COL4A5 genotypes and clinical characteristics of children with Alport syndrome / 中国当代儿科杂志

OBJECTIVES@#To investigate the genotypes of the pathogenic gene COL4A5 and the characteristics of clinical phenotypes in children with Alport syndrome (AS).@*METHODS@#A retrospective analysis was performed for the genetic testing results and clinical data of 19 AS children with COL4A5 gene mutations.@*RESULTS@#Among the 19 children with AS caused by COL4A5 gene mutations, 1 (5%) carried a new mutation of the COL4A5 gene, i.e., c.3372A>G(p.P1124=) and presented with AS coexisting with IgA vasculitis nephritis; 3 children (16%) had large fragment deletion of the COL4A5 gene, among whom 2 children (case 7 had a new mutation site of loss51-53) had gross hematuria and albuminuria at the onset, and 1 child (case 13 had a new mutation site of loss3-53) only had microscopic hematuria, while the other 15 children (79%) had common clinical phenotypes of AS, among whom 7 carried new mutations of the COL4A5 gene. Among all 19 children, 3 children (16%) who carried COL4A5 gene mutations also had COL4A4 gene mutations, and 1 child (5%) had COL4A3 gene mutations. Among these children with double gene mutations, 2 had gross hematuria and proteinuria at the onset.@*CONCLUSIONS@#This study expands the genotype and phenotype spectrums of the pathogenic gene COL4A5 for AS. Children with large fragment deletion of the COL4A5 gene or double gene mutations of COL4A5 with COL4A3 or COL4A4 tend to have more serious clinical manifestations.

Tratamento endovascular da síndrome do quebra-nozes em adolescente: relato de caso / Endovascular treatment of a teenager with nutcracker syndrome: a case report

Resumo A síndrome do quebra-nozes (ou síndrome de nutcracker) é causada pela compressão da veia renal esquerda pela artéria mesentérica superior e aorta, e está associada a uma sintomatologia característica, como dor no baixo ventre, varicocele e hematúria. O diagnóstico é frequentemente difícil e, portanto, demorado. O tratamento invasivo é controverso, especialmente nos pacientes pediátricos; no entanto, em casos de hematúria severa associada a anemia, insuficiência renal funcional, severa dor pélvica ou ineficácia de tratamento conservador, ele é indicado. É relatado o caso de uma criança do sexo masculino, 12 anos, com quadro de hematúria maciça por 12 horas, sem evidências de alterações à investigação inicial, que evoluiu com anemia intensa e retenção urinária. Investigações futuras evidenciaram imagens sugestivas da síndrome de nutcracker e foi optado pelo tratamento endovascular por implante de stent smart control seguido de balonamento. Paciente cessou a hematúria após o procedimento e permanece assintomático há 5 anos.

Abstract The nutcracker syndrome is caused by compression of the left renal vein by the superior mesenteric artery and aorta and is associated with characteristic symptoms, such as lower abdominal pain, varicocele, and hematuria. Diagnosis is often difficult and, therefore, is often delayed. Invasive treatment is controversial, particularly in pediatric patients. However, it is indicated in cases of gross hematuria associated with anemia, renal function impairment, severe pelvic pain, or ineffective conservative treatment. We report the case of a 12-year-old boy presenting with severe hematuria for 12 hours, with no abnormal findings at a first evaluation, who progressed with severe anemia and urinary retention. Further investigation provided images suggestive of nutcracker syndrome, and endovascular stenting (smart control stent) followed by balloon dilatation was the treatment of choice. Hematuria ceased after the procedure, and the patient is still asymptomatic at 5-year follow-up.

Fístula arteriovenosa renal pós-biópsia renal: relato de caso e revisão de literatura / Renal arteriovenous fistula after renal biopsy: a case report and literature review

Fístulas arteriovenosas (FAVs) renais adquiridas são raras, correspondendo a uma conexão anômala entre o sistema arterial e o sistema venoso. As FAVs renais se dividem em três grandes grupos: idiopáticas, congênitas e adquiridas, sendo as últimas as mais comuns. Atualmente, têm incidência aumentada em decorrência do crescente número de biópsias renais. Apesar de, atualmente, o procedimento de biópsia renal ser relativamente seguro, ele carrega como complicação a formação de FAV no território vascular renal. O tratamento de FAV renal é amplamente discutido na literatura e diversas modalidades terapêuticas podem ser aplicadas. Apresentamos um caso de FAV pós-biópsia renal que foi submetida a tratamento endovascular com sucesso mediante embolização com molas

Acquired renal arteriovenous fistulas (AVF) are rare conditions in which an anomalous connection arises between the arterial and venous systems. Renal AVFs can be classified into three main groups: idiopathic, congenital, and acquired, the last of which are the most common. Incidence has been increasing, due to the growing number of renal biopsies. Although the renal biopsy procedure is relatively safe nowadays, one possible complication is formation of an AVF in the renal vascular territory. Treatment of renal AVF is widely discussed in the literature and a variety of treatment methods can be employed. We report a case of arteriovenous fistula after renal biopsy that was successfully treated with endovascular coil embolization

Índice calcio-creatinina en una muestra matutina de orina para la estimación de hipercalciuria asociada con hematuria no glomerular observada en las edades infanto-juveniles / Calcium-creatinine ratio in a morning urine sample for the estimation of hypercalciuria associated with non-glomerular hematuria observed in children and adolescents

Resumen Introducción: La hipercalciuria suele revelarse durante el diagnóstico diferencial de la hematuria que acompaña a la litiasis renal. La exactitud diagnóstica de la excreción urinaria de calcio puede afectarse por las insuficiencias asociadas con la colección de orina de 24 horas. En este estudio se evaluó la utilidad diagnóstica del índice calcio/creatinina (ICaCre) en la estimación de la hipercalciuria asociada con hematuria y litiasis renal. Método: Se calculó el ICaCre de las concentraciones urinarias de calcio (mmol/l) y creatinina (µmol/l) en una alícuota de colección de 24 horas de orina en 169 niños y adolescentes atendidos por hematuria no glomerular (HNG) o litiasis renal (LR). La calciuria de 24 horas > 4.0 mg/kg en 24 horas se distribuyó según la presencia de HNG o LR. Resultados: El ICaCre promedio fue de 0.2 ± 0.1 mg/mg. La excreción urinaria de calcio estimada del ICaCre fue significativamente superior a la obtenida en colección de orina de 24 horas (p < 0.05). Los métodos de determinación de la calciuria concordaron en la frecuencia de hipercalciuria (ICaCre 39.5% vs. colección de 24 horas 32.1%; p > 0.05). Según la presencia de HNG o LR, la hipercalciuria se distribuyó de la siguiente manera: no HNG + no LR: 59%; no HNG + LR: 60% (diferencia: +1.0%); HNG + no LR: 68.2% (diferencia: +9.2%); HNG + LR: 73.3% (diferencia: +14.4%). Conclusiones: El ICaCre para estimar la excreción urinaria de calcio puede ser efectivo en el estudio de la hipercalciuria asociada con HNG y LR.

Abstract Background: Hypercalciuria might be revealed during the differential diagnosis of hematuria accompanying renal lithiasis (RL). In spite of this, diagnostic accuracy of calcium urinary excretion might be affected by incomplete 24-hour urine collections. In the present study, the diagnostic utility of calcium/creatinine (ICaCre) index for determining hypercalciuria associated with non-glomerular hematuria (NGH) and RL was assessed. Method: ICaCre (mg/mg) index was calculated from calcium (mmol/l) and creatinine (µmol/l) concentrations in an aliquot from a 24-hour urine collection in 169 children and adolescents with NGH or RL. Calciuria values > 4.0 mg/kg in 24 hours were distributed according to the presence of NGH or RL. Results: Mean ICaCre index was 0.2 ± 0.1 mg/mg. Calciuria values estimated from ICaCre were statistically higher to those from 24-hour urine collection (p < 0.05). The frequency of hypercalciuria was independent from the measurement method (estimated from ICaCre 39.5% vs. 24 h collection 32.1%; p > 0.05). Hypercalciuria distribution was as follows: no NGH + no RL: 59.0%; no NGH + RL: 60.0% (∆ = +1.0%); NGH + no RL: 68.2% (∆ = +9.2%); NGH + RL: 73.3% (∆ = +14.4%). Conclusions: The use of ICaCre index for determining calcium urine excretion might be effective in the study of hypercalciuria associated with NGH and RL.

Diagnóstico de infección por VIH por el hallazgo incidental de alteraciones ultraestructurales en una biopsia renal: Report of one case / Ultrastructural alterations in renal biopsy leading to the diagnosis of HIV infection

HIV infection has different clinical presentations. We report a 21-year-old male with longstanding isolated microscopic hematuria attributed to thin glomerular basement membrane disease, who after 15 years of follow-up presented with significant proteinuria. A kidney biopsy was performed, revealing the presence of tubulo-reticular inclusions in the glomerular endothelial cells. This finding led to suspect an HIV infection, which was verified. Antiretroviral therapy, angiotensin-converting enzyme and angiotensin II receptor blockers were prescribed. At 6 years of diagnosis the patient is asymptomatic and has normal kidney function. Microscopic hematuria and low level proteinuria persists.

Sarcoma renal fusocelular de alto grado con antecedente de carcinoma de células claras / Renal sarcoma with previous clear cell carcinoma

El carcinoma renal con diferenciación sarcomatoide es una entidad rara con muy pocos casos reportados en la literatura. Se presenta un caso de un paciente masculino de 65 años que por historia de hematuria macroscópica, se le realizan estudios diagnósticos, reportándose masa renal izquierda, efectuándos ele nefrectomía radical cuyo resultado de patología fue un carcinoma de células claras. Tres meses después de la cirugía el paciente refiere dolor localizado en el mismo sitio anatómico por lo que se le realiza tomografía, reportándose masa a nivel del retroperitoneo, debido a este hallazgo se decide efectuar tumorectomía, en la que se reporta sarcoma fusocelular de alto grado. Los resultados patológicos no corresponden a dos entidades patológicas diferentes, sino al mismo origen tumoral, con progresión de la enfermedad.

Sarcomatoid diferentaton in renal cell carcinoma is a rare disease with very few cases reported in the literature. A 65 year old man with hematuria anda lef renal mass underwent radical nephrectomy. Pathology reported a clear renal cell carcinoma. Three months afer surgery, the patent presentedwith abdominal complaints and a CT scan of the abdomen revealing fndings consistent with a retroperitoneal mass. The patent underwent a secondsurgical resecton and pathology revealed a high grade spindle cell sarcoma. The two diferent pathology reports are not two diferent pathologies; sarcomatoid diferentaton in renal cell carcinoma represents a common pathway of de-diferentaton of renal tumors.

Fenómeno y síndrome de cascanueces asociado a hematuria y proteinuria ortostática / Nutcracker phenomenon and syndrome associated to hematuria and orthostatic proteinuria

El fenómeno cascanueces es una compresión de la vena renal izquierda, lo más frecuente es el ángulo formado por la arteria aorta y la mesentérica superior, por una emergencia anormal de la mesentérica. Cuando aparecen síntomas derivados de esta anomalía se le denomina síndrome de cascanueces. Este síndrome puede producir síntomas y signos muy variados, pero entre ellos, la hematuria, la proteinuria ortostática, el varicocele, la congestión pélvica crónica, el dolor abdominal y en flanco, y la intolerancia ortostática son los más frecuentes. La hematuria y la proteinuria ortostática son 2 manifestaciones que frecuentemente tienen que enfrentar el médico general integral y el pediatra, y es necesario tener en cuenta al síndrome de cascanueces en el diagnóstico diferencial de estas alteraciones. La hematuria es muy frecuente y la proteinuria ortostática tiene como causa principal el síndrome cascanueces. Por tal motivo consideramos importante esta breve revisión del tema, para poder enfrentar estas situaciones teniendo en cuenta todas sus posibilidades diagnósticas

Nutcracker phenomenon is left renal vein compression, more frequently in the angle formed by the aorta artery and the superior mesenteric artery due to abnormal emergency of the mesentery. When symptoms derived from this anomaly occur, this situation is called nutcracker syndrome. It may cause very varied symptoms and signs such as hematuria, orthostatic proteinuria, varicocele, chronic pelvic congestion, abdominal pain and flank pain, and orthostatic intolerance as the most common ones. Hematuria and orthostatic proteinuria are two frequent manifestations that the family physician and the pediatrician must face, so it is necessary to take the nutcracker syndrome into account for the differential diagnosis of these alterations. Hematuria is more frequent and orthostatic proteinuria is mainly caused by the nutcracker syndrome. Therefore, we consider that this brief review on this topic is important to cope with these situations and to bear in mind all their diagnostic possibilities

Random Urinary Calcium/Creatinine Ratio for Screening Hypercalciuria in Children with Hematuria

BACKGROUND: Hypercalciuria is one of the most common causes of unexplained isolated hematuria. The diagnostic methods for hypercalciuria have not yet been standardized. The aim of this study was to assess whether random urinary calcium/creatinine ratio could be used as a screening tool for hypercalciuria in children with hematuria. METHODS: This prospective study included 264 children with primary hematuria for whom both random and 24 hr urinary evaluations were performed. Pearson correlation and ROC curve were used to assess the correlations. A multiple linear regression model was used to analyze effects of age, weight, height, body mass index, and body surface area on random urinary calcium/creatinine ratio. RESULTS: There was a moderately strong correlation between random urinary calcium/creatinine ratio and 24 hr urinary calcium excretion (r=0.584, P<0.001). The most appropriate cutoff value of random urinary calcium/creatinine ratio for the estimation of hypercalciuria was 0.075 mg/mg (sensitivity, 77.8%; specificity, 64.3%; area under the curve, 0.778). Body mass index and 24 hr urinary calcium excretion significantly affected random urinary calcium/creatinine ratio with a low coefficient of determination (r2=0.380, P<0.001). CONCLUSIONS: Random urinary calcium/creatinine ratio is not suitable for screening hypercalciuria in children with hematuria. Twenty-four hour urinary analysis should be performed to diagnose hypercalciuria in children with hematuria.

Renal Manifestations in 2007 Korean Patients with Behcet's Disease

PURPOSE: Behcet's disease (BD) theoretically affects all sizes and types of blood vessels and results in multi-organ involvement. However, renal BD has not been fully characterized, though the kidneys are histologically rich in blood vessels. MATERIALS AND METHODS: A total of 2007 patients who fulfilled the diagnostic criteria for BD were enrolled in this study. We reviewed the medical records and test results of the BD patients and used univariate and multivariate logistic regression analyses to determine the clinical significance of renal involvement in BD. RESULTS: Among the 2007 BD patients, we noted hematuria in 412 (20.5%) and proteinuria in 29 (1.4%). Univariate analysis showed that the BD patients with hematuria were predominantly female and older, had higher erythrocyte sedimentation rates (ESRs), and more frequently presented with genital ulcerations. BD patients with proteinuria had higher ESR levels compared to BD patients without proteinuria. In the multivariate analysis, age, sex, and ESR were found to be significantly associated with hematuria in BD patients, whereas only ESR was associated with proteinuria in BD patients. We also found that IgA nephropathy was the most common pathologic diagnosis in 12 renal BD patients who underwent renal biopsies. CONCLUSION: We suggest that routine urinalysis and serum renal function tests be performed for the early detection of renal BD, especially in older female BD patients with recurrent hematuria, high ESR levels, and frequent genital ulcers, as well as in BD patients with proteinuria and high ESR levels.

Hipercalciuria idiopática en el niño / Idiopathic hypercalciuria in children

Con el propósito de facilitar la orientación y el estudio de los pacientes con hematuria macroscópica recurrente o microscópica persistente, dolor abdominal recurrente, disuria, infección urinaria recurrente o litiasis nefrourológica en las que puede estar involucrada la hipercalciuria idiopática, se hace esta presentación de forma sencilla, para que pueda ser empleada por el médico de atención primaria. La hipercalciuria idiopática es tan frecuente que afecta aproximadamente al 10 por ciento de la población y ante sus síntomas que pueden ser muy variados, hay que pensar en esta alteración metabólica hereditaria que es la causa más frecuente de litiasis renal cálcica. En ocasiones con una orientación adecuada y pocos o ningún medicamento se puede evitar que los pacientes desarrollen una litiasis nefrourológica o que la enfermedad litiásica progrese, y a la vez, impedir que estos pacientes, niños principalmente, sean sometidos a investigaciones cruentas...

In order to make possible the orientation and the study of patients presenting with recurrent macroscopic hematuria or persistent microscopic hematuria, recurrent abdominal pain, dysuria, recurrent urinary infection or nephrourologic lithiasis where a idiopathic hypercalciuria could be involved, we make this simple presentation that primary care physician could be used. Idiopathic hypercalciuria is so frequent that involves approximately to 10 percent`of population and in face of its symptoms that could be varied, we must to consider this hereditary metabolic alteration as the more frequent cause of calcium renal lithiasis. Some times with an appropriate orientation and a few or none drug it is possible to avoid that patient develop a nephrourologic lithiasis or that the lithiasis disease be progressive, and at the same time, to prevent that these patients, mainly the children underwent difficult researches...

Natural History and Renal Pathology in Patients with Isolated Microscopic Hematuria

BACKGROUND/AIMS: No definite conclusions have been reached about the natural history of patients with isolated microscopic hematuria (IMH). In this study, we observed the natural history of patients with IMH and examined factors related to a pathologic diagnosis and subsequent prognosis. METHODS: We retrospectively evaluated 156 subjects with IMH who had a renal biopsy performed. Of the 156 subjects, 33.3% were diagnosed with IgA nephropathy, 23.7% with mesangial proliferative glomerulonephritis, 15.4% with glomerular minor lesion, and 12.8% with thin basement membrane nephropathy; 6.4% had normal biopsies. RESULTS: We followed up with 100 subjects for about 31 months. During this follow-up period, two subjects who had received a pathologic diagnosis of IgA nephropathy developed chronic kidney disease. During the course of the study, one of these subjects presented with proteinuria and hypertension and the other with proteinuria. The overall incidences of proteinuria and hypertension were 6% and 5% respectively. CONCLUSIONS: The prognosis for patients with IMH was relatively favorable, but patients developing proteinuria and/or hypertension require careful observation and management during the follow-up period.

Urogenital involvement in the Klippel-Trenaunay-Weber syndrome: treatment options and results

OBJECTIVE: Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital condition characterized by vascular malformations of the capillary, venous and lymphatic systems associated to soft tissue and bone hypertrophy in the affected areas. This syndrome may involve bladder, kidney, urethra, ureter and genitals. We report the treatment of 7 KTWS patients with urogenital involvement. MATERIALS AND METHODS: From 1995 to 2005, 7 patients with KTWS were evaluated and the charts of these patients were reviewed. RESULTS: PatientsÆ median age was 19-years (range 4 to 46-years) and only 1 was female. The clinical presentation included genital deformities in 3 cases, hematuria in 2 and urethrorragia in 2, one of which associated with cryptorchidism and phimosis. Three patients had an association of pelvic and genital malformations, including 2 patients with hematuria due to vesical lesions and 1 patient with left ureterohydronephrosis due to a pelvic mass. Two patients had urethral lesions. Treatment included endoscopic laser coagulation for 1 patient with recurrent hematuria and 1 patient with urethrorrhagia, pelvic radiotherapy for 1 patient with hematuria and circumcision in 2 patients with genital deformities. One patient required placement of a double-J catheter to relieve obstruction. Hematuria and urethrorragia were safely and effectively controlled with laser applications. Circumcision was also effective. The patient treated with radiotherapy developed a contracted bladder and required a continent urinary diversion. CONCLUSIONS: Urogenital involvement in patients with KTWS is not rare and must be suspected in the presence of hematuria or significant cutaneous deformity of the external genitalia. Surgical treatment may be warranted in selected cases.

Evaluación del reimplante ureteral extravesical según la técnica de Lich-Gregoir en el tratamiento del reflujo vesico renal primario / Evaluation of extravesical ureteral reimplant using the Lich-Gregoir in the treatment of primary renal bladder reflux

Entre los años 1995 y 2003, fueron reimplantados un total de 490 unidades renales por técnica extravesical según Lich-Gregoir. Todos los casos correspondieron a pacientes pediátricos de ambos sexos portadores de reflujo vesico renal primario. Del total de unidades renales tratadas. 2 por ciento experimentaron persistencia del reflujo. El éxito global en el tratamiento de reflujo se logró en 480 unidades renales (98 por ciento). El porcentaje de hematuria macroscópica (4.08 por ciento) y disuria (2.71 por ciento) fue significativamente menor que el observado en las técnicas transvesicales (100 por ciento). El tiempo de estancia hospitalaria fue menos de 24 horas en 58 por ciento de los casos; entre 24 y 48 horas en 26 por ciento de los mismos, y sólo 16 por ciento de los infantes necesitaron 2 o mas días de hospitalización. Considerando los resultados, recomendamos la utilización de la técnica extravesical de Lich-Gregoir para el tratamiento del reflujo vesico renal primario de II, III y IV grado ya que ofrece menor morbilidad que las técnicas transvesicales.

Xantinúria, nefrolitíase e insuficiência renal, relato de um caso / Xanthinuria, nephrolithiasis and kidney failure, a case report

Xantinúria é uma doença metabólica rara, caracterizada bioquimicamente por baixa produçäo de ácido úrico e acúmulo de xantina e hipoxantina no sangue. Como conseqüência, há uma quase ausência de ácido úrico na urina e elevada depuraçäo renal de oxipurinas. A deficiência isolada da atividade de enzima xantina oxidase é uma das causas possíveis. Cerca de 0,03 por cento dos cálculos urinários säo compostos por xantina, seja pura ou em associaçäo com ácido úrico, fosfato ou oxalato de cálcio. Descrevemos o caso de um paciente de 11 anos de idade, com insuficiência renal que formou vários cálculos urinários nos úlitmos quatro anos. Neste período o paciente apresentou infecçäo urinária recorrente e hematúria persistente. Näo havia história familiar de doença metabólica e nenhum outro membro da família havia formado cálculos renais. Uma informaçäo relevante é que os pais e os avós säo primos em primeiro grau. Os cálculos anteriormente retirados näo foram analisados. Há dois anos o paciente foi submetido a transplante renal, com boa evoluçäo clínica. Há 3 meses apresentou novos episódios de infecçäo urinária e hematúria macroscópica. Um ultrassom evidenciou atrofia bilateral dos rins remanescentes e um cálculo na bexiga, que foi retirado cirurgicamente. O cálculo analisado era de cor marrom, superfície lisa, forma oval e laminado no seu interior medindo 12 X 12 X 8 mm nos maiores diâmetros. As reaçöes para cálcio, oxalato, fosfato, magnésio, cistina, ácido úrico e amônia foram negativas. O teste para oxipurinas foi positivo.

Hemangioma vaginal en la infancia: presentación de caso clínico / Vaginal hemangioma in childhood: presentation of clinical case

La genitorragia en la infancia puede llegar a ser un problema diagnóstico si no se enfrenta apropiadamente. El objetivo de esta comunicación es presentar un caso clínico de una paciente con genitorragia a repetición cuyo diagnóstico final fue un hemangioma vaginal

Histopathological and immunological studies in a cohort of balanitis xerotica obliterans.

Balanitis xerotica obliterans consisting of 52 cases had been studied in the present series. This disease entity is an insidious sclerosing disease of unknown aetiology, affecting mainly the skin and mucous membrane of glans, prepuce and sometimes the fossa navicularis urethrae or even terminal urethra. Atrophic white patches on external genitalia and obstructive uropathy are two common presenting features. It has been classified as local form of lichen sclerosus et atrophicus. Poor local hygiene, circumcision and association of auto-immune diseases like vitiligo are responsible as suggested in this study. Routine tests detected associated cystitis in 8 cases, haematuria in 4 cases, non-reactive VDRL in all cases and impaired glucose tolerance in 5 cases. Immunoglobulin profile (39 cases) showed altered pattern, suggesting some chronic antigenic stimulation. Out of 33 cases where biopsy done, histologically proved typical balanitis xerotica obliterans was found in 19 cases. Malignant change was noted in one case only. Circumcision and dilatation offered temporary relief.

Factores pronóstico en la mortalidad por lupus eritematoso sistémico

Se trata de un estudio retrospectivo de casos y controles, donde se comparó un grupo de 25 pacientes con lupus eritematoso sistémico (LES) quienes murieron durante la hospitalización, con una muestra escogida al azar de 30 pacientes también con LES, de un total de 90 pacientes que estaban vivos en el momento de este análisis. Las muestras fueron comparadas en relación con algunas características (factores de riesgo), cuyas diferencias se relacionaban con la mortalidad. Los factores de riesgo asociados positivamente con la mortalidad fueron: edad menor de 18 años, compromiso renal manifestado por depuración de creatinina menor de 30 ml/min y hematuria masiva, vasculitis, compromiso cutáneo, compromiso del sistema nervioso central, leucopenia menor de 4.000 leucocitos/mm al cubo, presencia de IgA en la banda lúpica, aunque para definir este último factor de riesgo se necesitan mayores estudios. También fue posible demostrar el efecto sinérgico entre los factores de riesgo mencionados