Long-lasting recovery of renal function of the native kidneys after prolonged renal replacement therapy is rare. An 8-year-old girl and a 3-year-old boy had suffered from acute atypical and diarrhea-associated hemolytic uremic syndrome (HUS), respectively, with subsequent apparent end-stage renal failure. Both recovered renal function after long-lasting anuria and dialysis of 8 and 16months, respectively. After prolonged follow-up, i.e., 7 and 5years after cessation of dialysis, they attained normal or slightly reduced renal function (plasma creatinine 84 and 90µmol/l, respectively). In addition, growth and cognitive development were normal. We conclude that caution is appropriate before offering early renal transplantation to pediatric patients with presumed end-stage kidney disease secondary to HU

Bianchetti, Mario

Brunner, Kathrin

Neuhaus, Thomas

RERO DOC Digital Library

Pediatr Nephrol (2004) 19:229–231DOI 10.1007/s00467-003-1337-4B R I E F R E PORTKathrin Brunner · Mario G. Bianchetti ·Thomas J. NeuhausRecovery of renal function after long-term dialysisin hemolytic uremic syndromeReceived: 21 July 2003 / Revised: 11 September 2003 / Accepted: 11 September 2003 / Published online: 27 November 2003 IPNA 2003Abstract Long-lasting recovery of renal function of thenative kidneys after prolonged renal replacement therapyis rare. An 8-year-old girl and a 3-year-old boy hadsuffered from acute atypical and diarrhea-associatedhemolytic uremic syndrome (HUS), respectively, withsubsequent apparent end-stage renal failure. Both recov-ered renal function after long-lasting anuria and dialysisof 8 and 16 months, respectively. After prolonged follow-up, i.e., 7 and 5 years after cessation of dialysis, theyattained normal or slightly reduced renal function (plasmacreatinine 84 and 90 mol/l, respectively). In addition,growth and cognitive development were normal. Weconclude that caution is appropriate before offering earlyrenal transplantation to pediatric patients with presumedend-stage kidney disease secondary to HUS.Keywords Hemolytic uremic syndrome · Renalreplacement therapy · RecoveryIntroductionThe term end-stage kidney disease is used to indicateirreversible kidney failure requiring dialysis and renaltransplantation. Recovery of renal function of the nativeor transplanted kidneys after prolonged dialysis has rarelybeen reported, and mainly in adult patients [1, 2, 3, 4, 5, 6,7, 8, 9, 10]. We report on two children with hemolyticuremic syndrome (HUS) [11] who experienced a long-lasting recovery of renal function after being on dialysisfor 8 and 16 months, respectively.Case reportsBetween 1970 and 2002, 130 and 67 patients underwent chronicrenal replacement (i.e., necessary renal replacement therapy formore than 6 months) in Zurich and Bern, respectively. In bothcenters, HUS was an important cause of end-stage renal failure: 12patients (9%) from Zurich had HUS, two had diarrhea-related HUSand 10 atypical HUS. In Bern, HUS was the underlying cause infive (7%) patients, one had diarrhea-related HUS and four atypicalHUS. Two of the 197 patients, both with HUS, recovered renalfunction.Case 1An 8-year-old girl with a history of vomiting, fever and pallor for10 days was referred. There was no history of diarrhea. Physicalexamination revealed high blood pressure (132/102 mmHg: 95thcentile for gender, age and height: 115/75), pallor and a mild scleraljaundice. Hematology showed anemia (hemoglobin 37 g/l, normal100–150 g/l) and thrombocytopenia (85109/l, normal 200–400109/l); a blood film demonstrated moderate red blood cellfragmentation. Blood chemistry disclosed renal failure withelevated creatinine (399 mol/l, normal range for age <66 mol/l)and urea (54 mmol/l, normal <8.2 mmol/l), hyperbilirubinemia(45 mol/l, normal <30 mol/l) and high lactate dehydrogenase(1,360 U/l, normal <500 U/l). Complement C3, factor H and vonWillebrand cleavage protease were normal, antinuclear or antineu-trophil cytoplasmic autoantibodies were not detected and the directCoombs test was negative. Specific Escherichia coli lipopolysac-charide antibodies were not measured. Urinalysis showed hema-turia, red cell casts and marked proteinuria. No E. coli strainsharboring genes for shigatoxin were cultured from the stool. Hencethe diagnosis of atypical (diarrhea-negative) HUS was made. Arenal biopsy was not done. Within 10 days, the girl became anuric.Continuous automated peritoneal dialysis was started, amlodipinewas given to treat hypertension and fresh frozen plasma wasadministered on 15 occasions. During the following 4 weeks sheexperienced focal seizures with a tendency towards generalization.Intravenous diazepam and phenytoin were given repeatedly,followed by oral carbamazepine. One month after onset of thedisease the girl was switched from peritoneal dialysis to hemodi-K. Brunner · M. G. BianchettiDivision of Pediatric Nephrology,University of Bern,Bern, SwitzerlandK. Brunner · T. J. NeuhausDivision of Pediatric Nephrology,University of Zurich,Zurich, SwitzerlandT. J. Neuhaus ())Division of Pediatric Nephrology,University Children’s Hospital,Steinwiesstrasse 75, 8032 Zurich, Switzerlande-mail: thomas.neuhaus@kispi.unizh.chTel.: +41-1-2667111Fax: +41-1-2667171alysis (three sessions per week) because of two episodes ofbacterial peritonitis. She continued to be anuric.Six months after starting dialysis, the girl reported to pass smallportions of urine one to two times a week. One month later, urineproduction increased and hemodialysis was reduced to two weeklysessions. Eight months after onset, dialysis was finally stopped. Thesubsequent clinical course was uneventful.Recently, i.e., 7 years after cessation of dialysis, the girl waswell on treatment with irbesartan 300 mg daily, chlorthalidone25 mg daily, and carabamazepine 1,200 mg daily. Growth (bodyweight: 52 kg, height: 1.64 m) and cognitive development wereboth normal. Renal function, as assessed as plasma creatinine(84 mol/l, normal range for age <98 mol/l) and estimatedglomerular filtration rate (Schwartz formula: 78 ml/min/1.73 m2body surface area), was normal. In addition, casual blood pressure(109/50 mmHg: 95th centile: 128/83 mmHg) and 24-h ambulatoryblood pressure (108/62 mmHg: 95th centile: 124/76 mmHg), andurinalysis (normal sediment, albumin/creatinine 3.5 g/mol) werenormal. No further family members were suffering from HUS orrenal disease.Case 2A 3-year-old boy presented with a 5-day history of pallor andseveral blue bruises. A week earlier he had suffered from waterydiarrhea for 2 days after the consumption of raw cow milk. Physicalexamination demonstrated high blood pressure (120/66 mmHg:95th centile: 109/65 mmHg), mild scleral jaundice and swolleneyelids. Hematology revealed anemia (hemoglobin 59 g/l) andthrombocytopenia (123109/l); a blood film showed marked redblood cell fragmentation. Blood chemistry disclosed mild renalfailure (creatinine 103 mol/l, normal range for age <51 mol/l;urea 14 mmol/l), hyperbilirubinemia (53 mol/l) and high lactatedehydrogenase (1,034 U/l). Complement C3 was normal, antinu-clear or antineutrophil cytoplasmic autoantibodies were notdetected and the direct Coombs test was negative. Specific E. coliantibodies were not measured. Urinalysis revealed hematuria, redcell casts and marked proteinuria. E. coli strains harboring genes forshigatoxin could not be cultured from his stool at this stage.Systolic blood pressure further increased up to 140–160 mmHg,requiring intravenous administration of dihydralazine. Severalpacked red cells had to be given due to persistent microangiopathichemolytic anemia. A renal biopsy disclosed extensive lesions ofthrombotic microangiopathy including both intraglomerular capil-laries and extraglomerular arterioles and small arteries. Urinaryoutput decreased and the boy became anuric. Despite the unusualcourse—slow progression of renal failure over 3 weeks—apresumptive diagnosis of diarrhea-positive HUS was made.Hemodialysis had to be started 18 days after admission. Inaddition, plasma exchange with albumin and an infusion of freshfrozen plasma at the end of each session was applied on 12occasions. As renal function did not improve, plasma exchange wasstopped and the patient switched to automated peritoneal dialysis(duration: 12 h during the night; filling volume: 40 ml/kg bodyweight: number of exchanges: 12/night). Blood pressure controlwas only achieved with a triple therapy of atenolol, nifedipine andminoxidil.After being on a constant peritoneal dialysis regimen for14 months, the boy started to pass urine. Firstly, there were onlysmall volumes once a week, but urine output increased steadily.Finally, 16 months after onset of renal replacement therapy,peritoneal dialysis was ceased.Recently, i.e., 5 years after cessation of dialysis, the boy was ingood health. Growth (body weight: 28 kg, height: 1.31 m) andcognitive development were both normal. Renal function, asassessed as plasma creatinine (90 mol/l, normal range for age<75 mol/l) and estimated glomerular filtration rate (Schwartzformula: 58 ml/min/1.73 m2 body surface area), was slightlyreduced. There was mild microhematuria, but no proteinuria.Despite antihypertensive treatment with valsartane and amlodipine(20 and 7.5 mg daily, respectively), blood pressure (casual: 127/83 mmHg; 24-h ambulatory: 128/83 mmHg) remained persistentlyabove the 95th centile (casual: 116/77; 24-h: 117/75). Echocardi-ography and fundoscopy were normal. No further family memberswere suffering from HUS or renal disease.DiscussionWe report long-lasting recovery of renal function afterprolonged dialysis, i.e., more than 6 months, in pediatrichemolytic uremic syndrome. The term end-stage kidneydisease denotes irreversible deterioration of renal func-tion. A few patients, mainly adults, have been reported toregain independent renal function of their native kidneysafter long-term dialysis [1, 2, 3, 4, 5, 7, 8, 9]. Recoverymostly occurred within 6–12 months of initiating renalreplacement therapy and was generally partial, bothrelated to glomerular and tubular function. In addition,recovery appeared to be more common in kidney diseasessecondary to treatable malignancies, adverse effects ofdrugs as reported in two cases of atypical HUS [2, 4] or inhypertensive kidney disease [1, 9].Recovery after long-term dialysis has occasionallybeen reported in children [6, 10]: The duration of renalreplacement therapy had lasted less than 1 year in themajority of these children. In addition, the recovery wastransient and lasted less than 1 year in the majority ofpatients.Which protective factors might have contributed to thefavorable outcome in our patients with HUS are not clear,especially as two main risk factors of bad outcome inHUS [12]—long-term anuria in both patients and exten-sive thrombotic microangiopathy in one—were present.In particular, atypical HUS carries a substantial risk forend-stage renal failure and is an important cause of end-stage renal failure in childhood [11]. The high risk ofrecurrence—associated with high morbidity and mortal-ity—has prompted some centers, including our own, toavoid early renal transplantation in those children.In conclusion the present report suggests that caution isappropriate before offering early renal transplantation topediatric patients with presumed end-stage kidney diseasesecondary to diarrhea-associated or atypical HUS.References1. Nunan TO, Stevens EA, Croft DN, Hilton PJ, Jones NF, WingAJ (1983) Recovery of renal function after prolonged dialysisand transplantation. BMJ 287:248–2492. Verwey J, Boven E, van der Meulen J, Pinedo HM (1984)Recovery from mitomycin C-induced hemolytic uremic syn-drome. Cancer 54:2878–28813. Hansen BL, Birkeland SA (1985) Recovery of kidney functionafter cessation of graft function or prolonged dialysis treatment.Scand J Urol Nephrol 19:217–2194. Ortega Marcos O, Escuin F, Miguel JL, Gomez Fernandez P,Perez Fontan M, Selgas R, Sanchez Sicilia L (1985) Hemolyticuremic syndrome in a patient with gastric adenocarcinoma:partial recovery of renal function after gastrectomy. ClinNephrol 24:265–2682305. Sekkarie MA, Port FK, Wolfe RA, Guire K, Humphrys R, VanAmburg G, Ferguson CW (1990) Recovery from end-stagerenal disease. Am J Kidney Dis 15:61–656. Ehrich JH, Rizzoni G, Brunner FP, Brynger H, Geerlings W,Fassbinder W, Raine AE, Selwood NH, Tufveson G (1989)Combined report on regular dialysis and transplantation ofchildren in Europe. Nephrol Dial Transplant 6 (Suppl 1):37–477. Lindblad AS, Nolph KD (1992) Recovery of renal function incontinuous ambulatory peritoneal dialysis: a study of NationalCAPD Registry data. Perit Dial Int 12:43–478. Rottembourg J (1993) Residual renal function and recovery ofrenal function in patients treated by CAPD. Kidney Int Suppl40:S106–S1109. James SH, Meyers AM, Milne FJ, Reinach SG (1995) Partialrecovery of renal function in black patients with apparent end-stage renal failure due to primary malignant hypertension.Nephron 71:29–3410. Kaplan BS, Garcia CD, Chesney RW, Segar WE, Giugno K,Chem R (2000) Peripheral gangrene complicating idiopathicand recessive hemolytic uremic syndromes. Pediatr Nephrol14:985–98911. Neuhaus TJ, Calonder S, Leumann EP (1997) Heterogeneity ofatypical haemolytic uraemic syndromes. Arch Dis Child76:518–52112. Habib R, Levy M, Gagnadoux MF, Broyer M (1982) Prognosisof hemolytic uremic syndrome in children. Adv Nephrol 11:99–128231

Recovery of renal function after long-term dialysis in hemolytic uremic syndrome

http://doc.rero.ch/record/316718/files/467_2003_Article_1337.pdf

Recovery of renal function after long-term dialysis in hemolytic uremic syndrome

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library