Hurler Syndrome/(Mucopolysaccharidosis Type I MPH I): Aetiology, History, Symptoms, Treatment etc. An Overview.

· Dr.Hakim Saboowala
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Hurler Syndrome/(Mucopolysaccharidosis Type I MPH I): Aetiology, History, Symptoms, Treatment etc.

                                                    An Overview.

Hurler syndrome was first described by German paediatrician, Gertrud Hurler in 1919.

     It is one of the 11 disorders of the mucopolysaccharidoses (MPS).

     Hurler syndrome is considered as mucopolysaccharidosis type I (MPH I) and formerly known as gargoylism.

    It is an inherited lysosomal disorder caused by the absence of an enzyme responsible for the degradation of glycosaminoglycans (GAGs).

    This leads to a build-up of dermatan sulfate and heparin sulfate in multiple tissues, resulting in progressive deterioration and eventually, death.

     An attempt has been made in this Booklet to discuss the pathophysiology, evaluation, and management of Hurler Syndrome and the role of the interprofessional team along with relevant ILLUSTRATIONS for better understanding.  

…Dr. H. K. Saboowala.

                                 M.B (Bom) .M.R.S.H.(London)

 

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About the author

It is my passion to research for the Current/Recent Medical Topics and compile the E-Booklets as a reference to enrich the knowledge of busy Medicos at one CLICK, that too with an affordable price!

        Basically, I am a General Physician and a Dermatologist, practicing in Mumbai (India) since last 45 years.

          I am inspired for this ardent process of authoring after getting an overwhelming response (from Medical Community throughout India) for my Three E- Booklets titled:

1. "Medical Palmistry", (4000 copies distributed by "GLENMARK PHARMA'' -A multinational

 giant Pharmaceutical Company).

2. "Medical Mycoses" (2000 copies distributed by "MICROLAB."

3. “CRISPR Cas9 ”(An ENZYMATIC SCISSOR )

   (95 copies sold by “AMAZON KINDLE” in a year  

     worldwide).

 (All of them are available at Amazon.com. and Google Play at an affordable price at present).        

        At present there are 460 interesting & informative and a Safe Research-oriented E- Booklets are available at Amazon.com.at affordable prices .

      In view of this I have published a catalogue titled

"A Catalogue of a Elegant 200 compilation of E-Medical Booklets" enabling the readers to select the topic of choice and buy at leisure from:     

              amazon.com/author/dr.hakim_e-medicalbooks. 

     Moreover, I have been awarded recently the CERTIFICATES of Self Achievement and MEDALS for “The Record Number of 460 Multidisciplinary E – Medical Booklets Authored & Self Published by An Individual.” by “INDIASWORLD RECORDS” & “GOLDEN BOOK OF WORLD RECORDS

...Dr. Hakim. K. Saboowala.

                  M.B.(Bom) M.R.S.H. (London)

 

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