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Amyloidosis in PEDIATRICS
Kourosh Vahidshahi (MD)
Pediatric Cardiologist
Associate ProfessorShahid Beheshti University of Medical Sciences
July. 2021
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis: C0ngo-red stain
Amyloidosis
Amyloidosis
Amyloidosis in PEDIATRICS
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis
Amyloidosis in PEDIATRICS
• Amyloidosis is rare in children.
• Primary: genetic syndrome
- Peripheral nervous system and the heart.
• Secondary :juvenile rheumatoid arthritis, chronic infections, familial Mediterranean fever and malignancy.
• The most common : reactive AA amyloidosis due to chronic inflammatory diseases
Amyloidosis in PEDIATRICS
• Kidney
• Heart
• GI
• Neuropathy
• Hematologic
• Skin
• …
Amyloidosis in PEDIATRICS
Kidney
• Asymptomatic proteinuria
• the most common initial presentation
• Urinalysis routinely :risk of secondary amyloidosis.
• Gradually progressing to nephrotic syndrome
and/or renal dysfunction
Amyloidosis in PEDIATRICS
Kidney
• In the series reported by the Turkish FMF study : 32% proteinuria, 40% nephrotic syndrome, and 28% chronic renal failure .
• Hematuria and hypertension almost never occurs
• 10–30% of patients with renal amyloidosis might have only mild proteinuria and normal renal function
Amyloidosis in PEDIATRICS
Kidney
• Renal failure with little or no proteinuria:
- primary deposition of amyloid fibrils to blood
vessels or tubules.
• Tubular dysfunction such as type 1 (distal) renal
tubular acidosis, nephrogenic diabetes insipidus
related polyuria, and acquired Fanconi syndrome
Amyloidosis in PEDIATRICS
GI
• dyspepsia, abdominal pain, nausea, diarrhea, gastrointestinal bleeding, malabsorption, constipation, and obstruction.
• Acute gastrointestinal system symptoms may resemble FMF attacks.
• Hepatomegaly and splenomegaly .
• Serum liver enzymes can elevate
• Splenomegaly can cause hypersplenism and rarely splenic rupture
Amyloidosis in PEDIATRICS
Hematopoietic system
• Bone marrow
• bleeding diathesis due to factor X deficiency, liver
disease, or infiltration of blood vessels .
• Thrombotic events due to underlying disease,
nephrotic syndrome
Amyloidosis in PEDIATRICS
• Nervous system
• Mixed sensory and motor peripheral neuropathy and autonomic neuropathy
• Rare in the secondary forms.
• Hereditary amyloidoses : familial amyloidoticpolyneuropathy
• Central nervous system involvement is unusual.
Amyloidosis in PEDIATRICS
Attempts to treat amyloidosis by:
-immunosuppression
-steroids
-fat elimination diets
• have been unsuccessful.
• Kidney transplants
• recurrence has been reported.
Amyloidosis in PEDIATRICS
• Colchicine : great success for the prevention of the 'attacks' of FMF.
• inhibit the development of casein induced amyloidosis in mice.
• Perhaps, the onset of amyloidosis will be prevented or delayed in patients with FMF treated with colchicine
Amyloidosis in PEDIATRICS
The HEART
Amyloidosis in PEDIATRICS
• Cardiac amyloidosis results in a restrictive
cardiomyopathy
• Extracellular deposition of proteins in the
myocardium.
Amyloidosis in PEDIATRICS: The HEART
• Heart Systolic or diastolic dysfunction, and
arrhythmia are the main presenting features
of heart involvement.
• Cardiac involvement is more rare in AA
amyloidosis compared to AL amyloidosis and
may progress rapidly
Amyloidosis in PEDIATRICS: The HEART
Restrictive
Cardiomyopathy
Amyloidosis in PEDIATRICS: The HEART
Restrictive Cardiomyopathy
Clinical Manifestation
• Left sided backward :pulmonary congestion
- Exertional dyspnea / intolerance
- Recurrent Pneumonia
- Chronic cough
• Left sided forward:
-syncope, Angina,..
- Arrhythmia
• Right sided backward : Edema, Hepatomegaly,…
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy
Amyloidosis in PEDIATRICS
Kourosh Vahidshahi (MD)
Pediatric Cardiologist
Associate ProfessorShahid Beheshti University of Medical Sciences
July. 2021
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