JAFES-Booklet (English - pdf - 1103 Kb) - MEMS

Vol 24, No 1 (SUPPLEMENT) ISSN 0857-1074 May 2009
Proceedings of the First
Malaysian Endocrine And Metabolic Society Annual Congress,
Penang, Malaysia. 28th-31st May 2009.

Contents:
CLINICAL TRIALS AND STUDIES
A Genome Scan for Malay Type 2 Diabetes Mellitus Patients.
A Randomized Control Trial To Assess The Efficacy Of Structured Diabetic Counseling On Glycaemic And Metabolic Parameters Amongst
Patients With Type 2 Diabetes Mellitus.
A1C As A Screening Tool To Diagnose Dibetes Mellitus In A Rural Population Based Study.
Abnormal Glucose Tolerance In Tanjung Karang: The Burden Of Increasing Prevalence And Under-diagnosis.
Adiponectin In The Context Of Metabolic Syndrome Amongst Staff Of Ministry Of Health.
Binge Eating Behavior And The Preference For Carbohydrate Among The Staff Of The Malaysian Ministry of Health, Putrajaya.
Clinical And Endocrine Profile Of Patients With Precocious Puberty: A 4-year Experience In Putrajaya Hospital.
Gene Expression Profiling Associated With Diabetic Nephroapthy In Malay Type 2 Diabetes Mellitus.
Ghrelin And Its Association With Body- Mass Index And Waist Circumference.
Growth Hormone Deficiency And Cognitive Dysfunction Post Mild Traumatic Brain Injury.
Obesity-related Knowledge, Attitudes And Practices Among Adults In Rural Areas Of Tanjung Karang.
Prolonged QTc Interval Is Not Uncommonly Found In Recently Diagnosed Diabetes Mellitus.
Profile of Paediatric Obesity in Hospital Putrajaya.
Prevalence Of Glucose Intolerance In Women With Previous Gestational Diabetes Mellitus In Penang Hospital.
Prevalence Of Cardiac Autonomic Neuropathy In Recently Diagnosed Diabetes Mellitus.
Role of Adrenal Venous Sampling in Primary Aldosteronism – First Year Experience From Putrajaya Hospital.
The Biochemical Parameters Related To Glucose Intolerance In Women With Previous Gestational Diabetes Mellitus In Penang Hospital.
The Prevalence Of Metabolic Syndrome According To NCEP (ATP) III And IDF Criteria Among Rural Malay Population In Tanjung Karang, Selangor.
The Extent Of Nutritional Under-reporting Among Staff Of The Ministry Of Health In Nutritional Survey.
Treatment Outcome And Complications Of Acromegaly Patients (TOM CAT): A Malaysian Multicentre Retrospective Study.
Trimester-specific Reference Range For Thyroid Function Among Pregnant Women Attending UMMC, Malaysia.
CASE REPORTS
A Practical Approach to Diagnosing PCOS in the Adolescentt.
A Case Of Persistent Parathyroid Carcinoma: Dilemma In Management.
A Series Of Five Cases Of Adrenocortical Carcinoma.
Bilateral Adrenal Hyperplasia In Multiple Endocrine Neoplasia Type 2A.
Cardiac Paraganglioma In A Familial Paraganglioma Syndrome - Coronary Artery embolization For Nonresectable Tumor.
Carney Complex: Cardiac Myxoma And Cushing’s Syndrome.
Factitious Hypoglycemia - A Mystery Unravelled.
McCune-Albright Syndrome.
Osteogenesis Imperfecta : A Case Report.
The Case Of A Troubled Patient, Troubling Doctors With Troublesome Problems.
Thoraco-abdominal Surgery With Cardiopulmonary Bypass For Adrenocortical Carcinoma Extending Into The Inferior
Vena Cava And The Right Atrium : A Case Report
TSH- Secreting Pituitary Macroadenoma: A Rare Cause Of Thyrotoxicosis
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
23
24
25
26
27
28
29
30
31
32
33
34

CLINICAL TRIALS AND STUDIES

A Genome Scan for Malay
Type 2 Diabetes Mellitus Patients
Norhashimah AS a , Wan Nazaimoon WM a , Aziz Al-Safi b , Norlaila Mustafa c , Amir S Khir d , Zanariah Hussein e , Wan Mohamad WB b
a Institute For Medical Research, b Hospital Universiti Sains Malaysia,
c Hospital Universiti Kebangsaan Malaysia, d Penang Medical College, eHospital Putrajaya,
Genetics have been implicated to affect susceptibility to diabetic nephropathy. Genomewide
association study was carried out to elucidate and identify possible genetic markers
for diabetes nephropathy in Malay Type 2 Diabetes Mellitus patients. DNA samples from
84 subjects with normoalbuminuria, 51 subjects with microalbuminuria, 34 subjects with
macroalbuminuria and 27 subjects with ESRD were genotyped on the Affymetrix 100K
Single Nucleotide Polymorphism (SNP) array. Using Partek Genomic Suite to analyse the
data, our results suggested that deletions on chromosome 6 starting position 19064 KBps
to 73265 KBps and amplifications on chromosome 8 (5501 KBps to 137.1 MBps) were
associated with different stages of albuminuria; most significance being in ESRD. In addition,
there were significant amplifications and deletions (11719 KBps to 124.8 MBps and
132 KBps to 65694 KBps respectively) on chromosome 12 in with macroalbuminuria and
ESRD. The significance of these findings will be discussed.
1

A Randomized Control Trial To Assess The Efficacy Of Structured
Diabetic Counseling On Glycaemic And Metabolic Parameters
Amongst Patients With Type 2 Diabetes Mellitus.
SZ Lee 1 , SC Lim 1 , S. Ainul 1 , S Nitthya 1 , I. Erida 1 , Faizah 2 , Rohana J 2 , Aminuddin AK 2 , Nor Azmi K 2
1 Department of Physiology, 2 Department of Nursing,
3 Department of Medicine, Faculty of Medicine, National University of Malaysia, Kuala Lumpur.
This was a randomized control trial to assess the efficacy of diabetic counseling offered by trained
diabetic educators during regular diabetic clinic visits versus those who did not received any counseling.
A total of 37 patients (16 males, 21 females, median age: 58 years old) were either randomized
into an intervention group receiving individualised structured diabetic counseling at least twice
during a 3 month period or into a control group. At baseline, the glycaemic and metabolic parameters
of both groups were comparable. At the end of the study, the intervention group had a significant
reduction in HbA1c of 1.16% (p=0.01) when compared to baseline. However there was no
significant difference in the fasting blood glucose, between the intervention group with a reduction of
0.12mmol/L (p=0.85) compared to -0.79mmol/L (p=0.27) in the control group. The intervention group
had a significant weight loss of -1.92kg (p= 0.001) compared to their baseline weight. For percentage
body fat, the intervention group had a reduction of 1.91% (p=0.001) compared to their baseline
levels. For waist circumference, there was no significant difference between both groups. There were
also no difference between both groups in terms of triglyceride and total cholesterol (-0.05mmol/L,
p=0.677 and -0.22mmol/L, p=0.316 respectively for the intervention group compared to
+0.74mmol/L, p=0.237 and -0.02mmol/L, p=0.904 respectively for the control group). In conclusion,
patients who received diabetic counseling by trained diabetic educators were able to reduce their
HbA1c, weight and percentage body fat over a 3 month period.
Key words: Type II diabetes mellitus, diabetic educator, HbA1c, diabetic counseling.
2

A1c As A Screening Tool To Diagnose Diabetes
Mellitus In A Rural Population-based Study
Norlaila M, Nor Azmi K on behalf of the Malaysian Metabolic Syndrome Study (MSSM- UKM)*
Objective: The objective of this analysis was to evaluate the clinical relevance of a single measurement
of HbA1c as a screening tool for diabetes based on data derived from a small population
based study.
Methods: This was a cross sectional study of a rural population of five villages in Tanjung Karang.
The survey was conducted from April to August 2008, involving Malaysians aged 18 and above.
All subjects were randomly selected from households identified prior to the start of the study and
gave their consent. Of the 543 subjects who were recruited, 382 non-diabetic subjects (70.3%)
performed the 75 gram OGTT and 381 (99.7%) data were available for analysis. The sensitivity
and specificity of the various levels of A1c (range from 5 till 6%) were determined against the
results of the OGTT (gold standard) levels. Those with both sensitivity and specificity approaching
80% were deemed to have clinical potential as a cut-off point in the screening process.
Results: Based on the OGTT, 22.8% (n=87) were diagnosed with type 2 diabetes. Subjects with
diabetes had higher A1c levels than subjects without diabetes (6.8 ± 2.4% vs 5.3 ± 0.4%,
p

Abnormal Glucose Tolerance In Tanjung Karang:
The Burden Of Increasing Prevalence And Under-diagnosis
Norlaila M, Nor Azmi K on behalf of the Malaysian Metabolic Syndrome Study (MSSM-UKM)*
Objective: The aim of the study was to examine the prevalence of abnormal glucose tolerance
among rural adult Malay population in Tanjung Karang following a standard 75 gram oral glucose
tolerance test (OGTT).
Methods: This was a cross sectional study of a rural population of five villages in Tanjung Karang.
The survey was conducted from April till August of 2008, among Malaysians aged 18 and above.
The subjects were randomly selected from households which were identified with the aid of the
kirsh tables and the topographical charts of the district. Written consents were obtained prior to
the face to face interviews using structured questionnaires, followed by laboratory tests. Of the
543 subjects who were screened, 435 subjects (80.1%) answered the questionnaires and 382
with no known history of diabetes mellitus (T2DM) (87.8%) completed the OGTT.
Results: The prevalence of impaired fasting glucose (IFG), impaired glucose tolerance (IGT) and
newly diagnosed T2DM following OGTT was 30.1%, 23.3% and 22.8% respectively. Taking into
account those with known T2DM, 32.2% of the population has T2DM. Although OGTT yielded an
additional 20.0% to the T2DM prevalence, still the majority of subjects with diabetes (62.1%)
remained undiagnosed. This together with the fact that more than two-thirds of the population has
an abnormal glucose tolerance (an independent risk factor for cardiovascular diseases) poses an
enormous health burden to the population whose mean household income is far below that of
national average (45.0% of subjects reported a monthly household income of RM500 or less).
Conclusion: The health burden of abnormal glucose tolerance in Tanjung Karang is confounded
by two main issues; that of increasing prevalence as well as under diagnosis of T2DM.
* Norlaila M, Norasyikin AW, Suehazlyn Z, Wong Ming, Shahrul Azmin M, Noor Sharazat H, Wan Rosmaiza W, Shamita S, Norhaliza
A, Nor Shaffinaz YA, Rabizah M, Kamarulzaman H, Nurul Hani M, Fadlin M, Amiliyaton K, Alawiyah O, Azhar I, Nor Azmi K.
4

Adiponectin In The Context Of Metabolic
Syndrome Amongst Staff Of Ministry Of Health
Anilah AR and Nor Azmi K on behalf of the Malaysian Binge Eating Behaviour Study Team (MY-BEST)*
Introduction: The prevalence of metabolic syndrome is increasing parallels to the obesity
epidemic. It is a major concern as metabolic syndrome is an independent risk factor for cardiovascular
disease.
Objective: To determine the prevalence of metabolic syndrome among staff of MOH, Putrajaya
and its association with adiponectin levels.
Methods: This is a cross sectional study involving 738 staffs from Ministry of Health, Putrajaya.
Anthropometric measurements and blood pressures were recorded. Bloods were analyzed for
fasting blood glucose and lipids levels. Only 366 adiponectin levels were available for analysis.
The modified Asian criteria of NCEP (ATP) III and IDF criteria for diagnosing metabolic syndrome
were used in this study.
Results: Out of the 738 subjects, 74% (449) were female and 26% (189) were male while 94%
were Malays. Among the subjects, 8.9% had hypertension and 6% had Diabetes Mellitus (known
case and newly diagnosed).
The prevalence of Metabolic Syndrome was 22.6% (IDF Criteria) and 26.6% (ATP III Criteria)
respectively. Metabolic syndrome were more prevalent among males, (40.87%) compare to
females (24.87%) [using ATP III criteria, p

Binge Eating Behavior And The Preference For Carbohydrate
Among The Staff Of The Malaysian Ministry of Health, Putrajaya.
1 Barakatun Nisak MY, 2 Anilah AR on behalf of the Malaysian Binge Eating Behaviour Study Team (MY-BEST)*
1 Department of Nutrition & Dietetics, Faculty of Medicine & Health Sciences, Universiti Putra Malaysia, Serdang Selangor;
2 Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Aims: This study was conducted to determine the prevalence of binge eating (BE) behavior and
the constituents of food that are associated with the behavior among the staff of the Ministry of
Health (MOH), Putrajaya, Malaysia.
Methods: Although 738 subjects completed the Binge Eating Scale (BES) questionnaire only 401
subjects (Male= 22%) participated in the nutritional survey by completing the Food frequency
questionnaire. For the purpose of statistical analysis, only the subjects who completed both the
questionnaires were analysed and classified into two groups either BE (≥18) or non-BE (

Clinical And Endocrine Profiles Of Patients With Precocious Puberty:
A 4-year Experience In Putrajaya Hospital.
Hemlatha S., Zaihawa H., Nur Arina M.Z., Janet Y.H. Hong, Fuziah M.Z.
Paediatric Department, Hospital Putrajaya, Malaysia.
Objective: To evaluate the clinical and endocrine profile of patients with precocious puberty in
Putrajaya Hospital.
Methodology: Electronic medical records of 15 patients (9 girls, 6 boys) were reviewed.
Results: The mean age of patients was 5.65 ± 1.91 years (boys) and 4.93 ± 2.96 years (girls).
There were 7 girls (77.7%) who presented with early menses. All girls had breast enlargement.
Tall stature was seen in majority of the boys (83.3%) and girls (55.5%). There were 3 boys (50%)
who presented with masculinization and 4 (66%) had erection.
Majority of the girls (88.8%) had Tanner breast stage 2-3. There were 5 boys (83.3%) with
testicular volume > 4 ml (Tanner stage 2-3). All 6 boys (100%) and 7 girls (77.7%) had pubic
hair (Tanner stage 2-3).
Majority of patients (93.3%) had increase LH and FSH level with gonadotrophin releasing
hormone (GnRH) stimulation. Only one patient (6.7%) had elevated alpha-foetoprotein level.
The mean oestradiol level in girls was 115.2 ± 71 pmol/L. The mean testosterone level in boys
was 22.14 ± 24 nmol/L.
Central precocious puberty (CPP) was due to hypothalamic hamartoma (2 boys, 1 girl),
pituitary adenoma (1 boy, 3 girls), cerebral palsy (1 girl) and idiopathic precocious puberty (3
girls). Pseudoprecocious puberty was due to congenital adrenal hyperplasia (2 boys) and
adrenal adenoma (1 boy).
Majority of patients (80.0%) were treated with GnRH agonist. Reduction in testicular volume
was seen in 4 boys (66%) and cessation of menstruation in 7 girls.
Conclusion: Idiopathic precocious puberty was more common in girls whereas pathologic
causes were more common in boys.
7

Gene Expression Profiling Associated With Diabetic
Nephroapthy In Malay Type 2 Diabetes Mellitus
Faradianna EL a , Wan Nazaimoon WM a , Aziz Al-Safi b , Norlaila M c ,
Amir S Khir d , Zanariah H e , Wan Mohamad WB b
a Institute for Medical Research, , b HUSM, c HUKM, d Penang Medical College, e Hospital Putrajaya
The gene expression profiling of 308 samples obtained from Malay Type 2 Diabetes Mellitus patients
were analyzed using Affymetrix Hugene 1.0ST Array system. There were 82 patients with microalbuminuria
(MI), 59 with macroalbuminuria (MA), 41 with End-Stage-Renal-Failure (ESRD) and 126 with
normoalbuminuria (controls). Using Partek Analysis Software, MI was found to be associated with
up-regulation of 46 genes and down-regulation of 11 genes while 3043 genes were up-regulated and
774 genes were down-regulated in MA. ESRD caused up-regulation of 148 genes and downregulation
of 38 genes. Consistent with other studies, we found significant up-regulation of phosphatidylinositol
(PICALM) and major protein histocompatibility complex (HLA), and down-regulation
of bone morphogenic protein kinase (BPM2K) in association with progressive kidney malfunction.
These genes are involved in lipid different biological functions such as metabolic process, antigen
presentation and TGF- B growth factor. Interestingly in ESRD, there was a significant down-regulation
of the TTN gene which encodes the giant skeletal-muscle protein, titin. Further analysis will be
carried out to determine the significance of these findings.
8

Ghrelin And Its Association With Body-
Mass Index And Waist Circumference.
Anilah AR and Nor Azmi K on behalf of the Malaysian Binge Eating Behaviour Study Team (MY-BEST)*
1 Department of Nutrition & Dietetics, Faculty of Medicine & Health Sciences, Universiti Putra Malaysia, Serdang Selangor;
2 Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Introduction: Ghrelin is an appetite hormone whose levels increases with hunger and immediately
drops once food is consumed. Its level is also up-regulated in subjects who are on caloric
restriction either as a result of diet or pharmacotherapy. This phenomenon has been cited as one
of the main reasons for the dismal performance of some of the weight reducing programs.
Despite this, ghrelin levels had not been shown to be associated with obesity and this was generally
hypothesized, unsatisfactory though it may seemed, to the down-regulation of ghrelin in
obese human subjects. Our review of the medical literature revealed that the highest number of
subjects in any prior study involving the measurement of ghrelin was 85.
Objective: The objective of this study was to determine the association between ghrelin and the
indicators of obesity and cardiovascular risks.
Methods: In this study, 738 staff of the Ministry of Health, Malaysia participated in a binge eating
survey that included taking measurements of body mass index (BMI) and waist circumference
(WC). Bloods were also withdrawn from 659 of the subjects for fasting glucose and lipids profiles.
Three hundred and fifty subjects had their adiponectin and ghrelin levels analysed.
Results: Those who were obese (BMI > 27.5) had a higher level of Ghrelin compared to those
who were over -weight (BMI >23,

Growth Hormone Deficiency And Cognitive
Dysfunction Post Mild Traumatic Brain Injury
Foo Siew Hui 1 , Ainul SyahrilFazli Jaafar 2 , Nor Azmi Kamaruddin 1 , Dachayani Sinniah 3
1 Endocrine Unit, Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre.
2 Department of Surgery, Universiti Kebangsaan Malaysia Medical Centre.
3 Department of Psychology, Universiti Kebangsaan Malaysia Medical Centre.
Introduction: Mild traumatic brain injury (TBI) constitutes 70-90% of all TBI. It is defined as external
insult to the brain resulting in altered level of consciousness for less than 30 minutes with a
Glasgow Coma Score of 13-15. 10-25% of mild TBI are associated with longstanding cognitive
dysfunction. Growth hormone deficiency (GHD) is well reported in moderate to severe TBI but not
in mild TBI. The purpose of this study is to determine the prevalence of GHD following mild TBI
and any association between GHD and cognitive dysfunction.
Methods: A total of 51 adult patients with mild TBI were recruited from the Emergency Department
of Universiti Kebangsaan Malaysia Medical Centre. They were assessed three months
following the injury by an insulin tolerance test to determine their GH status. GHD was defined as
a peak GH of

Obesity-related Knowledge, Attitudes And Practices
Among Adults In Rural Areas Of Tanjung Karang.
Barakatun Nisak MY 1 , Norlaila M 2 , Norasyikin AW 2 , Suhazlyn Z 2 ,Wong Ming 2 , Kamaruzaman H 2 , Nurul Hani M 2 ,
Fadlin M 2 , Amiliyaton K 2 ,Rohana J 2 , Shaffinaz A 2 , Hartini M 2 , Rabizah M 2 , Siti Fatehah Y 2 , Noor Shahrazat H 2 ,
Shahrul Azmin MR 2 , Wan Rosmaiza W 2 , Shamita S 2 , Norhaliza A 2 & Nor Azmi K 2 .
1 Department of Nutrition & Dietetics, Universiti Putra Malaysia, Serdang.
2 Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur
Background: It is generally believed that non-obese adults are better equipped in terms of
obesity-related knowledge, attitude and practice (KAP) than their non-obese counterpart.
Objective: This cross-sectional survey was conducted to compare obesity-related knowledge,
attitude and practice among adult population aged 18 years and above in rural areas of Tanjung
Karang, Selangor.
Subjects and Methods: This study was part of a multi-centre Metabolic Syndrome Study of
Malaysia (MSSM) project. Subjects were selected randomly from households in five villages with
the aid of kirsch tables and topographical charts of the district. Among the 543 subjects who were
screened, 416 (77% response rate) participated in the survey by completing the structured questionnaires
followed by anthropometric measurements. The questionnaires assessed the subjects’
knowledge (34 items in true-false format), their attitude (9 items) and practice (19 items); both in
a likert-scale format involving various obesity related topics. Lastly seven questions related to
subjects’ intention to modify their food intake and lifestyles were also administered. For the
purpose of statistical analysis, subjects were divided into obese and non-obese groups based on
the World Health Organization classification.
Results: The prevalence of overweight and obesity in this study cohort was alarmingly high
(60.8%) especially among women (71.1%). Overweight and obese subjects [n= 253; mean
BMI+SD (95% CI) = 29.7 + 4.2 kgm-2 (29.2; 30.2)] were younger and had higher household
monthly income (p< 0.05) compared to the non-obese subjects [n= 163; mean BMI + SD (95%
CI) = 22.5 + 1.5 kgm-2(22.2; 22.7)]. The obese subjects had greater waist circumference (92+10
vs. 80+8 cm) and higher systolic/diastolic blood pressures (140/80 vs. 133/75 mmHg) compared
to the non-obese subjects (p

Prolonged QTc Interval Is Not Uncommonly Found
In Recently Diagnosed Diabetes Mellitus
Ijaz HR, Rohana AG, Hamizah R, Khairani O, Sheikh Anwar A, Nor Azmi K.
Departments of Medicine and Family Medicine, Universiti Kebangsaan Malaysia Medical Centre(UKMMC),
Jalan Yaacob Latiff, Bandar Tun Razak, Cheras 56000, Kuala Lumpur.
Introduction: It is well known that those with prolonged corrected QT interval (QTc) have an
increased preponderance to Torsades de pointes and sudden cardiac death. Prolonged QTc is
also found in long standing diabetic patients and it is associated with increased risk for cardiovascular
morbidity and mortality.
Objective: The purpose of this study is to determine the prevalence of prolonged QTc in recently
diagnosed diabetic subjects whose disease duration is less than 5 years.
Methods: A total of 115 patients with type 2 diabetes of less than 5 years duration were recruited
from an urban primary care clinic and had their QTc interval analysed using the standard 12-lead
ECG by an independent blinded investigator.
Results: Sixteen subjects or 15.2% had prolonged QTc interval and of these, 15 were females
(93.8% vs 6.3%, p= 0.001). Those with prolonged QTc had a higher incidence of autonomic
neuropathy (25.6% vs 9.1%, p= 0.023).
Conclusion: The presence of prolonged QTc in recently diagnosed diabetics is remarkably high
with a prevalence of 15.2%. It is common among female patients and is significantly associated
with autonomic neuropathy.
12

Profile of Paediatric Obesity in Hospital Putrajaya
JJ Ong, YL Lee, Jagdev S, Eugene CL Yeoh, Rodhyah AR, Janet YH Hong, Fuziah MZ.
Paediatric Department, Hospital Putrajaya, Putrajaya, Malaysia.
Objective: To eveluate the severity of obesity during presentation and complications. To assess
the change in weight in response to treatment and follow-up.
Methodology: This is a retrospective review of clinical notes of obese children under follow-up
in Obesity Clinic, Hospital Putrajaya from 2006-2008. Children with less than 2 follow-ups over the
study period were excluded from the study. criteria.
Results: A total of 33 children (15 girls, 18 boys), mean age 8.0 (±3.2) years were available but
8 patients were excluded from the study. Majority of patients had acanthosis nigricans (84.8%)
and few had striae (21.2%). Other features were fungal infections, dorsocervical fat pad and
hirsutism. 6 children had obstructive sleep apnoea with 1 on CPAP and 3 had adenotonsillectomy
done. Other complications were hypertension on treatment (18.2%), fatty liver (6.1%) and hyperuricemia
(12.1%). Two (6.1%) patients had impaired glucose tolerance with 1 having high fasting
insulin and 3 (9.1%) patients had metabolic syndrome. Maternal gestational diabetes was present
in 12.1% and positive first degree family history of obesity in 36.3% of patients. There were 60.6%
of patients who has history of defaulting follow-up. The average follow-up was 1.4 (±0.6)
visits/year. During their follow-ups, 12 (36.4%) children had reduction in BMI by 8.5% (±7.0) in
average.
Conclusion: Majority still fail to achieve reduction in BMI as evidenced by the high rate of defaulters.
Strategies to improve compliance to management of obesity are needed to improve the
outcome.
13

Prevalence Of Glucose Intolerance In Women With
Previous Gestational Diabetes Mellitus In Penang Hospital
Shanty Velaiutham 1 , Chong Hui Khaw 1 Shueh Lin Lim 1 , Malik Mumtaz 2 ,
Nor Azizah Aziz 1 , Jamilah Baharom 3 , Amir S Khir 2
1 Endocrine Unit, Medical Department, Penang Hospital,
2 Penang Medical College, 3 Pathology Department Penang Hospital
ABSTRACT
Gestational diabetes mellitus and diabetes have been linked to be part of the same disease
process that occurs at different points of time. Previous studies have indicated that these
women have a higher risk of developing type 2 diabetes mellitus and this could be related to
insulin resistance.
Method: Ninety-five women with previous history of GDM in the year 1999-2001, were recruited
from the Penang Maternity Hospital. Subjects had to undergo an oral glucose tolerance test
(OGTT) and measurements for fasting glucose together with anthropometrical measurements
and blood pressure readings were taken. Baseline maternal characteristics were recorded, for
example, age, parity, neonatal birth weight, and family history of diabetes mellitus.
Results: 95 (52 Malays, 28 Chinese, 15 Indian) subjects have been studied. Total of 74
subjects (78%) had glucose intolerance, out of which 9.5% had impaired fasting glucose (IFG),
9.5% had impaired glucose tolerance (IGT) and 9.5% had combined IFG and IGT. Diabetes
mellitus were diagnosed in 47(49.5%) of the women. The conversion rate to abnormal glucose
tolerance was 11% to 16% per year, The majority of women with abnormal glucose tolerance
were from age group of between 40-49 years old with median parity of 3 children. The median
neonatal birth weight was 3.2kg in those with abnormal glucose tolerance. Family history of
diabetes was reported by 64% of these women and there was no significant association.
Diastolic blood pressure showed significant association (p=0.007). Women with abnormal
glucose tolerance were found to have BMI greater than 25kg/m2 and larger waist circumference
> 80cm, however they were not statistically significant.
Conclusion: The prevalence of abnormal glucose metabolism in this cohort with previous
GDM was high at 78%, 5 to 7 years from index pregnancy. Women with previous history of GDM
must be seen as having a pre-diabetes state.
14

Prevalence Of Cardiac Autonomic Neuropathy
In Recently Diagnosed Diabetes Mellitus.
Ijaz HR, Rohana AG, Hamizah R, Khairani O, Sheikh Anwar A, Nor Azmi K.
Departments of Medicine and Family Medicine, Universiti Kebangsaan Malaysia Medical Centre(UKMMC),
Jalan Yaacob Latiff, Bandar Tun Razak, Cheras 56000, Kuala Lumpur.
Introduction: Autonomic neuropathy is a common complication of diabetes mellitus. Cardiovascular
autonomic neuropathy (CAN) is a form of diabetic autonomic neuropathy that results
in abnormalities in cardiovascular reflexes and is one of the early manifestation of this complication.
It is also associated with underlying coronary artery disease and erectile dysfunction.
Objective: To determine the prevalence of cardiac autonomic neuropathy (CAN) in diabetic
subjects whose disease duration is less than 5 years and its association with other complications
of diabetes.
Methods: A total of 115 patients who have type 2 diabetes of less than 5 years duration were
recruited from an urban primary care clinic. Each subject was made to undergo five cardiovascular
reflex measurements that form the standard CAN diagnostic test.
Results: The prevalence of CAN was remarkably high at 38.6%. Twenty four subjects had early
CAN, 11 had definite CAN, 8 had atypical combination and 1 had severe form of CAN. CAN is
significantly associated with age, height and the Chinese ethnicity. Male subjects with CAN had
higher HbA1c levels (7.61% ± 1.6 vs 6.76% ± 1.19, p= 0.031) while in the females, CAN is
associated with hypercholestrolaemia (3.04 mmol/l ± 0.98 vs 2.46mmol/l ± 0.66, p= 0.019).
Definite and severe CAN is associated with other microvascular complications such as
neuropathy (41.7% vs 4.2%, p= 0.04) and nephropathy (57.1% vs 7.1%, p= 0.016).
Conclusion: The prevalence of CAN in our recently diagnosed subjects was 38.6%. CAN in
our study is significantly associated with a shorter height, older age and Chinese ethnicity. The
definite and severe form of CAN is associated with neuropathy and nephropathy.
15

Role of Adrenal Venous Sampling in Primary Aldosteronism –
First Year Experience From Putrajaya Hospital
Gunavathy M 1 , Zanariah H 1 , Moorthy S 2
1 Endocrinology Unit, Department of Medicine, Putrajaya Hospital
2 Department of Diagnostic Imaging, Universiti Putra Malaysia, Serdang Hospital
Background: Primary aldosteronism (PA) is the most common form of secondary hypertension,
affecting between 5 – 10% of all patients with hypertension. The patients typically present with
hypertension and hypokalemia. Determination of the etiology of PA remains a diagnostic challenge.
The most common types of PA are bilateral adrenal hyperplasia (BAH), aldosterone
producing adenoma (APA) and primary adrenal hyperplasia. Computed tomography (CT)
remains the primary modality to identify the subtypes of PA however hormonal hyperfunction
cannot be ascertained from the mere presence of an adrenal nodule on CT. Adrenal venous sampling
(AVS) provides the most definitive means to distinguish between unilateral and bilateral
aldosterone hypersecretion.mortality.
Methodology: Between April 2008 and April 2009, ten patients with confirmed PA from our centre
underwent AVS. CT scan in all those revealed lesions in the adrenal gland, 7 had unilateral adrenal
lesions suggestive of adenoma with normal appearance of the contralateral adrenal. The
remaining three had bilateral adrenal abnormalities on CT. All of them were counseled for treatment
modality and all agreed for surgical intervention if deemed necessary.
They were optimized for AVS as per our centre protocol and underwent AVS in Serdang Hospital.
One single interventional radiologist performed all the ten AVS procedures. Simultaneous sampling
of both adrenal veins and a femoral vein were performed while on stimulation with ACTH
(Synacthen) infusion throughout the procedure. Aldosterone and cortisol concentrations were
measured from all three sites and cortisol corrected aldosterone ratios were then determined.
Results: All the ten patients had successful left adrenal vein cannulation. Right adrenal vein was
cannulated in 7 patients. There were no complications related to the procedure. In six patients,
AVS results showed significant lateralization indicative of unilateral disease, suggestive of APA
and one patient had features of BAH although CT reported a unilateral right adrenal nodule.
Among those with unilateral disease, one patient has undergone laparoscopic left adrenalectomy
with histopathological diagnosis of APA, another is planned for surgery in May 2009 and the
others are due for follow-up in clinic and consideration for surgery.
Conclusion: This review revealed that AVS is a technically difficult procedure and the most challenging
issue is the cannulation of the right adrenal vein as it is small and subject to variation,
making it difficult to identify. With this early experience at our centre, the rate of successful bilateral
adrenal vein cannulation of 70% is promising.
16

The Biochemical Parameters Related To Glucose
Intolerance In Women With Previous Gestational
Diabetes Mellitus In Penang Hospital
Shanty Velaiutham 1 , Chong Hui Khaw 1 , Shueh Lin Lim 1 , Malik Mumtaz 2 , Nor Azizah Aziz 1 ,
Jamilah Baharom 3 , Mohamed Ali Abdul Kader 4 , Wan Nazaimoon 5 , Amir S Khir 2
1 Endocrine Unit, Medical Department, Penang Hospital, 2 Penang Medical College, 3 Pathology Department Penang
Hospital, 4 Nuclear Medicine Department, Penang Hospital, 5 Institute of Medical Research, Kuala Lumpur
Objectives: To study the biochemical parameters in women with previous GDM .
Method: 95 women with previous history of GDM in the year 1999-2001, were recruited from the
Penang Maternity Hospital. Subjects had to undergo an oral glucose tolerance test (OGTT) and
measurements for fasting glucose, fasting lipids, fasting insulin, leptin, adiponectin and DEXA
measurement of total body fat and truncal fat.
Results: 95 (52 Malays, 28 Chinese, 15 Indian) subjects have been studied. Insulin resistance
(HOMA IR; p=0.003 and QUICKI; p=0.003) and pancreatic function (HOMA β cell; p=0.003)
showed significant association between women with normal and abnormal glucose tolerance.
Leptin and adiponectin did not show any significant association but there was a trend to indicate
that the women with abnormal glucose tolerance had lower adiponectin levels compared to those
normal tolerance group. Women with abnormal glucose tolerance had higher total fat and truncal
fat percentage (84% and 43% respectively),Despite this, the result did not show any significant
correlation.
Conclusion: The data in this study had indicated that women with previous GDM are at considerable
risk for development of an insulin resistance state and subsequent diabetes mellitus. Awareness
of this subgroup of women is worthy of a long term follow up and need of regular monitoring
to detect postpartum hyperglycaemia.
17

The Prevalence Of Metabolic Syndrome According To
NCEP (ATP) III And IDF Criteria Among Rural Malay
Population In Tanjung Karang, Selangor
Norlaila M, Nor Azmi K on behalf of the Malaysian Metabolic Syndrome Study (MSSM-UKM)*
1 Department of Nutrition & Dietetics, Faculty of Medicine & Health Sciences, Universiti Putra Malaysia, Serdang Selangor;
2 Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Objective: The objective of the study was to determine the prevalence of metabolic syndrome
(MS) among rural adult Malay population according to the revised criteria of the National Cholesterol
Education Programme (Adult Treatment Panel III) (NCEP ATP III) and the International Diabetes
Federation (IDF) and assess various components that contribute to the syndrome. It is part of
a nation-wide study to determine the prevalence of MS in Malaysia.
Methods: This was a cross sectional study of a rural population in Tanjung Karang, Selangor. The
survey was conducted from April till August of 2008, among Malaysians aged 18 and above. The
subjects were randomly selected from households which were identified with the aid of the Kirsh
tables and the topographical charts of the district. All subjects approached gave their written
consent. Face to face interviews were conducted using structured questionnaires, followed by
laboratory tests. Metabolic syndrome was defined using both IDF and revised ATP III criteria.
Results: Of the 543 subjects screened, 435 data were available for analysis. The prevalence of
metabolic syndrome according to IDF and modified ATP III criteria was 36.4% and 39.0% in men
and 50.5% and 45.9% in women, respectively. The concordance for both criteria was 82%. The
prevalence of metabolic syndrome by IDF and ATP III increased with age and body mass index
(BMI). High blood pressure and abdominal adiposity were found most frequently among the
metabolic syndrome subjects irrespective of the definitions used.
Conclusion: Nearly half of the women and more than a third of men in Tanjung karang Selangor
have metabolic syndrome. This alarming rate is of a major health concern as it poses a significant
risk of developing cardiovascular disease and type 2 diabetes mellitus.
* Norlaila M, Norasyikin AW, Suehazlyn Z, Wong Ming, Shahrul Azmin M, Noor Sharazat H, Wan Rosmaiza W, Shamita S, Norhaliza A, Nor Shaffinaz YA,
Rabizah M, Kamarulzaman H, Nurul Hani M, Fadlin M, Amiliyaton K, Alawiyah O, Azhar I, Nor Azmi K.
18

The Extent Of Nutritional Under-reporting In
Nutritional Survey Among Staff Of The Ministry Of Health.
Anilah AR, Barakatun Nisak MY and Nor Azmi K on behalf of the
Malaysian Binge Eating Behaviour Study Team (MY-BEST)*
Objectives: This analysis is part of the ‘Eating Habits, Obesity and Cardiovascular risk factors
among staff of the Ministry of Health’ study. Within this study, the under-reporters of energy intake
were identified and characterised.
Methods: Energy intake (EI) was assessed in 401 subjects (313 female and 88 male subjects) of
the ‘Obesity, Binge Eating among staff of MOH’ study (age range = 20-65 years) by means of food
frequency questionnaires. The basal metabolic rate (BMR) was calculated using age-specific
equations for Malaysian adult as reported by Ismail et al. EI was expressed as a multiple of BMR
and subjects with an EI:RMR ratio below 1.2 were classified as under-reporters.
Results: Among the subjects, 74.6% of obese, 63.6% over weight and 51.6% of lean subjects
were identified as under-reporters. The difference between the rate of underreporting among the
groups were statistically significant (p=0.001). We also found 65.5% of non binge subjects, 62%
moderately binge subjects and 36.8% of severe bingers underreported their energy intake
(p=0.042). There was no difference in age, race, marital status or occupation with regards to the
tendency to underreport their energy intakes.
Conclusion: The results indicate that underreporting of energy intake is common and relates to
the degree of obesity. The obese had the higher tendency to underreport their energy intake compared
to those who were overweight and lean. This is one of the major limitations in any nutritional
survey.
Key words: Underreporting, energy intake
*Anilah AR, Rohana AG, Norasyikin AW, Suehazlyn Z, Wong M, Norlaila M, Norhaliza MA, Noorlita A, Badrulnizam LB, Rizal Am, Kamaruzaman H, Nurul
Hani M, Norlinda D, Fadlin M, Norsyuhadah A, Intan Baizura R, Rohana J, Barakatun Nisak MY, Swee Chin, Nor Azmi K.
19

Treatment Outcome And Complications Of Acromegaly Patients
(TOM CAT): A Malaysian Multicentre Retrospective Study.
M Badrulnizam 1 , Zanariah Hussein 1 , Nor Azmi Kamaruddin 2
1 Hospital Putrajaya, Putrajaya.
2 Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur.
Introduction: Excess growth hormone associated with acromegaly results in increased cardiovascular
morbidity and mortality. It is pertinent that any therapy directed at acromegaly be
reviewed in the light of its clinical outcome.
Objectives: The aim of this study is to assess the outcome of the various treatment modalities of
acromegaly in Malaysia. In addition, the risk of hypopituitarism associated with these treatment
modalities were also reviewed.
Methodology: Out of the 70 patients reviewed only 53 were available for analysis. In terms of
size, 75% of the pituitary tumours were macroadenoma and 25% were microadenoma. The most
common co-morbidities seen were hypertension, diabetes mellitus, toxic multinodular goiter,
cardiovascular diseases and obstructive sleep apnoea. Among the patients with macroadenoma
(n = 38), 79% had surgery as the primary therapy and 21% had primary medical therapy. For
microadenomas (n = 15), 47% had surgery, 40% had medical therapy and 13% had radiotherapy
as their initial primary therapies. Based on the latest post-treatment IGF-1 levels, the overall
success rate for all modalities involved in the treatment of acromegaly was 42% with surgical
success rate being 43% and 67% for macroadenoma and microadenoma respectively. The
success rate with primary medical therapy was only 29% primarily due to the short term use of
octreotide. Hypopituitarism was seen in at least 50% of patients who had received radiotherapy,
double of those who did not receive any radiotherapy at all.
Conclusion: Treatment of acromegaly by various modalities results in a 42% normalization of
IGF-1 levels. The success rate of surgery was 43% and 67% for macroadenoma and microadenoma
respectively, while half of those receiving radiotherapy developed hypopituitarism.
20

Trimester-specific Reference Range For Thyroid
Function Among Pregnant Women Attending UMMC, Malaysia
SC Lim, SP Chan, R Raduan, N Sabir, S Zawiah, YY Chew, M Thevarajah.
University of Malaya Medical Centre, Kuala Lumpur
Introduction: Thyroid disease is common in women of reproductive age. The necessity to detect
and manage thyroidal illness in pregnancy is becoming increasingly important. Overt or even
subclinical hypothyroidism has been shown in many instances to result in adverse maternal and
fetal outcomes. In order to detect thyroid abnormalities during pregnancy, defining trimesterspecific
reference intervals is necessary.
Methods: This is a cross-sectional study carried out in the antenatal clinic and ward of University
of Malaya Medical Centre from July to December 2007. 631 consecutive pregnant women with no
known thyroid disorders were recruited. The ABBOTT AxSym system using microparticle enzyme
immunoassays were utilized for analysis of TPOAb, TSH, fT4 and TT4. Subjects who were TPOAb
positive or with moderate to large goiters were excluded from the analysis. 5th and 95th percentile
values were used to determine the reference ranges.
Results: A total of 537 subjects (1st trimester: 2nd trimester: 3rd trimester N=90:204:243) were
available for analysis.
Trimester-specific reference range for TSH, fT4 and TT4 were as follows:
TSH
FT4
TT4
1 st trimester
0.06 - 3.19
9.35 - 27.82
95.28 - 215.4
2 nd trimester
0.28 - 3.45
6.69 - 12.79
103.0 - 184.6
3 rd trimester
0.56 - 3.56
6.46 - 10.72
100.1 - 175.8
Conclusion: The main limitation of this study was we did not exclude patients with hyperemesis
gravidarum resulting in a possible skewed data for thyroid hormone level in the 1st trimester.
Nevertheless, we have established the trimester-specific reference intervals for TSH, fT4 and TT4
for second and third trimesters among the antenatal pregnant population in UMMC.
21

CASE REPORTS
22

A Practical Approach to
Diagnosing PCOS in the Adolescent
Hanifullah Khan
Women’s Health and Endometriosis Care Centre, Lumut, Malaysia
Polycystic ovary syndrome (PCOS) is the most common reproductive endocrinopathy of
women during their childbearing years and is associated with ovulatory dysfunction
which begins during the perimenarchal period. The importance of this condition lies with
its reproductive and endocrinological sequelae such as infertility and an increased risk of
metabolic syndrome. Early and prompt diagnosis would thus confer a distinct advantage
in its management with an overall improved quality of life on offer. However, the diagnosis
of PCOS in the adolescent is challenged due to a lack of consensus on the defining criteria,
a dearth of statistical and laboratory data and sensitivity issues. This is further compounded
by the symptoms frequently resembling normal occurrences during growing up.
Such a diagnosis can therefore only be achieved reliably through clinical history and
physical findings. The principle features to consider are menstrual irregularity, hirsutism,
persistent acne, obesity or precocious puberty. Consequently, the presence of acanthosis
nigricans and an impaired glucose profile is highly suggestive of an adolescent with
PCOS. Conditions that mimic PCOS should be excluded, while at the same time remembering
that these patients can appear totally normal. It is hoped that a review of these
features can aid the clinician in rapidly and reliably identifying the adolescent patient with
PCOS.
23

A Case Of Persistent Parathyroid Carcinoma:
Dilemma In Management
Foo Siew Hui 1 , Nor Azmi Kamaruddin 1 , Yong Sy Liang 1 , Norasyikin A. Wahab @ A. Rahman 1 , Rohaizak Muhammad 2 .
1 Endocrine Unit, Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre.
2 Breast & Endocrine Unit, Department of Surgery, Universiti Kebangsaan Malaysia Medical Centre.
Abstract
Parathyroid carcinoma (PTC) is a rare endocrine malignancy constituting 1-5% of primary hyperparathyroidism.
As it is mostly functional, metabolic complications of hypercalcemia due to
excess parathyroid hormone (PTH) secretion are the main cause of morbidity and mortality.
Our patient is a 49 year-old male presented with a cervical mass associated with primary hyperparathyroidism.
He had a history of renal calculi 4 years prior to presentation associated with
increased mood swings. The diagnosis of PTC was made pre-operatively based on the presence
of a palpable neck mass, the markedly elevated serum calcium (3.77mM)/PTH (132pM) and the
macroscopic evidence of local invasion intraoperatively. An en bloc resection of the tumor was
attempted. Histologically, the diagnosis was confirmed with the presence of capsular perforation.
Postoperatively, the patient’s serum calcium dropped to 2.73mM while the PTH level was not
suppressed. It was believed to be due to residual disease.
This case illustrates that the progression of symptoms of hypercalcemia secondary to PTC may
be insidious despite a markedly elevated serum calcium and PTH. En bloc resection is the gold
standard treatment for PTC but may not be possible in all cases. For persistent disease, the
choice of reoperation versus medical therapy should be individualized. Both modalities of treatment
have been shown to be effective in providing clinically and biochemical palliation of hypercalcemia
with no survival advantage of one over the other. Medical therapy was planned for our
patient whenever he is symptomatic from the hypercalcemia. Every attempt should be made to
remove any residual tumor surgically.
24

A Series Of Five Cases Of Adrenocortical Carcinoma
Norasyikin AW, Nor Shafinas NA, Norhaliza MA, Wong Ming,
Foo, Suezhazlyn Z, Norlaila M, Rohaizak M, Nor Azmi K
Departments of Medicine and Surgery, UKM Medical Center, Kuala Lumpur.
Abstract
Adrenocortical carcinoma (ACC) is an extremely rare tumor. Although it is potentially curable in
the early stages, only a third is confined to the adrenal gland at the time of diagnosis. Five cases
of ACC with various presentations, managed in our institution within the last 2 years are discussed
in the light of associated issues in their management.
Of the three ACC cases that presented with
Cushing’s syndrome, two were complicated by
uncontrolled hypertension and psychosis as a result
of excessive corticosteroid levels. Three of the
patients had large tumours, two of whom had left
adrenal tumor measuring greater than 6 cm in diameter
(fig 1) whilst the third had a right adrenal tumour
with the same dimension (fig 2).
Figure 1 Figure 2
Of the three functioning tumours, surgical intervention was not performed in two as they had
succumbed to overwhelming sepsis despite being on adrenalytic therapy. The remaining patient
underwent a successful debulking surgery followed by mitotane therapy.
The 4th case was a middle-aged Chinese man who presented with a back pain of two year duration.
The abdominal CT scan revealed an inhomogeneous right adrenal mass measuring 3x4x6
cm. He was subjected to a successful adrenalectomy. Unfortunately, he declined any further
treatment after the surgery.
The last case was that of a young Malay school girl who presented with
pulmonary embolism. Abdominal and thoracic CT scans revealed a huge
ACC measuring 10x12x13cm with inferior vena cava and right atrial
extensions (fig 3). She underwent an uneventful radical thoracoabdominal
surgery with the aid of a cardio-pulmonary bypass procedure.
Mitotane therapy was started before the operation and the dose was
titrated as tolerated (This case is described separately in detail elsewhere
in this proceeding).
Figure 3
Conclusion: Surgery seems to offer the only satisfactory mode of treatment in ACC regardless of
the stages while adrenocorticolytic therapy should be commenced as soon as possible. Efforts to
control excessive corticosteroid levels in functioning cases should be an immediate primary
concern as overwhelming sepis seems to carry the day.
25

Bilateral Adrenal Hyperplasia In
Multiple Endocrine Neoplasia Type 2A
Gunasekaran R, Zanariah H
Endocrinology Unit, Department of Medicine, Hospital Putrajaya
Multiple Endocrine Neoplasia (MEN) 2A is an autosomal dominant disease, characterized by the
development of Medullary Thyroid Carcinoma, Pheochromocytoma and hyperparathyroidism.
Genetic studies have identified the presence of germline mutations in the RET proto-oncogene in
almost 100 percent of MEN 2 patients.
In MEN 2A, pheochromocytoma occurs in 40 to 50 percent of patients, usually arising in the adrenal
glands and rarely occurs at extra-adrenal sites. In contrast to sporadic pheochromocytoma,
the adrenal involvement within MEN 2A begins with adrenal medullary hyperplasia and is multicentric
and bilateral in more than 50 percent of cases. Pheochromocytomas are almost always
benign, but some tend to recur locally. These tumors usually produce adrenaline disproportionately
to noradrenaline, in contrast to sporadic cases. Hypertension in MEN 2A patients with pheochromocytoma
is more often paroxysmal than sustained, in contrast to the usual sporadic case.
MEN 2A patients with pheochromocytomas may have paroxysmal palpitations, anxiety, headaches,
or sweating but many remain asymptomatic.
We describe a 52-year-old man who was referred to us for further evaluation and follow-up of his
elevated urinary catecholamines and adrenal abnormalities on the CT scan.
He was initially diagnosed to have medullary thyroid carcinoma with regional lymph node metastases.
He underwent total thyroidectomy and neck dissection 9 years back and is currently on
thyroxine replacement with persistent mild elevation of serum calcitonin levels in the absence of
clinically discernible disease. Serum calcium levels have remained within normal range. Genetic
testing had detected a mutation on codon 634. Regular screening of urinary catecholamines
revealed progressive elevation above normal and most recently, urinary adrenaline levels were
elevated five-fold without significant elevation of noradrenaline. Serial CT scan of the adrenals
showed gradual progressive nodularity of both adrenals in keeping with probable adrenal medullary
hyperplasia. An additional extra-adrenal lesion was identified adjacent to the left adrenal
gland. Further investigation with I-131-metaiodobenzylguanidine (MIBG) scintigraphy is planned
prior to considering the timing and extent of surgical approach for the adrenal disease.
26

Cardiac Paraganglioma In A Familial Paraganglioma Syndrome -
Coronary Artery embolization For Nonresectable Tumor.
Zanariah H 1 , Alfazir O 2 , Rosli MA 2
1 Endocrinology Unit, Department of Medicine, Hospital Putrajaya
2 Department of Cardiology, National Heart Institute (IJN)
Primary cardiac paraganglioma is a very rare tumor with less than 60 reported cases in literature to
date. Clinical presentation is variable,most commonly manifested by hypertension and symptoms
related to catecholamine excess such as palpitations, headache and sweating. Most are benign and
most commonly arise from the left atrial wall. The tumor is often poorly encapsulated and local
invasion can occur with metastases reported in up to 10% of cases. Surgical excision is the treatment
of choice, often complex and necessitating cardiopulmonary bypass. It is often difficult to detect
preoperatively the degree of extension of the tumor and its resectability. Patients with non-resectable,
intracardiac tumors have been considered for cardiac transplantation. Embolization of feeding coronary
arteries to the tumor has been performed, mainly as a preoperative procedure to reduce perioperative
bleeding.
We describe a case of non-resectable functioning cardiac paraganglioma who underwent embolization
of coronary arteries with subsequent improvement in clinical status, normalization of urinary
catecholamines and reduction in tumor size.
A 42-year-old Chinese male presented with recent onset hypertension and diabetes with an abnormal
chest radiograph. He was otherwise asymptomatic. He was on metformin monotherapy with a HbA1c
of 7.6% and had severe hypertension of 180/140 mmhg with a postural drop to 150/120 mmHg at
presentation to our center. He had previous surgical excision of bilateral carotid paragangliomas. His
sister was recently diagnosed to have abdominal paraganglioma with liver metastases.
Initial CT scan noted a mediastinal mass adjacent to the main pulmonary artery measuring 6.3 x 3.9
cm and normal appearance of both adrenal glands. Coronary angiogram revealed a highly vascular
cardiac mass adjacent to pulmonary artery with feeding vessels from the Right coronary (RCA) and
Left anterior descending (LAD) arteries. Twenty-four hour urinary noradrenaline levels were elevated
at 19236nmol (normal 40 – 780 nmol) with normal urinary adrenaline levels. MIBG scan revealed a
faint tracer uptake in the heart and otherwise physiological uptake elsewhere.
Cardiothoracic consultation concluded that the tumor was inoperable. The patient was referred to the
cardiologist for consideration of tumor embolization. He was prepared with oral phenoxybenzamine
and later addition of propranolol. Coronary angiogram was performed with alcohol ablation of the
tumor and placement of a helical coil in the main feeder artery to the tumor. On follow-up, blood pressure
gradually normalized and antihypertensives were withdrawn. Urinary catecholamines had
normalized at three months follow-up with CT scan showing tumor size reduction.
Follow-up to detect regrowth of cardiac tumor and development of additional paragangliomas at
other sites is important. He most likely has a succinate dehydrogenase (SDH) gene mutation giving
rise to familial and multifocal disease. Genetic studies are necessary for him and his family to enable
early diagnosis and disease prognostication.
27

Carney Complex: Cardiac Myxoma And Cushing’s Syndrome
Norhaliza MA, Zanariah H, Masni M
Endocrinology Unit, Department of Medicine, Hospital Putrajaya
Carney complex is a rare multiple neoplasia syndrome, comprising the association of cardiac and
cutaneous myxomas, pigmented skin lesions, endocrine tumors, and melanocytic schwannomas,
first reported by Carney in 1985. Endocrine tumors observed include growth hormone secreting
pituitary adenomas, thyroid adenomas or carcinomas, testicular tumors and ovarian cysts. Adrenocorticotropic
hormone (ACTH)-independent Cushing's syndrome due to primary pigmented nodular
adrenal disease (PPNAD) is observed in 25 to 30 % of patients. It is transmitted as an autosomal
dominant trait. We report two cases, both presenting with Cushing’s syndrome due to bilateral nodular
adrenal hyperplasia and cardiac myxoma.
The first case is a 39-year-old lady, recently diagnosed with ACTH-independent Cushing’s syndrome,
presented with uncontrolled hypertension and excessive weight gain. She had previous surgical
resection of cardiac myxoma at age 17 years and later underwent cystectomy for recurrent left ovarian
cyst. Her elder sister has acromegaly due to a pituitary adenoma. Computer tomography (CT)
showed a nodular right adrenal gland and normal appearance of the contralateral gland. With a probable
diagnosis of Carney complex and likelihood of bilateral adrenocortical hyperplasia, she underwent
laparoscopic bilateral adrenalectomy, which confirmed the diagnosis of primary pigmented
nodular adrenal disease (PPNAD) in both adrenals.
The second case is a 23 year-old-lady with initial presentation of Cushing’s syndrome, nondetectable
ACTH levels and CT scan evidence of bilateral nodular adrenal enlargement. She underwent a
bilateral adrenalectomy which confirmed bilateral macronodular adrenal hyperplasia in keeping with
PPNAD. She also had spotty facial hyperpigmentation. Six years later, she presented with acute
breathlessness and palpitations. Echocardiography showed a left atrial tumor, confirmed to be an
atrial myxoma following resection. She remained well until recent review revealed recurrence of
cardiac myxoma , 3 years after the initial removal. She had no family history of any of the components
of Carney complex.
Cardiac myxoma and PPNAD are characteristic components of the Carney complex. Cardiac tumors
and are often multi-centric, having a high risk for recurrence. Awareness of the Carney complex and
of the importance of its various components will facilitate identification of affected patients and
screening of their first-degree relatives.
28

Factitious Hypoglycemia - A Mystery Unravelled
K Nair, Tan ATB, S Sharma, NL Adam, SS Lim, M Badrulnizam, V Ananda, Rokiah P, SP Chan
Endocrine Unit, Department of Medicine, University Malaya Medical Centre
We describe a case of a 41 year old Chinese lady who presented to our institution with recurrent
hypoglycemic episodes since 1995. Her first presentation to the Endocrine team in UMMC was in
October 2005 when she was admitted for anaemia and was found to be profoundly hypoglycemic
requiring prolonged dextrose infusion for correction. She had been managed at various other
hospitals for this problem over the previous 10 years. Her past medical and surgical history was
significant, including 7 previous abdominal surgeries with no identifiable pathology, 3 prior ICU
admissions for hypoglycemia related events and recurrent symptomatic anaemia.
Laboratory investigations consistently revealed high insulin levels with corresponding low levels
of C-peptide. Insulin antibody levels were undetectable and other hormonal tests, in particular
ACTH, Synacthen and GH assay were normal. The patient repeatedly denied surreptitious use of
insulin despite all investigations pointing towards a factitious cause. Subsequent imaging and
EUS did not reveal the presence of an insulinoma. The diagnosis of factitious hypoglycemia was
confirmed more than 10 years after she initially presented when she was caught ‘red-handed’ with
an insulin syringe and Mixtard vial on her person during a recent admission to our institution in
March 2009. She was referred to the Psychiatric department but she discharged herself from the
hospital on the same day.
Factitious hypoglycemia carries a poor long term prognosis with a high fatality rate. This case
illustrates how the misuse of medication can pose a diagnostic challenge to medical professionals
and underscores the importance of early recognition of a factitious disorder as it might obviate
the need for lengthy and costly search for a non existent illness.
29

McCune-Albright Syndrome
Dr. Suhaimi Hussain
Introduction / Aims: McCune-Albright syndrome is diagnosed when at least two features of the triad
of polyostotic fibrous dysplasia, café au lait skin pigmentation and autonomous endocrine hyperfunction
are present.
It is most commonly diagnosed in young children. We report a 7 months old girl with McCune-
Albright syndrome
Results: A 7- months’ old girl presented with pervaginal bleeds, bilateral breast budding and
multiple popular rashes over the face. She is an ex-premature 25th week gestational age and was
born with birth weight of 700gram. She had a stormy neonatal period in which she was warded for 3
months.
She had fulfilled two out of three clinical triads for the diagnosis of McCune-Albright syndrome.
1. Peripheral precocious puberty
LH < 0.1, FSH 4.8 m IU/ ml
Oestradiol 1020 pmol/l and repeat test after one month 280 pmol/l
2. Typical café au lait pigmentation with irregular borders (Coast of Maine)
The source of high oestradiol is from the follicular cysts as confirmed by ultrasound study.
Treatment : Tamoxifen (aromatase inhibitor) 10 mg daily.
For the purpose of confirmation, tissue biopsy to look for mutation of Gs protein alpha subunits from
of follicular cysts fluid is needed.
30

Osteogenesis Imperfecta : A Case Report
Janet YH Hong , Jagdev S, Eugene CL Yeoh, Leong JJ, Fuziah MZ.
Paediatric Department Hospital Putrajaya, Malaysia.
Introduction: Osteogenesis imperfecta (OI) is a rare genetic disorder usually caused by a
dominant mutation to type 1 collagen genes. The clinical expression is usually polymorphic and
is characterised by increased osseous fragility, low bone mass, recurrent fractures leading to
severe immobilising deformities in patients. Other features include bluish sclera, dwarfism,
ligament laxity, dentinogenesis imperfecta, deafness and cardiopulmonary diseases. We report
a neonate with OI type III presenting with severe bony manifestations at birth.
Case Report: A term male infant delivered via spontaneous vertex delivery was referred for
bilateral lower limb deformities. His mother also had history of bilateral lower limb fractures at
birth. A routine antenatal scan showed shortening and bowing of long bones. Clinical examination
revealed bilateral lower limb deformity associated with bowing. There was a swelling over
the distal part of the left arm. Radiograph showed multiple bone fractures involving the ribs, left
humerus, bilateral femurs, tibia and fibula in different stages of healing. There was no blue
sclera and systemic examination was unremarkable. Serum calcium, phosphate and alkaline
phosphatase levels were normal. Ultrasound of the brain was normal. The infant was
discharged on 5th day of life with immobilisation of the left arm. A repeat radiograph at 18th day
of life showed callous formation and healing of left humerus with no new fracture.
Conclusion: OI requires utmost care and attention from a multidisciplinary team. Intranatal
detection of this condition may enable reduction of unnecessary iatrogenic trauma during delivery.
This patient will likely to benefit from bisphosphonate therapy.
31

The Case Of A Troubled Patient, Troubling Doctors
With Troublesome Problems
NL Adam, SS Lim, K Nair, V Ananda, ATB Tan, M Badrulnizam, S Sharma, Rokiah P, SP Chan.
Abstract
Vasoactive intestinal peptide producing tumour (VIPoma) or Verner-Morrison Syndrome is a very
rare neuroendocrine tumour. It occurs in less than 10% of pancreatic islet cell tumours. About 70
to 80% originate from the pancreas. At presentation, 60% of the cases have usually metastasized
either to lymph nodes, liver, bone or lung. The first line treatment is surgical. It may be curative in
40% of patients with benign and non-metastatic disease. Palliative surgery of the primary tumour
is indicated when the tumour is not completely respectable, followed by conventional somatostatin
analogue, (octreotide), therapy. Somatostatin analogues have a role in improving hormone
mediated symptoms, reducing tumour bulk and preventing local and systemic effects.
Here, we would like to present a case of VIPoma which has metastasized to the liver at diagnosis
that poses a management dilemma. The patient presented with a history of chronic diarrhoea.
She had been previously seen by many surgeons and thorough investigations had been carried
out. The symptoms portrayed and investigations done were very suggestive of VIPoma. She
underwent a palliative Whipple’s procedure and subsequently, had cytoreductive radiofrequency
ablation (RFA) to her liver metastases. However, the liver metastases did not respond to the ablation
therapy. On the contrary, new liver lesions were noted.
She continues to have persistent severe secretory diarrhea. Other alternative therapies such as
combination chemotherapy using streptozosin and 5- Flurouracil have limited roles in the management
of VIPoma but may have to be considered. She has refused more chemotherapy.
Somatostatin analogue, octreotide has been used to control hormone mediated symptoms, as
anti-proliferative effect as well as to control tumour growth. Patients with VIPoma had symptomatic
relief in about 80% and up to 70% has been reported to have reduction in VIP level. Octreotide
200-450 mcg/day associated with 60% symptomatic, 70% biochemical and 5-10% tumour
response. Stabilization of tumour growth documented by CT occurs in 30-50% pts. However,
tachyphylaxis may develop after prolong treatment and require escalating dose of octreotide.
Newer somatostatin analogue with more affinity to somatostatin receptors has been used with
encouraging result, such as SOM-230 or Passeriotide. Octreotide was also administered to alleviate
her symptoms. The symptoms have reduced in frequency but not completely aborted.
Currently she is on octreotide daily, achieving partial control of her symptoms.
32

Thoraco-abdominal Surgery With Cardiopulmonary Bypass
For Adrenocortical Carcinoma Extending Into The Inferior
Vena Cava And The Right Atrium : A Case Report
Nor Shaffinaz YA 1 , Norasyikin AW 1 , Anilah AR 1 , Foo SH 1 , Suehazlyn Z 1 , Wong Ming 2 ,
Norlaila M 1 , Normayah K 2 , Anita B 2 , Rohana A 2 , Roshila H 3 , Azhari Y 3 , Hisham AN 2 , Nor Azmi K 1 .
1 Department of Medicine, National University of Malaysia Medical Centre, Kuala Lumpur.
2 Department of Breast & Endocrine Surgery, Hospital Putrajaya, Putrajaya.
3 Department of Cardiothoracic Surgery, National Heart Institute, Kuala Lumpur.
We report a case of a 17 year old girl with a non-functioning left adrenocortical carcinoma (ACC)
with tumour extension along the inferior vena cava (IVC) and into the right atrium(RA). Her initial
presentation was that of progressive shortness of breath and abdominal pain of a week’s duration.
Thoraco-abdominal CT confirmed the diagnosis of pulmonary embolism together with the
findings of a large heterogeneously enhancing left adrenal mass measuring 10 x 12 x 15 cm with
extension into both renal veins and the inferior vena cava. She was promptly anticoagulated with
subcutaneous clexane. Histological diagnosis of ACC was obtained via an ultrasound-guided
biopsy of the primary tumour. The patient was commenced on mitotane, an adrenalytic agent.
Despite the pulmonary embolism, PET scan did not reveal any metastases to the lungs. However
attempts to titrate the mitotane to the recommended dose were thwarted by persistently raised
liver enzymes.
A repeat thoraco-abdominal CT, a month later, however revealed a further extension of the tumour
along the IVC into the RA of the heart. A decision was made to subject the patient for extensive
surgery in an attempt to debulk the tumour. She underwent a thoracotomy under cardiopulmonary
bypass procedure to remove the tumour from the RA with a simultaneous laparotomy to remove
the primary tumour and its IVC extension. The surgery was uneventful and three days later she
was recommenced on mitotane in multiple divided doses. A repeat PET scan a month after
surgery revealed minimal residual tumour in the adrenal bed and in a small segment of the IVC.
This case illustrates the importance of extensive thoraco-abdominal surgery in the overall management
of Stage 4 ACC with extension into the IVC and RA. In a big review of Stage 4 ACC with
IVC extension totaling 106 cases (28 had RA involvement), 35 of whom had thoraco-abdominal
surgery, survival beyond 2 years were reported in more than 40% of those who had thoracoabdominal
surgery.
33

TSH- Secreting Pituitary Macroadenoma:
A Rare Cause Of Thyrotoxicosis
Norhaliza MA, Zanariah H, Nurain MN
Endocrinology Unit, Department of Medicine, Hospital Putrajaya
TSH-secreting pituitary adenoma (TSH-oma) is a rare cause of thyrotoxicosis and accounts for
only 1-2% of all pituitary adenomas. The diagnosis is usually made much later in a patient who
has been diagnosed earlier with thyrotoxicosis. We report a case of a patient who was diagnosed
to have thyrotoxicosis during follow up of her pituitary adenoma, which was initially thought to be
a non-functioning pituitary adenoma.
A 25-year-old lady was diagnosed to have pituitary apoplexy when she presented with acute left
eye blindness and headache. MRI of the pituitary revealed the presence of a large pituitary mass
measuring 3.5 x 3.0 x 2.8 cm with suprasellar extension, compression of the optic chiasm, left
cavernous sinus and intracavernous portion of the left internal carotid artery (ICA). She underwent
transsphenoidal surgery and histological examination showed presence of infarcted
pituitary adenoma. The pituitary tissue was stained positive for prolactin and growth hormone,
while staining for ACTH, FSH, LH and TSH were negative. Serum FT4 was 13.2 pmol/l (9-19
pmol/l) and TSH was 0.38 mU/L (0.35-4.94 mU/L), while other anterior pituitary hormones were not
available.
Her vision improved post-operatively and a MRI performed three months later showed evidence
of a residual pituitary tumor measuring 2.0 x 1.8 x 1.8 cm with encasement of the cavernous
portion of the left ICA. Her thyroid function was normal; serum FT4 level was 18.1 pmol/l (9-19
pmol/l) and TSH level was 1.94 mU/L (0.35-4.94 mU/L). Other anterior pituitary hormones were
also within normal limits. However six months later, thyroid function tests noted elevated serum
FT3 and FT4 levels with non-suppressed TSH.
In view of the underlying pituitary adenoma, diagnosis of thyrotoxicosis secondary to TSH-oma
was made. This was supported by an elevated _-subunit of 3.09 IU/L (0.05-0.4 IU/L). However
TRH- stimulation test was positive, which is not classical of TSH-oma. She was planned for a trial
of medical therapy with a somatostatin analogue.
34