Pregnanetriol in the Range of 1.2-2.1mg/m2/day as an Index of Optimal Control in CYP21A2 Deficiency

Izawa, Masako; Aso, Keiko; Higuchi, Asako; Ariyasu, Daisuke; Hasegawa, Yukihiro

doi:10.1297/cpe.16.45

Cited by 10 publications

(29 citation statements)

References 9 publications

(19 reference statements)

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“…Thus, an overtreatment state was defined using Cushing-like symptoms instead of increase in body weight in our previous study (11). …”

Section: Methodsmentioning

confidence: 99%

“…Therefore, we previously analyzed an ideal 24-h PT range based on auxological data and reported that the range of 1.2–2.1 mg/m 2 /day could be used as an index of optimal control in patients with CYP21 deficiency ( 11 ). The first morning urine sample can be obtained easily compared with collection of urine for 24 h. However, the utility of PT in the first morning urine samples (morning PT) as an index of therapeutic condition has never been reported.…”

Section: Introductionmentioning

confidence: 99%

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The Range of 2.2-3.3 mg/gCr of Pregnanetriol in the First Morning Urine Sample as an Index of Optimal Control in CYP21 Deficiency

Izawa

Aso

Higuchi

et al. 2008

Clin Pediatr Endocrinol

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Auxological data are the gold standard indexes of the therapeutic conditions in patients with CYP21 deficiency over long-term periods, whereas urinary pregnanetriol (PT) for 24 h has been used as an index for short-term periods. We previously reported that the range of 1.2–2.1 mg/m2/day of PT for 24 h (24-h PT) could be used as an index of optimal control in patients with CYP21 deficiency. The purpose of this study was to analyze the range of PT in the first morning urine samples (morning PT) as an index of optimal control in patients with CYP21 deficiency. First, the therapeutic periods of 15 participants (aged 2 yr and 5 mo to 17 yr and 4 mo) were classified into excessive, good or poor control periods using auxological data and Cushing-like symptoms, and 24-h PT levels were analyzed in each period, retrospectively. The 95% confidence intervals for the means of 24-h PT levels in the excessive, good and poor control periods were 0.24–2.24 (n=25), 2.88–4.92 (n=114) and 13.26–21.28 (n=72) mg/gCr, respectively. Subsequently, 24-h PT and morning PT levels collected on the same day were analyzed for 14 participants (aged 9 mo to 29 yr and 8 mo). There was a significant correlation between the above two PT levels (n=25, p<0.0001). When the 24-h PT range of the good control period, 2.88–4.92 mg/gCr, was adjusted by the correlation, the ideal morning PT range became 2.15–3.34 mg/gCr. In conclusion, a morning PT in the range of 2.2–3.3 mg/gCr can be used as an index of optimal control in patients with CYP21 deficiency.

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“…Thus, an overtreatment state was defined using Cushing-like symptoms instead of increase in body weight in our previous study (11). …”

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The Range of 2.2-3.3 mg/gCr of Pregnanetriol in the First Morning Urine Sample as an Index of Optimal Control in CYP21 Deficiency

Izawa

Aso

Higuchi

et al. 2008

Clin Pediatr Endocrinol

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“…Complete normalization of serum 17-OHP usually suggests doses of GC that are too high. As another method of monitoring, a urine concentration of pregnantriol (a urine metabolite of 17-OHP) of 1.2-2.1 mg/m 2 /day has been proposed [91]. In adult patients with 21-OHD who no longer have the problem of growth retardation, various types of GC have been used.…”

Section: [Growth Retardation and Therapy Monitoring In 21ohd]mentioning

confidence: 99%

Diagnosis and treatment of adrenal insufficiency including adrenal crisis: a Japan Endocrine Society clinical practice guideline [Opinion]

et al. 2016

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Abstract. This clinical practice guideline of the diagnosis and treatment of adrenal insufficiency (AI) including adrenal crisis was produced on behalf of the Japan Endocrine Society. This evidence-based guideline was developed by a committee including all authors, and was reviewed by a subcommittee of the Japan Endocrine Society. The Japanese version has already been published, and the essential points have been summarized in this English language version. We recommend diagnostic tests, including measurement of basal cortisol and ACTH levels in combination with a rapid ACTH (250 µg corticotropin) test, the CRH test, and for particular situations the insulin tolerance test. Cut-off values in basal and peak cortisol levels after the rapid ACTH or CRH tests are proposed based on the assumption that a peak cortisol level ≥18 µg/ dL in the insulin tolerance test indicates normal adrenal function. In adult AI patients, 15-25 mg hydrocortisone (HC) in 2-3 daily doses, depending on adrenal reserve and body weight, is a basic replacement regime for AI. In special situations such as sickness, operations, pregnancy and drug interactions, cautious HC dosing or the correct choice of glucocorticoids is necessary. From long-term treatment, optimal diurnal rhythm and concentration of serum cortisol are important for the prevention of cardiovascular disease and osteoporosis. In maintenance therapy during the growth period of patients with 21-hydroxylase deficiency, proper doses of HC should be used, and long-acting glucocorticoids should not be used. Education and carrying an emergency card are essential for the prevention and rapid treatment of adrenal crisis.Key words: Adrenal insufficiency, Adrenal crisis, Cortisol, Hydrocortisone, Congenital adrenal hyperplasia Summary of Recommendations I. Chronic adrenal insufficiency (AI) I-1.0 SymptomsWe recommend suspecting AI in patients who have the following symptoms.

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“…Normalized 17-OHP suggests an overdose of glucocorticoids. Monitoring using pregnanetriol (PT), a urinary metabolite of 17-OHP, by urine collection has also been proposed ( 7 , 112 , 113 , 114 ). In a study of 21-OHD in Japanese children, excluding the neonatal and pubertal stages, a PT range of 1.2–2.1 mg/m 2 /d was shown to indicate good control ( 113 ).…”

Section: Treatment Monitoring For Growing Childrenmentioning

confidence: 99%

“…Monitoring using pregnanetriol (PT), a urinary metabolite of 17-OHP, by urine collection has also been proposed ( 7 , 112 , 113 , 114 ). In a study of 21-OHD in Japanese children, excluding the neonatal and pubertal stages, a PT range of 1.2–2.1 mg/m 2 /d was shown to indicate good control ( 113 ). If 21-OHD cannot be determined before administration of glucocorticoids early in the morning, determination of the 17-OHP level and monitoring of PT by urine collection may be useful, while keeping the circadian rhythm and daily variance of 17-OHP in mind.…”

Section: Treatment Monitoring For Growing Childrenmentioning

confidence: 99%

Guidelines for diagnosis and treatment of 21-hydroxylase deficiency (2014 revision)

Ishii¹,

Anzo

Adachi

et al. 2015

Clin Pediatr Endocrinol

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Purpose of developing the guidelines: The first guidelines for diagnosis and treatment of 21-hydroxylase deficiency (21-OHD) were published as a diagnostic handbook in Japan in 1989, with a focus on patients with severe disease. The “Guidelines for Treatment of Congenital Adrenal Hyperplasia (21-Hydroxylase Deficiency) Found in Neonatal Mass Screening (1999 revision)” published in 1999 were revised to include 21-OHD patients with very mild or no clinical symptoms. Accumulation of cases and experience has subsequently improved diagnosis and treatment of the disease. Based on these findings, the Mass Screening Committee of the Japanese Society for Pediatric Endocrinology further revised the guidelines for diagnosis and treatment. Target disease/conditions: 21-hydroxylase deficiency. Users of the guidelines: Physician specialists in pediatric endocrinology, pediatric specialists, referring pediatric practitioners, general physicians; and patients.

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Pregnanetriol in the Range of 1.2-2.1mg/m2/day as an Index of Optimal Control in CYP21A2 Deficiency

Cited by 10 publications

References 9 publications

The Range of 2.2-3.3 mg/gCr of Pregnanetriol in the First Morning Urine Sample as an Index of Optimal Control in CYP21 Deficiency

The Range of 2.2-3.3 mg/gCr of Pregnanetriol in the First Morning Urine Sample as an Index of Optimal Control in CYP21 Deficiency

Diagnosis and treatment of adrenal insufficiency including adrenal crisis: a Japan Endocrine Society clinical practice guideline [Opinion]

Guidelines for diagnosis and treatment of 21-hydroxylase deficiency (2014 revision)

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