Wimj january1.qxd

Paediatric Nephrology at the University Hospital of the West Indies
A Walk through Time
ABSTRACT
Paediatric nephrology at the University Hospital of the West Indies has grown over the last five decades into an established paediatric subspecialty of ering to Jamaica and other Caribbean territories the benefit of paediatricians with training and exposure in this field. Dissemination of information to medical practitioners within the island has reduced mortality and morbidity associated with potential y treatable atypical renal disease. Clear investigative guidelines for urinary tract infection have made earlier diagnosis of urological malformations possible. Pat erns of glomerular disease in Jamaican children have now been aetiological y and histological y documented. Children with chronic kidney disease now have clear management guidelines and the possibility of renal replacement therapy. Future goals include community education about renal disease and the development of a paediatric dialysis Keywords: Glomerulonephritis, nephrotic syndrome, renal failure
Nefrología Pediátrica en el Hospital Universitario de West Indies
Un Recorrido a Través del Tiempo
RESUMEN
La nefrología pediátrica en el Hospital Universitario de West Indies se ha desarrol ado en las últimas cinco décadas, convirtiéndose en una sub-especialidad pediátrica establecida, que ofrece a Jamaica y otros territorios caribeños, el beneficio de pediatras con entrenamiento y experiencia en este campo. La diseminación de la información entre los médicos dentro de la isla, ha reducido la mortalidad y la morbosidad asociadas con la enfermedad renal atípica potencialmente tratable. Los claros linea- mientos de la investigación de las infecciones del tracto urinario, han hecho posible un diagnóstico más temprano de las malformaciones urológicas. Los patrones de enfermedad glomerular en los niños jamaicanos han sido ahora documentados tanto etiológica como histológicamente. Los niños con enfermedad renal crónica tienen a hora a su disposición lineamientos claros para el tratamiento así como la posibilidad de la terapia del reemplazo renal. Las metas futuras incluyen la educación de la comunidad sobre la enfermedad renal y el desarrol o de una unidad pediátrica de diálisis y trasplante. Palabras claves: Glomerulonefritis, síndrome nefrótico, insuficiencia renal
West Indian Med J 2012; 61 (4): 415
INTRODUCTION
until December 1984 with the arrival of Dr Maolynne Mil er, Paediatric nephrology as a subspecialty did not formal y the first paediatric nephrologist. However the first paedia- begin at the University Hospital of the West Indies (UHWI) trician with interest in this field was Dr Dawn Swaby.
From: Department of Child and Adolescent Health, The University of the Glomerulonephritis
It was a common practice of physicians then, in some hos- Correspondence: Dr M Mil er, Department of Child and Adolescent Health, pitals, to deem al types of acute glomerulonephritis, post- The University of the West Indies, Kingston 7, Jamaica, West Indies. E-mail: streptococcal (PSGN), and expect a good prognosis in spite of atypical features. Many of these atypical children did not West Indian Med J 2012; 61 (4): 415
undergo renal biopsy and progressed to chronic renal failure in whom the resistance pattern is similar to amoxil (1). The initial thrust of paediatric nephrology at UHWI was education at al levels – medical students, residents, con- sultants, paediatricians and physicians in general practice – to Nephrotic Syndrome
recognize presentations of renal disease which required In the early days, outpatient urine protein testing was not urgent nephrological referral. This information was spread done because of the prohibitive cost of commercial Lab- through islandwide meetings with various medical groups.
stix™. Children with nephrotic syndrome frequently needed Residents in training were able to take back to their territory admission as oedema was the only indicator of relapse. With (if non-Jamaican) and to their practices, information on the use of home testing by test tube using sulphosalicylic paediatric nephrology. To facilitate this, two paediatric acid, relapses are diagnosed earlier and admissions for nephrology manuals were writ en – in 1988 (2) and a revision nephrotic syndrome have drastical y reduced.
in 2002 (3) with paediatric norms, investigative and man- Complete serological evaluation of al children with nephrotic syndrome is now practised resulting in improved ability to make aetiological diagnoses. Hepatitis B infection Renal Failure
was found to be the cause of nephrotic syndrome in 6% of Prior to 1984/85, the management of chronic renal failure in otherwise asymptomatic children with nephrotic syndrome children was not standardized and the absence of 1-hydro- and the commonest cause of childhood membranous nephro- xylated 25-OH Vitamin D resulted in rampant rachitic de- pathy (8). These data were able to support the appeal for formities. Dialysis for acute renal failure was general y Hepatitis B immunization in Jamaican children, and since the unavailable and pulmonary oedema from renal failure was implementation of vaccination, no new cases have been ob- treated with mechanical ventilation. There were no facilities served. The first published cases of HTLV-1 associated renal for chronic dialysis or transplantation. After December 1984, disease were Jamaican children with infective dermatitis (9) acute peritoneal dialysis became possible and paediatric renal resulting in the inclusion of this investigation in children with biopsies could be performed with greater ease. As the asso- ciation with the adult nephrology team evolved into the dyna- mic unit it now is, children were able to benefit from haemo- Patterns of Renal Disease
and peritoneal dialysis. In fact, a nine-year old actual y re- Glomerulonephritis
ceived a renal transplant in 2001 which served him wel . We Over the years, the pat ern of renal disease has evolved. In a are now able to offer chronic automated peritoneal dialysis retrospective review of glomerulonephritis in Jamaican chil- and haemodialysis to children weighing as lit le as 13 kilo- dren from 1978–1982, 95% of cases were postinfectious with PSGN accounting for 98% of infections. The only two renal biopsies performed revealed mesangial proliferative glo- Urinary Tract Infection
merulonephritis (MesPGN) with focal sclerosis and diffuse Prior to December 1984, children with urinary tract infection proliferative glomerulonephritis (DPGN), respectively (10).
(UTI) were investigated only if infections were recurrent, A review of renal biopsies in al Jamaican children less associated with a clinical y obvious urological abnormality, than 12 years of age was conducted between 1985–2008 to or if the child was male. Children with urological pathology obtain information on the pat ern of renal disease seen local y were often diagnosed late, already with chronic kidney (11). Children were biopsied if they had nephrotic syndrome disease (1). Since then, local guidelines were formalized and atypical for minimal change disease (MCD), unexplained or disseminated to ensure adequate investigation of Jamaican atypical glomerulonephritis, unexplained haematuria, pro- teinuria, or to stage renal involvement in systemic lupus In a 1984–2005 retrospective study of 301 children erythematosus. Of the 270 children biopsied, the commonest with UTI in a combined general paediatric and paediatric histology was DPGN (27.7%), followed by MesPGN nephrology referral service, renal ultrasound was normal in 88% with vesico-ureteric reflux and 75% of those with pos- Glomerular disease was associated with infection in terior urethral valves, making it an inadequate screening tool fewer cases in the current study (32.4% vs 95%) with PSGN accounting for 75% of these compared with 98% in the Prospective research has documented that Escherichia earlier review. During the 24-year period, PSGN peaked be- coli is the commonest uropathogen in Jamaican children < tween 1985 and 1988 and again 1993–1995 with corres- age 12 years with first UTI (7). Escherichia coli is highly ponding trends in DPGN and MesPGN both of which resistant to ampicil in which is thus an inappropriate first line histological appearances may be seen in PSGN.
treatment for UTI. Overal , the greatest sensitivity was to In sickle cel (SS) disease, DPGN was again the com- gentamycin fol owed by amoxil clavulanate and cotrimoxa- monest histology (47.4%), fol owed by MesPGN and mem- zole. It now seems reasonable to use cotrimoxazole as first branoproliferative glomerulonephritis. There was no statis- line treatment in children in the 1 to under 12-year age group tical y significant difference between the frequency of DPGN, PSGN and MesPGN in the children with SS disease being diagnosed and treated earlier, renal failure persisted in and the remaining children, suggesting that children with SS some. It appeared that education and early referral had re- disease have the childhood nephropathies endemic to duced mortality from atypical PSGN. However, with the Jamaica rather than renal disease due to the sickling process longer survival of HIV infected children, progressive renal disease was now apparent, usual y associated with treatment noncompliance or failure. As before, about half of the chil- Nephrotic Syndrome
dren were already in CRF at first presentation with renal Prior to 1984, children less than 12 years of age with nep- disease. Mortality had fal en to 44.4%. At the UHWI, 36% hrotic syndrome most commonly had MCD fol owed by of children under 12 years old accessed chronic haemo- MesPGN (15%) and DPGN (7%). Most children did not have dialysis. There was one renal transplant in this group (16).
Between 1984–1987, 26/27 Jamaican children present- Plans for the Future
ing with nephrotic syndrome had features atypical for MCD It seems that physicians now recognize and refer atypical and were biopsied. The commonest histology overal was cases earlier with improvement in outcome. The next goal is MesPGN (30%) fol owed by MCD (26%) and focal seg- to take basic information about kidney disease to the com- mental glomerulosclerosis (FSGS) [18%]. Diffuse proli- munity to ensure prompt presentation for medical care. The ferative glomerulonephritis accounted for only 4%. Neph- paediatric nephrology service wil be strengthened in 2012 rotic syndrome was idiopathic in 41% and postinfectious in when a second paediatric nephrologist joins the University the majority (59%) with PSGN being the commonest Hospital of the West Indies. Renal transplantation and a infection (81%). In unreferred patients, MCD was stil the separate paediatric dialysis unit are our aspirations.
most common histology/presentation (45%) while in those referred for nephrological consultation, the frequency of REFERENCES
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(PSGN in 67%). Overal , MesPGN was the commonest ap- Departmental Handbook for Paediatric Residents.
pearance (31.2%) fol owed by MCD (22.9%) and DPGN 4. Mil er M, Wil iams J, Abel C, Duncan N. Management guidelines for (16.6%). In idiopathic nephrotic syndrome, MesPGN was urinary tract infections in Jamaican children. A consensus document again the most frequent histological appearance (43%) fol- 5. Mil er M, Abel C, Dundas S. Management guidelines for urinary tract lowed by MCD (29%) and FSGS and DPGN (10% res- infections in Jamaican children. Consensus document for medical pectively). The rise in frequency of FSGS observed with time in the international literature was not evident in 6. Mil er M. Is the micturating cystogram real y necessary? [Manuscript 7. Morgan R, Mil er M, Olugbuyi O, Trotman H. Aetiology and antibiotic resistance of uropathogens in Jamaican children. Doctoral Thesis for Chronic Renal Failure
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