| 1. |
- Falhammar, H, et al.
(författare)
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Fractures and Bone Mineral Density in Adult Women with 21-Hydroxylase Deficiency.
- 2007
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Ingår i: J Endocrinol Metab. - : The Endocrine Society. - 0021-972X .- 1945-7197.
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Tidskriftsartikel (refereegranskat)abstract
- Context: Patients with classic congenital adrenal hyperplasia (CAH) receive lifelong, often supraphysiological, glucocorticoid therapy. Pharmacologic doses of glucocorticoids are an established risk factor for osteoporosis. Objectives: To evaluate bone mineral density (BMD), fracture prevalence and markers of bone metabolism in adult females with CAH. Design: This was a cross-sectional observational study. Setting: Tertiary care referral centers. Participants: We studied 61 women, aged 18-63 years, with genetically verified CAH due to 21-hydroxylase deficiency. They were patients with salt-wasting (n = 27), simple virilising (n = 28) and non-classic 21-hydroxylase deficiency (n = 6). Sixty-one age-matched women were controls. Main outcome measures: History of fractures was recorded. Total body, lumbar spine and femoral neck BMD were measured by dual-energy X-ray absorptiometry. The WHO criteria for osteopenia and osteoporosis were used. Serum marker of bone resorption, beta-C telopeptide (CTX) was studied. Results: The mean glucocorticoid dose in hydrocortisone equivalents was 16.9 +/- 0.9 mg/m(2). Patients had lower BMD than controls at all measured sites (P < 0.001). In patients < 30 years old 48% were osteopenic vs 12% in controls (P < 0.009). In patients >/= 30 years old 73% were osteopenic or osteoporotic vs 21% in controls (P < 0.001). BMD was similar in the two classic forms and had no obvious relationship to genotypes. CTX was decreased in older patients. More fractures were reported in patients than controls (P < 0.001). The number of vertebrae and wrist fractures almost reached significance (P = 0.058). Conclusion: Women with CAH have low BMD and increased fracture risk. BMD should be monitored, adequate prophylaxis and treatment instituted, and glucocorticoid doses optimized from puberty.
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| 2. |
- Falhammar, Henrik, et al.
(författare)
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Metabolic profile and body composition in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
- 2007
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Ingår i: The Journal of clinical endocrinology and metabolism. - : The Endocrine Society. - 0021-972X .- 1945-7197. ; 92:1, s. 110-6
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Tidskriftsartikel (refereegranskat)abstract
- CONTEXT: The chronic, often supraphysiological glucocorticoid doses used in congenital adrenal hyperplasia (CAH) might increase morbidity in cardiovascular disease and diabetes. OBJECTIVE: Our aim was to assess risk factors for cardiovascular disease and diabetes in CAH women. SUBJECTS, METHODS, AND DESIGN: We compared 61 women, 18-63 yr, with CAH due to 21-hydroxylase deficiency with 61 age- and sex- matched controls. Twenty-seven were younger than 30 yr, and 34 were 30 yr or older. Anthropometry, fat and lean mass measured by dual-energy x-ray absorptiometry, serum lipids, insulin, and adrenocortical steroids were studied. MAIN OUTCOME MEASURE: Body composition and cardiovascular risk factors were the main outcome measures. RESULTS: Younger patients and controls had similar waist to hip ratio, lean and fat mass, and insulin. Older patients had higher waist to hip ratio, lean mass, and insulin than controls. Fat mass was similar to controls but higher than in younger patients. Lipid profiles were slightly more favorable in older patients than controls. Gestational diabetes was more common in patients (21% of pregnancies vs. 0, P < 0.026). Few older patients had hypertension, cardiovascular disease, or diabetes. Despite moderate glucocorticoid doses, most patients had suppressed androgens. CONCLUSIONS: No clear evidence of unfavorable cardiovascular risk factors were found. Increased fat mass and higher insulin levels were, however, found in patients older than 30 yr. High frequency of gestational diabetes is a risk marker for future diabetes. Lifelong follow-up, lifestyle modifications, and attempts to adjust and reduce the glucocorticoid doses seem important.
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| 3. |
- Holmdahl, Gundela, 1956, et al.
(författare)
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Self-catheterization during adolescence.
- 2007
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Ingår i: Scandinavian journal of urology and nephrology. - : Informa UK Limited. - 0036-5599 .- 1651-2065. ; 41:3, s. 214-7
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: It has been suggested that clean intermittent catheterization (CIC) during the teenage years is associated with poor motivation to comply with the treatment, recurrent urinary tract infections and epididymitis. The aim of this study was to identify the main problems and complications associated with self-CIC in a group of adolescents with no overt neurological problems. MATERIAL AND METHODS: The medical records of 24 boys and girls aged >/=12 years on self-CIC with severe bladder dysfunction and intact perineal sensibility and motor function in the lower limbs were reviewed. The median age of the patients was 16.5 years (range 12-24 years) and they had been on CIC for a median of 11 years (range 2-16 years). RESULTS: No urethral damage was reported. Of the 11 patients (46%) who had good CIC routines, two had cystitis and none had epididymitis. Thirteen patients (54%) reported a poor CIC regimen at a median age of 11.5 years (range 9-16 years). In this group, 10 had recurrent cystitis, five had pyelonephritis, four had increasing creatinine levels and three of nine boys had epididymitis. CONCLUSIONS: One of the main problems associated with CIC during adolescence is poor compliance with the treatment. Epididymitis and recurrent urinary tract infections were seen more frequently in connection with poor CIC routines.
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| 4. |
- Sillén, Ulla, 1946, et al.
(författare)
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Treatment of bladder dysfunction and high grade vesicoureteral reflux does not influence the spontaneous resolution rate.
- 2007
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Ingår i: The Journal of urology. - : Ovid Technologies (Wolters Kluwer Health). - 0022-5347 .- 1527-3792. ; 177:1
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: We investigated whether the treatment of bladder dysfunction in infants with congenital high grade vesicoureteral reflux could influence the spontaneous resolution rate of the reflux and the number of recurrent urinary tract infections. MATERIALS AND METHODS: A total of 115 infants with high grade vesicoureteral reflux were included in a followup study of bladder function and reflux resolution between 1993 and 1999. The present study deals with 20 of these infants with mainly grade V reflux, most of whom had recurrent urinary tract infections in combination with high post-void residual and high bladder capacity. Treatment with clean intermittent catheterization was instituted during infancy in these 20 patients and continued until a median age of 4 years. RESULTS: Bladder capacity was high at presentation and at all followup investigations in the clean intermittent catheterization treated group. Moreover, residual urine was high at presentation and especially between ages 1 and 2 years. However, after bladder control was achieved this residual decreased and consisted mainly of reflux urine. In only 1 girl did reflux resolve spontaneously during the 4-year followup period. A total of 18 patients were treated surgically at the end of this period, and clean intermittent catheterization could be stopped a few months later in all but 2. Urinary tract infection recurrences were uncommon after the institution of treatment. CONCLUSIONS: The treatment of bladder dysfunction characterized by a high capacity bladder with poor emptying in infants with congenital high grade reflux does not influence the rate of spontaneous resolution. On the other hand, the tendency toward recurrent urinary tract infections appears to decrease with treatment.
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