[Expression of TGF-beta and hepatocyte growth factor in kidney tissues of children with primary focal segmental glomerular sclerosis].

OBJECTIVE: To study the expression of transforming growth factor-beta (TGF-beta) and hepatocyte growth factor (HGF) in kidney tissues of children with primary focal segmental glomerular sclerosis (FSGS) and the possible role of the two growth factors in the development of FSGS. METHODS: Kidney specimens were obtained from 33 children with primary FSGS and 7 children with isolated haematuria but without FSGS (control group). Of the 33 children with primary FSGS, 6 children had no renal tubule interstitial pathological damage (Experimental I group) and 27 children had renal tubule interstitial pathological damage (Experimental II group). Expression of TGF-beta and HGF in kidney tissues was ascertained by the immunohistochemical method. RESULTS: TGF-beta and HGF were expressed in the three groups, but there were significant differences among the three groups. The expression of TGF-beta and HGF in the two experiment groups increased significantly compared with that in the control group. The Experimental II group had increased TGF-beta expression but a significantly decreased HGF expression compared with the Experimental I group. The index of tubule interstitial pathological changes was positively correlated with the TGF-beta expression (r=0.763, P<0.01), but negatively correlated with the HGF expression (r=-0.461, P<0.05) in the Experimental II group. There was a negative correlation between TGF-beta and HGF expression in children with primary FSGS (r=-0.425, P<0.05). CONCLUSIONS: The expression of TGF-beta and HGF in kidney tissues is increased in children with primary FSGS. TGF-beta might be a fibrogenic factor and HGF might be an anti-fibrotic factor in the kidney in primary FSGS.

Acute pancreatitis in childhood-onset systemic lupus erythematosus: Case report. / Pancreatitis aguda en el lupus eritematoso sistémico de inicio en la niñez: A propósito de un caso.

Systemic lupus erythematosus (SLE) is a multisystem disease with protean manifestations. Lupus pancreatitis is the second most common disease associated with SLE-related acute abdomen. Although acute pancreatitis is rare it is clinically important because this condition can be life threatening if not treated promptly. Here, we report a case of a 10-year-old girl who developed SLE-associated pancreatitis after steroids therapy that was subsequently complicated by fungal septicaemia. Her clinical symptoms and signs markedly improved after administration of glucocorticoids and cyclophosphamide.

El lupus eritematoso sistémico (LES) es una enfermedad multisistémica con manifestaciones proteicas. La pancreatitis lúpica es la segunda enfermedad más frecuentemente asociada con el abdomen agudo en relación con el LES. Si bien la pancreatitis aguda es rara, es clínicamente importante porque puede ser potencialmente mortal si no se trata de inmediato. En este artículo, describimos el caso de una niña de 10 años que desarrolló pancreatitis asociada a LES después del tratamiento con corticoesteroides que se complicó posteriormente debido a septicemia fúngica. Los signos y síntomas clínicos mejoraron marcadamente después de la administración de glucocorticoides y ciclofosfamida.

[A prospective multicenter clinical control trial on treatment of refractory nephrotic syndrome with mycophenolate mofetil in children].

OBJECTIVE: To evaluate the efficacy and safety of mycophenolate mofetil (MMF) plus prednisone on refractory nephrotic syndrome (RNS) in children. METHODS: One hundred and forty-two children with RNS from ten clinical trial centers were divided into two groups: MMF (n=87) and control (n=55). The MMF group patients were administered with oral MMF (30-40 mg/kg daily) for at least 6 months. Afterwards the patients who responded to MMF received another 6 months MMF treatment at a dosage of 10-20 mg/kg daily. The controls were treated with pulse intravenous infusion of cyclophosphamide (CTX) (10 mg/kg daily) for 2 days every 2 weeks for 3 months. Then CTX was administered at a dosage of 500 mg/m2 once a month 4, 7 and 10 months after treatment. While the patients received MMF or CTX treatment, they were treated with oral prednisone (0.5-1 mg/kg daily) for 2 to 3 months, and then the dosage of prednisone was gradually reduced. Urinary protein, liver and renal functions, and side effects of drugs were examined at regular intervals for one year. RESULTS: Of the 87 patients, 58 achieved complete remission, 16 achieved partial remission, 9 achieved early remission and 4 had no response to treatment. In the control group, 35 achieved complete remission, 9 achieved partial remission, 1 achieved early remission and 10 had no response to treatment. The total remission rate in the MMF group (95.4%) was significantly higher than that in the control group (81.8%) (P<0.01). After treatment 67 patients (65.4%) in the MMF group had negative proteinuria compared with 36 patients (65.4%) in the control group (P>0.05). MMF was found to be more effective in reducing proteinuria, and improving hypoproteinemia, oliguria, hyperlipemia, and edema than CTX. MMF was better tolerated with lower incidences of adverse reactions than CTX. CONCLUSIONS: The combined therapy of MMF and prednisone is more effective and tolerable than pulse intravenous infusion of CTX for treatment of RNS in children.

Pancreatitis aguda en el lupus eritematoso sistémico de inicio en la niñez: A propósito de un caso / Acute pancreatitis in childhood-onset systemic lupus erythematosus: Case report

El lupus eritematoso sistémico (LES) es una enfermedad multisistémica con manifestaciones proteicas. La pancreatitis lúpica es la segunda enfermedad más frecuentemente asociada con el abdomen agudo en relación con el LES. Si bien la pancreatitis aguda es rara, es clínicamente importante porque puede ser potencialmente mortal si no se trata de inmediato. En este artículo, describimos el caso de una niña de 10 años que desarrolló pancreatitis asociada a LES después del tratamiento con corticoesteroides que se complicó posteriormente debido a septicemia fúngica. Los signos y síntomas clínicos mejoraron marcadamente después de la administración de glucocorticoides y ciclofosfamida.

Systemic lupus erythematosus (SLE) is a multisystem disease with protean manifestations. Lupus pancreatitis is the second most common disease associated with SLE-related acute abdomen. Although acute pancreatitis is rare it is clinically important because this condition can be life threatening if not treated promptly. Here, we report a case of a 10-year-old girl who developed SLE-associated pancreatitis after steroids therapy that was subsequently complicated by fungal septicaemia. Her clinical symptoms and signs markedly improved after administration of glucocorticoids and cyclophosphamide.

Efficacy of urine screening at school: experience in Shanghai, China.

To explore the prevalence of hematuria or proteinuria in school children in Shanghai and to evaluate the screening methods, we conducted urine screening in more than 40,000 school children between 2003 and 2005. Children were tested with dipsticks read manually (method A) or dipsticks read by machines (method B) combined with a sulfosalicylic acid test or microscopy. Some children were tested once, and others who had abnormal results in the first screening were tested again 2 weeks later. The prevalence of urine abnormalities in the first screening was more than 5.00% and of the second screening about 1.00%. Either method B or testing two urine samples for each child had higher specificity. As to the direct cost, that of screening twice with method A was lower than just screening once with method B. So using method A to screen twice for each child was not only convenient and economical, but also could reduce the false positive rate effectively. More than 10 months of follow-up diagnosed two cases of IgA nephropathy. Asymptomatic chronic renal diseases in school children could be detected through school urine screening. For Shanghai, China, screening twice using method A might be the best choice.