Amyloid Disease, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

By Kenneth Kee

This book describes Amyloid Disease, Diagnosis and Treatment and Related Diseases
Amyloid Disease is a progressive disease affecting the tissue infiltrates of insoluble proteins or amyloid proteins leading to poor function of the organs involved.
Amyloid Disease is a medical disorder produced by extra-cellular and or intra-cellular deposition of insoluble abnormal amyloid fibrils that alter the normal function of tissues.
In humans there are about 23 different unrelated proteins known to form amyloid fibrils in vivo.
Proteins that develop into amyloid fibrils differ in size function, amino acid effect and native structure but become non-soluble clumps that are similar in structure and in properties.
In the past amyloid disease was classified as primary amyloid disease 70% or secondary amyloid disease but with chemical testing of the amyloid protein, Amyloid is categorized chemically.
The amyloid diseases are termed with a capital A (for amyloid) accompanied by an abbreviation for the fibril protein:
1.AA amyloid disease contains a normal-sequence serum amyloid A protein
The kidneys, joints, liver and spleen are the main targets of AA amyloid deposits
2.AL amyloid Disease was previously known as primary amyloid disease
Organs involved are the heart, kidney, peripheral nerve, gastrointestinal tract, respiratory tract and nearly any other organ.
AL amyloid disease is the most frequent type of systemic amyloid disease in developed countries
3.Transthyretin amyloid disease (ATTR) is the most frequent inherited systemic type of amyloid disease
ATTR is an autosomal-dominant disease but is linked with at least 100 different transthyretin (TTR) mutations
There are many other amyloid diseases.
Symptoms:
This disease can involve the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen and kidneys
1.Fatigue,
2.Weight loss,
3.Easy bruising,
4.Breathlessness,
5.Peripheral edema,
6.Sensory change,
7.Carpal tunnel syndrome
8.Postural hypotension
Signs:
1.Unexplained renal disease
2.Massive proteinuria
3.Cardiac amyloid disease
4.Marked hepatomegaly with kidney disease:
5.Sensory glove- and stocking-type polyneuropathy:
a.Particularly carpal tunnel syndrome
6.Vitreous opacities
7.Gastrointestinal amyloid
8.Skin lesions:
a.Spontaneous periorbital purpura - racoon eye
9.A firm, symmetrical, non-tender goiter
Diagnosis:
Biopsy of an affected organ, or simple subcutaneous aspiration of abdominal fat - stains red with Congo red stain giving red-green birefringence under polarized light
Diagnosis is validated in 80% of cases
Immunofixation electrophoresis can confirm the type of amyloid disease
Treatment
No treatment is present that specifically targets the amyloid deposits,
The treatment is therefore directed at suppressing the underlying plasma cell dyscrasia along with supportive measures to support and possibly preserve organ function.
Supportive
1.Symptoms must be treated, e.g. diuretics for renal failure, erythropoietin for anemia.
2.Congestive heart failure may respond to diuretics, but larger doses are often required as the disease progresses.
Underlying Disorder
Any underlying causes, e.g. inflammatory disorders, chronic infection or carcinoma, should be treated
Specific treatment
Alkylator-based chemotreatment is effective to some degree in almost 66% of patients.
Trials with novel medicines such as thalidomide, lenalidomide, pomalidomide and bortezomib have shown promising results.
Stem cell transplantation are for:
1.Normally < 70 years old,
2.Minimal heart failure,
3.Serum creatinine ≤177 μmol/L and
4.Fewer than 3 organs involved
Surgical
Liver and kidney transplant may be transiently successful

TABLE OF CONTENT
Introduction
Chapter 1 Amyloid Disease<

• Language: English
• Publisher: Kenneth Kee
• Release date: Apr 30, 2018
• ISBN: 9780463146965

Kenneth Kee

Medical doctor since 1972.Started Kee Clinic in 1974 at 15 Holland Dr #03-102, relocated to 36 Holland Dr #01-10 in 2009.Did my M.Sc (Health Management ) in 1991 and Ph.D (Healthcare Administration) in 1993.Dr Kenneth Kee is still working as a family doctor at the age of 74However he has reduced his consultation hours to 3 hours in the morning and 2 hours inthe afternoon.He first started writing free blogs on medical disorders seen in the clinic in 2007 on http://kennethkee.blogspot.com.His purpose in writing these simple guides was for the health education of his patients which is also his dissertation for his Ph.D (Healthcare Administration). He then wrote an autobiography account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.comThis autobiography account “A Family Doctor’s Tale” was combined with his early “A Simple Guide to Medical Disorders” into a new Wordpress Blog “A Family Doctor’s Tale” on http://ken-med.com.From which many free articles from the blog was taken and put together into 1000 eBooks.He apologized for typos and spelling mistakes in his earlier books.He will endeavor to improve the writing in futures.Some people have complained that the simple guides are too simple.For their information they are made simple in order to educate the patients.The later books go into more details of medical disorders.He has published 1000 eBooks on various subjects on health, 1 autobiography of his medical journey, another on the autobiography of a Cancer survivor, 2 children stories and one how to study for his nephew and grand-daughter.The purpose of these simple guides is to educate patient on health disorders and not meant as textbooks.He does not do any night duty since 2000 ever since Dr Tan had his second stroke.His clinic is now relocated to the Buona Vista Community Centre.The 2 units of his original clinic are being demolished to make way for a new Shopping Mall.He is now doing some blogging and internet surfing (bulletin boards since the 1980's) startingwith the Apple computer and going to PC.The entire PC is upgraded by himself from XT to the present Pentium duo core.The present Intel i7 CPU is out of reach at the moment because the CPU is still expensive.He is also into DIY changing his own toilet cistern and other electric appliance.His hunger for knowledge has not abated and he is a lifelong learner.The children have all grown up and there are 2 grandchildren who are even more technically advanced than the grandfather where mobile phones are concerned.This book is taken from some of the many articles in his blog (now with 740 posts) A Family Doctor’s Tale.Dr Kee is the author of:"A Family Doctor's Tale""Life Lessons Learned From The Study And Practice Of Medicine""Case Notes From A Family Doctor"

Book preview

Amyloid Disease,

A

Simple

Guide

To

The Condition,

Diagnosis,

Treatment

And

Related Conditions

By

Dr Kenneth Kee

M.B.,B.S. (Singapore)

Ph.D (Healthcare Administration)

Copyright Kenneth Kee 2018 Smashwords Edition

Published by Kenneth Kee at Smashwords.com

Dedication

This book is dedicated

To my wife Dorothy

And my children

Carolyn, Grace

And Kelvin

This book describes Amyloid Disease, Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.

(What The patient Need to Treat Amyloid Disease)

This eBook is licensed for your personal enjoyment only. This eBook may not be re-sold or given away to other people. If the patient would like to share this book with another person, please purchase an additional copy for each reader.

If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashwords.com and purchase your own copy.

Thank you for respecting the hard work of this author.

Introduction

I have been writing medical articles for my blog http://kennethkee.blogspot.com (A Simple Guide to Medical Disorder) for the benefit of my patients since 2007.

My purpose in writing these simple guides was for the health education of my patients.

Health Education was also my dissertation for my Ph.D (Healthcare Administration).

I then wrote an autobiolographical account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.com.

This autobiolographical account A Family Doctor’s Tale was combined with my early A Simple Guide to Medical Disorders into a new Wordpress Blog A Family Doctor’s Tale on http://kenkee481.wordpress.com.

From which many free articles from the blog was taken and put together into 800 eBooks.

Some people have complained that the simple guides are too simple.

For their information they are made simple in order to educate the patients.

The later books go into more details of medical disorders.

The first chapter is always from my earlier blogs which unfortunately tends to have typos and spelling mistakes.

Since 2013, I have tried to improve my spelling and writing.

As I tried to bring the patient the latest information about a disorder or illness by reading the latest journals both online and offline, I find that I am learning more and improving on my own medical knowledge in diagnosis and treatment for my patients.

Just by writing all these simple guides I find that I have learned a lot from your reviews (good or bad), criticism and advice.

I am sorry for the repetitions in these simple guides as the second chapters onwards have new information as compared to my first chapter taken from my blog.

I also find repetition definitely help me and maybe some readers to remember the facts in the books more easily.

I apologize if these repetitions are irritating to some readers.

Chapter 1

Amyloidosis

What is Amyloid Disease?

Amyloid Disease is a progressive disease affecting the tissue infiltrates of insoluble proteins or amyloid proteins leading to poor function of the organs involved.

Amyloid Disease is a medical disorder produced by extra-cellular and or intra-cellular deposition of insoluble abnormal amyloid fibrils that alter the normal function of tissues.

Only 10% of amyloid disease deposits comprise components such as glycosaminoglycans (GAGs), apolipoprotein-E (apoE), and serum amyloid P-component (SAP)

Nearly 90% of the deposits comprise amyloid fibrils produced by the clustering of misfolded proteins.

Pathophysiology

Amyloids are proteins where secondary structure changes occur, inducing the proteins to fold in a typical form, the beta-pleated sheet.

When the normally soluble proteins fold to become amyloids, they become not soluble and deposit in organs or tissues, interrupting normal function

These proteins either develop from proteins secreted by cells at the deposition site (localized), or they form systemically after production at a local site (systemic).

In humans there are about 23 different unrelated proteins known to form amyloid fibrils in vivo.

Proteins that develop into amyloid fibrils differ in size function, amino acid effect and native structure but become non-soluble clumps that are similar in structure and in properties.

Amyloid is defined as deposited material differentiated by fibrillar electron micrograph appearance, amorphous eosinophilic appearance on hematoxylin and eosin staining.

All types of amyloid comprise a major fibrillar protein that defines the type of amyloid (about 90%) plus different minor components

All types of amyloid comprise one main fibrillar protein that defines the type of amyloid.

Polymorphisms that slightly change native peptides or inflammatory processes produce the stage for abnormal protein folding and amyloid fibril deposition.

What are the types of Amyloidosis?

The Amyloid Disease comprises two main types:

1. Primary form 70% of cases

Primary amyloidosis can result in disorders that are:

a. Carpal tunnel syndrome

b. Heart muscle damage (cardiomyopathy) leading to congestive heart failure

c. Intestinal malabsorption

d. Liver swelling

e. Kidney failure

f. Nephrotic syndrome

g. Nerve problems (neuropathy)

h. Orthostatic hypotension (drop in blood pressure when the person stands up)

Primary amyloidosis is rare.

It is similar to multiple myeloma.

2. Secondary amyloid disease that is linked with chronic granulomatous diseases or infections

Other milder forms are:

3. Familial Amyloid disease – present with a family history of amyloid disease

4. Mixture of Primary and secondary amyloidosis

Classification

The modern amyloidosis classification started in the late 1960s with the formation of methods to solubilize amyloid fibrils.

These methods permitted chemical amyloid studies.

Amyloid is categorized chemically.

The amyloid diseases are termed with a capital A (for amyloid) accompanied by an abbreviation for the fibril protein:

AA amyloid disease

AA is a normal-sequence serum amyloid A protein which is an acute-phase chemical produced mainly in the liver in reaction to multiple cytokines.

Only a small amount of patients with raised serum amyloid A levels form amyloid disease

Systemic AA amyloid disease is a long-term complication of several chronic inflammatory disorders – e.g., rheumatoid arthritis, ankylosing spondylitis, Crohn's disease, malignancies and disorders predisposing to repeat infections.

The incidence of AA in rheumatoid arthritis and other chronic arthritides has reduced as a result of the use of more effective anti-inflammatory and immunosuppressive treatments.

Organ injury occurs from the extracellular deposition of proteolytic fragments of the acute-phase reactant serum amyloid A (SAA) as amyloid fibrils.

Only a small amount of patients with long-standing inflammation really occur with AA amyloid disease.

The kidneys, joints, liver and spleen are the main targets of AA amyloid