T. Schurmans - Academia.edu (original) (raw)
Papers by T. Schurmans
Revue Médicale de Bruxelles, 2008
Le departement pediatrique d’uro-nephrologie, dialyse et transplantation a ete cree en 1976 sur l... more Le departement pediatrique d’uro-nephrologie, dialyse et transplantation a ete cree en 1976 sur le campus Brugmann. Les differents secteurs developpes concernent l’hemodialyse, la dialyse peritoneale, la transplantation renale, la chirurgie urologique et genitale, le depistage antenatal et la prise en charge medico-chirurgicale des malformations congenitales du rein et du tractus urinaire, le traitement des troubles mictionnels et des vessies neurogenes, et le traitement des pathologies tubulaires et glomerulaires. Les progres dans les domaines de la genetique, l’imagerie medicale, l’obstetrique, la neonatologie et la chirurgie nous ont permis de constituer des equipes multidisciplinaires qui traitent les enfants malades renaux de maniere concertee. Les contributions cliniques et scientifiques les plus originales ont porte sur la greffe hepato-renale dans l’hyperoxalurie primaire, sur la determination de l’histoire naturelle des malformations congenitales du rein et de l’appareil ur...
Pediatric Nephrology, 2001
Renal dysplasia (RD) is a common cause of chronic renal failure (CRF) in children. The evolution ... more Renal dysplasia (RD) is a common cause of chronic renal failure (CRF) in children. The evolution towards end-stage renal failure is unpredictable due to the paucity of early prognostic factors. In order to identify early prognostic clinical criteria, we have retrospectively analyzed renal function and growth in 11 infants with RD and CRF from birth up to 4 years of age. Children with obstructive RD were not included. Glomerular filtration rate (GFR) was estimated from Schwartz formula. In infants with a GFR below 15 ml/min per 1.73 m2 at 6 months of age (group A, n=5), kidney function did not further improve; 4 reached end-stage renal failure between 8 months and 6 years of age. In contrast, infants with a GFR above 15 ml/min per 1.73 m2 at 6 months of age (group B, n=6) experienced a significant improvement in renal function during follow-up, and none required renal replacement therapy. During the first 3 months of life all infants with RD and CRF developed severe growth retardation. Between 6 months and 4 years of age, children from group B grew significantly better than those from group A. In conclusion, our experience suggests that GFR, estimated from Schwartz formula at 6 months of age, is a useful prognostic factor in infants with RD and CRF. Infants with a GFR below 15 ml/min per 1.73 m2 are at risk of severe growth delay and the need for early renal replacement therapy, whereas those with a GFR above 15 ml/min per 1.73 m2 have a relatively favorable long-term prognosis.
Nephrology Dialysis Transplantation, 1995
Brief Reports should be submitted online to www.editorialmanager.com/ amsurg. (See details online... more Brief Reports should be submitted online to www.editorialmanager.com/ amsurg. (See details online under ''Instructions for Authors''.) They should be no more than 4 double-spaced pages with no Abstract or sub-headings, with a maximum of four (4) references. If figures are included, they should be limited to two (2). The cost of printing color figures is the responsibility of the author. In general, authors of case reports should use the Brief Report format.
Kidney International, 1996
BJU International, 2006
endogenous vasopressin production and thereby ensure that any antidiuresis could be attributed to... more endogenous vasopressin production and thereby ensure that any antidiuresis could be attributed to treatment. Dosing with desmopressin or placebo occurred when urinary production was > 0.13 mL/min/kg. Urinary volume, osmolality and duration of urinary-concentrating action (above three threshold levels: 125, 200 and 400 mOsm/kg) were determined as endpoints. RESULTS All 72 participants receiving desmopressin had a pharmacodynamic response to the drug, while there was no change in urinary output in the 12 placebo-treated patients. There was a clear relationship between desmopressin dose and duration of action and osmolality during action, although the three highest-dose groups had similar results. The mean duration of action of desmopressin at the lowest osmolality threshold level was 3.6-10.6 h, according to dose; for the highest threshold, the values were 1.3-8.6 h. CONCLUSION Desmopressin, as the oral lyophilisate, causes a marked decrease in urinary output in hydrated children with PNE. A small dose range (120-240 µ g) is likely to control diuresis for a period corresponding to a night's sleep (7-11 h) in most children with PNE. However, some patients might require a higher dose to obtain antidiuresis for the complete night.
Archives de Pédiatrie, 1996
Archives de Pédiatrie, 1998
European Journal of Pediatrics, 1999
Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disorder characterised by an inc... more Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disorder characterised by an increased urinary excretion of calcium oxalate, leading to recurrent urolithiasis, nephrocalcinosis and accumulation of insoluble oxalate throughout the body (oxalosis) when the glomerular ®ltration rate falls to below 40±20 mL/min per 1.73 m 2. The disease is due to a functional defect of the liver-speci®c peroxisomal enzyme alanine: glyoxylate aminotransferase (AGT), the gene of which is located on chromosome 2q37.3. The diagnosis is based on increased urinary oxalate and glycollate, increased plasma oxalate and AGT measurement in a liver biopsy. AGT mistargeting may be investigated by immuno-electron microscopy and DNA analysis. End-stage renal failure is reached by the age of 15 years in 50% of PH1 patients and the overall death rate approximates 30%. The conservative treatment includes high¯uid intake, pyridoxine and crystallisation inhibitors. Since the kidney is the main target of the disease, isolated kidney transplantation (Tx) has been proposed in association with vigorous peri-operative haemodialysis in an attempt to clear plasma oxalate at the time of Tx. However, because of a 100% recurrence rate, the average 3-year graft survival is 15%±25% in Europe, with a 5±10-year patient survival rate ranging from 10% to 50%. Since the liver is the only organ responsible for the detoxi®cation of glyoxylate by AGT, de®cient host liver removal is the ®rst rationale for enzyme replacement therapy. Subsequent orthotopic liver Tx aims to supply the missing enzyme in its normal cellular and subcellular location and thus can be regarded as a form of gene therapy. Because of the usual spectrum of the disease, isolated liver Tx is limited to selected patients prior to having reached an advanced stage of chronic renal failure. Combined liver-kidney Tx has therefore become a conventional treatment for most PH1 patients: according to the European experience, patient survival approximates 80% at 5 years and 70% at 10 years. In addition, the renal function of survivors remains stable over time, between 40 and 60 mL/min per 1.73 m 2 after 5 to 10 years. In addition, liver Tx may allow the reversal of systemic storage disease (i.e. bone, heart, vessels, nerves) and provide valuable quality of life. Whatever the transplant strategy, the outcome is improved when patients are transplanted early in order to limit systemic oxalosis. According to the European experience, it appears that combined liver-kidney Tx is performed in PH1 patients with encouraging results, renal Tx alone has little role in the treatment of this disease, and liver Tx reverses the underlying metabolic defect and its clinical consequences.
Archives de Pédiatrie, 1997
Compte rendu de reunion 1271 trait des signes de pancrratite chronique, sur laquelle se sont ajou... more Compte rendu de reunion 1271 trait des signes de pancrratite chronique, sur laquelle se sont ajoutEes quatre poussdes de pancrEatite aiguE au cours des 7 mois snivant, imposant la poursuite de la nutrition parentErale. Dans le cas 3, une hypoalbuminrmie (20 g/L) avec hypogammaglobufinrmie (1,8 g/L) et lymphopdnie (450lmm3), en l'absence de protEinufie, ont ErE les seuls symptrmes digestifs. Le diagnostic de jEjunite a EtE pose par le TOGD. Le renforcement du traitement par cyclophosphamide intraveineuse a permis la correction des anomalies biologiques. L'atteinte rEnale (respectivement GN proliferative endo-et extracapillaire, GN extramembraneuse, GN proliferative endocapillaire) ne s'est aggravde parallrlement au probl~me digestif que dans le cas 2, nEcessitant 2 mois d'hdmodialyse. Conclusion. Les atteintes digestives du LED sont rares. II s'agit essentiellement de pan-crEatites aiguEs, ou chroniques (exceptionnelles), d'ent~ropathies exsudatives, de sErites et/ou d'ascites aiguEs ou rarement chroniques, et d'h6patites. Elles peuvent survenir au cours d'une poussEe aiguE ou au conrs d'un LED apparemment ma]trisr, voire rEvEler la maladie. Elles rrpondent le plus souvent au traitement immunosuppresseur.
Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysacc... more Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysaccharide (LPS) and LPS-binding protein. Methods. We examined by flow cytometry the effect of in vitro and in vivo haemodialysis on cuprophane membrane and recombinant C5a on the expression of CD14 molecules at the surface of monocytes. Monocyte CD 14 expression was also studied during in vitro and in vivo haemodialysis on polyacrylonitrile AN69 membrane. Results. In vitro haemodialysis of whole blood from healthy volunteers on cuprophane membrane resulted within 30 min in upregulation of monocyte CD 14 expression. The reuse of the cuprophane membrane
Neurourology and Urodynamics, 2005
Hypothesis / aims of study Evidence-based treatment of primary nocturnal enuresis (PNE) is based ... more Hypothesis / aims of study Evidence-based treatment of primary nocturnal enuresis (PNE) is based on the threefold pathophysiology of the problem: high urinary output, high arousal status and eventual nocturnal overactive bladder. The administration of the antidiuretic drug desmopressin is an effective treatment for PNE, being particularly beneficial for children with relative nocturnal polyuria and normal functional bladder capacity. However, the dosage of desmopressin is not standardized, and pharmacokinetic (PK) data are extremely limited in children. To determine the pharmacodynamic (PD) properties of desmopressin in children with documented PNE, we have, therefore, performed a multicentre study using a new oral lyophilisate formulation of desmopressin. In addition, this study identified dosages that provide a duration of antidiuretic action corresponding to the length of night-time sleep.
Revue médicale de Bruxelles, 2008
The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, ... more The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, various sectors have been developed including: hemodialysis and peritoneal dialysis, kidney transplantation, urological and genital surgery, antenatal screening and rapid management of uronephropathies, treatment of voiding dysfunction and neurogenic bladder, management of tubular and glomerular diseases. The progress in genetics, medical imaging, obstetrics, neonatology and surgery has allowed us to take care of our young patients within a multidisciplinary framework. The most original contributions of the department are related to the performance of combined liver-kidney transplantation in primary hyperoxaluria, to the determination of the natural history of several congenital anomalies of the kidney and urinary tract, to the assessment of the role of genetic mutations on tubular and glomerular diseases, to the usefulness of radioisotopic tracers in the measurement of renal function in...
Diabetes & metabolism, 2006
Haemolytic-uraemic syndrome (HUS) is a rare cause of insulin-dependent diabetes mellitus during t... more Haemolytic-uraemic syndrome (HUS) is a rare cause of insulin-dependent diabetes mellitus during the acute stage. We previously reported the case of a 3-year-old girl having presented with typical HUS with diarrhea, microangiopathic anaemia, thrombocytopenia and acute renal failure (17 days of anuria). Transient hyperglycaemia (highest level: 513 mg/dl) was observed, requiring continuous intravenous insulin infusion for 9 days. Subcutaneous insulin injections were stopped after 24 days. Oral glucose tolerance test performed 4 months after normalization of blood glucose was normal. HLA DQ genotype (DQA1-DQB1.AZH/DQA3-DQB3.1) was not at risk for type 1 diabetes and there were no auto-antibodies (ICA and IAA). The 3-years follow-up was marked by persistent arterial hypertension, proteinuria and slight renal insufficiency despite angiotensin-converting enzyme inhibitor treatment. Ten years after HUS occurred (the patient had been lost to follow-up for 7 years), she came back with complai...
Revue médicale de Bruxelles
Advances in immunosuppressive therapy over the past decade have led to dramatic improvements of p... more Advances in immunosuppressive therapy over the past decade have led to dramatic improvements of patient and graft survival. The immunosuppression that is used is constantly evolving. The goal remains to find the best combination that will optimize long-term graft survival, while minimizing the adverse effects. It is likely that in the near future the results will even be improved further by the development of new medications with a better therapeutic index and the induction of transplant tolerance.
Disseminated intravascular coagulation (DIC) is a serious complication of meningococcal septicaem... more Disseminated intravascular coagulation (DIC) is a serious complication of meningococcal septicaemia. It often results in infarction of various tissues namely the skin, adrenal glands, kidneys, brain and, much less commonly, bones. We describe a patient who presented bone lesions after meningococcal septicaemia. In addition to plain radiography and scintigraphy the lesions were evaluated with MRI and have proved to be extensive and still progressive, approximately 18 months after the onset of the disease.
Pediatric Uroradiology, 2001
Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysacc... more Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysaccharide (LPS) and LPS-binding protein. Methods. We examined by flow cytometry the effect of in vitro and in vivo haemodialysis on cuprophane membrane and recombinant C5a on the expression of CD14 molecules at the surface of monocytes. Monocyte CD 14 expression was also studied during in vitro and in vivo haemodialysis on polyacrylonitrile AN69 membrane. Results. In vitro haemodialysis of whole blood from healthy volunteers on cuprophane membrane resulted within 30 min in upregulation of monocyte CD 14 expression. The reuse of the cuprophane membrane abolished both complement activation and CD 14 upre- gulation. Moreover, incubation of whole blood with recombinant C5a led to an increased monocyte CD 14 expression supporting a role for complement activation in the rapid cuprophane-induced CD14 upregulation. During AN69 dialysis which is not associated with complement activation in the blood...
Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysacc... more Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysaccharide (LPS) and LPS-binding protein. Methods. We examined by flow cytometry the effect of in vitro and in vivo haemodialysis on cuprophane membrane and recombinant C5a on the expression of CD14 molecules at the surface of monocytes. Monocyte CD 14 expression was also studied during in vitro and in vivo haemodialysis on polyacrylonitrile AN69 membrane. Results. In vitro haemodialysis of whole blood from healthy volunteers on cuprophane membrane resulted within 30 min in upregulation of monocyte CD 14 expression. The reuse of the cuprophane membrane abolished both complement activation and CD 14 upre- gulation. Moreover, incubation of whole blood with recombinant C5a led to an increased monocyte CD 14 expression supporting a role for complement activation in the rapid cuprophane-induced CD14 upregulation. During AN69 dialysis which is not associated with complement activation in the blood...
Diabetes Research and Clinical Practice, 1999
S25 increasing families' participation in its activities from 27.7% to 64.4% in 1998. We conclude... more S25 increasing families' participation in its activities from 27.7% to 64.4% in 1998. We conclude that pediatric diabetes care in our region is improving with time. There is still much to achieve in organising ambulatory care, building a complete diabetes team and supplying means of self-control. These will further improve diabetes control and reduce the relative share of acute and chronic diabetes complications.
Archives de Pédiatrie, 1996
Soci~t~ de n6phrotogie pediatrique 1157 de la pression intrap6riton6ale (PIP) et sur le test de p... more Soci~t~ de n6phrotogie pediatrique 1157 de la pression intrap6riton6ale (PIP) et sur le test de perm6abi-lit6 de la membrane p6riton6ale. La PIP est d6termin6e telle une pression veineus¢ c~.ntmle, pression hydrostatique en cmH~O, mesure ais6¢ tout particuli~:ren',ent avec le syst~me d6connectable double poehe et tubulure en Y type Baxter. La pression est fonction du volume de remplis.~age l~riton6a~ et de I'~ge. En cas de misc en route de la DP d~s t.-. page du catheter p6:;ton6ai, le suivi de la PIP pennet d'adapter le volume intra#ri*on6al r6duisam les risques de douleur et de fuite, Le test de perm6abilit6 p6riton6ale permet de d~finir la vitesse de d6saturatRm "lu dialysat cn glucose ct la vitesse de saturation du dialysat en toxines ur6miques par rapgort au plasma (D/P~. Lc point de croisement de la courtm de d~saturation en glucose et de saxuration en ur6e d6finit le, temps APEX ou temps d'ultrafiltration; un temps de contact p~us long r6duit la capacit6 d'ultrafiltration de la DP par perle du gradiant osmiotique d~pendant du glucose. Le temps de saturation du dialysat, par convention Dm ~2al ~ 60 % pour le phosphate, toxine ur~miqae ~ diffusion 'ente, d6finit un temps d'dpuration. La d~tennination de ees temps d'ultrafiltration et dMpuration (n = 142 mesures) durant ies 5 demi~res ann6es ehez 17 enfants en DP a pennis de montrer I'opposition entre le temps de contact efficace pour I'ultrafiltmtion: 39 4-23 (18 71 rain) et le temps n6eessaire/~ I'dpuration: 193 + 69 (105 238 rain). Ainsi, la d6tennination individuelle de ees param~:tres aklc au ehoix de la modalit6 de DP. Si I'ultafiltralion est I'objectif principal, des temps de contacts courts sont ipdispensables, imposant la dialyse p6riton6ale automatique par eycleur. Si I'@uration est prioritairc, des temps de conSarts longs sont souhaitables, r6alis6s on DPCA. Si I'ultrafiltration et I'~puration sont n6ce.~aires, la dialyse p~riton~ale fluetuante fTidal) peut ~tre propose.
Revue Médicale de Bruxelles, 2008
Le departement pediatrique d’uro-nephrologie, dialyse et transplantation a ete cree en 1976 sur l... more Le departement pediatrique d’uro-nephrologie, dialyse et transplantation a ete cree en 1976 sur le campus Brugmann. Les differents secteurs developpes concernent l’hemodialyse, la dialyse peritoneale, la transplantation renale, la chirurgie urologique et genitale, le depistage antenatal et la prise en charge medico-chirurgicale des malformations congenitales du rein et du tractus urinaire, le traitement des troubles mictionnels et des vessies neurogenes, et le traitement des pathologies tubulaires et glomerulaires. Les progres dans les domaines de la genetique, l’imagerie medicale, l’obstetrique, la neonatologie et la chirurgie nous ont permis de constituer des equipes multidisciplinaires qui traitent les enfants malades renaux de maniere concertee. Les contributions cliniques et scientifiques les plus originales ont porte sur la greffe hepato-renale dans l’hyperoxalurie primaire, sur la determination de l’histoire naturelle des malformations congenitales du rein et de l’appareil ur...
Pediatric Nephrology, 2001
Renal dysplasia (RD) is a common cause of chronic renal failure (CRF) in children. The evolution ... more Renal dysplasia (RD) is a common cause of chronic renal failure (CRF) in children. The evolution towards end-stage renal failure is unpredictable due to the paucity of early prognostic factors. In order to identify early prognostic clinical criteria, we have retrospectively analyzed renal function and growth in 11 infants with RD and CRF from birth up to 4 years of age. Children with obstructive RD were not included. Glomerular filtration rate (GFR) was estimated from Schwartz formula. In infants with a GFR below 15 ml/min per 1.73 m2 at 6 months of age (group A, n=5), kidney function did not further improve; 4 reached end-stage renal failure between 8 months and 6 years of age. In contrast, infants with a GFR above 15 ml/min per 1.73 m2 at 6 months of age (group B, n=6) experienced a significant improvement in renal function during follow-up, and none required renal replacement therapy. During the first 3 months of life all infants with RD and CRF developed severe growth retardation. Between 6 months and 4 years of age, children from group B grew significantly better than those from group A. In conclusion, our experience suggests that GFR, estimated from Schwartz formula at 6 months of age, is a useful prognostic factor in infants with RD and CRF. Infants with a GFR below 15 ml/min per 1.73 m2 are at risk of severe growth delay and the need for early renal replacement therapy, whereas those with a GFR above 15 ml/min per 1.73 m2 have a relatively favorable long-term prognosis.
Nephrology Dialysis Transplantation, 1995
Brief Reports should be submitted online to www.editorialmanager.com/ amsurg. (See details online... more Brief Reports should be submitted online to www.editorialmanager.com/ amsurg. (See details online under ''Instructions for Authors''.) They should be no more than 4 double-spaced pages with no Abstract or sub-headings, with a maximum of four (4) references. If figures are included, they should be limited to two (2). The cost of printing color figures is the responsibility of the author. In general, authors of case reports should use the Brief Report format.
Kidney International, 1996
BJU International, 2006
endogenous vasopressin production and thereby ensure that any antidiuresis could be attributed to... more endogenous vasopressin production and thereby ensure that any antidiuresis could be attributed to treatment. Dosing with desmopressin or placebo occurred when urinary production was > 0.13 mL/min/kg. Urinary volume, osmolality and duration of urinary-concentrating action (above three threshold levels: 125, 200 and 400 mOsm/kg) were determined as endpoints. RESULTS All 72 participants receiving desmopressin had a pharmacodynamic response to the drug, while there was no change in urinary output in the 12 placebo-treated patients. There was a clear relationship between desmopressin dose and duration of action and osmolality during action, although the three highest-dose groups had similar results. The mean duration of action of desmopressin at the lowest osmolality threshold level was 3.6-10.6 h, according to dose; for the highest threshold, the values were 1.3-8.6 h. CONCLUSION Desmopressin, as the oral lyophilisate, causes a marked decrease in urinary output in hydrated children with PNE. A small dose range (120-240 µ g) is likely to control diuresis for a period corresponding to a night's sleep (7-11 h) in most children with PNE. However, some patients might require a higher dose to obtain antidiuresis for the complete night.
Archives de Pédiatrie, 1996
Archives de Pédiatrie, 1998
European Journal of Pediatrics, 1999
Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disorder characterised by an inc... more Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disorder characterised by an increased urinary excretion of calcium oxalate, leading to recurrent urolithiasis, nephrocalcinosis and accumulation of insoluble oxalate throughout the body (oxalosis) when the glomerular ®ltration rate falls to below 40±20 mL/min per 1.73 m 2. The disease is due to a functional defect of the liver-speci®c peroxisomal enzyme alanine: glyoxylate aminotransferase (AGT), the gene of which is located on chromosome 2q37.3. The diagnosis is based on increased urinary oxalate and glycollate, increased plasma oxalate and AGT measurement in a liver biopsy. AGT mistargeting may be investigated by immuno-electron microscopy and DNA analysis. End-stage renal failure is reached by the age of 15 years in 50% of PH1 patients and the overall death rate approximates 30%. The conservative treatment includes high¯uid intake, pyridoxine and crystallisation inhibitors. Since the kidney is the main target of the disease, isolated kidney transplantation (Tx) has been proposed in association with vigorous peri-operative haemodialysis in an attempt to clear plasma oxalate at the time of Tx. However, because of a 100% recurrence rate, the average 3-year graft survival is 15%±25% in Europe, with a 5±10-year patient survival rate ranging from 10% to 50%. Since the liver is the only organ responsible for the detoxi®cation of glyoxylate by AGT, de®cient host liver removal is the ®rst rationale for enzyme replacement therapy. Subsequent orthotopic liver Tx aims to supply the missing enzyme in its normal cellular and subcellular location and thus can be regarded as a form of gene therapy. Because of the usual spectrum of the disease, isolated liver Tx is limited to selected patients prior to having reached an advanced stage of chronic renal failure. Combined liver-kidney Tx has therefore become a conventional treatment for most PH1 patients: according to the European experience, patient survival approximates 80% at 5 years and 70% at 10 years. In addition, the renal function of survivors remains stable over time, between 40 and 60 mL/min per 1.73 m 2 after 5 to 10 years. In addition, liver Tx may allow the reversal of systemic storage disease (i.e. bone, heart, vessels, nerves) and provide valuable quality of life. Whatever the transplant strategy, the outcome is improved when patients are transplanted early in order to limit systemic oxalosis. According to the European experience, it appears that combined liver-kidney Tx is performed in PH1 patients with encouraging results, renal Tx alone has little role in the treatment of this disease, and liver Tx reverses the underlying metabolic defect and its clinical consequences.
Archives de Pédiatrie, 1997
Compte rendu de reunion 1271 trait des signes de pancrratite chronique, sur laquelle se sont ajou... more Compte rendu de reunion 1271 trait des signes de pancrratite chronique, sur laquelle se sont ajoutEes quatre poussdes de pancrEatite aiguE au cours des 7 mois snivant, imposant la poursuite de la nutrition parentErale. Dans le cas 3, une hypoalbuminrmie (20 g/L) avec hypogammaglobufinrmie (1,8 g/L) et lymphopdnie (450lmm3), en l'absence de protEinufie, ont ErE les seuls symptrmes digestifs. Le diagnostic de jEjunite a EtE pose par le TOGD. Le renforcement du traitement par cyclophosphamide intraveineuse a permis la correction des anomalies biologiques. L'atteinte rEnale (respectivement GN proliferative endo-et extracapillaire, GN extramembraneuse, GN proliferative endocapillaire) ne s'est aggravde parallrlement au probl~me digestif que dans le cas 2, nEcessitant 2 mois d'hdmodialyse. Conclusion. Les atteintes digestives du LED sont rares. II s'agit essentiellement de pan-crEatites aiguEs, ou chroniques (exceptionnelles), d'ent~ropathies exsudatives, de sErites et/ou d'ascites aiguEs ou rarement chroniques, et d'h6patites. Elles peuvent survenir au cours d'une poussEe aiguE ou au conrs d'un LED apparemment ma]trisr, voire rEvEler la maladie. Elles rrpondent le plus souvent au traitement immunosuppresseur.
Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysacc... more Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysaccharide (LPS) and LPS-binding protein. Methods. We examined by flow cytometry the effect of in vitro and in vivo haemodialysis on cuprophane membrane and recombinant C5a on the expression of CD14 molecules at the surface of monocytes. Monocyte CD 14 expression was also studied during in vitro and in vivo haemodialysis on polyacrylonitrile AN69 membrane. Results. In vitro haemodialysis of whole blood from healthy volunteers on cuprophane membrane resulted within 30 min in upregulation of monocyte CD 14 expression. The reuse of the cuprophane membrane
Neurourology and Urodynamics, 2005
Hypothesis / aims of study Evidence-based treatment of primary nocturnal enuresis (PNE) is based ... more Hypothesis / aims of study Evidence-based treatment of primary nocturnal enuresis (PNE) is based on the threefold pathophysiology of the problem: high urinary output, high arousal status and eventual nocturnal overactive bladder. The administration of the antidiuretic drug desmopressin is an effective treatment for PNE, being particularly beneficial for children with relative nocturnal polyuria and normal functional bladder capacity. However, the dosage of desmopressin is not standardized, and pharmacokinetic (PK) data are extremely limited in children. To determine the pharmacodynamic (PD) properties of desmopressin in children with documented PNE, we have, therefore, performed a multicentre study using a new oral lyophilisate formulation of desmopressin. In addition, this study identified dosages that provide a duration of antidiuretic action corresponding to the length of night-time sleep.
Revue médicale de Bruxelles, 2008
The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, ... more The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, various sectors have been developed including: hemodialysis and peritoneal dialysis, kidney transplantation, urological and genital surgery, antenatal screening and rapid management of uronephropathies, treatment of voiding dysfunction and neurogenic bladder, management of tubular and glomerular diseases. The progress in genetics, medical imaging, obstetrics, neonatology and surgery has allowed us to take care of our young patients within a multidisciplinary framework. The most original contributions of the department are related to the performance of combined liver-kidney transplantation in primary hyperoxaluria, to the determination of the natural history of several congenital anomalies of the kidney and urinary tract, to the assessment of the role of genetic mutations on tubular and glomerular diseases, to the usefulness of radioisotopic tracers in the measurement of renal function in...
Diabetes & metabolism, 2006
Haemolytic-uraemic syndrome (HUS) is a rare cause of insulin-dependent diabetes mellitus during t... more Haemolytic-uraemic syndrome (HUS) is a rare cause of insulin-dependent diabetes mellitus during the acute stage. We previously reported the case of a 3-year-old girl having presented with typical HUS with diarrhea, microangiopathic anaemia, thrombocytopenia and acute renal failure (17 days of anuria). Transient hyperglycaemia (highest level: 513 mg/dl) was observed, requiring continuous intravenous insulin infusion for 9 days. Subcutaneous insulin injections were stopped after 24 days. Oral glucose tolerance test performed 4 months after normalization of blood glucose was normal. HLA DQ genotype (DQA1-DQB1.AZH/DQA3-DQB3.1) was not at risk for type 1 diabetes and there were no auto-antibodies (ICA and IAA). The 3-years follow-up was marked by persistent arterial hypertension, proteinuria and slight renal insufficiency despite angiotensin-converting enzyme inhibitor treatment. Ten years after HUS occurred (the patient had been lost to follow-up for 7 years), she came back with complai...
Revue médicale de Bruxelles
Advances in immunosuppressive therapy over the past decade have led to dramatic improvements of p... more Advances in immunosuppressive therapy over the past decade have led to dramatic improvements of patient and graft survival. The immunosuppression that is used is constantly evolving. The goal remains to find the best combination that will optimize long-term graft survival, while minimizing the adverse effects. It is likely that in the near future the results will even be improved further by the development of new medications with a better therapeutic index and the induction of transplant tolerance.
Disseminated intravascular coagulation (DIC) is a serious complication of meningococcal septicaem... more Disseminated intravascular coagulation (DIC) is a serious complication of meningococcal septicaemia. It often results in infarction of various tissues namely the skin, adrenal glands, kidneys, brain and, much less commonly, bones. We describe a patient who presented bone lesions after meningococcal septicaemia. In addition to plain radiography and scintigraphy the lesions were evaluated with MRI and have proved to be extensive and still progressive, approximately 18 months after the onset of the disease.
Pediatric Uroradiology, 2001
Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysacc... more Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysaccharide (LPS) and LPS-binding protein. Methods. We examined by flow cytometry the effect of in vitro and in vivo haemodialysis on cuprophane membrane and recombinant C5a on the expression of CD14 molecules at the surface of monocytes. Monocyte CD 14 expression was also studied during in vitro and in vivo haemodialysis on polyacrylonitrile AN69 membrane. Results. In vitro haemodialysis of whole blood from healthy volunteers on cuprophane membrane resulted within 30 min in upregulation of monocyte CD 14 expression. The reuse of the cuprophane membrane abolished both complement activation and CD 14 upre- gulation. Moreover, incubation of whole blood with recombinant C5a led to an increased monocyte CD 14 expression supporting a role for complement activation in the rapid cuprophane-induced CD14 upregulation. During AN69 dialysis which is not associated with complement activation in the blood...
Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysacc... more Background. The CD 14 molecule is a high-affinity receptor for the complex formed by lipopolysaccharide (LPS) and LPS-binding protein. Methods. We examined by flow cytometry the effect of in vitro and in vivo haemodialysis on cuprophane membrane and recombinant C5a on the expression of CD14 molecules at the surface of monocytes. Monocyte CD 14 expression was also studied during in vitro and in vivo haemodialysis on polyacrylonitrile AN69 membrane. Results. In vitro haemodialysis of whole blood from healthy volunteers on cuprophane membrane resulted within 30 min in upregulation of monocyte CD 14 expression. The reuse of the cuprophane membrane abolished both complement activation and CD 14 upre- gulation. Moreover, incubation of whole blood with recombinant C5a led to an increased monocyte CD 14 expression supporting a role for complement activation in the rapid cuprophane-induced CD14 upregulation. During AN69 dialysis which is not associated with complement activation in the blood...
Diabetes Research and Clinical Practice, 1999
S25 increasing families' participation in its activities from 27.7% to 64.4% in 1998. We conclude... more S25 increasing families' participation in its activities from 27.7% to 64.4% in 1998. We conclude that pediatric diabetes care in our region is improving with time. There is still much to achieve in organising ambulatory care, building a complete diabetes team and supplying means of self-control. These will further improve diabetes control and reduce the relative share of acute and chronic diabetes complications.
Archives de Pédiatrie, 1996
Soci~t~ de n6phrotogie pediatrique 1157 de la pression intrap6riton6ale (PIP) et sur le test de p... more Soci~t~ de n6phrotogie pediatrique 1157 de la pression intrap6riton6ale (PIP) et sur le test de perm6abi-lit6 de la membrane p6riton6ale. La PIP est d6termin6e telle une pression veineus¢ c~.ntmle, pression hydrostatique en cmH~O, mesure ais6¢ tout particuli~:ren',ent avec le syst~me d6connectable double poehe et tubulure en Y type Baxter. La pression est fonction du volume de remplis.~age l~riton6a~ et de I'~ge. En cas de misc en route de la DP d~s t.-. page du catheter p6:;ton6ai, le suivi de la PIP pennet d'adapter le volume intra#ri*on6al r6duisam les risques de douleur et de fuite, Le test de perm6abilit6 p6riton6ale permet de d~finir la vitesse de d6saturatRm "lu dialysat cn glucose ct la vitesse de saturation du dialysat en toxines ur6miques par rapgort au plasma (D/P~. Lc point de croisement de la courtm de d~saturation en glucose et de saxuration en ur6e d6finit le, temps APEX ou temps d'ultrafiltration; un temps de contact p~us long r6duit la capacit6 d'ultrafiltration de la DP par perle du gradiant osmiotique d~pendant du glucose. Le temps de saturation du dialysat, par convention Dm ~2al ~ 60 % pour le phosphate, toxine ur~miqae ~ diffusion 'ente, d6finit un temps d'dpuration. La d~tennination de ees temps d'ultrafiltration et dMpuration (n = 142 mesures) durant ies 5 demi~res ann6es ehez 17 enfants en DP a pennis de montrer I'opposition entre le temps de contact efficace pour I'ultrafiltmtion: 39 4-23 (18 71 rain) et le temps n6eessaire/~ I'dpuration: 193 + 69 (105 238 rain). Ainsi, la d6tennination individuelle de ees param~:tres aklc au ehoix de la modalit6 de DP. Si I'ultafiltralion est I'objectif principal, des temps de contacts courts sont ipdispensables, imposant la dialyse p6riton6ale automatique par eycleur. Si I'@uration est prioritairc, des temps de conSarts longs sont souhaitables, r6alis6s on DPCA. Si I'ultrafiltration et I'~puration sont n6ce.~aires, la dialyse p~riton~ale fluetuante fTidal) peut ~tre propose.