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- Sun Jul 08, 2007 2:27 pm
Muna is now 16-year-old female with multiple medical problems including the following:
1- Fatty acid oxidation defect [ SCHAD ]
2- Hypothyrodism, central.
3- Hypocorticalism, central.
4- Severe short stature.
5- Bilateral congenital dislocated hips.
Muna has been seen in King Faisal Specialist Hospital and Research Center since she was an infant. She was initially referred to the metabolic service where she was diagnosed to have Acyl-CoA Dehydrogenase Deficiency and was started on carnitin and riboflavin and Polycose for several years for her recurrent hypoglycemia. Subsequent to that, she was treated for bilateral congenital dislocated hips by the orthopedic team using harness and then a surgery. Unfortunately, it seemed to have recurred, and she has acetabular dysplasia bilaterally. As far as her endocrine problems, I started seeing her in 2000 because of Hypothyrodism which seems to be central, i.e., low TSH ( thyroid stimulating hormone) and low free T4. She was started on thyroxine and her thyroid function was monitored thereafter every three to four months with stable thyroid function on treatment. She was screened for other hormonal deficiency and was found to have both ACTH deficiency and Growth hormone deficiency. She was treated with hydrocortisone 5 mg p.o. two times a day and still receiving this treatment. Growth hormone stimulation test was done in 2001 and was found to be low. The peak level was 12 mU/L, which is low for her age. She was started on growth hormone injection in April 2002. Her height at that time was 120.5 cm, and her weight was 39 kg. Almost a year later, she gained 6.5 cm, which is considered a good response at this point. However, a year after, in March 2004, she had only gained 2 cm on a higher dose of growth hormone. So growth hormone was discontinued after two years of treatment because of what we could say a lack of response to growth hormone. Skeletal survey was done in 2004 showed scoliosis of the mid dorsal spine, severe hypoplasia of the iliac bones and bilateral acetabular dysplasia, short fourth metacarpal bones and thinning of the bone. Subsequently, she was referred to the pediatric orthopedics, and it was decided that she needs posterior spine fusion and instrumentation. The parents preferred to take a second opinion as far as the surgery is concerned. Currently, Muna is 16 years of age, She is 129.5 cm, well below the 3rd percentile, and she crossed her centile since the age of seven years which could be typical of some form of skeletal dysplasia. She is receiving thyroxine 50 mcg once a day and hydrocortisone 5 mg twice daily, and she is off the riboflavin and carnitine. She is followed by genetic metabolic team, endocrinology, orthopedics and nutrition service.
Muna has doen the Scoliosis surgery .
The question is : in a way or another, can she get more height ?
Thank you .
- Wed Jul 11, 2007 5:50 am