Grand Rounds in Pediatric Nephrology

Recorded On: 05/03/2021

  1. A tale of two pathologies: Rapidly Progressive Glomerulonephritis and Acute Tubulointerstitial Nephritis
  2. Nephropathic Cystinosis: How to Diagnose and Treat
  3. Make the Peritoneal Membrane Work for You
  4. Approach to Infantile Hypercalcemia
  5. Pediatric Renal Genetics: New Advances 
  6. Management of Renal Bone Disease
  7. Resistant Hypertension in Children on Dialysis 
  8. C3 Glomerulonephritis and Dense Deposit Disease – A Tale of Two Twins
  9. Immunosuppression vs post-transplant viral infections: Walking a fine line
  10. A Challenging Case of Nephrotic Syndrome
  11. The Spectrum of Alport Syndrome: Who and how to investigate?
  12. Renal amyloidosis presenting as glucocorticoid resistant nephrotic syndrome
  13. Approach to Treating Lupus Nephritis in Children
  14. All Cysts Are Not Equal
  15. Diagnosis and Management of the Child with Atypical Peritonitis
  16. Renal Tubular Acidosis: Let T(r)ooth Be Told…
  17. Diagnosis and Treatment of Hyperoxaluria Type 1: Old and New
  18. Management of a Child with Posterior Urethral Valves
  19. Evaluation and Management of a Child with a Neurogenic Bladder
  20. Nephro-Uro Crosstalk – A Nephrologist’s Perspective on Bladder Management

Rukshana Shroff

UK

Neha Bhongale

India

Jyoti Sharma

India

John Mahan

USA

Sukanya Govindan

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

Sukanya Govindan (Moderator)

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

Priya Pais

India

Iftikhar Ijaz

Pakistan

Zubair Hussain

Pakistan

Mignon McCulloch

South Africa

Felicia U. Ek

Nigeria

Tochi Uchenwa

Nigeria

Kimberly Reidy

USA

Miriam Harel

USA

Lorna Lourdes Simangan

Philippines

Marnelle Gay Yandra Aramburo

Philippines

Gayathri Raman

Australia

Aniruddh Deshpande

Australia

Key:

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Approach to Treating Lupus Nephritis in Children
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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.
Resistant Hypertension in Children on Dialysis
Open to view video.
Open to view video.
Management of Renal Bone Disease
Open to view video.
Open to view video.
Pediatric Renal Genetics: New Advances
Open to view video.
Open to view video.
Nephropathic Cystinosis: How to Diagnose and Treat
Open to view video.
Open to view video.
Approach to Infantile Hypercalcemia
Open to view video.
Open to view video.
A tale of two pathologies: Rapidly Progressive Glomerulonephritis and Acute Tubulointerstitial Nephritis
Open to view video.
Open to view video.
Make the Peritoneal Membrane Work for You
Open to view video.
Open to view video.
Immunosuppression vs post-transplant viral infections: Walking a fine line
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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?
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. “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
Open to view video.
Open to view video. 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…
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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
Open to view video.
Open to view video. 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.