Symptoms
The symptoms of Sly syndrome are similar to those of Hurler syndrome (MPS I). The symptoms include:
- in the head, neck, and face: coarse (Hurler-like) facies and macrocephaly, frontal prominence, premature closure of sagittal lambdoid sutures, and J-shaped sella turcica
- in the eyes: corneal opacity and iris colobmata
- in the nose: anteverted nostrils and a depressed nostril bridge
- in the mouth and oral areas: prominent alveolar processes and cleft palate
- in the thorax: usually pectus carinatum or exacavatum and oar-shaped ribs; also a protruding abdomen and inguinal or umbilical hernia
- in the extremities: talipes, an underdeveloped ilium, aseptic necrosis of femoral head, and shortness of tubular bones occurs
- in the spine: kyphosis or scoliosis and hook-like deformities in thoracic and lumbar vertebrate
- in the bones: dysostosis multiplex
In addition recurrent pulmonary infections occur. Hepatomegaly occurs in the gastrointestinal system. Splenomegaly occurs in the hematopoietic system. Inborn mucopolysaccharide metabolic disorders due to β-glucuronidase deficiency with granular inclusions in granulocytes occurs in the biochemical and metabolic systems. Growth and motor skills are affected, and mental retardation also occurs.
Read more about this topic: Sly Syndrome
Famous quotes containing the word symptoms:
“For anyone addicted to reading commonplace books ... finding a good new one is much like enduring a familiar recurrence of malaria, with fever, fits of shaking, strange dreams. Unlike a truly paludismic ordeal, however, the symptoms felt while savoring a collection of one man’s pet quotations are voluptuously enjoyable ...”
—M.F.K. Fisher (1908–1992)
“A certain kind of rich man afflicted with the symptoms of moral dandyism sooner or later comes to the conclusion that it isn’t enough merely to make money. He feels obliged to hold views, to espouse causes and elect Presidents, to explain to a trembling world how and why the world went wrong. The spectacle is nearly always comic.”
—Lewis H. Lapham (b. 1935)
“Social movements are at once the symptoms and the instruments of progress. Ignore them and statesmanship is irrelevant; fail to use them and it is weak.”
—Walter Lippmann (1889–1974)