Persistent hematuria among children with sickle cell anemia in steady state

ABSTRACT Introduction: Persistent hematuria is a chronic complication of sickle cell anemia (SCA) which can progress to chronic kidney disease. The practice of early detection of persistent hematuria in children with SCA in steady state is important for timely intervention. Objective: To determine the prevalence of persistent hematuria among children with sickle cell anemia in steady state and compare the result with that of a group of HbAA controls. The outcome will possibly strengthen the health policy on the need for regular screening for persistent hematuria in children with SCA. Methods: Children with sickle cell anemia, aged 2-18 years in steady state, were recruited consecutively from the sickle cell clinic at the University of Nigeria teaching Hospital Enugu. The controls were similarly recruited from the children's outpatient clinic. To determine persistent hematuria, dipstick urinalysis and microscopy were performed for both subjects and controls at enrollment and repeated after four weeks. Results: Out of the 122 children with SCA studied, 5 (4.1%) had persistent hematuria. None (0%) of the 122 age- and gender-matched HbAA controls had persistent hematuria. This difference in prevalence of persistence between HbSS patients and HbAA controls was statistically significant (p= 0.02). Conclusion: Persistent hematuria still occurs significantly more among children with SCA, even among those in steady state. Routine urinalysis at follow-up visits in children with SCA is strongly recommended, as this will aid early detection and prompt management to prevent progression to chronic kidney disease.

Pathogenesis and clinical significance in kidney injury induced by persistent hematuia / 国际儿科学杂志

Hematuria,which hasn't attracted enough attention of the clinicians during the last twenty years,is one of the common symptoms of urinary system.At present,it is believed that persistent hematuria may be involved in the further development of renal injury and the prognosis of renal disease.This article mainly describes the diagnosis,the prognosis and the possible mechanism of hematuria,as well as paying attention to the hematuria symptom,making the correct diagnosis and the appropriate intervention in time.

La nefropatía IgA, la glomerulopatía más frecuente en el mundo / IgA nephropathy, the most common glomerulopathy worldwide

La nefropatía inmunoglobulina A es una glomerulonefritis mediada por inmunocomplejos caracterizada por el depósito de inmunoglobulina A1 en el mesangio glomerular. Es la forma más frecuente de glomerulonefritis primaria en el mundo. La probabilidad de deterioro de la función renal a largo plazo, está aumentada por los hallazgos siguientes: hipertensión arterial, hematuria microscópica persistente, proteinuria mayor de 0,5 g/día, descenso de la función renal al comenzar las manifestaciones o hallazgos en la biopsia renal de esclerosis glomerular, esclerosis vascular, fibrosis intersticial, atrofia tubular, formación de crecientes o distribución de IgA en la pared de los capilares glomerulares en la inmunofluorescencia. Son manifestaciones clínicas en la nefropatía inmunoglobulina A la hematuria macroscópica en aproximadamente la mitad de los pacientes al primero o segundo día del inicio de síntomas de infección respiratoria, y está asociada con dolor en el flanco en pacientes menores de 40 años. En los más viejos, la hematuria microscópica es generalmente asintomática, y en ocasiones, detectada en análisis de orina de pesquisa. Entre 10 y 20 % de los pacientes, por lo general aquellos que tienen hematuria y proteinuria ligera, pueden lograr remisión espontánea; pero, entre 25 y 30 % pueden mostrar progresión hacia la enfermedad renal crónica terminal, y la progresión por lo general es lenta (5-20 años). La biopsia renal es la única prueba específica para confirmar el diagnóstico. Los pacientes con hematuria y proteinuria menor de 0,3 g/día que están normotensos, no requieren tratamiento específico con medicamentos, pero necesitan ser controlados periódicamente con análisis de orina, creatinina sérica y medida de la tensión arterial. Los pacientes con proteinuria o hipertensión deben ser tratados enérgicamente con inhibidores de la enzima convertidora. La hipertensión, la proteinuria significativa (> 0,5 g/día), la glomerulonefritis rápidamente progresiva (rara) y el síndrome nefrótico necesitan ser tratados inmediatamente. La amigdalectomía, frecuentemente realizada en Japón, puede ser de beneficio para los que se presentan con hematuria macroscópica y amigdalitis. Se consultaron varias fuentes para esta revisión.

IgA nephropathy is immunocomplex-mediated glomerulonephritis that is characterized by the A1 immunoglobulin deposition in the glomerular mesangium. It is the most frequent form of primary glomerulonephritis worldwide. The probabilities of long-term renal function deterioration increased due to the following findings: blood hypertension, persistent microscopic hematuria, proteinuria greater than 0.5 g/day, decrease of renal function when manifestations or findings of glomerular sclerosis are observed in the renal biopsy; vascular sclerosis, interstitial fibrosis, tubular atrophy, formation or distribution of IgA in the glomerular capillary walls in the immunofluorescence test. Among the clinical manifestations of IgA nephropathy is macroscopic hematuria in roughly half of patients in the first or second day after onset of the respiratory infection symptoms and is associated to flank pain in patients less than 40 years. In the oldest people, macroscopic hematuria is generally asymptomatic and occasionally detected in screening urinalysis. Ten to twenty percent of patients with mild proteinuria and hematuria may reach spontaneous remission, but 25 to 30 % of them may also progress into the terminal chronic renal disease at a general slow rate (5 to 20 years). Renal biopsy is the only specific test to confirm diagnosis. Those patients with hematuria and proteinuria of less than 0.3 g/day, whose blood pressure is normal, do not require specific drug treatment but they need to be systematically controlled through urinalysis, serum creatinine and blood hypertension taking. The patients suffering from proteinuria and hypertension must be strictly treated with converting enzyme inhibitors. Hypertension, significant proteinuria (0.5 g/day), rapidly progressive glomerulonephritis (rare) and nephrotic syndrome must be immediately managed. Tonsillectomy, frequent method in Japan, could be beneficial for those patients presenting with macroscopic hematuria and tonsillitis. Several sources were consulted to make this review.

A Case of Idiopathic Membranous Glomerulonephritis in association with Thin Glomerular Basement Membrane Nephropathy / 대한신장학회잡지

We report a rare case of the idiopathic membranous glomerulonephritis (IMGN) in association with the thin glomerular basement membrane nephropathy (TGBMN) in a 63-year-old female with hematuria. This is the first case reported in Korea. In renal biopsy of this case, direct immunofluorescence demonstrated anti-IgG Ab along the glomerular capillary wall with granular pattern. The basement membrane was thin, about 170-220 nm and small epimembranous electron dense deposits were observed by electron microscopy. As this case, the combination of TGBMN and IMGN is very uncommon because the IMGN is characterized morphologically by diffuse global thickening of the glomerular capillary wall, while the TGBMN is defined as an extreme thinning of the glomerular basement membrane, less than 200 nm. Our case showed no renal function deterioration and benign prognosis as other reports showed.