Based on the information provided, the most likely diagnosis is idiopathic nephrotic syndrome. Some key points:
- The child is 5 years old, which is a common age for nephrotic syndrome to present.
- There is generalized body swelling (edema), hypoalbuminemia (serum albumin 17), heavy proteinuria (4+ on urine dipstick), and hyperlipidemia (cholesterol 12), meeting the criteria for nephrotic syndrome.
- No obvious underlying cause is identified, so it is likely primary/idiopathic in nature.
The main possible etiologies/causes of nephrotic syndrome include minimal change disease, focal segment
The document discusses different types of kidney diseases and disorders:
- It describes the normal anatomy and histology of the kidney, including structures like the glomerulus.
- Nephrotic syndrome is discussed, which is characterized by proteinuria and low albumin levels, causing fluid retention and edema. Investigations include urine tests and kidney biopsies.
- Different glomerular diseases are described based on their pathology, including membranous glomerulonephritis, minimal change disease, focal segmental glomerulosclerosis (FSGS), and membranoproliferative glomerulonephritis. Their features on light microscopy, immunofluorescence and electron microscopy are provided.
- IgA nep
Glomerulonephritis is a group of inflammatory diseases of the kidneys with primary defeat of renal glomeruli and subsequent involvement of renal channels, interstitial tissue, and vessels. Infectious agents are the most common inciting antigens associated with immune complex-mediated glomerulonephritis. Post-streptococcal glomerulonephritis is the most common form in children and occurs following a skin or pharyngeal infection with Group A beta-hemolytic streptococci. The immune deposits are usually located on the subepithelial aspect of the glomerular basement membrane and are associated with an increase in mesangial cellularity. The major pathogenic categories are inflammatory (nephritic
Minimal Change Disease (MCD) is a type of nephrotic syndrome characterized by proteinuria and swelling without significant findings on light microscopy. It is caused by a circulating permeability factor that leads to foot process effacement on electron microscopy. MCD is most common in children and responds well to steroid treatment, though relapses are common. Second line treatments include cyclophosphamide or rituximab if relapses are frequent or the patient is steroid dependent.
1. Acute pyelonephritis is a sudden, severe bacterial infection of the kidneys that commonly affects young women. It is caused by bacteria like E. coli traveling from the bladder to the kidneys.
2. Symptoms include fever over 102°F, flank pain, painful or frequent urination, and other urinary symptoms. Risk factors include female anatomy, kidney stones, diabetes, and other underlying conditions.
3. Diagnosis involves urine and blood tests showing white blood cells and bacteria. Imaging tests can confirm infection in the kidneys. Treatment consists of intravenous or oral antibiotics like ciprofloxacin or trimethoprim/sulfamethoxazole for 5-14 days.
Minimal change disease (MCD) is a histologic pattern seen on kidney biopsy that is characterized by nephrotic syndrome without immune deposits or inflammation. It is most common in children but can occur in adults. The pathogenesis involves circulating permeability factors that cause foot process effacement and proteinuria. MCD is highly responsive to steroids, with remission occurring in 80-85% of adults, though relapses are common. Treatment involves corticosteroids, with slower tapers needed in adults to minimize relapses. Refractory cases may require additional immunosuppression. The prognosis is generally excellent if the disease is steroid-responsive.
Let's learn the pharmacology related to nephrotic syndrome - features of nephrotic syndrome with underlying mechanisms, objectives of treatment, and management of the nephrotic syndrome.
Chronic glomerulonephritis is characterized by progressive scarring of the glomeruli and kidneys that leads to reduced kidney function over time. If left untreated, it can progress to end-stage renal disease requiring dialysis or kidney transplantation. The rate of progression depends on the underlying cause of the chronic glomerulonephritis. Treatment aims to slow progression through controlling blood pressure and proteinuria with ACE inhibitors, ARBs, and other medications. Dietary modifications and management of complications help preserve kidney function for as long as possible.
This document provides an overview of acute kidney injury (AKI). It discusses the definition, epidemiology, etiology, pathophysiology, diagnosis and treatment of AKI. Some key points:
- AKI accounts for 5-7% of acute care hospital admissions and 30% of ICU admissions, with mortality rates as high as 50%. It can worsen chronic kidney disease and increase the risk of end-stage renal disease.
- Causes include pre-renal issues like hypovolemia, renal issues like acute tubular necrosis, and post-renal issues like obstruction. Diagnosis involves history, physical exam, lab tests of kidney function and imaging.
- Treatment focuses on optimizing
The document discusses different types of kidney diseases and disorders:
- It describes the normal anatomy and histology of the kidney, including structures like the glomerulus.
- Nephrotic syndrome is discussed, which is characterized by proteinuria and low albumin levels, causing fluid retention and edema. Investigations include urine tests and kidney biopsies.
- Different glomerular diseases are described based on their pathology, including membranous glomerulonephritis, minimal change disease, focal segmental glomerulosclerosis (FSGS), and membranoproliferative glomerulonephritis. Their features on light microscopy, immunofluorescence and electron microscopy are provided.
- IgA nep
Glomerulonephritis is a group of inflammatory diseases of the kidneys with primary defeat of renal glomeruli and subsequent involvement of renal channels, interstitial tissue, and vessels. Infectious agents are the most common inciting antigens associated with immune complex-mediated glomerulonephritis. Post-streptococcal glomerulonephritis is the most common form in children and occurs following a skin or pharyngeal infection with Group A beta-hemolytic streptococci. The immune deposits are usually located on the subepithelial aspect of the glomerular basement membrane and are associated with an increase in mesangial cellularity. The major pathogenic categories are inflammatory (nephritic
Minimal Change Disease (MCD) is a type of nephrotic syndrome characterized by proteinuria and swelling without significant findings on light microscopy. It is caused by a circulating permeability factor that leads to foot process effacement on electron microscopy. MCD is most common in children and responds well to steroid treatment, though relapses are common. Second line treatments include cyclophosphamide or rituximab if relapses are frequent or the patient is steroid dependent.
1. Acute pyelonephritis is a sudden, severe bacterial infection of the kidneys that commonly affects young women. It is caused by bacteria like E. coli traveling from the bladder to the kidneys.
2. Symptoms include fever over 102°F, flank pain, painful or frequent urination, and other urinary symptoms. Risk factors include female anatomy, kidney stones, diabetes, and other underlying conditions.
3. Diagnosis involves urine and blood tests showing white blood cells and bacteria. Imaging tests can confirm infection in the kidneys. Treatment consists of intravenous or oral antibiotics like ciprofloxacin or trimethoprim/sulfamethoxazole for 5-14 days.
Minimal change disease (MCD) is a histologic pattern seen on kidney biopsy that is characterized by nephrotic syndrome without immune deposits or inflammation. It is most common in children but can occur in adults. The pathogenesis involves circulating permeability factors that cause foot process effacement and proteinuria. MCD is highly responsive to steroids, with remission occurring in 80-85% of adults, though relapses are common. Treatment involves corticosteroids, with slower tapers needed in adults to minimize relapses. Refractory cases may require additional immunosuppression. The prognosis is generally excellent if the disease is steroid-responsive.
Let's learn the pharmacology related to nephrotic syndrome - features of nephrotic syndrome with underlying mechanisms, objectives of treatment, and management of the nephrotic syndrome.
Chronic glomerulonephritis is characterized by progressive scarring of the glomeruli and kidneys that leads to reduced kidney function over time. If left untreated, it can progress to end-stage renal disease requiring dialysis or kidney transplantation. The rate of progression depends on the underlying cause of the chronic glomerulonephritis. Treatment aims to slow progression through controlling blood pressure and proteinuria with ACE inhibitors, ARBs, and other medications. Dietary modifications and management of complications help preserve kidney function for as long as possible.
This document provides an overview of acute kidney injury (AKI). It discusses the definition, epidemiology, etiology, pathophysiology, diagnosis and treatment of AKI. Some key points:
- AKI accounts for 5-7% of acute care hospital admissions and 30% of ICU admissions, with mortality rates as high as 50%. It can worsen chronic kidney disease and increase the risk of end-stage renal disease.
- Causes include pre-renal issues like hypovolemia, renal issues like acute tubular necrosis, and post-renal issues like obstruction. Diagnosis involves history, physical exam, lab tests of kidney function and imaging.
- Treatment focuses on optimizing
This document discusses acute kidney injury (AKI), including its definition, diagnosis criteria, epidemiology, classification, pathogenesis, etiology, treatment, and management. AKI is defined as an abrupt reduction in kidney function, diagnosed by changes in serum creatinine, BUN, and urine output. Between 5-7% of hospitalized patients and a greater percentage of ICU patients develop AKI. Mortality from AKI exceeds 50% despite improvements in care. AKI is classified using criteria like RIFLE, AKIN, and KDIGO which consider risk, injury, failure, and loss of kidney function. Causes include prerenal issues like dehydration, intrinsic renal damage, and postrenal obstruction
The document provides an outline for pathology lectures on nephrotic syndrome, nephritic syndrome, rapidly progressive glomerulonephritis (RPGN), and chronic kidney disease (CKD). It discusses the objectives, key topics, and clinical manifestations of these conditions. It then focuses on RPGN/crescentic glomerulonephritis, describing the types (I-III) based on etiology, light microscopy findings, and clinical features. Finally, it covers CKD/CRF, discussing common causes, clinical features, and morphology under light microscopy of end-stage kidneys.
1. Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems.
2. SLE is characterized by the production of autoantibodies directed against cell nuclei.
3. Treatment of lupus nephritis involves induction therapy with corticosteroids and cytotoxic agents to control disease activity, followed by long-term maintenance treatment to prevent flares and preserve renal function.
This document provides information about pyelonephritis (kidney infection) including:
1. It defines pyelonephritis as an infection of the kidney and ureters that can be life-threatening.
2. Causes include urinary tract infections ascending from the bladder or entering via the bloodstream.
3. Symptoms vary depending on age but usually include fever, flank pain, nausea, and urinary symptoms.
4. Diagnosis involves urinalysis, urine culture, blood tests and imaging. Treatment is usually antibiotics selected based on likely causative organisms.
Minimal Change Disease (MCD), also known as Minimal Change Nephropathy, is a kidney disorder characterized by diffuse effacement and loss of foot processes in the glomeruli seen on electron microscopy. It is the most common cause of nephrotic syndrome in children aged 1-7 years and accounts for about 90% of cases. Boys are more commonly affected than girls. MCD is thought to be caused by an abnormal immune response involving T-cells and cytokines that damages the glomerular filtration barrier. This results in proteinuria but no significant pathology on light microscopy. The clinical features include nephrotic syndrome. The prognosis is generally good as over 90% will respond to corticosteroid therapy
This document provides a review of renal amyloidosis. It begins by defining amyloidosis as a group of diseases caused by the misfolding and accumulation of various proteins. 27 human proteins are known to cause amyloidosis. The kidney is a common site of deposition for several types of amyloidosis. The document reviews the pathogenesis of amyloidosis, determinants of renal deposition, how it causes renal disease, classification, epidemiology including statistics from India, pathology findings including staining techniques, and methods to determine the type of amyloidosis involved.
This document discusses glomerular diseases, including their classification, clinical manifestations, and pathogenesis. It covers the following key points:
- Glomerular diseases are classified as primary (involving the glomeruli predominantly) or secondary (affecting the glomeruli due to systemic/hereditary diseases).
- Major clinical manifestations include proteinuria, hematuria, hypertension, and impaired renal function. The main glomerular syndromes discussed are nephritic, nephrotic, acute/chronic renal failure, and asymptomatic proteinuria/hematuria.
- The pathogenesis involves immunological mechanisms like antibody-mediated immune complex deposition, as well as non-immunological mechanisms. Injury
Chronic glomerulonephritis is a kidney disorder caused by slow, cumulative damage and scarring of the glomeruli, or tiny blood filters in the kidneys, usually due to inflammation. This results in reduced kidney function over time and can lead to chronic kidney disease, end-stage renal disease, cardiovascular disease, renal failure, and death if left untreated. Treatment focuses on slowing disease progression, managing symptoms like high blood pressure and fluid retention, and renal replacement therapy with dialysis or kidney transplantation for kidney failure.
Pancreatitis is an inflammation of the pancreas that can be acute or chronic. Acute pancreatitis is often caused by gallstones blocking the pancreatic duct or heavy alcohol use, while chronic pancreatitis results from repeated acute attacks or long-term alcohol abuse. Symptoms include abdominal pain radiating to the back, tender swollen abdomen, fever, nausea, and increased heart rate. Blood tests measuring amylase and lipase levels indicate pancreatitis. Treatment focuses on pain management, intravenous fluids, dietary changes like a low-fat diet, and potentially surgery to drain fluid or remove diseased tissue. Maintaining a healthy diet and lifestyle through modifications can help manage and prevent pancreatitis.
Progressive damage to the nephrons and glomeruli causes chronic renal failure, leading to a decreased glomerular filtration rate and tubular dysfunction. As kidney function declines, waste products accumulate and patients eventually require dialysis or transplantation. Dialysis modalities include peritoneal dialysis, which uses the peritoneal membrane for diffusion and ultrafiltration, and hemodialysis, which uses an external dialyzer. Post-transplant complications include hyperacute, acute, and chronic rejection as the immune system attacks the donor kidney. Careful immunosuppression management aims to prevent rejection while monitoring for other complications.
This document provides an overview of acute kidney injury (AKI), formerly known as acute renal failure. It discusses the definition and epidemiology of AKI and describes the main causes as pre-renal, intrinsic renal, and post-renal. Pre-renal AKI is the most common type and is caused by reduced renal blood flow. The document outlines the diagnostic evaluation, complications, treatment approaches including dialysis indications, and outcomes of AKI. It emphasizes the importance of identifying and eliminating nephrotoxic agents to optimize management of this condition.
This document provides an overview of nephritic and nephrotic syndrome, describing their pathophysiology and clinical features. Nephritic syndrome is characterized by inflammation of the glomeruli, resulting in hematuria, hypertension, and mild proteinuria. Glomerulonephritis causes include post-streptococcal and rapidly progressive crescentic glomerulonephritis. Nephrotic syndrome is caused by increased glomerular permeability, leading to massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Specific causes discussed include minimal change disease, membranous nephropathy, focal segmental glomerulosclerosis, and membranoproliferative glomerulone
This document discusses acute kidney injury (AKI) in patients with sepsis. It recommends using isotonic crystalloids for initial volume expansion in at-risk patients and those with AKI. Albumin may have a role in patients requiring substantial crystalloid volumes or those with low serum albumin. Sodium bicarbonate administration has failed to improve hemodynamics in human studies of lactic acidosis and may increase risks. Guidelines are missing data on albumin benefits in low albumin patients and bicarbonate risks.
lupus nephritis is a autoimmune disease, commonly seen in adult and child and the medical or nursing care is also very important for this type of disease condition.
This document provides an overview of acute glomerulonephritis (AGN) and several related kidney conditions. It discusses the pathogenesis, pathology, clinical manifestations, diagnosis, treatment, and prognosis of AGN and other glomerular diseases including post-streptococcal glomerulonephritis, membranous glomerulonephritis, membranoproliferative glomerulonephritis, lupus nephritis, Henoch-Schonlein purpura nephritis, and rapidly progressive glomerulonephritis. Key pathological features, diagnostic tests, and treatment approaches are described for each condition.
Acute Kidney Injury (AKI) is a common complication, affecting 5-7% of hospital admissions and 30% of intensive care unit patients. The top causes of AKI in India are diarrheal diseases, sepsis, malaria, drug toxicity, and hospital-acquired injuries. Biomarkers like cystatin C and kidney injury molecule 1 can help detect AKI earlier than creatinine. Treatment involves fluid resuscitation, eliminating nephrotoxins, and renal replacement therapy for complications like electrolyte imbalances or uremia. Outcomes depend on the underlying cause, with pre-renal and post-renal AKI having a better prognosis than intrinsic renal injury.
This document discusses glomerulonephritis (GN) and nephrotic syndrome. It begins by defining acute glomerulonephritis (AGN) as a set of kidney diseases caused by immunological inflammation and proliferation in the glomeruli. It then covers the classification, etiology, pathophysiology, presentation, workup, treatment and prognosis of AGN. Nephrotic syndrome is defined as proteinuria, hypoalbuminemia, edema and low serum albumin. The pathophysiology, causes, complications and treatment of nephrotic syndrome are also outlined.
Primary glomerular diseases include various forms of glomerulonephritis and nephrotic syndrome. Acute glomerulonephritis is defined by the sudden onset of hematuria, proteinuria, and red blood cell casts. It is caused by an immunological reaction, often due to a streptococcal infection, that results in inflammation and proliferation in the glomerulus. Symptoms include edema, hypertension, and decreased kidney function. Treatment involves a low sodium diet, fluid restriction, antibiotics, corticosteroids, diuretics, and dialysis if needed. With treatment, most cases of acute glomerulonephritis resolve though some may progress to chronic kidney disease.
This document discusses acute kidney injury (AKI), including its definition, diagnosis criteria, epidemiology, classification, pathogenesis, etiology, treatment, and management. AKI is defined as an abrupt reduction in kidney function, diagnosed by changes in serum creatinine, BUN, and urine output. Between 5-7% of hospitalized patients and a greater percentage of ICU patients develop AKI. Mortality from AKI exceeds 50% despite improvements in care. AKI is classified using criteria like RIFLE, AKIN, and KDIGO which consider risk, injury, failure, and loss of kidney function. Causes include prerenal issues like dehydration, intrinsic renal damage, and postrenal obstruction
The document provides an outline for pathology lectures on nephrotic syndrome, nephritic syndrome, rapidly progressive glomerulonephritis (RPGN), and chronic kidney disease (CKD). It discusses the objectives, key topics, and clinical manifestations of these conditions. It then focuses on RPGN/crescentic glomerulonephritis, describing the types (I-III) based on etiology, light microscopy findings, and clinical features. Finally, it covers CKD/CRF, discussing common causes, clinical features, and morphology under light microscopy of end-stage kidneys.
1. Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems.
2. SLE is characterized by the production of autoantibodies directed against cell nuclei.
3. Treatment of lupus nephritis involves induction therapy with corticosteroids and cytotoxic agents to control disease activity, followed by long-term maintenance treatment to prevent flares and preserve renal function.
This document provides information about pyelonephritis (kidney infection) including:
1. It defines pyelonephritis as an infection of the kidney and ureters that can be life-threatening.
2. Causes include urinary tract infections ascending from the bladder or entering via the bloodstream.
3. Symptoms vary depending on age but usually include fever, flank pain, nausea, and urinary symptoms.
4. Diagnosis involves urinalysis, urine culture, blood tests and imaging. Treatment is usually antibiotics selected based on likely causative organisms.
Minimal Change Disease (MCD), also known as Minimal Change Nephropathy, is a kidney disorder characterized by diffuse effacement and loss of foot processes in the glomeruli seen on electron microscopy. It is the most common cause of nephrotic syndrome in children aged 1-7 years and accounts for about 90% of cases. Boys are more commonly affected than girls. MCD is thought to be caused by an abnormal immune response involving T-cells and cytokines that damages the glomerular filtration barrier. This results in proteinuria but no significant pathology on light microscopy. The clinical features include nephrotic syndrome. The prognosis is generally good as over 90% will respond to corticosteroid therapy
This document provides a review of renal amyloidosis. It begins by defining amyloidosis as a group of diseases caused by the misfolding and accumulation of various proteins. 27 human proteins are known to cause amyloidosis. The kidney is a common site of deposition for several types of amyloidosis. The document reviews the pathogenesis of amyloidosis, determinants of renal deposition, how it causes renal disease, classification, epidemiology including statistics from India, pathology findings including staining techniques, and methods to determine the type of amyloidosis involved.
This document discusses glomerular diseases, including their classification, clinical manifestations, and pathogenesis. It covers the following key points:
- Glomerular diseases are classified as primary (involving the glomeruli predominantly) or secondary (affecting the glomeruli due to systemic/hereditary diseases).
- Major clinical manifestations include proteinuria, hematuria, hypertension, and impaired renal function. The main glomerular syndromes discussed are nephritic, nephrotic, acute/chronic renal failure, and asymptomatic proteinuria/hematuria.
- The pathogenesis involves immunological mechanisms like antibody-mediated immune complex deposition, as well as non-immunological mechanisms. Injury
Chronic glomerulonephritis is a kidney disorder caused by slow, cumulative damage and scarring of the glomeruli, or tiny blood filters in the kidneys, usually due to inflammation. This results in reduced kidney function over time and can lead to chronic kidney disease, end-stage renal disease, cardiovascular disease, renal failure, and death if left untreated. Treatment focuses on slowing disease progression, managing symptoms like high blood pressure and fluid retention, and renal replacement therapy with dialysis or kidney transplantation for kidney failure.
Pancreatitis is an inflammation of the pancreas that can be acute or chronic. Acute pancreatitis is often caused by gallstones blocking the pancreatic duct or heavy alcohol use, while chronic pancreatitis results from repeated acute attacks or long-term alcohol abuse. Symptoms include abdominal pain radiating to the back, tender swollen abdomen, fever, nausea, and increased heart rate. Blood tests measuring amylase and lipase levels indicate pancreatitis. Treatment focuses on pain management, intravenous fluids, dietary changes like a low-fat diet, and potentially surgery to drain fluid or remove diseased tissue. Maintaining a healthy diet and lifestyle through modifications can help manage and prevent pancreatitis.
Progressive damage to the nephrons and glomeruli causes chronic renal failure, leading to a decreased glomerular filtration rate and tubular dysfunction. As kidney function declines, waste products accumulate and patients eventually require dialysis or transplantation. Dialysis modalities include peritoneal dialysis, which uses the peritoneal membrane for diffusion and ultrafiltration, and hemodialysis, which uses an external dialyzer. Post-transplant complications include hyperacute, acute, and chronic rejection as the immune system attacks the donor kidney. Careful immunosuppression management aims to prevent rejection while monitoring for other complications.
This document provides an overview of acute kidney injury (AKI), formerly known as acute renal failure. It discusses the definition and epidemiology of AKI and describes the main causes as pre-renal, intrinsic renal, and post-renal. Pre-renal AKI is the most common type and is caused by reduced renal blood flow. The document outlines the diagnostic evaluation, complications, treatment approaches including dialysis indications, and outcomes of AKI. It emphasizes the importance of identifying and eliminating nephrotoxic agents to optimize management of this condition.
This document provides an overview of nephritic and nephrotic syndrome, describing their pathophysiology and clinical features. Nephritic syndrome is characterized by inflammation of the glomeruli, resulting in hematuria, hypertension, and mild proteinuria. Glomerulonephritis causes include post-streptococcal and rapidly progressive crescentic glomerulonephritis. Nephrotic syndrome is caused by increased glomerular permeability, leading to massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Specific causes discussed include minimal change disease, membranous nephropathy, focal segmental glomerulosclerosis, and membranoproliferative glomerulone
This document discusses acute kidney injury (AKI) in patients with sepsis. It recommends using isotonic crystalloids for initial volume expansion in at-risk patients and those with AKI. Albumin may have a role in patients requiring substantial crystalloid volumes or those with low serum albumin. Sodium bicarbonate administration has failed to improve hemodynamics in human studies of lactic acidosis and may increase risks. Guidelines are missing data on albumin benefits in low albumin patients and bicarbonate risks.
lupus nephritis is a autoimmune disease, commonly seen in adult and child and the medical or nursing care is also very important for this type of disease condition.
This document provides an overview of acute glomerulonephritis (AGN) and several related kidney conditions. It discusses the pathogenesis, pathology, clinical manifestations, diagnosis, treatment, and prognosis of AGN and other glomerular diseases including post-streptococcal glomerulonephritis, membranous glomerulonephritis, membranoproliferative glomerulonephritis, lupus nephritis, Henoch-Schonlein purpura nephritis, and rapidly progressive glomerulonephritis. Key pathological features, diagnostic tests, and treatment approaches are described for each condition.
Acute Kidney Injury (AKI) is a common complication, affecting 5-7% of hospital admissions and 30% of intensive care unit patients. The top causes of AKI in India are diarrheal diseases, sepsis, malaria, drug toxicity, and hospital-acquired injuries. Biomarkers like cystatin C and kidney injury molecule 1 can help detect AKI earlier than creatinine. Treatment involves fluid resuscitation, eliminating nephrotoxins, and renal replacement therapy for complications like electrolyte imbalances or uremia. Outcomes depend on the underlying cause, with pre-renal and post-renal AKI having a better prognosis than intrinsic renal injury.
This document discusses glomerulonephritis (GN) and nephrotic syndrome. It begins by defining acute glomerulonephritis (AGN) as a set of kidney diseases caused by immunological inflammation and proliferation in the glomeruli. It then covers the classification, etiology, pathophysiology, presentation, workup, treatment and prognosis of AGN. Nephrotic syndrome is defined as proteinuria, hypoalbuminemia, edema and low serum albumin. The pathophysiology, causes, complications and treatment of nephrotic syndrome are also outlined.
Primary glomerular diseases include various forms of glomerulonephritis and nephrotic syndrome. Acute glomerulonephritis is defined by the sudden onset of hematuria, proteinuria, and red blood cell casts. It is caused by an immunological reaction, often due to a streptococcal infection, that results in inflammation and proliferation in the glomerulus. Symptoms include edema, hypertension, and decreased kidney function. Treatment involves a low sodium diet, fluid restriction, antibiotics, corticosteroids, diuretics, and dialysis if needed. With treatment, most cases of acute glomerulonephritis resolve though some may progress to chronic kidney disease.
This document defines and compares nephrotic syndrome and nephritic syndrome. It provides details on various causes of glomerular disease including minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, IgA nephropathy, lupus nephritis, anti-GBM disease, and rapidly progressive glomerulonephritis. Evaluation of glomerular disease involves history, exam, urinalysis, and complement levels with renal biopsy used to confirm specific diagnoses.
1. Acute post-streptococcal glomerulonephritis (APSGN) is a type of acute nephritic syndrome that occurs after a streptococcal infection.
2. It is characterized by edema, hematuria, proteinuria, and decreased kidney function.
3. The infection triggers an immune response where antibodies form complexes that deposit along the glomerular basement membrane, causing inflammation and kidney damage.
Acute Poststreptococcal Glomerulonephritis (APSGN) is caused by certain strains of Group A streptococci that infect the throat or skin. It presents with hematuria, edema, hypertension, and sometimes acute renal failure. The kidneys show enlarged glomeruli with immune complex deposition on the glomerular basement membrane. Treatment focuses on supportive care with bed rest, salt restriction, diuretics for hypertension or edema, and antibiotics to limit spread of infection. The condition usually resolves spontaneously within a few weeks.
1. The document discusses glomerular diseases in children, describing the anatomy of the kidneys and glomerulus.
2. It then focuses on acute glomerulonephritis (AGN), the most common form in children which typically follows a streptococcal infection.
3. AGN is characterized by hematuria, proteinuria, edema, hypertension, and other symptoms. It results from immune complexes depositing in the glomerular basement membrane.
The patient, a 23-year-old male, presented with abdominal pain and was found to have palpable purpura on his lower extremities. He was initially diagnosed with acute appendicitis but developed a skin rash. Biopsy of the skin rash showed leukocytoclastic vasculitis. He was ultimately diagnosed with Henoch-Schonlein purpura (HSP), characterized by palpable purpura, arthritis/arthralgia, gastrointestinal involvement, and renal involvement. HSP is an immune complex-mediated vasculitis that commonly affects children and is generally self-limiting, though steroids may help with symptoms. Prognosis depends on factors like age of onset, severity
1) Acute Poststreptococcal Glomerulonephritis (APSGN) is caused by certain strains of Group A streptococci that lead to the formation of immune complexes and activation of the complement system, causing inflammation in the glomeruli.
2) It typically presents with hematuria, edema, hypertension, and sometimes acute renal failure. Treatment involves bed rest, antibiotics, controlling hypertension and edema, and dialysis in severe cases.
3) The prognosis is generally good, with over 95% of patients making a full recovery without long-term effects on kidney function. However, a small percentage can develop chronic kidney disease if the acute phase is severe.
This document discusses systemic lupus erythematosus (SLE), a complex autoimmune disorder with multifactorial origins. It causes activation of T and B cells leading to autoantibody production. The pathogenesis involves genetic, hormonal and environmental factors interacting to cause immune dysregulation and failure of self-tolerance. SLE can affect many organ systems and presents with a variety of clinical manifestations involving the skin, kidneys, joints, blood, and more. Diagnosis is based on identifying 4 out of 11 diagnostic criteria. Investigations help assess disease activity and organ involvement. Treatment involves immunosuppression with corticosteroids and other drugs depending on disease severity and organ involvement.
This document discusses several diseases that present with hematuria or proteinuria in children. It describes a case of acute post-streptococcal glomerulonephritis in a 10-year-old boy presenting with edema and Coca-Cola colored urine. It then provides details on the etiology, clinical presentation, diagnosis, complications and treatment of this condition. It also summarizes several other conditions like IgA nephropathy, Alport syndrome, hemolytic uremic syndrome, polycystic kidney disease, nephrotic syndrome, undescended testes, testicular torsion, and epididymitis.
Nervous. System nephrilogy. System clinicsaneesshahzad3
Nephrotic syndrome is characterized by heavy proteinuria, hypoalbuminemia, edema, and normal kidney function. It has multiple primary and secondary causes, including diseases like membranous glomerulonephritis or diabetes. Patients present with edema, fatigue, and abdominal pain. Laboratory tests show protein in the urine and low serum albumin. Treatment involves addressing the underlying cause, diuretics, and immunosuppressants like prednisone. Complications can include infections, blood clots, and kidney damage if left untreated.
This document provides an overview of nephro-pathological correlation and the approach to kidney disease. It discusses the reasons for referral to a nephrologist, major kidney syndromes, indications for renal biopsy, urinalysis findings including hematuria and proteinuria, causes of glomerular and secondary proteinuria, history and exam findings, and laboratory evaluation for nephritic and nephrotic syndrome. Specific diseases discussed include IgA nephropathy, anti-GBM disease, ANCA-associated vasculitis, cryoglobulinemia, infection-related glomerulonephritis, and FSGS.
Nephrotic syndrome is defined by heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia. It is caused by damage to the glomerular basement membrane that increases pore size and protein leakage. Loss of protein like albumin leads to edema as serum oncotic pressure decreases. Steroids are first line treatment but some patients are steroid resistant. Kidney biopsy guides treatment, which may include immunosuppressants for steroid resistant forms. Complications include infection, hypercoagulability, and renal failure.
Nephrotic syndrome and nephritic syndrome are types of kidney diseases that involve glomerular injury. Nephrotic syndrome is characterized by proteinuria, hypoalbuminemia, edema, and hyperlipidemia. It is often caused by minimal change disease, membranous nephropathy, or focal segmental glomerulosclerosis. Nephritic syndrome involves glomerular inflammation and is characterized by hematuria, hypertension, and decreased kidney function. Post-streptococcal glomerulonephritis is a common cause in children, presenting with hematuria, edema, and low complement levels. Evaluation involves kidney biopsy and lab tests to determine the specific cause and guide treatment options like steroids,
The document discusses nephrotic syndrome and nephritic syndrome. It covers:
- Mechanisms of glomerular injury including proteinuria, hematuria, loss of filtration function, and hypertension.
- Causes of nephrotic syndrome including minimal change disease, membranous nephropathy, and focal segmental glomerulosclerosis which often involve podocyte dysfunction.
- Causes of nephritic syndrome include inflammatory diseases that break the glomerular basement membrane such as post-streptococcal glomerulonephritis.
A 7-year-old boy presented with facial puffiness, decreased urinary output, and fever for one week. Examination found pallor, elevated blood pressure, and a skin lesion on his elbow. Urine tests found protein and red blood cells. Blood tests showed elevated urea and low C3 levels. Ultrasound showed enlarged pale kidneys. He was diagnosed with acute nephritis likely due to a preceding streptococcal infection based on the clinical presentation and serological tests. Treatment focused on controlling blood pressure and supporting kidney function until recovery, which generally occurs within 6-8 weeks.
The document discusses acute glomerulonephritis (AGN), which is an immune-mediated inflammatory disease of the renal glomeruli capillary loops caused by a streptococcal infection. It presents in children ages 2 to 10 most commonly in males. Symptoms include fever, headache, edema, hematuria, oliguria and hypertension. Investigations include urine analysis, blood tests to check kidney function, and throat swab culture. Treatment involves antibiotics, diuretics, vasodilators, and dialysis if renal failure occurs. Nursing management focuses on rest, vital sign monitoring, nutritional support, skin care, parental education, and follow up care.
2. Glomerulonephritis & hypertension in children 01.04.15 lecture.pptxIvwananjisikombe1
Acute glomerulonephritis (AGN) is characterized by edema, hematuria, proteinuria, and hypertension resulting from inflammation of the glomeruli. It is commonly caused by a prior streptococcal infection. The inflammation damages renal tissue through immune complex deposition and cellular proliferation. Treatment focuses on controlling blood pressure and fluid balance. Medical management includes diuretics, ACE inhibitors, calcium channel blockers, and other antihypertensives. Outcomes are generally good if the inflammatory process is self-limited, but progressive kidney damage can occur if inflammation persists long-term.
Rapidly progressive glomerulonephritis in childrenNishatTasnim46
Rapidly progressive or crescentic glomerulonephritis is a medical emergency and diagnostic challenge in paediatric population. There is a significant risk of development of complications such as CKD in the long term. This seminar was prepared to increase knowledge about early diagnosis and management of this condition in a tertiary level hospital.
Executive Directors Chat Leveraging AI for Diversity, Equity, and InclusionTechSoup
Let’s explore the intersection of technology and equity in the final session of our DEI series. Discover how AI tools, like ChatGPT, can be used to support and enhance your nonprofit's DEI initiatives. Participants will gain insights into practical AI applications and get tips for leveraging technology to advance their DEI goals.
A workshop hosted by the South African Journal of Science aimed at postgraduate students and early career researchers with little or no experience in writing and publishing journal articles.
This slide is special for master students (MIBS & MIFB) in UUM. Also useful for readers who are interested in the topic of contemporary Islamic banking.
This presentation was provided by Steph Pollock of The American Psychological Association’s Journals Program, and Damita Snow, of The American Society of Civil Engineers (ASCE), for the initial session of NISO's 2024 Training Series "DEIA in the Scholarly Landscape." Session One: 'Setting Expectations: a DEIA Primer,' was held June 6, 2024.
A review of the growth of the Israel Genealogy Research Association Database Collection for the last 12 months. Our collection is now passed the 3 million mark and still growing. See which archives have contributed the most. See the different types of records we have, and which years have had records added. You can also see what we have for the future.
বাংলাদেশের অর্থনৈতিক সমীক্ষা ২০২৪ [Bangladesh Economic Review 2024 Bangla.pdf] কম্পিউটার , ট্যাব ও স্মার্ট ফোন ভার্সন সহ সম্পূর্ণ বাংলা ই-বুক বা pdf বই " সুচিপত্র ...বুকমার্ক মেনু 🔖 ও হাইপার লিংক মেনু 📝👆 যুক্ত ..
আমাদের সবার জন্য খুব খুব গুরুত্বপূর্ণ একটি বই ..বিসিএস, ব্যাংক, ইউনিভার্সিটি ভর্তি ও যে কোন প্রতিযোগিতা মূলক পরীক্ষার জন্য এর খুব ইম্পরট্যান্ট একটি বিষয় ...তাছাড়া বাংলাদেশের সাম্প্রতিক যে কোন ডাটা বা তথ্য এই বইতে পাবেন ...
তাই একজন নাগরিক হিসাবে এই তথ্য গুলো আপনার জানা প্রয়োজন ...।
বিসিএস ও ব্যাংক এর লিখিত পরীক্ষা ...+এছাড়া মাধ্যমিক ও উচ্চমাধ্যমিকের স্টুডেন্টদের জন্য অনেক কাজে আসবে ...
Strategies for Effective Upskilling is a presentation by Chinwendu Peace in a Your Skill Boost Masterclass organisation by the Excellence Foundation for South Sudan on 08th and 09th June 2024 from 1 PM to 3 PM on each day.
Biological screening of herbal drugs: Introduction and Need for
Phyto-Pharmacological Screening, New Strategies for evaluating
Natural Products, In vitro evaluation techniques for Antioxidants, Antimicrobial and Anticancer drugs. In vivo evaluation techniques
for Anti-inflammatory, Antiulcer, Anticancer, Wound healing, Antidiabetic, Hepatoprotective, Cardio protective, Diuretics and
Antifertility, Toxicity studies as per OECD guidelines
Thinking of getting a dog? Be aware that breeds like Pit Bulls, Rottweilers, and German Shepherds can be loyal and dangerous. Proper training and socialization are crucial to preventing aggressive behaviors. Ensure safety by understanding their needs and always supervising interactions. Stay safe, and enjoy your furry friends!
it describes the bony anatomy including the femoral head , acetabulum, labrum . also discusses the capsule , ligaments . muscle that act on the hip joint and the range of motion are outlined. factors affecting hip joint stability and weight transmission through the joint are summarized.
2. Case
A 9 yr old presents with h/o of seizures for 1 day.
Mom gives a history that the boy was well until 2
days ago when he started c/o a headache,
swelling of feet and eyes, and passing tea
colored urine. No h/o travel but mom says the boy
had sore throat 2 weeks ago
3. Introduction
Glomerulonephritis, as the term states, is
inflammation of the glomerulus
Acute Glomerular injury with the following
features:
Haematuria (Microscopic or Macroscopic)
Hypertension (due to water and salt retention)
AKI (Oliguria, Uraemia, elevated creatinine)
Oedema (Peripheral or Pulmonary)
The major underlying pathology is inflammation of the
glomerulus
The primary pathology can be in the kidney, or it can
be a consequence of systemic disorders
4. Etiology Causative agent Examples
Post-Infectious
(Immune complex mediated
following infection)
Bacterial
Post-Streptococcal
(80% of cases)
S. aureus, S. pneumoniae, M,
pneumoiae, E. coli, Sypilis, TB
Viral EBV, CMV, HSV, VZV, Hepatitis
B and C, HIV
Parasites Malaria, Schistosomiasis,
Toxoplamosis, Trypanosomiasis
Fungi Candida, Aspergillus,
Cryptococcus, etc
Autoimmune Immune Complex Mediated
Systemic Lupus Erythematosus
Ig A vasculitis (HSP)
IgA Nephropathy
Anti GBM antibodies Anti GBM
Pauciimmune ANCA Vasculitis GPA, MPA
5. Pathophysiology
Depends on the underlying cause
There is a structural disruption of the glomerular basement
membrane
Glomerular filtration barrier (GFB) is formed by a meshwork
of laminin, proteoglycans, and type IV collagen
The GFB allows the filtration of water and small and
medium-sized solutes
The three layers of the glomerular filtration barrier are the
endothelium, glomerular basement membrane (GBM), and
podocytes
7. Pathophysiology
The GFB can be damaged by various
mechanisms
Direct damage to the endothelial cell layer
Deposition of immune complex in subendothelial,
subepithelial, and mesangial space
Disruption of glomerular basement membrane by
primary renal or secondary systemic diseases
Damage to the podocytes' cellular layer
8. Pathophysiology
ofAPSGN
In children, the most common cause of acute
glomerulonephritis is post-streptococcal glomerulonephritis
PSGN appears to be caused by glomerular immune complex
disease induced by specific nephritogenic strains of group A
beta-hemolytic streptococcus (GAS)
The resulting glomerular immune complex disease triggers
complement activation and inflammation
The latent period between GAS infection and PSGN is
dependent upon the site of infection: between one and three
weeks following GAS pharyngitis and between three and six
weeks following GAS skin infection
10. HISTORY
PertinentQuestionstoAsk
Periorbital puffiness in the early morning
Edema in legs in the evening
The change in color, odor, consistency, and output of urine
Recent upper respiratory tract or skin infection
Fever, fatigue
Headache and seizures
Ulcers and rash on the extremities to rule out vasculitis
Hemoptysis and dyspnea may be present in
Goodpasture syndrome (due to cross-reacting antibodies to the alveolar
epithelium)
GPA or MPA (if lungs are affected)
11. SYMPTOMS
The classic symptoms of the nephritic syndrome are:
Periorbital and pedal edema
Hematuria with red or cola-colored urine
Proteinuria in non-nephrotic range (i.e., less than 3.5 gm/day)
and may cause foamy urine when protein content is high
Hypertension or poorly controlled blood pressure (BP) in
patients with previously controlled BP
Seizures due to Hypertension
Renal insufficiency characterized by oliguria (reduced urine
output), and azotemia, due to decreased glomerular filtration
rate (GFR)
12. Physical
Examination
Patient may be Pale
Elevated blood pressure.
The signs of fluid overload may be present
JVP distention
Pitting edema
Crackles on chest auscultation (pulmonary edema)
On cardiovascular examination, a new heart murmur
can be auscultated in patients with infective
endocarditis.
Palpable purpura and painful and swollen joints are
present in patients with systemic diseases like
vasculitis, Henoch-Schönlein purpura, and SLE.
13. Evaluation
Urine analysis is the first step in the evaluation of
nephritic syndrome
Urine can be discoloured by food, medication,
exercise, haemoglobinuria and myoglobinuria
The upper limit of normal excretion of blood in the
urine is 3 RBCs/HPF
In nephritic syndrome
The urine has greater than 5 RBCs/ HPF along with
acanthocytes and dysmorphic RBCs
RBCs casts and in a few cases WBCs casts
Glomerular Hematuria is usually marked with
brownish (cola or tea) coloured urine
15. Investigations
U&Es, Creatinine
Full Blood Count, PBS, ESR, C-reactive Protein
Blood Culture
ASOT, Anti-DNAse B
Urine Culture and Microscopy for casts
UPCR
Throat Swab
C3, C4
ANA, Anti-dsDNA, ANCA, Anti-GBM
KUB Ultrasound
Kidney Biopsy (when indicated)
RF to r/o cryoglobulinemia if suspected
Hepatitis B Surface Antigen and HCV Antibodies
16. Differential
Diagnosis
The following renal diseases have a clinical
presentation similar to the nephritic
syndrome:
Nephrotic syndrome – FSGS, ICGN
Familial nephritis – Alports, Thin Basement
Membrane Disease
Idiopathic hematuria
Anaphylaxis
17. Treatment
The treatment is mainly supportive and consists of:
Fluid and Dietary salt restriction
If fluid overloaded, restrict intake to insensible losses only
The reduced intake of sodium and potassium helps reduce the
retention of water
Bed rest
Antihypertensives
Some patients may come in Hypertensive emergency and this
should be managed with drugs such as Labetalol, Sodium
Nitroprusside and Hydralazine
In those who are not in hypertensive crisis, hypertension can be
treated with ACE inhibitors, ARBs, Amlodipine or Nifedipine
Diuretics: Loop diuretics may be administered to excrete
excess sodium and water retained in the body
Corticosteroids: Help relieve the inflammation in the kidney
and promote healing. This is indicated in patients with RPGN
18. Treatment
Immunomodulators: Immunosuppressive drugs reduce and
block the antigenic effects of the inciting agents especially in
RPGN
Use of corticosteroids and immunomodulators is controversial in
certain causes of the nephritic syndrome, including staphylococcal
endocarditis. It can aggravate the sepsis and result in increased
mortality
Antibiotics: Post streptococcal GN patients with evidence of
streptococcal infection are administered penicillin. Erythromycin
is preferred for patients allergic to penicillin.
Dialysis: In some cases, the disease has a fulminating course
leading to severe AKI requiring renal replacement therapy.
19. Complications
The nephritic syndrome can grossly compromise
renal function and lead to the following
complications.
Acute Kidney Injury and progression to RPGN
Uncontrolled hypertension
Azotemia
Hyperkalemia
Hyperphosphatemia
Hypocalcemia
Heart failure
Hypertensive encephalopathy presenting as seizures and
altered consciousness
20. Prognosis
The prognosis of the nephritic syndrome depends
upon the underlying aetiology, age of the patients
and timely intervention
APSGN is the commonest in children and has
excellent outcome
Adults typically have a chronic fulminating course.
The disease is not resolved in 20 to 74% of the adults
In these patients, the renal function derangement
persists and will result in Chronic Kidney Disease
21. SUMMARY
The nephritic syndrome is a common presentation of most proliferative glomerulonephritides (GN) and
is characterised by haematuria, oliguria, hypertension and edema
The primary pathology can be in the kidney, or it can be a consequence of systemic disorders
Poststrep GN is the commonest cause of Nephritic Syndrome in children and has a good prognosis
Nephritic syndrome may have a variable clinical picture depending on the underlying aetiology
The acanthocytes, dysmorphic red blood cells, and RBC casts are pathognomonic of glomerular
inflammation
The severity of clinical symptoms at the time of presentation determines the prognosis of the disease
Patients with severe oliguria, azotemia, raised creatinine level at the time of presentation have a worse
prognosis.
Supportive treatment should be initiated immediately along with carrying out the evaluation tests for a
specific diagnosis
24. Scenario
5 year old presents with generalized body
swelling is referred to the ER.
His lab tests are as follows:
Urine 4+ proteinuria; Serum albumin 17mmol/l
Cholesterol 12mmol/l; Creatinine 38umol/l; BP 90/60
Questions
Take relevant history
What is the most likely diagnosis
What are the possible aetiologies?
What further investigations would like to do?
25. INTRODUCTION
Nephrotic Syndrome is the commonest
glomerular disease in children
It manifests with oedema, hypoalbuminemia,
heavy proteinuria and hyperlipidaemia.
26. Epidemiology
Incidence 2-7/100000 Children per year - Nandlal
L. et al-2019
Sex predilection Male:Female 2:1
Commonly affects Children between 2- 10 years.
27. Definition:NephroticSyndrome
Nephrotic Range Proteinuria
UPCR ≥ 200 mg/mmol (2 mg/mg) in 1st morning void, or 24hr urine
sample ≥ 1000 mg/m2 per day corresponding to 3 + or 4 + by urine
dipstick
Hypoalbuminemia
Serum Alb <30g/L
OR
Edema
When serum albumin is
not available
28. Classification
Congenital Nephrotic Syndrome
Manifests in the first 3 months of life
Infantile N.S
Manifests after 3 months of life but before 12 months of
life
Primary/Idiopathic NS
Most common and cause is unknown
Secondary NS
Has an identifiable cause
Can present at any age
29. 1. Idiopathic 85% of cases
2. Genetics
3. Infections e.g Syphilis, malaria,toxoplasimosis,schistosomiasis
4.Drugs NSAIDS, Lithium, Mercury, Lead.
5. Autoimmune and connective tissue disorders e.g, SLE, Granulomatosus with
Polyangiitis, Microscopic polyangiitis, IgA nephropathy
6. Malignancies; Lymphoma, leukemia
CAUSES OF CHILDHOOD NEPHROTIC SYNDROME
30. IdiopathicNS
Commonest form of NS
Affecting from 1.15 to 16.9 per 100,000 children per year
globally
85–90% are Steroid Sensitive (SSNS)
70–80% will have at least one relapse during follow-up
50% of these will experience frequent relapses (FRNS) or
become Steroid dependent (SDNS)
10–30% continue to have a relapsing course into young
adulthood
A small percentage will show no response to steroid therapy
of which some will progress to ESKD
32. The podocyte is a polarised epithelial cell with
interdigitating foot processes with a unique cell–cell
junction known as the slit diaphragm
The podocyte, along with the glomerular basement
membrane and the fenestrated glomerular
endothelium, forms a trilayered structure— the
glomerular filtration barrier
The podocyte and filtration barrier allow an
ultrafiltrate almost completely devoid of protein to
pass into the Bowman’s space and proceed onto
the proximal tubule
Normal
33. Podocyte architecture is maintained by an extensive
actin cytoskeleton that enables the glomerular filtration
barrier to withstand the substantial capillary hydrostatic
pressure
Loss of normal podocyte structure, the foot processes
or the slit diaphragm that spans these interdigitations
can lead to loss of albumin in the ultrafiltrate
Podocytes are terminally differentiated cells with
minimal regeneration and thus, vulnerable to injury
Abnormal
36. Pathophysiology
25-Jul-23
Edema:
Under fill theory: hypoalbuminemia
Over fill theory:↑ tubular NaCl reabsorption
secondary to RAAS → intravascular expansion
→ fluid shift following pressure gradient
Hypercholesterolemia
hypoproteinemia → hepatic lipoprotein
synthesis
→ ↑serum lipid (cholesterol, lipoprotein) → lipid
metabolism
37. 25-Jul-23
Sudden onset of dependent pitting oedema
Periorbital
scrotal or vulva
ankle or leg
Weight gain
Ascites
abdominal pain
Diarrhea (due to intestinal oedema)
Respiratory distress (due to pulmonary edema/
pleural effusions or massive ascites)
Decreased urine output
±HTN and Hematuria
Clinical
Manifestation
38. 25-Jul-23
Infection
Spontaneous bacterial peritonitis, cellulitis,
bacteriemia (S.pneumoniae, E.coli)
Steroid and immunosuppressant toxicity
Hypovolaemia
abdominal pain and may feel faint, cold
peripheries, poor pulse volume, hypotension, and
haemoconcentration.
A low urinary sodium (<20mmol/L) and a high
packed cell volume
Complications
39. Thromboembolism
Hypercoagulable state due to decreased
fibrinolytic factors (urinary losses of antithrombin
III, proteins C and S)
Thrombocytosis
exacerbated by steroid therapy
Increased synthesis of clotting factors
Increased blood viscosity from the raised
haematocrit,
This is usually arterial and may affect the brain,
limbs and splanchnic circulation
Hypercholesteroleamia
Acute Kidney Injury (rare)
Complications
41. First time or relapse???
History of oedema noted on awakening in the morning or sudden swelling??
Distribution
Colour changes
Initiating factor? (bee sting)
Painful??
Weight gain (edema)
Respiratory distress (Breathlessness)
Infection, Pleural Effusion/Pulmonary Edema, Ascites
Diarrhea
Urine: frothy
Past medical and drug history: recent illness, allergies, asthma
Nutrition history
Family history
History Taking
42. 25-Jul-23
Assessment of hydration status identifies fluid imbalances (dehydration,
overhydration)
Blood pressure: hypertension
Henoch-Schönlein purpura (purpura)
Systemic lupus erythematosus (e.g. malar rash)
Rales heard on lung auscultation suggest extravascular fluid from overload or
hypoalbuminemia
Palpation and percussion of the abdomen may reveal ascites or masses
Liver enlargement is present in several multisystem diseases (systemic lupus
erythematosus, infections, polycystic disease) and in glomerulosclerosis
Physical
Examination
Physical Examination
48. InitialEpisode
High protein diet
Salt moderation
Treatment of infections
If significant edema – diuretics can be used but cautiously
Mainstay of therapy is Corticosteroid treatment with Prednisolone
First episode:
Prednisone 2mg/kg(60mg/m2) daily for 6weeks then taper
Second episode:
Prednisone 2mg/kg(60mg/m2) until protein free for 3 days then taper
Prevention:
Prednisone 1mg/kg daily x 5 days when URTI/Vaccines
Initial Episode
49. Treatment Related Definitions
• Complete remission
o UPCR (based on first morning void or 24 h urine sample) ≤ 20 mg/mmol (0.2
mg/mg) or < 100 mg/m2 per day, respectively, or negative or trace dipstick on
three or more consecutive days
• Steroid-sensitive nephrotic syndrome (SSNS)
o Complete remission within 4 weeks of PDN at standard dose (60 mg/m2/day or
2 mg/kg/day, maximum 60 mg/day)
• Steroid-resistant nephrotic syndrome (SRNS)
o Lack of complete remission within 4 weeks of treatment with PDN at standard
dose
• SSNS late responder
o A patient achieving complete remission during the confirmation period (i.e.
between 4 and 6 weeks of PDN therapy) for new onset NS
50. Treatment Related Definitions
• Infrequently relapsing Nephrotic Syndrome
o < 2 relapses in the 6 months following remission of the initial episode or <3
relapses in any subsequent 12-month period (modified definition)
• Frequently relapsing Nephrotic Syndrome (FRNS)
o ≥ 2 relapses in the first 6-months following remission of the initial episode or ≥
3 relapses in any 12 months (modified definition)
• Steroid-dependent nephrotic syndrome (SDNS)
o A patient with SSNS who experiences 2 consecutive relapses during
recommended Prednisolone therapy for first presentation or relapse or within
14 days of its discontinuation
51. Definition: Relapse
• Relapse
o Urine dipstick ≥3+ or UPCR ≥ 200 mg/mmol (≥ 2 mg/mg) on a spot urine sample
on 3 consecutive days, with or without reappearance of edema in a child who
had previously achieved complete remission
• Complicated Relapse (New Definition)
o A relapse requiring hospitalization due to one or more of the following: severe
edema, symptomatic hypovolemia or AKI requiring IV albumin infusions,
thrombosis, or severe infections (e.g., sepsis, peritonitis, pneumonia, cellulitis)
52. Treatment-Related Definitions: SSNS
• Steroid toxicity
o New or worsening obesity/overweight
o Sustained hypertension
o Hyperglycemia
o Behavioral/psychiatric disorders, sleep disruption
o Impaired statural growth (height velocity < 25th percentile and/or height
< 3rd percentile) in a child with normal growth before start of steroid
treatment
o Cushingoid features, striae rubrae/distensae,
o Glaucoma, ocular cataract
o Bone pain, avascular necrosis
53. Mx of oedematous state
Bed rest to be avoided as there is a tendency of hypercoagulability
Dietary advice: no added salt, normal protein with adequate calories
Prophylactic antibiotics: oral penicillin particularly in during relapse with
gross oedema
Hypovolaemia: infuse salt poor albumin or 5% albumin, plasma protein
derivatives or human plasma.
• Note that the patient should be passing urine as you risk causing pulmonary oedema
• Diuretics: to be given cautiously
MANAGEMENT
55. Steroidtoxicity
• Stunting of growth
• Cataracts
• Striae
• Severe cushingoid features
• behavioural changes, a rounded face, central obesity
and the tendency to bruise more easily, hirsutism
• Osteoporosis
• Proximal myopathy
• Recurrent infection due low immunity
58. Managementof
Complications
Infection: parenteral penicillin and a third
generation cephalosporin (in primary
peritonitis)
If exposed to chickenpox and measles
varicella-zoster immunoglobulin (VZIG) should
be given within 72 hours after exposure to
chickenpox / single dose of intravenous
immunoglobulin
Thrombosis : Warfarin, low-dose aspirin, and
dipyridamole all have been used to minimize
the risk of clots.
25-Jul-23
59. EDUCATION
Parents and school teachers should be
provided with information regarding the disease
which includes:
1. Advice and precaution of infection
2. Danger of sudden steroid withdrawal (adrenal
crisis)
3. Immunization:
While the child is on corticosteroid treatment and
within 6 weeks after its cessation, only killed
vaccines may be safely administered to the child.
Live vaccines can be administered 6 weeks after
cessation of corticosteroid therapy
In immune complex–mediated diseases
Antibody is produced against and combines with a circulating antigen that is usually unrelated to the kidney
The immune complexes accumulate in GBMs and activate the complement system, leading to immune injury
anti-GBM antibody disease
Auto- antibodies are formed against Type IV collagen in the basement membrane
Nephritogenic antigens
There are two leading candidates for the putative streptococcal antigen(s) responsible for PSGN
Nephritis-associated plasmin receptor (NAPlr), a glycolytic enzyme, which has glyceraldehyde-3-phosphate dehydrogenase (GAPDH) activity. NAPlr has a plasmin-like activity which may promote a local inflammatory reaction.
Streptococcal pyrogenic exotoxin B (SPE B), a cationic cysteine proteinase, has been localized in the subepithelial deposit
angiotensin-converting enzyme (ACE) inhibitors should be used with caution due to the risk of hyperkalemia
Rationale: Change in definitions was necessitated by the need to reduce exposure to Prednisolone