Peds- Nephrology

When a patient is lying down overnight, there is often no protein detected in the urine.
If the same patient stands up for a few hours and walks around, protein appears in the urine upon testing.
This occurrence is typically transient and not considered dangerous.

Persistent or high-grade proteinuria is a primary indicator of nephrotic syndrome.
Evaluation of this condition often involves bilinear analysis.
Nephrotic syndrome is characterized by five distinct features, with four classic features frequently highlighted:
- Massive proteinuria: Defined as being more than $1000$ .
- Hypoalbuminemia: The excessive loss of protein leads to albumin levels becoming less than $3$ .
- Edema: Resulting from fluid retention in the body.
- Hyperlipidemia: Specifically hypercholesterolemia, occurring because the liver attempts to compensate for rapid albumin loss by synthesizing more proteins and lipids.

Significant protein loss in the urine causes hypoalbuminemia (albumin $< 3$ ).
Decreased levels of albumin lead to a reduction in plasma oncotic pressure.
The decrease in oncotic pressure causes the body to retain fluids, leading to clinical edema.
The liver increases synthesis of proteins and lipids (hypercholesterolemia) to replace lost plasma albumin.

The treatment course typically lasts between $4$ to $6$ weeks, often extending up to $12$ weeks total.
Standard initial treatment involves Prednisolone:
- Phase 1: Administer $2\,mg/kg$ of Prednisolone daily for the first $6\,weeks$ .
- Phase 2: Administer $1\,mg/kg$ per day on alternate days for the following $6\,weeks$ .
- Phase 3: The drug is subsequently stopped.
Steroid treatment is generally successful in resolving proteinuria.
Renal biopsies are often unnecessary if the patient falls within the typical age range of $1$ to $10\,years$ and lacks high-risk features such as hematuria, hypertension, or other significant signs of renal disease. In these cases, steroids are the primary treatment of choice.

A relapse is defined by the recurrence of proteinuria after initial resolution.
Relapses are treated with daily steroids until the proteinuria resolves, followed by $4\,weeks$ of alternate-day steroid administration.
Steroid dependence occurs when proteinuria recurs whenever the steroid dosage is stopped or transitioned to an alternate-day schedule.
For steroid-dependent patients, steroid-sparing agents are utilized, including:
- Mycophenolate
- Moccitin
- Cyclosporic
- Tacrolimus
- Levamacil

Glomerulonephritis involves inflammation of the glomerulus, leading to glomerular hematuria.
The clinical presentation is referred to as nephritic syndrome, which is distinct from nephrotic syndrome.
Key characteristics of nephritic syndrome include:
- Hematuria (often appearing like Coca-Cola diluted with water).
- Proteinuria.
- Acute kidney injury (AKI).
- Edema.
- Hypertension.
Nephritic syndrome is typically a short-lasting, transient process from which the kidneys soon recover.
In contrast, nephrotic syndrome can involve repeated episodes of proteinuria, edema, and hospitalizations, persisting until the second decade of life (the twenties). Most nephrotic patients recover by the second or third decade of life and do not develop chronic kidney disease.

Certain forms of glomerulonephritis are produced when poorly glycosylated IgA is present in the body.
This IgA deposits in the mesentium of the kidneys, leading to chronic glomerulonephritis.
Clinical features often include hematuria occurring immediately after a Upper Respiratory Infection (URI).
Episodes of hematuria typically start within $3$ to $7\,days$ of a cough or cold.
This specific condition can lead to episodic hematuria throughout a patient's life and may eventually progress to chronic kidney disease.

Question: Have you seen hematuria ever?
Response: It looks like Coca-Cola diluted with water. This presentation is mostly associated with glomerulonephritis, where inflammation of the glomerulus causes glomerular hematuria.