health management of adult turner women by gynecologists...
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Health management of Health management of Adult Turner Women by Adult Turner Women by Gynecologists in Japan Gynecologists in Japan
Hideya Sakakibara, M.D & Ph.DHideya Sakakibara, M.D & Ph.D
Dept. OB/GYNDept. OB/GYN
Yokohama City UniversityYokohama City University
Contents
3. Situation of adult Turner women’s health management
4. Screening of complications based on the “guidelines” for care of
Turner women
5. Earlier detection and prevention of complications according to the
“risk factors”
Situation of adult Turner women’s health management
Yokohama City Univ.
Complications in Women with Turner Syndrome
< childhood>
< adolescence>
< adulthood>
short stature
cardiovascular malformationrenal diseaseotitis media
infertilityosteoporosis
thyroid disorderhypertensiondyslipidaemiadiabetes mellitusneoplasm of gonads
無月経第二次性徴の欠如
other complications
gonadal dysfunction
primary amenorrhea
disorder of sexual development
Yokohama City Univ.
Complications in adult Turner Women in Australia
(Internal Medicine Journal ; 2006)
Yokohama City Univ.
Situation of adult health care in Australia
• Questionnaire survey to 39 adult Turner women
• Situation of follow-up regular follow-up 24
(63.2%) adequate surveillance 17 (43.6%)
• Attending physician endocrinologists 16 (66.7%)
general practitioners 6 (25.0%)gynecologists 2 (8.3%)
(Internal Medicine Journal ; 2006)
Situation of adult health care in Japan
• Questionnaire survey to of 83 Turner Adults
• Adequate surveillance blood test 88% urine test 78% BMD 61% chest XP 48% audiometry 41% etc.
• Attending physician 1 pediatricians2 gynecologists3 general practitioners / endocrinologists
(Turner Society in Osaka and Tokyo) Yokohama City Univ.
Yokohama City Univ.
Transition is an important problem in Japan
⇒ endocrinologistpediatricians ⇒ gynecologist
⇒ general practitioner ⇒ other physicians
pediatric to adult healthcare
Yokohama City Univ.
Summary(1)Situation of the adult health care in Japan
• There are few physicians who are familiar with the health problems associated with adult Turner women among adult health care providers.
• The ratio of adequate surveillance was about 40% as well as Australia.
• Transition is an important problem in Japan.
Screening of complications
based on the “guidelines”
Yokohama City Univ.
Recommendation of Transition
The transition from pediatric to adult healthcare supervision of women with TS should occur at the completion of puberty, usually by 18 yr of age. Ideally, the process of transition should take place over a period of 2-3 yr during the late pubertal period and should involve a gynecologist with expertise in fertility problems. Physicians familiar with the natural history and unique problems associated with TS should be responsible for organizing adult care. .
(Recommendations for the Diagnosis and Management of Turner Syndrome ;JCEM 2001 )
during the late pubertal period (by 18 yr of age) to physicians familiar with the natural history and
unique problems associated with TS should involve a gynecologist with expertise in
fertility problems.
• Management of reproductive health in the patients with gonadal dysfunction.
2. Providing a comprehensive health care throughout their life stage.
3. Taking a primary responsibility for the multidisciplinary treatment.
childhood ⇒ adolescence ⇒ adulthood ⇒ climacteric
pediatricians ⇒ gynecologists ⇒ experts in other fields
「 Reproductive Health Clinic 」
Yokohama City Univ.
Objectives of health management of adult Turner women
1 Management of gonadal dysfunction
by Hormone Replacement Therapy (HRT)
2 Care of complications developed in present or past history of the patients
3 Screening of possible complications developed in the future based on the “guidelines”
Yokohama City Univ.
Yokohama City Univ.
J clin Endocrinol Metab;2007
Yokohama City Univ.
Check up list for the health management of adult Turner women
General
Blood pressure
Body weight
Laboratory test
Hematological
Liver enzymes
Renal functions
Lipids
Blood sugar
Coagulation system
Urine analysis
Chest X ray
Bone mineral density
Gynecological
Pap smear
Sonographic evaluation
Digital examination
Others
Hearing test
Sonographic evaluation
heart
kidney
Cardiovascular MRI
Endocrinological Laboratory test Gonadotropins Estradiol Prolactin Teststerone TSH free T3 free T4
Yokohama City Univ.
Characteristics of the patients
Total Number of the Patients 57 Number of referred Patients 52
from Pediatrician 40 Gynecologist 11
Physician 1 Mean Age at the First Visit (y.o.) 23.9 Height (cm) 144.8±6.2Weight (kg) 46.7±7.9 Body Mass Index (kg/m2)
22.4±4.0History of Growth Hormone or Anabolic Steroid Therapy (No. of cases) 31
Yokohama City Univ.
Gonadal function of the patients
Status of menstruation No. of casesPrimary Amenorrhea 47 Secondary Amenorrhea 3 Spontaneous menstrual cycles 7
History of hormone replacement therapy No. of casesReceived 35 Non-received 20 Uncertain 2
Yokohama City Univ.
Complications in present or past history of the patients
Complications No. of cases . Osteoporosis/ osteopenia 21Otitis Media 14
Hearing loss 3 Thyroid dysfunction 10
Hyperthyroidism 2 Hypothyroidism 8
Cardiovascular disease 7 Urinary tract abnormality 6 Liver dysfunction 6 Glucose intolerance 5
Type 2 diabetes 2 Bone fracture 3
fracture of femur neck 2 Gonadoblasatoma 3 Scoliosis 2 Adenoma of mammary gland 1 Dyslipidemia 1
Yokohama City Univ.
Relationship between BMD (L2-4) and menstrual status of the patients
Menstrual status No. of cases BMD ( g/cm 2 )
Spontaneous Cycle 6 0.993 ± 0.129
Primary Amenorrhea 41 0.809 ± 0.116*
Receiving HRT 29 0.834 ± 0.106Non-receiving HRT 10 0.717 ± 0.099**
*P<0.05 compared to patients with spontaneous cycle **P<0.01 compared to patients with receiving HRT .
Comparison of BMD (L2-4) by menstrual status of the patients
0
0.2
0.4
0.6
0.8
1
SC PA HRT(+) HRT(- )
g /cm2 *
Yokohama City Univ.
*P<0.05
**P<0.01
**
Yokohama City Univ.
Complications developed in the patients during management at our institution
Complications No. of cases
Glucose intolerance 4
Type 2 diabetes 2 Liver dysfunction 3
Dyslipidemia 3 Cholesteatoma 2
Surgical treatment 2 Inflammatory bowel disease 2
Crohn's disease 1 Ulcerative colitis 1
Adenoma of mammary gland 2 Secondary amenorrhea 1Hypothyroidism 1 Hypertension 1
Yokohama City Univ.
Summary(2) Screening of complications based on the “guidelines”
• Comprehensive health care for TS adults in our Reproductive Health Clinic functions well.
• In this system, gynecologists play an important role by checking and managing their patient’s medical problems in cooperation with experts from other fields.
Earlier detection and prevention of complications according to the
“risk factors”
•Thyroid antibodies for earlier detection of thyroid dysfunction •BMI for prevention of metabolic disorders
Yokohama City Univ.
Thyroid dysfunction
J clin Endocrinol Metab;2007
Yokohama City Univ.
Thyroid dysfunction & Thyroid antibodies
24519
18513
606(-)
total(+)(-)dysfunction
antibodies
( no. of patients )
n.s.(+)
total
Yokohama City Univ.
Metabolic disorders
J clin Endocrinol Metab;2007
Yokohama City Univ.
BMI & metabolic disorders
BMI No. of TS No. of MDIncidence (%
)
< 25 44 6 13.6
≧25 12 7 58.3
Total 56 13 23.2
p=0.004
Yokohama City Univ.
Summary(3)Earlier detection and prevention
of complications according to the “risk factors
• Thyroid antibodies could be a factor for earlier detection of thyroid dysfunction, because all of the patients with thyroid dysfunction had the antibodies while all antibody-negative patients had normal thyroid function.
• BMI should be a good factor for prevention for metabolic disorders, because the incidence of metabolic disorders in obese patients was significantly higher than that of non-obese patients.
Yokohama City Univ.
For the future
The following observations should be considered in the future: In this study the timing of the transitionwas relatively late; an early referral in cooperation with pediatricians is preferable.
Secondly, as the patient ages, more careful management will be needed because complications, such as hypertension and dyslipidemia are more likely to appear.
Yokohama City Univ.
but ・・・
Ideal health care is not available for all adult Turner women.
They should manage their own health problems by themselves in adulthood.
so !
For the health care of Adult Turner Women
Yokohama City Univ.
Yokohama City Univ.
Thyroid function
HeightWeight
BMI
Acknowledgement
Stuff of the Reproductive Health Clinic
Hideya SakakibaraMariko MuraseKazuko SumitomoYosiyuki NomuraTomoko NagataYasuko SuzukiTomonari HayamaRyoko AsanoAkiko NaraAiko KawanoReiko NakamuraYuko Tagami
Thank you for your kind attention !Thank you for your kind attention !
Hideya Sakakibara, M.D & Ph.DHideya Sakakibara, M.D & Ph.D
Dept. OB/GYNDept. OB/GYN
Yokohama City UniversityYokohama City University
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