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Mucopolysaccharidosis type I (MPS I) is a condition that affects many parts of the body. It is a progressively debilitating disorder; however, the rate of progression varies among affected individuals. MPS I is caused by mutations in the IDUA gene. These mutations lead to reduced levels or the complete lack of the IDUA enzyme.

Mucopolysaccharidosis type III (MPS III), also known as Sanfilippo syndrome, is a progressive disorder that primarily affects the brain and spinal cord (central nervous system). It is characterized by deterioration of neurological function (neurodegeneration), resulting in many of the features of the condition. Other body systems can also be involved, although the physical features are usually mild in the early stages.

People with MPS III generally do not display any features of the condition at birth, but they begin to show signs and symptoms of the disorder during early childhood. Affected children often initially have delayed speech and behavior problems. They may become restless, destructive, anxious, or aggressive, and some display features of autism spectrum disorder, which is a condition characterized by difficulty with social interactions and communication. Sleep disturbances are also very common in children with MPS III. This condition causes progressive intellectual disability and the loss of previously acquired skills (developmental regression). In later stages of the disorder, people with MPS III may develop seizures and movement disorders.

The physical features of MPS III are less pronounced than those of other types of mucopolysaccharidosis. Individuals with MPS III typically have mildly 'coarse' facial features, a large head (macrocephaly), a slightly enlarged liver (mild hepatomegaly), and a soft out-pouching around the belly-button (umbilical hernia) or lower abdomen (inguinal hernia). Some people with MPS III have short stature, joint stiffness, or mild dysostosis multiplex, which refers to multiple skeletal abnormalities seen on x-ray. Affected individuals often experience chronic diarrhea and recurrent upper respiratory and ear infections. People with MPS III may also have hearing loss and vision problems.

MPS III is divided into types IIIA, IIIB, IIIC, and IIID, which are distinguished by their genetic cause. The different types of MPS III have similar signs and symptoms, although the features of MPS IIIA typically appear earlier in life and progress more rapidly. People with MPS III usually live into adolescence or early adulthood.

Frequency

MPS III is the most common form of mucopolysaccharidosis; the estimated incidence of all four types combined is 1 in 70,000 newborns. MPS IIIA and MPS IIIB are much more common than MPS IIIC and MPS IIID.

Causes

Mutations in the GNS, HGSNAT, NAGLU, and SGSH genes cause MPS III. These genes provide instructions for making enzymes involved in the breakdown of large sugar molecules called glycosaminoglycans (GAGs). GAGs were originally called mucopolysaccharides, which is where this condition gets its name. The GNS, HGSNAT, NAGLU, and SGSH enzymes are involved in the step-wise breakdown of a subset of GAGs called heparan sulfate.

MPS IIIA is caused by mutations in the SGSH gene, and MPS IIIB is caused by NAGLU gene mutations. Mutations in the HGSNAT gene result in MPS IIIC, and GNS gene mutations cause MPS IIID. Mutations in these genes reduce or eliminate enzyme function. A lack of any one of these enzymes disrupts the breakdown of heparan sulfate. As a result, partially broken down heparan sulfate accumulates within cells, specifically inside the lysosomes. Lysosomes are compartments in the cell that digest and recycle different types of molecules. Conditions such as MPS III that cause molecules to build up inside the lysosomes are called lysosomal storage disorders. Researchers believe that the accumulation of GAGs interferes with the functions of other proteins inside the lysosomes and disrupts the normal functions of cells. It is unknown why the buildup of heparan sulfate mostly affects the central nervous system in MPS III.

Inheritance

This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

Other Names for This Condition

• MPS III
• Mucopolysaccharidosis III
• Sanfilippo syndrome

Additional Information & Resources

Genetic Testing Information

• Genetic Testing Registry: Mucopolysaccharidosis, MPS-III-A
• Genetic Testing Registry: Mucopolysaccharidosis, MPS-III-B
• Genetic Testing Registry: Mucopolysaccharidosis, MPS-III-C
• Genetic Testing Registry: Mucopolysaccharidosis, MPS-III-D

Genetic and Rare Diseases Information Center

• Mucopolysaccharidosis type III

Patient Support and Advocacy Resources

Research Studies from ClinicalTrials.gov

• ClinicalTrials.gov

Catalog of Genes and Diseases from OMIM

References

• Bodamer OA, Giugliani R, Wood T. The laboratory diagnosis ofmucopolysaccharidosis III (Sanfilippo syndrome): A changing landscape. Mol Genet Metab. 2014 Sep-Oct;113(1-2):34-41. doi: 10.1016/j.ymgme.2014.07.013. Epub 2014Jul 16. Review. Citation on PubMed
• Gilkes JA, Heldermon CD. Mucopolysaccharidosis III (Sanfilippo Syndrome)-disease presentation and experimental therapies. Pediatr Endocrinol Rev. 2014Sep;12 Suppl 1:133-40. Review. Citation on PubMed
• Malm G, Månsson JE. Mucopolysaccharidosis type III (Sanfilippo disease) inSweden: clinical presentation of 22 children diagnosed during a 30-year period.Acta Paediatr. 2010 Aug;99(8):1253-7. doi: 10.1111/j.1651-2227.2010.01800.x. Epub2010 Mar 14. Citation on PubMed
• Meyer A, Kossow K, Gal A, Mühlhausen C, Ullrich K, Braulke T, Muschol N.Scoring evaluation of the natural course of mucopolysaccharidosis type IIIA(Sanfilippo syndrome type A). Pediatrics. 2007 Nov;120(5):e1255-61. Epub 2007 Oct15. Citation on PubMed
• Ruijter GJ, Valstar MJ, van de Kamp JM, van der Helm RM, Durand S, vanDiggelen OP, Wevers RA, Poorthuis BJ, Pshezhetsky AV, Wijburg FA. Clinical andgenetic spectrum of Sanfilippo type C (MPS IIIC) disease in The Netherlands. Mol Genet Metab. 2008 Feb;93(2):104-11. Epub 2007 Nov 19. Citation on PubMed
• Valstar MJ, Bertoli-Avella AM, Wessels MW, Ruijter GJ, de Graaf B, Olmer R,Elfferich P, Neijs S, Kariminejad R, Suheyl Ezgü F, Tokatli A, Czartoryska B,Bosschaart AN, van den Bos-Terpstra F, Puissant H, Bürger F, Omran H, Eckert D,Filocamo M, Simeonov E, Willems PJ, Wevers RA, Niermeijer MF, Halley DJ,Poorthuis BJ, van Diggelen OP. Mucopolysaccharidosis type IIID: 12 new patientsand 15 novel mutations. Hum Mutat. 2010 May;31(5):E1348-60. doi:10.1002/humu.21234. Citation on PubMed
• Valstar MJ, Ruijter GJ, van Diggelen OP, Poorthuis BJ, Wijburg FA. Sanfilippo syndrome: a mini-review. J Inherit Metab Dis. 2008 Apr;31(2):240-52. doi:10.1007/s10545-008-0838-5. Epub 2008 Apr 4. Review. Citation on PubMed

On March 23, 2021, the Milwaukee Board of School Directors approved the Administration’s recommendation to return to in-person learning.

Students will attend four days of in-person learning and virtual instruction on Wednesdays. In-person learning will begin on the following dates:

• Wednesday, April 14, 2021: K3 through grade 3 (attending Wednesday, Thursday, and Friday this week).
• Monday, April 19, 2021: Grade 4 through grade 8.
• Monday, April 26, 2021: High school seniors and students in need of support. All other freshmen, sophomores and juniors will remain in virtual learning for the remainder of the school year.
• Note that all students will learn virtually on Wednesdays beginning April 21.

A virtual option will be available for families who do not wish to return to in-person learning.

Mps Schoology

Families and staff can review the questions and responses from the Milwaukee Board of School Directors.

Roadmap to Readiness Return to Classroom Survey

Schools will survey families to determine if their child will be attending in-person or remain virtual for the remainder of the school year. Families will also be surveyed regarding transportation needs.

Families can take the survey online. The survey will be open until Thursday, April 1, 2021.

Parent Engagement Sessions

The district will hold three virtual family engagement sessions to help families learn about school routines, procedures, and safety measures that will allow students to safely return to in-person learning. Topics that will be covered include:

• Personal protective equipment in schools
• Bus transportation
• Supports provided by school nurses
• Procedures for sick children
• Morning wellness checks at home
• Before- and after-school care

Parent engagement sessions will be held from 5:30-7:00 p.m. on the following dates:

• Wednesday, March 31, 2021: K3 through grade 3
• Wednesday, April 14, 2021: Grades 4 through grade 8
• Wednesday, April 21, 2021: High school

Registration is open for the Virtual Family Engagement Sessions and families can request interpreters when they register. All sessions will be recorded and posted on the Roadmap to Readiness website.

Flyers

Download the Ready, Set, Return to Classroom – A Family Guide Flyer

Mpsos

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Summer Academy

Registration for Summer Academy is open! For information, please visit the MPS Summer Academy webpage.

Stay Connected

It is vital that your contact information is up-to-date in our records so you can be sure to receive Back to School information from the district and your school.

The district is now utilizing the SchoolMessenger system to send text messages to the mobile phones of MPS staff and families to keep everyone up-to-date with important information, safety alerts, school closing and more.

MPS staff and families will need to opt-in to continue to receive important district and school messages to their phone. Everyone will need to opt-in by sending a text message to 67587 with “Y”. You can do this step with any mobile phone you would like to receive messages. For questions or assistance, please contact the Department of Communications and Marketing at 414-475-8274 or comm@milwaukee.k12.wi.us

Parent Portal Technical Support

To set up a new parent portal account or receive assistance with an existing account, please visit the Technical Help webpage.

Mps Email

Milwaukee Public Schools
5225 W. Vliet Street
Milwaukee, WI 53208
Switchboard: (414) 475-8393