health management of adult turner women by gynecologists...

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)


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”


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.


J clin Endocrinol Metab;2007


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


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


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


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


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

**


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


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


Thyroid dysfunction



Thyroid dysfunction & Thyroid antibodies

２４５１９

１８５１３

６０６(-)

total(+)(-)dysfunction

antibodies

（ no. of patients ）

n.s.(+)

total


Metabolic disorders



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


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.


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.


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



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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