Lymphedema Hypoparathyroidism Syndrome
A very rare form of lymphedema.
Genetic/Inheritance: Autosomal recessive and X-linked recessive inheritance
Synonyms: Lymphedema hypoparathyroidism syndrome, Hypoparathyroidism lymphedema syndrome, Dahlberg borer newcomer syndrome, Dahlberg syndrome
Symptoms: hypoparathyroidism (A condition where the parathyroid glands don't produce enough parathyroid hormone for normal body functioning. The primary function of these hormones is to regulate body calcium levels.), nephropathy (kidney disease), congenital lymphedema, mitral valve prolapse (when the valve between your heart's left upper chamber (left atrium) and the left lower chamber (left ventricle) doesn't close properly. When the left ventricle contracts, the valve's leaflets bulge (prolapse) upward or back into the atrium.) and short end bones in fingers and toes.
Treatment: Treatment focuses on the symptoms/complications. This would include manual lymphatic drainage for the lymphedema. Treatment of hypothydroidism may include oral calcium carbonate tablets, Vitamin D, a diet ridh in calcium and low in phosphorus items. Treatment for the mitral valve prolaspse might include beta blockers, aspirin, prescription anticoagulants and surgery for the most severe cases.
May 29, 2008
Alternative titles; symbolsHYPOPARATHYROIDISM-LYMPHEDEMA SYNDROME
Dahlberg et al. (1983) described 2 adult brothers with congenital lymphedema, hypoparathyroidism, nephropathy, mitral valve prolapse and brachytelephalangy. The older sib was found to have bilateral cataracts on routine examination at age 19 years. Swelling of his arms and legs, noted soon after his birth, increased after he began walking. Progressive renal failure necessitated renal transplantation at age 26 years. The brother had similar findings. Both have a broad nasal bridge and lateral displacement of the inner canthi. Pulmonary lymphangiectasia (see 265300) was suspected on the basis of radiologic findings. The mode of inheritance is not clear but includes autosomal recessive and X-linked recessive inheritance.
Victor A. McKusick : 6/3/1986
supermim : 3/17/1992
supermim : 3/20/1990
ddp : 10/26/1989
marie : 3/25/1988
reenie : 6/3/1986
Copyright © 1966-2004 Johns Hopkins University
D I S E A S E : Dahlberg borer newcomer syndrome
Lymphedema hypoparathyroidism syndrome
respiratory system (Very frequent sign)
absent/small fingernails (Very frequent sign)
autosomal recessive inheritance (Very frequent sign)
brachydactyly (Very frequent sign)
broad nose (Very frequent sign)
cataract (Very frequent sign)
decreased sweating (Very frequent sign)
hypocalcemia (Very frequent sign)
hypothyroidy (Very frequent sign)
increased body hair (Very frequent sign)
long qt/preexcitation/abnormal ecg (Very frequent sign)
lymphoedema/oedema (Very frequent sign)
mitral valve prolapse/incompetence (Very frequent sign)
ptosis (Very frequent sign)
renal disease (Very frequent sign)
renal failure (Very frequent sign)
short foot/brachydactyly of toes (Very frequent sign)
short stature/dwarfism (Very frequent sign)
synophris (Very frequent sign)
telecanthus (Very frequent sign)
thick skin (Very frequent sign)
Hypoparathyroidism Patient/Family Resources
Hypothyroidism - primary
Hypothyroidism - secondary
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Lymphedema People - Support Groups
The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.
No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.
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Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.
Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.
Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.
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Associated with Lymphedema
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Page Updated: Dec. 28, 2011