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Sly syndrome

Mucopolysaccharidosis Type VII or Sly syndrome (named after its discoverer William Sly in 1969) is also sometimes called MPS. The defective gene lies on chromosome 7. MPS is transmitted as an autosomal recessive trait. more...

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It is an extremely rare inherited metabolic disorder characterized by a deficiency of the enzyme β-glucuronidase, a lysosomal enzyme. Sly syndrome belongs to a group of disorders known as the mucopolysaccharidoses, which are lysosomal storage diseases. In Sly syndrome, the deficiency in β-glucuronidase leads to the accumulation of certain complex carbohydrates (mucopolysaccharides) in many tissues and organs of the body.

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: dysotosis 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.

MPS type VII occurs in only 1:250,000 people.

Mucopolysaccahridosis Type VII is also known as β-glucurondinase deficiency, β-glucurondinase deficiency mucopolysaccahridosis, GUSB deficiency, mucopolysaccahride storage disease VII, MCA, and MR.

Read more at Wikipedia.org


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Toxic thoughts syndrome - Sisterspeak - Brief Article
From Ebony, 9/1/98 by Laura B. Randolph

I was talking with a Sister-friend on the phone the other night, a woman who, as anyone who knows her will tell you, is one of the most optimistic people on the planet. A modern-day Pollyanna with dreadlocks, Lucy always sees the glass half full. Which is why, 10 minutes into our conversation, I knew something was very wrong.

By very wrong I don't mean that my Sister-friend was facing some unexpected crisis (the loss of a job) or enforceable catastrophe (the loss of a loved one). I mean she was facing an emotional emergency, a psychic urgency. She had a bad case of TTS--toxic thoughts syndrome.

At some point in their life, most Sisters I know have experienced TTS--feelings of self-doubt that are so deep and so debilitating they make you question your smarts, your skills, yourself. An equal opportunity disease, TTS strikes Sisters of every age and occupation. No one is to it. Not the beautiful, not the powerful, not exert the truly gilled.

I continue to be amazed by the extraordinary women who have experienced TTS. Take, for instance, Patti LaBelle. When LaBelle, the ahead-of-its-time trio with whom she sang lead in the '70s, broke up, Patti says she wondered if she had what it took to make it solo.

"As much as I loved to sing, just the thought of going onstage all alone--without Sarah and Nona--terrified me," she confided to me. "My sell-doubt was just too deep." Then there is Oprah Winfrey. "I have struggled with my own sell-value for many, many years," she once confessed to me. Even when she was winning award after award for her history-making talk: show, Oprah said she was filled with toxic thoughts that, at their root, sprang from the same fear: She just wasn't good enough.

You would think that, in light of those eye-opening encounters, not to mention my own experience with TTS, the moment Lucy called, I would have recognized it. But I didn't. I thought Lucy was just going through the end-of-summer blues; you know; the sadness we all feel about saying good-bye to sun-drenched days at the beach, moonlit nights on the porch, and all the other sensational, singularly summer stuff Sly and the Family Stone immortalized in the group's unforgettable classic, "Hot Fun In the Summertime."

The more Lucy talked, however; the more I knew this was no run-of-the-mill case of the end-of-summer blues. This was something much more serious, much more insidious. Still, I didn't realize it was TTS until Lucy exhibited its defining, tell-tale symptom: She started questioning everything. And when I say everything, I mean everything!

She started with her looks. "You won't believe how much weight I've put on," she groaned. "At this rate, I'm going to have to hire Omar the tentmaker to make my dresses."

She had barely finished trashing her looks when she started questioning her competence. "I just can't compete at work," she lamented. "I'm never going to get that promotion."

Over the next 10 minutes, Lucy questioned everything from her finances ("I'm never going to be able to save enough money for a house.") to her future ("The odds of me finding Mr. Right are slim to none, so I just need to accept the fact that I'm going to be alone for the pest of my life").

"What am I going to do?" she finally asked.

Occasionally, I say the right thing. And this, I think, was one of those occasions. I told Lucy the stray of something that happened to Maya Angelou, something amazing, when she was 24 years old and living in San Francisco. It began with a request by her voice teacher, Frederick Wilkerson, that she read to him. As Sister Maya tells us in her wise and wonderful book, Wouldn't Take Nothing For My Journey Now, Mr. Wilkerson didn't want her to read just anything. He wanted her to read a specific section from the book, Lessons in Truth, a section which ended with the words "God loves me."

As Mr. Wilkerson requested, Sister Maya read the passage. Then she closed the book. Mr. Wilkerson told her to read it again. "I pointedly opened the book," she writes, "and I sarcastically read, `God loves me.'" Unfazed, Mr. Wilkerson told her to read it again.

After about the seventh repetition, something magical happened. As Sister Maya was saying the words, she started to feel them. She started to believe in their truth. She started to know their power. She started to accept and embrace them way down deep at her center, in her very core.

"I suddenly began to cry at the grandness of it all," she writes. "I knew that if God loved me, then I could do wonderful things, I could try great things, learn anything, achieve anything."

As I told Lucy, if, like Sister Maya, we soak up these words, we would know it, too. We would have no reason or room for insecurity and self-doubt. If, like Sister Maya, we soak up these words, we would know our value. Whenever TTS tried to rear its ugly head, we could stop it by following Sister Maya's lead: By reciting those three powerful words--"God loves me"--until we feel the essential, empowering truth of them in our soul.

And when we feel it, we will know what Sister Maya knows, that life-changing lesson she learned that day in San Francisco: "For what could stand against me with God, since one person, any person, with God, constitutes the majority?"

COPYRIGHT 1998 Johnson Publishing Co.
COPYRIGHT 2000 Gale Group

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