Rituximab therapy for juvenile-onset systemic lupus erythematosus

Rituximab (RTX), an anti-CD20 monoclonal antibody, has been proposed for use in the therapy of systemic lupus erythematosus (SLE). We present the initial long-term experience of the safety and efficacy of rituximab for treatment of SLE in children. Eighteen patients (mean age 14 ± 3 years) with severe SLE were treated with rituximab after demonstrating resistance or toxicity to conventional regimens. There was a predominance of female (16/18) and ethnic African (13/18) patients. All had lupus nephritis [World Health Organization (WHO) classes 3–5] and systemic manifestations of vasculitis. Clinical disease activity of the SLE was scored with the SLE-disease activity index 2K (SLEDAI-2K). Patients were followed-up for an average of 3.0 ± 1.3 years (range 0.5 to 4.8 years). B-cell depletion occurred within 2 weeks in all patients and persisted for up to 1 year in some. Clinical activity scores, double-stranded DNA (dsDNA) antibodies, renal function and proteinuria [urine protein to creatinine ratio (Upr/cr)] improved in 93% of the patients. Five patients required multiple courses of RTX for relapse, with B-cell repopulation. One died of infectious endocarditis related to severe immunosuppression. In conclusion, our data support the efficacy of rituximab as adjunctive treatment for SLE in children. Although rituximab was well tolerated by the majority of patients, randomized controlled trials are required to establish its long-term safety and efficacy

Manejo del edema en el sindrome nefrósico del niño

Sin resume

HIV Nephropathy in Children

The evaluation and management of children with HIV nephropathy provide a great challenge to the pediatric nephrologist caring for patients with this tragic illness, of which the nephropathy is only part of their problem and extends beyond the patient to the family and all of the supportive team involved. Care decisions should be based on a multi-disciplinary approach in which the infectious disease component is included. There are many areas that require a better understanding, particularly the pathogenesis of HIV nephropathy and the approach to treatment of the nephropathy with specific drugs. Early identification of children affected with this condition and an aggressive approach to management seem essential to the improvement of the outcome of these patients

Hypertensive crisis in children

Hypertensive crisis is rare in children and is usually secondary to an underlying disease. There is strong evidence that the renin-angiotensin system plays an important role in the genesis of hypertensive crisis. An important principle in the management of children with hypertensive crisis is to determine if severe hypertension is chronic, acute, or acute-on-chronic. When it is associated with signs of end-organ damage such as encephalopathy, congestive cardiac failure or renal failure, there is an emergent need to lower blood pressures to 25-30% of the original value and then accomplish a gradual reduction in blood pressure. Precipitous drops in blood pressure can result in impairment of perfusion of vital organs. Medications commonly used to treat hypertensive crisis in children are nicardipine, labetalol and sodium nitroprusside. In this review, we discuss the pathophysiology, differential diagnosis and recent developments in management of hypertensive crisis in children

Renal manifestations of sexually transmitted diseases: sexually transmitted diseases and the kidney

The adolescent population is particularly vulnerable to STDs. Those that cause significant kidney disease are of viral origin. The primary VVD are HIV-1, HBV, and HCV. Screening of high-risk populations should include quantitation of proteinuria, including total protein and microalbumin, to assess severity of renal damage and potential for progression. Renal biopsy is indicated for diagnosis and for planning important treatment interventions if there is significant proteinuria or decreased renal function. Causes of acute renal failure are frequently reversible and should be treated aggressively. These include HUS, vaso-motor or ischemic acute tubular necrosis, and drug toxicities. The spectrum of chronic kidney disease associated with VVD is broad and may include systemic manifestations of vasculitis. HIV-associated nephropathy is the prototype, with the most prevalent lesion remaining FSGS. Progression occurs in up to 15% of the patients, who are overwhelmingly of African lineage. Significant advances in management include ongoing development of HAART, angiotensin antagonists to control proteinuria, and novel immune-modulating drugs such as MMF, CsA, and rituximab. Dialysis therapies have offered improved survival, especially in pediatric patients. Moreover, transplantation is no longer considered experimental and should be offered to select patients

Abnormal urinary acidification in infants with hydronephrosis

Distal renal tubular abnormalities have been observed in patients with dilated urinary tract disorders. The present study was undertaken to look for patterns in urinary acidification in infants with varying degrees of hydronephrosis due to either reflux or obstruction and occurring as unilateral or bilateral disease. Three groups of infants (mean age 3.7±3.8 months) were studied prospectively. Groups IA and IB included patients with hydronephrosis who were acidotic and non-acidotic, respectively. Group II served as controls and consisted of patients with diarrhea and secondary metabolic acidosis with no known renal disease. Serum electrolytes, creatinine, and urine pH were measured in all patients. Urinary titratable acidity, ammonium (NH4), and net acid excretion (NAE) were measured by the titrimetric method. Infants with hydronephrosis demonstrated lower urinary buffering capacity, reflected in low NAE in the face of acidosis. Deficiencies were noted in both titratable acid and NH4 excretion compared with control infants. Acidosis was as common in unilateral as in bilateral disease, regardless of severity score. These data confirm a defect in distal urinary acidification in infants with hydronephrosis, whether unilateral or bilateral. Immaturity and endogenous acid load may play a significant role in the manifestation of metabolic acidosis with unilateral disease