Grand Rounds in Pediatric Nephrology
Recorded On: 05/03/2021
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- A tale of two pathologies: Rapidly Progressive Glomerulonephritis and Acute Tubulointerstitial Nephritis
- Nephropathic Cystinosis: How to Diagnose and Treat
- Make the Peritoneal Membrane Work for You
- Approach to Infantile Hypercalcemia
- Pediatric Renal Genetics: New Advances
- Management of Renal Bone Disease
- Resistant Hypertension in Children on Dialysis
- C3 Glomerulonephritis and Dense Deposit Disease – A Tale of Two Twins
- Immunosuppression vs post-transplant viral infections: Walking a fine line
- A Challenging Case of Nephrotic Syndrome
- The Spectrum of Alport Syndrome: Who and how to investigate?
- Renal amyloidosis presenting as glucocorticoid resistant nephrotic syndrome
- Approach to Treating Lupus Nephritis in Children
- All Cysts Are Not Equal
- Diagnosis and Management of the Child with Atypical Peritonitis
- Renal Tubular Acidosis: Let T(r)ooth Be Told…
- Diagnosis and Treatment of Hyperoxaluria Type 1: Old and New
- Management of a Child with Posterior Urethral Valves
- Evaluation and Management of a Child with a Neurogenic Bladder
- Nephro-Uro Crosstalk – A Nephrologist’s Perspective on Bladder Management
- Peritoneal Dialysis in low resource settings – pearls and pitfalls
- Renovascular hypertension in children
- Challenges in diagnosis and treatment of lupus nephritis in a lower-middle income country
- Small kidneys at birth: implications into adulthood
Rukshana Shroff
UK
Neha Bhongale
India
Jyoti Sharma
India
John Mahan
USA
Sukanya Govindan (Moderator)
India
Oluseyi Oniyangi
Nigeria
Gabriel Obiorah Ezeh
Nigeria
Shaegan Irusen
South Africa
Priya Pais
India
Dept of Pediatric Nephrology,St John's National Academy of Health Sciences, Bangalore, India
Soumya Reddy
India
Paul Goodyer
Canada
Elena Levtchenko
Belgium
Larisa Prikhodina
Russia
Svetlana Papizh
Russia
Felicia Eke
Nigeria
Ifeoma Anochie
Nigeria
Frederick Kaskel
USA
James Pullman
USA
Kimberly Reidy
USA
Nicole Hayde
USA
Pooja G N
India
Marcelo Orias
USA
Indira Agarwal
India
Rouba Garro
USA
Naorem Lakshmee Devi
India
Nadira Sultana
Bangladesh
Ng Kar Hui
Singapore
Syed Saimul Huque
Bangladesh
Fiona Mackie (Moderator)
Australia
Rajiv Sinha
India
Sanjukta Poddar
India
Mordi Mourah
UK
Asheeta Gupta
UK
Alexey N. Tsygin
Russia
Komiljon Khamzaev
Uzbekistan
Fayzakhmatova Feruza
Uzbekistan
Arvind Bagga
India
Iftikhar Ijaz
Pakistan
Zubair Hussain
Pakistan
Mignon McCulloch
South Africa
Felicia U. Ek
Nigeria
Tochi Uchenwa
Nigeria
Miriam Harel
USA
Lorna Lourdes Simangan
Philippines
Marnelle Gay Yandra Aramburo
Philippines
Gayathri Raman
Australia
Aniruddh Deshpande
Australia
Eugene Yu Hin Chan
Hong Kong
Natasa Stajic
Serbia
Jelena Stojanovic
UK
Marina Peric
Serbia
Hui Kim Yap
Singapore
Judith Exantus
Haiti
Toussaint Jeanne d’Arc
Haiti
Aditya Pawar
USA
Valerie Luyckx
Switzerland
Nancy Rodig
USA
Onex Ton-Aimé
Haiti
Jaime Restrepo
Colombia
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Approach to Treating Lupus Nephritis in Children
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Kidney involvement is common in lupus and the prognosis depends upon the underlying pathology and optimal and early immunosuppressive treatment. The approach should be based on kidney biopsy data, kidney function and immune tests. Different immunosuppressive regimens are indicated in pathology classes III to V based on steroids, cyclophosphamide, MMF and AZA. Monoclonal antibodies as Rituximab and Belimumab are extensively used in the last decades. The results of clinical trials and guidelines will be discussed.
C3 Glomerulonephritis and Dense Deposit Disease – A Tale of Two Twins
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C3 glomerulopathy is defined by the Consensus Group 2013 as a rare group of kidney disorders due to complement dysregulation. The single diagnostic criteria is dominant C3 deposition in kidney with clinical features of glomerulonephritis (GN). It includes both Dense Deposit Disease (DDD) and C3 GN which have overlapping clinicopathological features. Electron microscopy is necessary to distinguish both. Investigations include tests for complements, antibodies to complement regulating proteins, C3 NeF and genetic analysis. 70% of children and 30 to 50% of adults reach ESKD in 10 years. No treatment is universally effective, though immunosuppression may help.
A tale of two pathologies: Rapidly Progressive Glomerulonephritis and Acute Tubulointerstitial Nephritis
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Immunosuppression vs post-transplant viral infections: Walking a fine line
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BK virus (BKV) is a common infection after kidney transplant (KT), with rising prevalence in the era of more potent immunosuppression. Uncontrolled, BKV infection can lead to BK-associated nephropathy, irreversible damage, and graft loss. Concurrent BKV nephropathy and acute rejection is not uncommon, and treatment is challenging. We discuss the case of a child who presented three years after living donor KT with fever, weight loss, GI symptoms, and acute graft dysfunction. Virology screen revealed CMV, BKV, and EBV viremia. Kidney biopsy showed acute cellular rejection. Optimized immunosuppression and antiviral therapy resulted in resolution of viremia and nearly baseline creatinine.
A Challenging Case of Nephrotic Syndrome
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Nephrotic syndrome refractory to standard treatment poses a challenging problem to paediatric nephrologists. Management issues involve many inter-dependent factors, including fluid status, kidney function, nutrition, infection and thrombosis risks. We will present a case of a child with primary nephrotic syndrome refractory to standard treatment. This session will address common management challenges of a nephrotic child, immunosuppressive strategies and general approach to genetic testing.
The Spectrum of Alport Syndrome: Who and how to investigate?
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Early diagnosis and pharmacologic intervention can delay progression of chronic kidney disease and the onset of kidney failure in patients with Alport syndrome. We aim to highlight the phenotypic spectrum of this condition through a series of cases. We will cover key updates in diagnostics with a focus on genomics, as well as longer term surveillance and management of patients with Alport syndrome.
Renal amyloidosis presenting as glucocorticoid resistant nephrotic syndrome
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Amyloidosis is a rare multi-system disease. In children, amyloid A (AA) amyloidosis (previously known as reactive or secondary amyloidosis) may occur due to chronic inflammation. Pediatric AA amyloidosis mainly involves the kidneys and presents with proteinuria, hematuria, hypertension and nephrotic syndrome. Therapy for AA amyloidosis is targeted towards control of the underlying inflammatory disease and suppression of serum amyloid protein production.
In this webinar, we will present a case of renal amyloidosis, successfully treated with IL-6 antibody.
All Cysts Are Not Equal
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“Simple cysts” are extremely rare in children and any cyst in the kidney of a child should be appropriately evaluated. Major clinical entities include multicystic dysplastic kidney, juvenile nephronopthisis, glomerulocystic kidney and Polycystic kidney diseases.
Clinical case: A 12-year-old, male, JE, was referred from peripheral hospital with recurrent fever and abdominal pain of 4 and 1 month(s) respectively. Accompanying abdominal ultrasound report showed diffuse renal cysts which was confirmed on repeat scan. Some cysts contained debris suggestive of haemorrhage or infection. Autosomal Dominant Polycystic Kidney Disease (ADPKD) was suspected and both parents had KUB ultrasound scan done. Mother had bilateral multiple cysts. Genetic work up is not feasible in our center. Child had 100mls of serous aspirate drained from multiple locules Child’s BP remained normal (between 50th -90th). Fever persisted for 3 weeks and finally settled on Piperacin-Tozobactam. ADPKD, though rare in children, should be suspected if diffuse cysts without dysplasia can be demonstrated in either parent even in areas without facilities for genetic testing so that maximal anticipatory care could be instituted. Recalcitrant infected cyst(s) should be aggressively treated.
Learning Objectives:
How to approach kidney cyst in a child
When to suspect and diagnose Autosomal Dominant Polycystic Kidney Disease in children
Discuss anticipatory care, monitoring and complications of ADPKD in children
Diagnosis and Management of the Child with Atypical Peritonitis
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Peritoneal dialysis (PD)-related peritonitis is one of the most frequent and severe complications of PD. In this webinar, we present a case of fungal peritonitis and discuss the literature on fungal peritonitis and peritonitis caused by atypical organisms and their impact on patient outcomes. A high index of suspicion is required to recognise peritonitis caused by atypical organisms and implement appropriate treatment in a timely manner.
A delay in diagnosis and treatment can lead to peritoneal damage, necessitating the need for PD catheter removal and may even be life-threatening.
Learning objectives:
Understand when to suspect atypical peritonitis
Assessment of appropriate diagnostic strategy
To acquire knowledge towards high-risk patients management
Prevention and treatment including PD catheter removal where necessary
Renal Tubular Acidosis: Let T(r)ooth Be Told…
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We present 2 children with poor growth, polyuria, dental abnormalities, hypokalemia, and hyperchloremic metabolic acidosis. Detailed evaluation suggested the diagnosis of rate-dependent distal renal tubular acidosis with bilateral medullary nephrocalcinosis. There was transient proximal tubular dysfunction with hypophosphatemia and aminoaciduria. Genetic screening helped clarify the clinical diagnosis, and enable genetic counselling.
Diagnosis and Treatment of Hyperoxaluria Type 1: Old and New
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A child presented with a history of multiple kidney stones and end-stage kidney disease. Diagnostic evaluation demonstrated hyperoxaluria type 1. We will review the different clinical scenarios when one should suspect hyperoxaluria type 1 and how to make the diagnosis. Finally, we will discuss therapeutic options, including pyridoxine, liver transplantation and lumasiran, a recent advance in the treatment of hyperoxaluria type 1.
Learning objectives:
Describe 3 different clinical presentations of hyperoxaluria type 1.
Plan the diagnostic strategy for a patient with suspected hyperoxaluria type 1.
Explain how lumasiran decreases liver oxalate production in hyperoxaluria type 1.
Management of a Child with Posterior Urethral Valves
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The case of a 21-year-old boy diagnosed with posterior urethral valves (PUV) at one month and had valve ablation at two months of age will be presented during the webinar. He has been showing lately with deterioration of kidney function and bladder outlet obstruction. We will review the immediate and long-term management of posterior urethral valves. Our urologist will discuss urology management and our pediatric nephrologist will provide input about nephrology management and collaboration with the pediatric urology team.
Learning objectives:
Recognize the importance of joint pediatric urology and nephrology management of posterior urethral valves.
Understand the unique complications of PUV.
Describe the management of CKD secondary to PUV
Evaluation and Management of a Child with a Neurogenic Bladder
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A neurogenic bladder may result from several causes, such as spina bifida, sacral agenesis, anorectal and cloacal malformations, spinal cord injury, transverse myelitis and sometimes as a part of a progressive systemic neurologic disorder. Every pediatric nephrologist should be equipped with knowledge about the close monitoring and management of children with neurogenic bladder (and bowel) to be able to prevent, recurrent urinary tract infections (UTIs), irreversible deterioration of kidney function and urinary and bowel incontinence. This requires a multidisciplinary team approach.
In this webinar a nephro-urology duo discuss the investigations and management of a child with sacral agenesis, including discussions on the adaptations that may be considered in low-resource settings.
Learning objectives:
Recognize the child with a neurogenic bladder
Advise relevant investigations
Discuss the management options
Further reading:
Brownrigg N, Lorenzo AJ, Rickard M, Dos Santos J. The urological evaluation and management of neurogenic bladder in children and adolescents-what every pediatric nephrologist needs to know. Pediatr Nephrol. 2023 Jul 31. doi: 10.1007/s00467-023-06064-z
Fairchild RJ, Aksenov LI, Hobbs KT et al (2022) Medical management of neurogenic bladder in patients with spina bifida: a scoping review. J Pediatr Urol 19:55–63
Nephro-Uro Crosstalk – A Nephrologist’s Perspective on Bladder Management
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This webinar addresses bowel and bladder dysfunction, common in children with conditions like vesical-ureteric reflux (VUR), leading to recurrent urinary tract infections that can damage the kidneys and accelerate the decline to kidney failure. The session focuses on a case of complicated infections, discussing practical aspects of urotherapy, bowel management, and our current approach to bladder management in children with VUR.
Learning objectives:
Understand the Role of Bowel and Bladder Dysfunction as a Risk Factor for Recurrent Urinary Tract Infections
Grasp General Principles for Managing Recurrent Urinary Tract Infections in Children to prevent Kidney Failure
Develop Practical Skills in Performing Urotherapy
Gain Proficiency in Bowel Management for the Pediatric Nephrologist
Further reading:
Nieuwhof-Leppink AJ, Hussong J, Chase J, Larsson J, Renson C, Hoebeke P, Yang S, von Gontard A. Definitions, indications and practice of urotherapy in children and adolescents: – A standardization document of the International Children’s Continence Society (ICCS). J Pediatr Urol. 2021 Apr;17(2):172-181. doi: 10.1016/j.jpurol.2020.11.006. Epub 2020 Nov 5. PMID: 33478902.
Yang, S., Chua, M.E., Bauer, S. et al. Diagnosis and management of bladder bowel dysfunction in children with urinary tract infections: a position statement from the International Children’s Continence Society. Pediatr Nephrol 33, 2207–2219 (2018). https://doi.org/10.1007/s00467-017-3799-9
Klijn AJ, Asselman M, Vijverberg MA, Dik P, de Jong TP. The diameter of the rectum on ultrasonography as a diagnostic tool for constipation in children with dysfunctional voiding. J Urol. 2004 Nov;172(5 Pt 1):1986-8. doi: 10.1097/01.ju.0000142686.09532.46. PMID: 15540772.
Peritoneal Dialysis in low resource settings – pearls and pitfalls
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Explore the intricacies of Peritoneal Dialysis in resource-limited settings through an insightful session titled “Peritoneal Dialysis in low resource settings – pearls and pitfalls.” Delve into the nuances of Acute Peritoneal Dialysis (PD) with a comprehensive overview, specifically focusing on pediatric Acute Kidney Injury (AKI). The session includes a compelling case study on Haemolytic Uraemic Syndrome presented by Handsome Deksiso, followed by expert insights into the pearls of Acute PD shared by Mignon McCulloch.
Additionally, the discussion extends to the practical aspects of using acute PD catheters, including insights into locally made fluid. Explore troubleshooting techniques and gain valuable knowledge on Continuous Flow PD (CFPD) led by Peter Nourse. The session addresses common catheter issues, providing a platform to discuss essential concepts related to CFPD. Join us as we navigate through the challenges and best practices associated with Peritoneal Dialysis in settings with limited resources.
Learning objectives:
Understand the indications and techniques for implementing Acute Peritoneal Dialysis (PD) in the context of Acute Kidney Injury (AKI).
Gain insights into the different types and applications of Acute PD catheters.
Explore improvisation strategies for situations where there is a lack of available fluid and catheters for Acute PD.
Acquire troubleshooting skills specific to Peritoneal Dialysis in the context of AKI, addressing common challenges and complications.
Explore the principles and applications of Continuous Flow Peritoneal Dialysis, understanding its role and significance in clinical practice.
Further reading:
Nourse P, Cullis B, Finkelstein F, Numanoglu A, Warady B, Antwi S, McCulloch M. ISPD guidelines for peritoneal dialysis in acute kidney injury: 2020 Update (paediatrics). Perit Dial Int. 2021 Mar;41(2):139-157. doi: 10.1177/0896860820982120. Epub 2021 Feb 1. PMID: 33523772.
Renovascular hypertension in children
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Open to view video. | Closed captions available
Primary arterial hypertension is an emerging problem in the pediatric population and is often seen in obese children. Among the patients with secondary hypertension, renovascular causes are the third most common cause and are the cause of high blood pressure in 5 – 25% of hypertensive children. Renovascular hypertension is most associated with fibromuscular dysplasia, starts early in life, and can be asymptomatic for years making it challenging for diagnosis. We will present a patient with renovascular hypertension who initially started treatment in Serbia and continued at Great Ormond Street Hospital for Children where the patient was subsequently, successfully treated.
Learning objectives:
Demonstrate effective techniques for presenting and conducting investigations on patients diagnosed with renovascular hypertension.
Evaluate and compare the medical, endovascular, and surgical treatment options available for managing pediatric patients afflicted with renovascular hypertension.
Analyze the clinical outcomes and devise strategies for long-term follow-up care in pediatric patients undergoing treatment for renovascular hypertension.
Further reading:
Patel P. and Stojanovic J. Diagnosis and treatment of renovascular disease. Roentgenol. 2019 Oct;54(4):367-383.
Kurt-Sukur E, Brennan E, Davis M, Forma C, Hamilton G, Kessaris N, Marks S, McLaren C, Minhas K, Patel P, Roebuck D, Stojanovic J, Stuart S, Tullus K. Presentation, treatment, and outcome of renovascular hypertension below 2 years of age. Eur J Pediatr. 2022 Sep;181(9):3367-3375.PMID:35792951
Redhead ECG, Paessler A, Arslan Z, Patel P, Minhas K, Forman C, Hollis P, Lava S, Ionescu F, Manuel D, Ray S, Kessaris N, Giardini A, Ratnamma V, Dobby N, Tullus K, Simmonds J, Stojanovic J. Pediatr Nephrol. Cardiovascular outcomes improve in children with renovascular hypertension following endovascular and surgical interventions 2024 Feb;39(2):521-530. PMID:37658875
Arslan Z, Patel P, Minhas K, Tullus K, Stojanovic J. Importance of the arc of Riolan in children with severe middle aortic syndrome. Dis Child. 2021 April 14
Mudalige N, Ranasinghe C, Stojanovic J. The Clinical and Radiological Cerebrovascular Abnormalities Associated with Renovascular Hypertension in Children: A systematic review. Nephrol. 2021 Jul 8
Tullus K, Brennan E, Hamilton G, Lord R, McLaren CA, Marks SD, Roebuck DJ. Renovascular hypertension in children. Lancet. 2008 Apr 26;371(9622):1453-63. doi: 10.1016/S0140-6736(08)60626-1. PMID: 18440428.
Salice P, Mircoli L, Butera G, Burdick L, Borzani I, Mastrangelo A, Ardissino G, Beretta C, Ferraresso M, Ughi L, Montini G, Carugo S, Morganti A. Percutaneous cutting balloon angioplasty for the treatment of renovascular hypertension in children and adolescents. J Hypertens. 2022 Oct 1;40(10):1902-1908. doi: 10.1097/HJH.0000000000003162. Epub 2022 Aug 18. PMID: 35983863.
de Oliveira Campos JL, Bitencourt L, Pedrosa AL, Silva DF, Lin FJJ, de Oliveira Dias LT, Simões E Silva AC. Renovascular hypertension in pediatric patients: update on diagnosis and management. Pediatr Nephrol. 2021 Dec;36(12):3853-3868. doi: 10.1007/s00467-021-05063-2. Epub 2021 Apr 13. PMID: 33851262.
Challenges in diagnosis and treatment of lupus nephritis in a lower-middle income country
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Open to view video. | Closed captions available
Systemic lupus erythematosus is a lifelong multi-systemic autoimmune condition. Kidney involvement occurs in over 50% of children and treatment decisions are guided by histological classification. However,kidney biopsies and their histological interpretation is not available in some LMIC such as Haiti where it may be impossible to get histopathology on a kidney biopsy and where regular immunological testing may not be easily available. The aim of this webinar is to discuss the management of a child with lupus nephritis, relying more on clinical examination than advanced imaging tests. We present an adolescent girl with florid systemic lupus erythematosus and discuss the management of complex lupus nephritis.
Learning objectives:
Diagnose Lupus Nephritis in Low- and Middle-Income Countries (LMICs): Identify and apply diagnostic criteria and techniques suitable for resource-limited settings.
Adapt Treatment Protocols for Lupus Nephritis Based on Medication Availability: Develop and adjust treatment regimens considering the availability and accessibility of medications in LMICs.
Evaluate Outcomes and Implement Follow-Up Strategies for Patients with Lupus Nephritis: Assess patient outcomes and establish effective follow-up protocols to monitor disease progression and treatment response.
Small kidneys at birth: implications into adulthood
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Open to view video. | Closed captions available
It has been well recognized that individuals born with low birth weight, small for gestation age and/or delivered prematurely are at increased risk for the development of hypertension and chronic kidney disease (CKD). The basis of this risk is believed to be related to disruption of developmental programming leading to reduced nephron number.
Supportive measures and treatments that promote renal health and retard progression of CKD are broadly available. However, many individuals who are at-risk based upon their birth history do not receive adequate screening to permit early detection and intervention.
We will present the case of a teenager who was delivered both prematurely and at a very low birth weight and subsequently developed CKD and hypertension.
We will then review the relationship of abnormal birth history and subsequent kidney disease and hypertension. We will discuss the importance of identifying at-risk individuals and ensuring disease screening during childhood and adulthood. We will also discuss the opportunity to reduce the risk of preterm birth and abnormal intrauterine growth by optimizing maternal health, thereby reducing the burden of CKD globally
Learning objectives:
Being born too early (preterm) or too small (small for gestational age) are risk factors for longer term hypertension, kidney disease, cardiovascular disease and pregnancy complications
Preterm birth and being small for gestational age are associated with smaller kidneys with fewer nephrons.
Being born with smaller kidneys constitutes a first kidney “hit” which enhances vulnerability further kidney “hits”
Long-term follow up of at-risk infants is advised to permit early diagnosis, and intervention to prevent or delay kidney disease
Addressing the Social Determinants of Health across the life course is important to reduce the cycle of risk
Further reading:
Bianchi, M. E., & Restrepo, J. M. (2022). Low birthweight as a risk factor for non-communicable diseases in adults. Frontiers in medicine, 8, 793990.
Iyengar, A., Nesargi, S., George, A., Sinha, N., Selvam, S., & Luyckx, V. A. (2016). Are low birth weight neonates at risk for suboptimal renal growth and function during infancy?. BMC nephrology, 17, 1-9.
Luyckx, V. A., Shukha, K., & Brenner, B. M. (2011). Low nephron number and its clinical consequences. Rambam Maimonides medical journal, 2(4).
Weight, The Low Birth, and Nephron Number Working Group. “The impact of kidney development on the life course: a consensus document for action.” Nephron. Clinical Practice 136.1 (2017): 3.
Anna Gjerde et al. Intrauterine growth restriction and risk of diverse forms of kidney disease during the first 50 years of life. CJASN 2020.