QuestionSBA
QuestionChoice
Sodium would usually be low due to increased ADH levels leading to hypervolaemic hyponatraemia.
10017219
d
High serum sodium
QuestionChoice
Serum triglycerides would likely be raised in nephrotic syndrome.
10017217
b
Low serum triglycerides
QuestionChoice
Serum albumin is low in nephrotic syndrome.
10017218
c
High serum albumin
QuestionChoice
This would likely be raised in nephrotic syndrome. This is due to a combination of increased production and impaired catabolism/clearance of LDLs.
10017220
e
Low serum cholesterol
QuestionChoice
This describes a case of minimal change disease, a form of nephrotic syndrome. It commonly follows viral upper respiratory tract infections. This classically causes a low serum albumin and elevated serum triglycerides/cholesterol.
10017216
a
Low serum albumin
Concept
Chapter
# Summary
 

Minimal Change Disease (MCD) is the leading cause of nephrotic syndrome in children, responsible for roughly 70% of cases. It shows minimal changes to nephrological structures on light microscopy. Typically characterised by the presence of oedema and frothy urine, MCD often follows a viral upper respiratory tract infection. Key investigations include urine tests and possibly a kidney biopsy. Management strategies primarily involve corticosteroid therapy, fluid restriction, and reduced salt intake. It can lead to complications like spontaneous peritonitis, thrombosis, renal damage, and an increased risk of infections.
 

# Definition
 

Minimal Change Disease is a condition that primarily affects the kidneys. Despite causing significant clinical symptoms, the disease is marked by minimal or no changes visible under light microscopy in nephrological structures. However, more subtle changes can be detected using electron microscopy. This condition is the most common cause of nephrotic syndrome in children, and while it may also affect adults, it is much less common in this population.
 

# Epidemiology
 

 -  The incidence of minimal change disease is 2 per 100,000 children in the UK per year. 
 -  Minimal Change Disease most commonly affects children aged 1-8 years old.
 -  It is the primary cause of nephrotic syndrome in children, accounting for around 70% of all cases.
 

# Aetiology
 

While the exact cause of Minimal Change Disease is unknown, it has been associated with certain factors:
 

 -  Recent viral upper respiratory tract infection
      -  There may be an immune response causing damage to the kidneys.
 - Being male (2x more prevalent in boys than girls)
 - Being South Asian (4-6x more prevalent compared to white British children)
 

# Signs and Symptoms
 

Patients with Minimal Change Disease typically present with:
 

 -  Oedema, including facial swelling and puffiness around the eyes most prominent in the morning, followed by pedal oedema 
 -  Frothy urine, caused by excessive protein in the urine (proteinuria)
 -  Fatigue
 -  Weight gain due to fluid retention

# Differential Diagnosis
 

In a patient presenting with symptoms suggestive of nephrotic syndrome, several other conditions should be considered:
 

 -  **Focal segmental glomerulosclerosis (FSGS)**: Presents with proteinuria, hypertension, and oedema. However, FSGS often progresses to kidney failure and responds less well to corticosteroids compared to MCD.
 -  **Membranous nephropathy**: Characterized by heavy proteinuria, hypertension, and oedema. It typically affects adults and is less common in children.
 -  **Secondary causes of nephrotic syndrome**: Conditions such as diabetes, lupus, or certain infections and medications can lead to nephrotic syndrome.
 

# Investigations
 

Patients suspected to have Minimal Change Disease should undergo:
 

 -  Urine tests: to identify proteinuria and to look for blood or white blood cells
       -  Urine protein : creatinine >200 mg/mmol 
 -  Blood tests: to check for low albumin and elevated cholesterol
       -  Hypoalbuminaemia defined by serum albumin < 25 g/L
 -  Kidney biopsy: often reserved for patients who do not respond to initial treatment, to confirm diagnosis. Light microscopy of renal biopsy specimens from patients with MCD shows minimal to no change, hence the name.
 

# Management
 

The treatment of Minimal Change Disease involves:
 

-  Corticosteroid therapy: Prednisolone is the first-line treatment, used for 4 weeks. 
-  Second-line therapy: Includes other oral immunosuppressive agents such as ciclosporin if initial treatment fails.
-  Fluid restriction and reduced salt intake: To manage oedema and prevent further complications.
     -  Fluid can be restricted to 750 mL/day for children < 5 years, or 1,000 mL/day for children over the age of 5. 
-  Diuretics (i.e. furosemide) may be used to reduce fluid overload.
     -  If severe, IV albumin infusions may also be used to manage hypoalbuminaemia. 

Children are also advised to have the pneumococcal vaccine due to relative immunodeficiency and the use of immunosuppressive medications. 
 
#  Complications
 
Complications of minimal change disease include:
 
- Infection
    - Through the loss of immunoglobulins in urine, the children are at increased risk of infections
    - Antibiotic prophylaxis is not routinely recommended 
-  Thrombosis due to loss of anti-thrombin III in the urine and hypovolaemic state 
    -  Consider DVT, PE and cerebral venous sinus thrombosis in a child presenting with leg, chest or head pain
- Steroid-related side effects (i.e. hyperglycaemia/diabetes, hypertension, psychosis and sleep disturbance) 
-  Vitamin D deficiency 
-  Hypertension 
-  Hypercholesterolaemia 
 

# Prognosis

Initially, over 80% of children respond to high-dose prednisolone. Factors associated with poor response to steroid treatment include age <1 year or >10 years, hypertension at presentation, macroscopic haematuria, and a family history of nephrotic syndrome. 

The long-term prognosis, however, for Minimal Change Disease varies:
 

 -  1/3 of patients resolve completely and never have another episode.
 -  1/3 have further relapses requiring additional steroid treatment.
 -  1/3 are dependent on continued steroid/immunosuppression therapy.

 
Patients require monitoring via urinary dipsticks. 
 

# References

[NHS Nephrotic syndrome in children](https://www.nhs.uk/conditions/nephrotic-syndrome/) 
 
 [BMJ Best Practice Summary](https://bestpractice.bmj.com/topics/en-gb/940)
 
 [National Kidney Federation Minimal Change Nephropathy](https://www.kidney.org.uk/minimal-change-nephropathy#:~:text=Minimal%20change%20causes%20fluid%20retention,This%20is%20not%20usually%20affected.)
542
556
Minimal change disease
Topic
29
Paediatrics
Video
Concept
556
Minimal change disease
590
erivGUb
images/videos/nephrology.png
Quesmed Tutorial: Paediatric Nephrology
15987
Low serum albumin is commonly seen in minimal change disease, a type of nephrotic syndrome, due to increased urinary loss of protein, leading to hypoalbuminemia and subsequent oedema.
A 5-year-old boy has facial swelling. His mother states he had a cough and runny nose the week prior to the swelling. His urine dipstick shows 3+ protein.

Given the likely diagnosis, which of the following investigation results would you expect to see?
a