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Septo-optic dysplasia

Septo-optic dysplasia (SOD) is a congenital malformation syndrome manifested by hypoplasia (underdevelopment) of the optic nerve, hypopituitarism, and absence of the septum pellucidum (a midline part of the brain). In a severe case, this results in pituitary hormone deficiencies, blindness, and mental retardation. However, there are milder degrees of each of the three problems, and some children only have one or two of the three. more...

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The optic nerve hypoplasia is generally manifested by nystagmus (involuntary eye movements, often side-to-side) and a smaller-than-usual optic disk. The degree of visual impairment is variable, and ranges from normal vision to complete blindness. When nystagmus develops, it typically appears by 1-4 months of age, and usually indicates that there will be a significant degree of visual impairment, but the severity is difficult to predict in infancy. Although there are many measures to compensate for visual impairment, no treatment is available to induce normal optic nerve function.

The degree of pituitary deficiency is also variable, and ranges from normal function, to deficiency of a single hormone, to deficiency of both anterior and posterior hormones (termed panhypopituitarism). Hypopituitarism in this syndrome is most often manifested by growth hormone deficiency. If severe, it can lead to diagnosis in the first days of life by causing hypoglycemia, jaundice, and micropenis (if a boy). The cause of the jaundice is unknown, and an unusual aspect of it (compared to most neonatal jaundice) is that it can be largely a conjugated (direct) hyperbilirubinemia suggested of obstructive liver disease. It typically resolves over several weeks once hormone replacement is begun. All of the pituitary hormones can be replaced, and this is the treatment for deficiencies. Septo-optic dysplasia is one of the most common forms of congenital growth hormone deficiency.

The brain effects are also variable and range from normal intelligence to severe mental retardation. Seizures sometimes occur. Prediction of intellectual outcome in infancy is difficult. Various types of early intervention or equivalent programs can help a child reach full developmental potential, but if brain impairment is significant, it cannot be made normal by any treatment.

The cause of septo-optic dysplasia is not known. Rare familial recurrence has been reported, suggesting at least one genetic form, but in most cases it is a sporadic birth defect of unknown cause and does not recur again with subsequent pregnancies.

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24/7: JORDAN EXPECTS
From Sunday Mirror, 1/5/03 by POLLY GRAHAM

IF it was anyone else I wouldn't believe it, but here goes... Jordan is pregnant again. Well, that's what the glamour model has been telling friends.

She's told them she's expecting a baby with current boyfriend Scott Sullivan and she is already eight weeks gone.

And she was seen clutching her stomach as she posed with Scott at a New Year's Eve party in Brighton.

It is, of course, only eight months since she gave birth to her son Harvey, who is sadly blind.

Jordan, 24, has already gone to pains to deny my story - but then she would. It appears she isn't too happy about the new pregnancy, and friends say she plans to get rid of the baby.

Obviously she's got her hands full with Harvey, the son of Manchester United star Dwight York. And she's just been getting her career back on track after the birth, oh, and that ludicrous cancer scare.

But apparently 20-year-old Scott, who has been dating Jordan since October, is all for her having it.

Jordan has hardly won any mother-of-the-year awards since Harvey's birth. Some critics have blamed his condition - called septo-optic dysplasia - on his mum's constant partying throughout her pregnancy.

Then her babysitter claimed Jordan was more interested in nightclubbing than being a mum and was always dumping Harvey on other people while she had a good time.

A friend says: "Jordan has been trying to find a discreet London clinic, rather than where she lives in Brighton, to get rid of the baby.

"For once in her life she doesn't want the world to know.

"Scott has been very supportive and said he would stand by her but he's only young and probably wouldn't be able to cope with the reality of it all."

When Jordan got wind that I planned to reveal her secret she was straight on the phone.

She screeched: "I am not pregnant. Why would I go and get myself pregnant? I've already got a baby." Exactly. But why then have you been telling friends about your condition, my dear? Surely not to remain the centre of attention and to keep Scott interested in you?

Who knows in the weird world of the silicon girl.

Copyright 2003 MGN LTD
Provided by ProQuest Information and Learning Company. All rights Reserved.

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