Long-term renal prognosis of typical haemolytic-uraemic syndrome suffered in infancy
Pronóstico renal a largo plazo en síndrome hemolítico urémico típico padecido en la infancia
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We report a relevant case.</p><p class="elsevierStylePara">Our patient is a 33-year-old woman. When she was 9 months old, she suffered typical HUS that required acute peritoneal dialysis for 15 days, resulting in subsequent full recovery of renal function. She was discharged from consultations when she was 16 years of age. Since then, she has had no other relevant illness. Normotensive. She sought consultation due to progressive proteinuria with 5 years of evolution was determined by urine test strip. She is a woman of normal size, height 167cm, weight 67kg, blood pressure: 123/80. No oedema. The remainder was without findings. In complementary tests: CRP was 0.89mg/dl, creatinine clearance was 87ml/min, non-selective glomerular proteinuria was 292mg/dl (2.29g/d) urine analysis with absence of microhaematuria. Cholesterol was 252mg/dl. Total protein was 6.8g/d, albuminaemia was 4.0g/d. Complete blood count and angiotensin converting enzyme were normal. Immunology, thyroid profile and basic viral serology were negative. Renal ultrasound with kidneys showed a diameter of 97 and 95mm, with cortex of 13mm and 10mm and resistance index of 0.6 and 0.65. Immunoelectrophoresis of proteins in blood and urine and normal light chains. Computerised tomography of the chest, abdomen and pelvis without findings. Mantoux test negative. Renal biopsy was performed, revealing 7 glomeruli: 1 sclerotic, 4 normal and 2 with signs of focal segmental hyalinosis (Figure 1). Immunofluorescence: negative. Electron microscopy: not performed. Treatment was started with angiotensin converting enzyme inhibitors with reduction in proteinuria at 3 months to 1 g/d and glomerular filtration stability.</p><p class="elsevierStylePara">In childhood HUS, the number of affected capillaries determines long-term glomerular damage by developing glomerular hyperfiltration (GHF). Many articles<span class="elsevierStyleSup">1-5</span> recognise this fact. Garg et al.<span class="elsevierStyleSup">6</span> include 49 studies, 3476 patients with a mean follow-up period of 4.4 years (1 to 22), patients from 1 month to 18 years old. It shows a combined incidence of mortality/end-stage renal failure (ESRF) of 12%. 64% displayed renal complications (defined as high blood pressure [HBP], a decrease in the glomerular filtration rate [GFR] <80ml/min and/or significant proteinuria), with a combined incidence of 25%. 15% had proteinuria, 10% HBP and there was a 15.8% drop in GFR. The long-term prognosis was worse in those who suffered cortical necrosis and who required renal replacement therapy for longer than 8 days. Between 8% and 61% of those who had fully recovered renal function suffered renal complications that even began 20 years later.</p><p class="elsevierStylePara">Moghal et al.<span class="elsevierStyleSup">7</span> biopsied 7 normotensive patients with complete recovery of renal function and late damage, with the following result: overall glomerulosclerosis (85.7%), segmental sclerosis lesions (28.6%), tubular atrophy (14%) and overall glomerulomegaly with intimal thickening of small vessels (100%).</p><p class="elsevierStylePara">Caletti et al.<span class="elsevierStyleSup">8</span> biopsied 30 children with renal complications with 11.2 years evolution. They found 56.6% of focal segmental glomerulosclerosis with hyalinosis, 30% of diffuse mesangial proliferative glomerulonephritis (GN), 6.6% of GN with minimal changes and 6.6% of diffuse glomerulosclerosis. The findings were interpreted as mesangial GN followed by focal segmental glomerulosclerosis with hyalinosis culminating in diffuse glomerulosclerosis. After follow-up, only 25% of the latter had a normal GFR.</p><p class="elsevierStylePara">Tönshoff et al.<span class="elsevierStyleSup">9</span> studied 89 patients after 16 years. 10.4% progressed to advanced chronic renal failure (CRF) and 3% to ESRF.</p><p class="elsevierStylePara">Gagnadoux<span class="elsevierStyleSup">4</span> followed up 29 patients for 15-28 years. 41.4% had sequelae, 10.3% progressed to CRF and 13.8% to TRF between 16 and 24 years after it started. 6.9% had normal GF at 10 years and developed CRF after this time.</p><p class="elsevierStylePara">Kelles et al.<span class="elsevierStyleSup">5</span> followed up 95 patients for 10 years and found that 65% did not suffer sequelae, 26% had mild renal disorder and 9% had progressed to severe CKD.</p><p class="elsevierStylePara">In all series, the possibility of progression was greater if they suffered mesangial GN.</p><p class="elsevierStylePara">These data coincide with the possibility of developing late renal failure after suffering pre-eclampsia in pregnancy.<span class="elsevierStyleSup">10</span> In both cases, a noxa, self-limiting in time determines functional renal sequelae in the very long-term.</p><p class="elsevierStylePara">In our case, the histology ruled out the possibility of primary glomerulopathy. In spite of the short follow-up time, we found a significant decrease in proteinuria, giving the patient a good renal function prognosis.</p><p class="elsevierStylePara">This case illustrates that a typical epidemic HUS in children may cause renal failure, HBP and proteinuria. These complications may even begin 20 years after the illness has been considered cured. As such, we indicate long-term follow-up of these patients. Treatment should be aimed at controlling risk factors that accentuate GHF (obesity, HBP) and specifically inhibition of the renin-angiotensin-aldosterone axis.</p><p class="elsevierStylePara"> </p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">The authors declare that they have no conflicts of interest related to the contents of this article.</p><p class="elsevierStylePara"><a href="grande/11751_16025_46859_en_11751_f1.jpg" class="elsevierStyleCrossRefs"><img src="11751_16025_46859_en_11751_f1.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 1. Kidney biopsy</p>" "pdfFichero" => "P1-E550-S4088-A11751-EN.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "11751_16025_46859_en_11751_f1.jpg" "Alto" => 497 "Ancho" => 654 "Tamanyo" => 206040 ] ] "descripcion" => array:1 [ "en" => "Kidney biopsy" ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Milford DV, White RH, Taylor CM. Prognostic significance of proteinuria one year after onset of diarrhea-associated hemolytic-uremic syndrome. 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| 2020 January | 54 | 27 | 81 |
| 2019 December | 57 | 19 | 76 |
| 2019 November | 45 | 9 | 54 |
| 2019 October | 30 | 8 | 38 |
| 2019 September | 42 | 20 | 62 |
| 2019 August | 31 | 12 | 43 |
| 2019 July | 42 | 22 | 64 |
| 2019 June | 27 | 10 | 37 |
| 2019 May | 19 | 13 | 32 |
| 2019 April | 36 | 33 | 69 |
| 2019 March | 33 | 21 | 54 |
| 2019 February | 23 | 18 | 41 |
| 2019 January | 29 | 12 | 41 |
| 2018 December | 64 | 37 | 101 |
| 2018 November | 70 | 11 | 81 |
| 2018 October | 68 | 15 | 83 |
| 2018 September | 51 | 11 | 62 |
| 2018 August | 65 | 26 | 91 |
| 2018 July | 44 | 11 | 55 |
| 2018 June | 51 | 11 | 62 |
| 2018 May | 48 | 9 | 57 |
| 2018 April | 57 | 4 | 61 |
| 2018 March | 52 | 12 | 64 |
| 2018 February | 47 | 8 | 55 |
| 2018 January | 37 | 2 | 39 |
| 2017 December | 52 | 9 | 61 |
| 2017 November | 43 | 14 | 57 |
| 2017 October | 36 | 8 | 44 |
| 2017 September | 42 | 13 | 55 |
| 2017 August | 44 | 9 | 53 |
| 2017 July | 30 | 16 | 46 |
| 2017 June | 40 | 5 | 45 |
| 2017 May | 35 | 12 | 47 |
| 2017 April | 27 | 10 | 37 |
| 2017 March | 22 | 12 | 34 |
| 2017 February | 21 | 33 | 54 |
| 2017 January | 22 | 14 | 36 |
| 2016 December | 43 | 11 | 54 |
| 2016 November | 57 | 19 | 76 |
| 2016 October | 90 | 9 | 99 |
| 2016 September | 109 | 3 | 112 |
| 2016 August | 181 | 12 | 193 |
| 2016 July | 208 | 10 | 218 |
| 2016 June | 132 | 0 | 132 |
| 2016 May | 124 | 0 | 124 |
| 2016 April | 97 | 0 | 97 |
| 2016 March | 80 | 0 | 80 |
| 2016 February | 117 | 0 | 117 |
| 2016 January | 103 | 0 | 103 |
| 2015 December | 124 | 0 | 124 |
| 2015 November | 79 | 0 | 79 |
| 2015 October | 76 | 0 | 76 |
| 2015 September | 67 | 0 | 67 |
| 2015 August | 60 | 0 | 60 |
| 2015 July | 65 | 0 | 65 |
| 2015 June | 39 | 0 | 39 |
| 2015 May | 63 | 0 | 63 |
| 2015 April | 10 | 0 | 10 |
Show all
