss, Inc.     

长疗程环孢素A治疗儿童激素耐药型肾病综合征的疗效和预后

目的 观察激素耐药的儿童肾病综合征(SRNS)应用环孢素A(CsA)长疗程治疗的疗效并分析影响疗效的相关因素.方法 回顾性分析疗程2年的CsA治疗SRNS病例20例,男:女为3:1;平均年龄5.5岁,肾病病理类型微小病变(MCNS)15例,局灶节段肾小球硬化(FSGS)4例,系膜增生性肾炎(MsPGN)1例.结果 (1)完全缓解13例(65%),部分缓解4例(20%),CsA无效3例(15%),总有效率85%.平均随访时间40.5个月,复发率45%.(2)CsA治疗微小病变肾病的有效率为93%,非微小病变为60%,但两者差异无统计学意义.(3)毒副作用:多毛、齿龈增生和高血压的发生率分别为75%、25%和10%,2例出现可逆性的肾损伤,4例出现尿NAG/Cr增高,神经系统症状2例.3例重复肾活检未发现CsA相关的肾小管间质纤维化病变.结论 对儿童激素耐药型肾病综合征CsA长疗程治疗有良好的疗效.小剂量CsA长期治疗未发现药物相关的肾小管间质纤维化和肾功能损伤.CsA长期治疗的安全性和预后有待进一步研究.     

Neonatal gram negative pyogenic meningitis: therapeutic evaluation

Thirty babies with gram negative neonatal pyogenic meningitis were studied for therapeutic evaluation of various drug combinations. Group I received ampicillin and gentamycin and group II received ampicillin with chloramphenicol all intravenously. These drugs were continued for 2 wk after bacteriological cure. Group III include those from group I and II who did not show bacteriological cure even after 7 days of therapy and were given sulphamethoxazole—trimethoprim preparation intravenously for 3 to 4 days and continued orally for another 2 wk. Mortality was 75 per cent (9 cases) in group I compared to 16·6 percent (9 cases) in group II. Group III children developed more complications like cerebral palsy and hydrocephalus.     

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.     

Body electrolytes in bronchopulmonary dysplasia and the effects of diuretic therapy

Body electrolytes and their regulatory hormones were studied in preterm infants who suffered from bronchopulmonary dysplasia under two groups: those who were not treated with diuretics (Group II), and those who were treated with diuretics (Group III). The values were compared with a group of matched healthy controls (Group I). Lower serum Na levels, a need of higher Na intake, and higher urinary Na concentrations and urinary specific gravity were found in Group II infants. FeNa was normal and the urinary flow rate was lower than the controls. These data suggest an inability of these infants to dilute urine. Group III infants who were treated with diuretics showed higher serum Na levels and lower urinary specific gravity than Group II infants. These values, as well as water and Na intake/output ratios, were all similar to the control values. Serum aldosterone level was highest in Group II but did not reach significance. Intracellular K concentration was not different between the groups indicating an optimum total body K balance. A significant negative correlation between serum Na and aldosterone levels was found in Group II infants, which was not noted in the controls. Significant correlations were also found between FeNa and plasma aldosterone level in the BPD groups, unlike the controls. The control group of infants showed significant positive correlation between Na balance and serum Na levels. Our results suggest that inability to dilute urine appropriately might be the reason for the BPD patients to retain body water. Water restriction and diuretic therapy therefore are reasonable therapeutic approaches in such cases.     

IgA nephropathy presenting clinicopathological features of acute post-streptococcal glomerulonephritis

Abstract A 5-year-old Japanese girl was affected with acute nephritis. The patient had hypo-complementaemia and an elevation of anti-streptolysin O with positive throat culture of Group A streptococci. Four weeks after onset of the disease, serum complement level returned to normal, but proteinuria increased into the nephrotic range with a deterioration in renal function. Four weeks after onset, light microscopy of a renal biopsy showed diffuse endocapillary proliferation, and immunofluoroscopy revealed predominant IgA deposition in the mesangium. Electron microscopy showed electron dense deposits in the mesangial and subendothelial area, but subepithelial deposits were not found in the glomeruli. Histological diagnosis was IgA nephropathy, while her clinico-serological features were typical of acute post-streptococcal glomerulonephritisConclusion These results suggest that in some patients, IgA nephropathy may be triggered by streptococcal infection and misdiagnosed as acute post-streptococcal glomerulonephritis if renal histological examinations are not done.     

Immunological and Endocrinological Response to Growth Hormone Therapy in Short Children

ABSTRACT. We investigated the influence of human growth hormone (hGH) on mitogen-stimulated lymphoproliferation, in vitro IgM production, serum levels of immunoglobulins, somatomedin-C (Sm-C) values and serum growth-promoting activity (Thymidine Activity, TA) in 18 short children, aged between 6.6–14.5 years, undergoing a 3-month course of hGH therapy. Blood was collected the day before treatment (Group A), on the 5th day after patients were administered hGH daily (0.1 U/kg) i.m. for 4 days (Group B), after a 3-month course of hGH injected three times weekly, and finally before (Group C) and 24 h after an extra injection (Group D). In vitro IgM production from the patients' unstimulated lymphocytes decreased from 277±41 (Group A) to 168±38 (Group B), to 119±43 (Group C) and then to 119±28 ng/ml (Group D) ( p <0.05). Using PWM-stimulated lymphocytes in vitro IgM production decreased from 2015±464 (Group A) to 1116±316 (Group B), then to 511±170 (Group C) and 968±295 ng/ml (Group D) ( p <0.02). The variation of this decrease could be correlated with the variation of growth velocity during treatment ( r =0.619, p <0.05). In contrast, no significant changes were found following therapy either in serum levels of IgA, IgE, IgG, IgM, Sm-C and TA, or in phytohemagglutinin, concanavalin A and pokeweed mitogen-stimulated lymphoproliferation. Our data suggest that there is some relationship between growth hormone, growth and immunity.     

T cell subsets in glomerulonephritis

Abnormal lymphocyte function has been postulated to have a pathogenetic role in nephrotic syndrome. In an attempt to investigate the pathogenetic role of lymphocyte subsets in human glomerular disease, we studied 110 children suffering from nephritis during the acute nephrotic phase or nephritis without steroid treatment, 4 weeks later after steroid treatment, in remission and relapse. These patients included minimal change nephrotic syndrome (MCNS) 15 cases, focal segmental glomerular sclerosis (FGS) 6 cases, mesangial cell proliferative nephropathy (MesPGN) 42 cases, membranoproliferative glomerulonephritis (MPGN) 2 cases, hepatitis B surface antigenemia associated with membranous nephropathy (HBVMN) 10 cases, IgA mesangial nephropathy (IgAN) without nephrotic syndrome 7 cases, poststreptococcal glomerulonephritis (PSGN) 24 cases and chronic glomerulonephritis (CGN) 4 cases. There was no significant difference in the total lymphocyte count of each different pathological group of nephritis except that lymphopenia was noted in the CGN patients. When the lymphocyte phenotypic profile was examined, OKT8 cells were significantly increased in the MesPGN patients and both OKT4 and OKT8 cells were significantly increased in HBVMN. Comparison of MCNS and MesPGN during the acute nephrotic phase showed the OKT4/OKT8 ratio decreased significantly in MesPGN. Four weeks after steroid treatment, OKT4 cells decreased both in MCNS and MesPGN being pronounced in MCNS. In the remission stage with steroid treatment the OKT4/OKT8 ratio decreased in MCNS and was mildly elevated in MesPGN. In relapse, the OKT4/OKT8 ratio was the same as it was during the onset of nephrotic phase. MCNS cases were steroid responsive whereas in MesPGN there were frequent relapses or partial steroid response.(ABSTRACT TRUNCATED AT 250 WORDS)