Manual of Pediatric Nephrology

Kishore Phadke • Paul Goodyer

Martin Bitzan
Editors

Manual of Pediatric
Nephrology
Editors
Kishore Phadke Martin Bitzan
Department of Pediatric Nephrology Division of Pediatric Nephrology
Children’s Kidney Care Center Montreal Children’s Hospital
St. John’s Medical College Hospital McGill University
Bangalore, KA Montreal, QC
India Canada

Paul Goodyer
Division of Pediatric Nephrology
Montreal Children’s Hospital
McGill University
Montreal, QC
Canada

ISBN 978-3-642-12482-2 ISBN 978-3-642-12483-9 (eBook)

DOI 10.1007/978-3-642-12483-9
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Foreword I

The International Society of Nephrology strives to improve access to the best possible
kidney care worldwide. An important step is the training of nephrologists and physicians
treating patients with renal diseases. These goals form the basis of the ISN “Global
Outreach” (GO) programs – to build health equality worldwide and improve kidney care
and prevention strategies around the globe by making the knowledge and experience of the
developed world accessible to kidney doctors and other specialists in emerging countries.
Children are a particularly vulnerable population. Unacceptable delays in the
recognition and treatment of (severe) renal disease due to lack of access to basic and
comprehensive care often leads to preventable, acute and chronic adverse effects
caused by malfunctioning kidneys. Kidney disease during infancy to adolescence
due to (intrauterine) malnutrition, genetic inheritance, nephrotoxic agents or poorly
treated primary diseases has devastating, often irreversible, effects on bone health,
growth and intellectual development.
Disparities in preventive and curative or supportive care arise from the lack of
knowledgeable health care professionals, lack of (public) health care resources and
lack of individual financial means to initiate and support therapies that are taken for
granted in more affluent countries.
To come to grips with the global challenge of rising numbers of patients with
renal diseases, the ISN created the Sister Renal Center program pairing emerging
and supporting (established) centers across all continents. As the chair and ardent
supporter of our GO programs, I am proud to witness the involvement of dedicated
pediatric nephrologists in this endeavor.
This Manual is the collaborative product of one of the few and successful pedi-
atric sister center pairs, the Children’s Kidney Care Center at the St. John’s Medical
College Hospital in Bangalore, India, and the Nephrology Division at the Montreal
Children’s Hospital in Montreal, Canada. Written in a collaborative spirit, it empha-
sizes a global perspective.
May it serve health care professionals, trainees, and physicians to improve the
diagnosis and treatment of children with renal diseases worldwide.

Paul N. Harden
Professor of Nephrology
Chair, International Society of Nephrology Sister Renal Program
Oxford, UK

v
Foreword II

Today, kidney issues dramatically impact global health, especially the health of
children. In developing countries, millions of people – most of them children – die
each year from diseases that are preventable and treatable. For many of these chil-
dren, kidney problems represent a serious threat to their survival.
Important examples include – but are not limited to – the delayed recognition and
treatment for congenital abnormalities of the kidney and urinary tract, which are a
major cause of chronic kidney disease and secondary health problems; severe fluid
and electrolyte disturbances and acute kidney injury due to diarrhea, the #2 cause of
death in children worldwide; infants with low birth weight in low income groups
due to malnutrition and lack of prenatal care, with an increased risk of small kid-
neys, hypertension in adolescence and chronic kidney disease; HIV associated
nephropathy, particularly in areas where highly active antiretroviral therapy is not
accessible.
A major goal of the International Pediatric Nephrology Association (IPNA) is to
reach physicians throughout the world through educational and fellowship programs
and enable them to care for children with kidney diseases. This Manual of Pediatric
Nephrology is designed to give pediatrician and general physicians as well as train-
ees and other health care professionals a quick and practical approach to the diag-
nosis and treatment of children with different types of kidney diseases. The
well-structured text is easy to read and covers all important areas in the field.
On behalf of IPNA, I welcome this new manual as a valuable resource for prac-
titioners and trainees in (pediatric) nephrology alike. Its appeal lies in the combined
perspective provided by experienced nephrologists from different continents that
takes into account the realities in emerging countries. It corresponds well to IPNA’s
goals to “work to disseminate knowledge about kidney disease in children in the
areas where care is needed most”.

Isidro B, Salusky, M.D.

Secretary General IPNA
Distinguished Professor of Pediatrics
David Geffen School of Medicine at UCLA
Los Angeles, CA, USA

vii
Preface

This manual does not want to replace standard textbooks of nephrology or pediatric
nephrology. Rather, it offers a first, quick and practical approach for the care of
children with kidney disease. It is meant to serve practitioners, trainees, pediatri-
cians, general physicians, and other health care professionals.
The manual is the product of a long-lasting collaboration between two pediatric
nephrology units, the Children’s Kidney Care Center at the Saint John’s Medical
College Hospital (St-John’s) in Bangalore, India, and the Division of Nephrology at
the Montreal Children’s Hospital (MCH) in Montreal, Canada.
The authors approached each chapter with the practical reality in emerging and
resource-poor countries in mind. We hope that this concept renders the manual use-
ful and versatile in a variety of settings and diverse medical practices. To this end,
we are proud of the manual’s endorsement by the International Society of Nephrology
Global Outreach initiative and the support by the International Pediatric Nephrology
Association.
It is unavoidable in a practically oriented, abbreviated book like ours that topics
are missing or only mentioned cursorily. The editors purposefully focused on rela-
tively frequent and clinically important disorders and practical, mainly diagnostic
and therapeutic, aspects. Detailed pathological and pathophysiological reviews
were omitted to keep the text short and lean. The reader is strongly encouraged to
seek in-depth information in more comprehensive textbooks.
The editors appreciate specific suggestions that help improve the utility and ame-
liorate the shortcomings of this manual. Please, email the editors your comments.
We hope that the global pediatric nephrology community will adopt the manual as
a dynamic, interactive project with input from trainees and practitioners alike.
The editors have an enduring commitment to research and education in pediatric
renal diseases locally and internationally with the ultimate goal of improving chil-
dren’s access to renal care – irrespective of monetary resources, societal status, or
geography. They are connected through a long-standing collaboration within the
ISN Sister Renal Center program. Kishore Phadke, Professor of Pediatrics and
Nephrology, Director of the Children’s Kidney Care Center and former Chair of
Pediatrics at St-John’s was the driving force behind this manual. He established a
successful, internationally recognized Pediatric Nephrology Training Program,
whose trainees now work across India, and in Sri Lanka, Bangladesh and the Middle

ix
x Preface

East. Paul R. Goodyer, McGill Professor, former Director of the Division of

Nephrology at the MCH, is a successful investigator of kidney development and
genetic renal diseases with a number of international collaborations, including
St-John’s. Martin Bitzan, Associate Professor of Pediatrics and Pediatric Nephrology
and current Director of the Division of Nephrology and of the Pediatric Nephrology
Training program at the MCH has a focused research and clinical interests in glom-
erular and systemic renal diseases and international education in pediatric
nephrology.
The editors wish to acknowledge their collaborators from the St-John’s and the
MCH. Their input and contributions were instrumental in the genesis of this man-
ual. Not all of them are named individually. “Thank you” also to the associate/
publishing editors and “midwives” of this oeuvre, Sandra Lesny, Rosmarie Unger,
Marion Krämer, and Dr. Tobias Kemme from Springer, and project manager Steven
Muthu Raj Joe Arun from SPi Global, India, for their patient professionalism. We
apologize to our families, particularly our spouses, for enduring long absences and
distractions associated with this manual and with our clinical work. Last, but not
least, we thank our students and trainees for reminding us of our educational mis-
sion, and our patients and their families, who taught us so much and who continue
inspiring us.

Bangalore, India Kishore Phadke

Montreal, Canada Paul Goodyer
Montreal, Canada Martin Bitzan
Contents

1 Evaluation of Renal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

Paul Goodyer and Kishore Phadke
2 Fluids, Electrolytes, and Acid–Base Disorders . . . . . . . . . . . . . . . . . . . 65
Anil Vasudevan and Kishore Phadke
3 Glomerular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
Martin Bitzan
4 Tubular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231
Paul Goodyer
5 Cystic Renal Diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 249
Indra Gupta and Martin Bitzan
6 Issues Related to Pediatric Urology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263
Arpana A. Iyengar, John-Paul Capolicchio,
Mohamed El-Sherbiny, Roman Jednak,
and Konrad M. Szymanski
7 Hypertension. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315
Martin Bitzan
8 Acute Kidney Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
Indra Gupta and Martin Bitzan
9 Chronic Kidney Disease (CKD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 373
Arpana A. Iyengar and Bethany J. Foster
10 Chronic Dialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 401
Kishore Phadke, Martin Bitzan, and Sudarshan Shetty
11 Renal Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 429
Kishore Phadke, Paul Goodyer, and Arpana A. Iyengar
12 Oncology and Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445
Anand Prakash and David Mitchell
13 The Kidney and the Tropics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461
Anita Shet

xi
xii Contents

14 HIV and the Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479

Anita Shet
15 Newborn and the Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493
P.N. Suman Rao
16 Nephrotoxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517
Anil Vasudevan and Nivedita Kamath
17 Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 533
Jyoti Sharma and Anil Vasudevan

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 611
Abbreviations

mg Micrograms
ABG Arterial blood gases
ABPM Ambulatory blood pressure monitoring
ACE Angiotensin-converting enzyme
ADH Anti diuretic hormone
ADH rickets Autosomal dominant hypophosphatemic rickets
ADPKD Autosomal dominant polycystic kidney disease
AG Anion gap
AKI Acute kidney injury
ANA Antinuclear antibodies
ANCA Anti neutrophilic cytoplasmic antibodies
anti-DsDNA Anti-deoxyribonuclease B
anti-GBM antibodies Anti glomerular basement antibodies
APLA Antiphospholipid antibodies
ARB Angiotensin receptor blocker
ARH rickets Autosomal recessive hypophosphatemic rickets
ARPKD Autosomal recessive polycystic kidney disease
ATN Acute tubular necrosis
ATN Acute interstitial nephritis
AVP Arginine vasopressin
BE Base excess
BMD Bone mineral density
BP Blood pressure
BUN Blood urea nitrogen
BUN Blood urea nitrogen
CAPD Continuous ambulatory peritoneal dialysis
CCPD Continuous cycling peritoneal dialysis
CDI Central diabetes insipidus
CHD Coronary heart disease
CKD Chronic kidney disease
Cl Chloride
CNI Calcineurin inhibitors
COX-2 Cyclooxygenase type 2
Cr Creatinine

xiii
xiv Abbreviations

Cr Creatinine clearance
CTA CT angiography
CVD Cerebrovascular disease
D5 5 % dextrose
DABP Diastolic ambulatory blood pressure
DBP Diastolic blood pressure
DDS Dialysis equilibrium syndrome
DIC Disseminated intravascular coagulation
DKA Diabetic ketoacidosis
DMSA Dimercaptosuccinic acid
DRCG Direct nuclide cystography
DXA Dual-energy X-ray absorptiometry
e GFR Estimated GFR
e.g. For example
EKG Electrocardiogram
ELBW Eextremely low birth weight
ESRD End-stage renal disease
ESWL Extracorporeal lithotripsy
GBM Glomerular basement membrane
GCD Glomerulocystic disease
GFR Glomerular filtration rate
GI Gastrointestinal
gm Gram
HAART Highly active antiretroviral therapy
HBPM Hemoglobin
HBPM Home blood pressure monitoring
HCO3 Bicarbonate
HIVAN HIV-associated nephropathy
HTN Hypertension
HUS Hemolytic uremic syndrome
IRIS Immune reconstitution inflammatory syndrome
kDa Kilodaltons
kDa Potassium
KIM-1 Kidney injury molecule −1
LDH Lactic dehydrogenase
LVH Left ventricular hypertrophy
mL Milliliters
MA Metabolic acidosis
MAG 3 Mercaptoacetyltriglycine 3
MCKD Multicystic kidney disease
mEq Milliequivalent
mg Milligrams
MH Masked hypertension
MNE Monosymptomatic enuresis
mosm Milliosmoles
Abbreviations xv

MRA MR angiography
MRV MR venography
Na Sodium
NDI Nephrogenic diabetes insipidus
NGAL Neutrophil gelatinase-associated lipocalin
NHBPEP National high blood pressure education program
NPHP Nephronophthisis
NRTI Nucleoside reverse transcriptase inhibitor
NS Normal saline
ORS Oral rehydration solution
PCNL Percutaneous nephrolithotomy
PCO2 Partial pressure of carbon dioxide
PET Peritoneal equilibration test
PH Primary hyperoxaluria
PI Protease inhibitors
PO2 Partial pressure of oxygen
PRA Panel reactive antibodies
PRA Plasma renin activity
RBC Red blood cell
rhGH Human recombinant growth hormone
RPGN Rapidly progressive glomerulonephritis
RTA Renal tubular acidosis
RTA Renal tubular acidosis
SABP Systolic ambulatory blood pressure
SBP Systolic blood pressure
SIAD Syndrome of inappropriate antidiuresis
TIN Tubulointerstitial nephritis
TmP Renal tubular phosphate threshold maximum
TRP Tubular reabsorption of phosphate
TTKG Transtubular potassium gradient
UAG Urine anion gap
UAG Urinary anion gap
UOG Urine osmolal gap
UTI Urinary tract infection
VACTERL Vertebral, anorectal, cardiac, tracheoesophageal, renal, limb defects
VATER Vertebral, anorectal, tracheal, esophageal, renal
VCUG Voiding cystourethrography
VUR Vesicoureteral reflux
WBC White blood cell
XLH rickets X-linked hypophosphatemic rickets