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Prof Oreste Battisti, Faculté de médecine, néphrologie pédiatrique, 3° cycle, édition 2009
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Néphrologie pédiatrique
à l’usage du
3° cycle ou
des masters complémentaires
Prof Oreste Battisti
ULG Faculté de médecine

Prof Oreste Battisti, Faculté de médecine, néphrologie pédiatrique, 3° cycle, édition 2009
Prof Oreste Battisti, néphrologie pédiatrique, - 2 -
Table du contenu
- évaluation de la fonction rénale
whats’new in pediatric nephrology and hypertension?
Le bilan renal et les valeurs normales
Introduction to renal function
glomerular anatomy and function
Water balance and regulation of plasma osmolality
- Maintenance fluid therapy in children, in newborns and clinical assessment of hypovolemia
- Les néphropathies héréditaires
- Evaluation de l’hématurie
- Nephrolithiase
- Diagnostic des malformations et des obstructions des voies urinaires
- L’insuffisance rénale aiguë
Acute renal failure in the newborn
Clinical presentation, evaluation and diagnosis of acute renal failure in children
dialyse péritonéale
Clinical manifestations and diagnosis of Henoch-Schönlein purpura
- Syndrome néphritique aigu
- Syndromes néphrotiques de l'enfant
- La biopsie rénale
- L’obstruction des voies urinaires
- L’infection urinaire
- Préhypertension et hypertension artérielle ; la décompensation cardiaque
- Vitamine et ostéodystrophie
- Reins polykystiques
- Remplacement rénal

Prof Oreste Battisti, Faculté de médecine, néphrologie pédiatrique, 3° cycle, édition 2009
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Evaluation de « la fonction rénale »
Introduction
Le rein possède plusieurs fonctions :
- Co-gestion de l’équilibre hydro-électrolytique ;pour cette raison, certains aspects de la
gestion des fluides et électrolytes seront traités ici.
- Co-gestion de la tension artérielle ; pour cette raison, l’abord de l’hypertension
artérielle sera traité ici.
- Co-gestion de l’équilibre acido-basique ;
- Co-gestion de l’équilibre phospho-calcique ; pour cette raison, le métabolisme de la
vitamine D sera traité ici.
- Co-gestion de l’hémopoièse ;
- Co-gestion de la croissance.
Il est aussi la cible de malformations ou d’autres états malades.
Il intervient aussi dans la gestion et l’excrétion de médicaments.

Prof Oreste Battisti, Faculté de médecine, néphrologie pédiatrique, 3° cycle, édition 2009
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What's new in nephrology and hypertension
GLOMERULAR DISEASE AND VASCULITIS
In the largest series of patients with idiopathic membranous nephropathy treated with rituximab, 10 of 50
consecutive patients achieved remission of proteinuria to less than 500 mg/day at one year [1].
Among patients with IgA nephropathy and baseline proteinuria greater than 1 g/day, a prospective controlled
study showed that tonsillectomy plus steroid therapy increased the rate of remission of proteinuria and
hematuria compared to steroid therapy alone [2].
Forty patients with lupus nephritis with concurrent diffuse proliferative and membranous lesions were
randomly assigned to induction therapy with either intravenous cyclophosphamide or mycophenolate,
tacrolimus and corticosteroids [3]. At six and nine months, there was a higher rate of complete remission
(defined as proteinuria <0.4 g/day, normal urinary sediment and normal creatinine) in patients treated with
combination therapy (50 and 65 percent) compared to those who received cyclophosphamide (5 and 15
percent).
HYPERTENSION
A secondary analysis of the ONTARGET trial in patients with known atherosclerotic vascular disease or with
diabetes and end organ damage revealed that, compared to ramipril alone, combined therapy with ramipril and
telmisartan was associated with a significantly higher rate of a composite outcome of dialysis, doubling of the
serum creatinine, and death at 4.5 years [4].
ACUTE AND CHRONIC KIDNEY DISEASE
Among predialysis patients with chronic kidney disease, a retrospective study found that the use of oral
calcitriol may be associated with improved survival [5].
A study of over 1700 patients found that even moderate chronic kidney disease (estimated GFR between 30
and 59 mL/min per 1.73 m2) is a major risk factor for the development of acute kidney injury during
hospitalization [6].
Secondary analysis of the CHOIR trial has found that high doses of erythropoietin, rather than a higher target
hemoglobin level, is associated with an increased risk of adverse events [7]. To achieve target hemoglobin
levels, we suggest that the dose of epoetin-alpha NOT exceed 20,000 units per week in predialysis patients
with chronic kidney disease.
Among 212 outpatients with estimated glomerular filtration rate between 45 and 60 mL/min per 1.73 m2,
fewer than one percent had an increase in serum creatinine greater than 0.5 mg/dL within 48 to 96 hours
following intravenous contrast administration for a nonemergent CT scan [8].

Prof Oreste Battisti, Faculté de médecine, néphrologie pédiatrique, 3° cycle, édition 2009
Prof Oreste Battisti, néphrologie pédiatrique, - 5 -
DIALYSIS
Fibroblast growth factor 23 (FGF-23), an osteoblastic hormone involved in the regulation of phosphate and
vitamin D, stimulates the renal excretion of phosphate and inhibits the synthesis of 1,25-dihydroxyvitamin D.
Among patients initiating hemodialysis, a nested case control study found that increased FGF-23 levels are
significantly associated with increased mortality [9]. Further study is required to better characterize the role of
FGF-23 as a marker of increased mortality in this setting.
In a small randomized study of patients with end-stage renal disease, survival was found to be superior with
hemofiltration compared with hemodialysis [10]. However, given the small number of patients in this trial, any
possible survival benefit with hemofiltration must be confirmed in larger studies.
TRANSPLANTATION
The addition of rituximab to a high dose intravenous immune globulin (IVIG) regimen was safe and effective
in lowering panel reactive antibody levels and increasing kidney transplantation rates among highly sensitized
patients [11].
An observational study found that sirolimus is associated with impaired spermatogenesis, resulting in a
decreased fathered pregnancy rate [12]. Men who desire to father children should be informed of the risks and
benefits associated with exposure to sirolimus.
GENETIC DISEASES
Gene polymorphisms that involve the MYH9 gene, which encodes the podocyte protein, nonmyosin heavy
chain IIA, are highly associated with nondiabetic end-stage renal disease and both HIV- and non-HIV-related
focal and segmental glomerulosclerosis (FGS) [13,14]. The MYH9 alleles that confer risk are more commonly
present in African Americans (60 percent of alleles) compared to European Americans (4 percent of alleles)
and may explain both the increased risk of kidney failure and the heightened incidence of FGS among African
Americans.
NEPHROLITHIASIS
Some patients with idiopathic hypercalciuria have increased calcitriol levels, due possibly to a urinary
phosphate leak. In some cases, the mechanism for the urinary phosphate leak may be mutations in the sodium-
hydrogen exchanger regulator factor 1 (NHERF1), which interacts with both renal sodium phosphate
transporters to facilitate normal phosphate regulation. This was shown in a study of 94 patients with
nephrolithiasis or bone demineralization, of whom seven had mutations in NHERF1 [15]. All seven had low
maximal reabsorption of phosphate, hypophosphatemia, and increased calcitriol levels. Further study is
required to better clarify the increased risk of renal stone formation with abnormalities in NHERF1.
PEDIATRIC NEPHROLOGY
The American Heart Association (AHA) has published a scientific statement outlining the indications for 24-
hour ambulatory blood pressure monitoring (ABPM) and the necessary criteria for accurate and valid ABPM