1.Single-stage adrenalectomy and hysterectomy for pheochromocytoma with giant uterine fibroid: A multidisciplinary perioperative challenge
Fei Bing Yong ; Sarojini Devi Simanchalam ; Hidayatil Alimi Keya Nordin ; Nithiya Devi Kandasami ; Sadhana Sadar Mahamad ; Suhaimi Jaafar ; Mohd Wajdi Zanuddin ; Poh Shean Wong ; Chin Voon Tong ; Noor Lita Adam ; Zanariah Hussein
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):20-
Introduction:
Pheochromocytoma is a catecholamine-secreting adrenal
tumor associated with major perioperative hemodynamic
instability. When concurrent major pelvic pathology
requires surgery, operative planning becomes particularly
challenging. We describe the successful single-stage
management of pheochromocytoma and a giant uterine
fibroid, highlighting the importance of multidisciplinary
coordination and perioperative optimization.
Case:
A 49-year-old female with symptomatic uterine fibroid
was found to have proliferative endometrium on a pipelle
biopsy. Computed tomography (CT) abdomen incidentally
detected a right adrenal mass alongside a large posterior
uterine fibroid (11.9 × 17.4 × 14.6 cm). CT adrenal protocol
demonstrated a heterogeneously enhancing right adrenal
mass (6.9 × 6.9 × 9.6 cm) with high unenhanced attenuation.
Biochemical evaluation revealed markedly elevated
24-hour urinary metanephrine (4.7× upper limit) and
normetanephrine (2.4× upper limit).
Following multidisciplinary discussions, a single-stage
surgical approach was planned after careful assessment of
feasibility and perioperative risk in view of the uncertain malignant potential of the pelvic mass and to minimize
repeated exposure to anesthesia. Preoperative optimization
included transitioning from terazosin to phenoxybenzamine, with subsequent addition of bisoprolol for
hemodynamic control. The operative strategy prioritized
pheochromocytoma resection first, given its potential
for significant hemodynamic instability. Progression to
hysterectomy was contingent upon achieving adequate
intraoperative hemodynamic stability following adrenalectomy, with continuous reassessment by the anesthetic
and surgical teams.
Right adrenalectomy was performed first, followed by
total abdominal hysterectomy with bilateral salpingooophorectomy. Significant hemodynamic lability occurred
during tumor manipulation, with hypertensive surges
managed using sodium nitroprusside and remifentanil
infusions. Following adrenal vein ligation and tumor
removal, hypotension was managed with noradrenaline
and additional adrenaline support as required. Total
operative time was approximately 6 hours. Postoperatively,
transient noradrenaline support was required but was
rapidly weaned as hemodynamic stability was achieved.
Conclusion
Single-stage adrenalectomy and major pelvic surgery
can be safely performed in selected patients with pheochromocytoma when guided by meticulous preoperative
optimization, clear intraoperative sequencing, and close
multidisciplinary coordination.
Pheochromocytoma
;
Adrenalectomy
;
Leiomyoma
;
Hysterectomy
2.Recurrent Diabetic Ketoacidosis: Predictors and Clinical Outcomes in a 24-Year Retrospective Cohort
Liang Wei Wong ; Lisa Mohamed Nor ; Raja Nurazni binti Raja Azwan ; Adilah Zulaikha binti Abd Latib ; Hidayatil Alimi bin Keya Nordin ; Qin Zhi Lee ; Kean Heng Lim ; Jia Ling Low ; Mohd Fyzal bin Bahrudin ; Syaza binti Izhar Hisham ; Jia Whey Jacelyn Ong ; Chin Voon Tong
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):33-34
Introduction:
Diabetic ketoacidosis (DKA) is a life-threatening complication associated with significant morbidity and healthcare
burden. Despite advances in diabetes care, recurrent
DKA remains common, often reflecting gaps in treatment
adherence and patient education. Identifying predictors
of recurrence is crucial for risk stratification and targeted
intervention.
Methodology:
We conducted a retrospective observational study of all
adult DKA admissions to a tertiary centre between 2001
and 2025. Electronic medical records were reviewed for
demographic data, biochemical parameters, precipitating
factors, and clinical outcomes. DKA was defined using standard biochemical criteria. Recurrent DKA was defined as ≥2 admissions during the study period. Factors associated
with recurrent DKA admissions were analyzed. Patients
under the age of 18 years and those with missing vital
information were excluded.
Results:
A total of 667 DKA admissions, comprising 566 patients,
were identified, of which 101 admissions (15.1%) were
recurrent, involving 65 patients. Among recurrent DKA
episodes, the most common precipitating factors were
infection (64.4%) and insulin omission (62.4%). After
multivariate analyses, patients with type 1 diabetes
mellitus (T1DM) were more likely to develop recurrent
DKA compared to those with type 2 diabetes mellitus
(aOR 4.16; 95% confidence interval [CI] 2.58–6.70; p <0.001).
Insulin omission was strongly associated with recurrent
DKA (aOR 2.29; 95% CI 1.46–3.60; p <0.001). In contrast,
baseline glycated hemoglobin and chronic kidney disease
were not significantly associated with recurrence. Diabetic
counseling during the first DKA admission did not reduce
recurrent DKA. There were no significant differences in
mortality (3.9% vs 6.2%, p = 0.524) or critical care admission
rates (40.6% vs 38.7%, p = 0.718) between recurrent and first
DKA episodes.
Conclusion
Recurrent DKA accounts for a substantial proportion of
DKA admissions and is strongly associated with insulin
omission and T1DM. Our findings suggest that recurrent
DKA is driven predominantly by behavioral and adherencerelated factors, indicating the need for multidisciplinary
interventions beyond standard inpatient counseling.
Diabetic Ketoacidosis
;
Retrospective Studies
3.Clinical and Biochemical Characteristics of Adult Diabetic Ketoacidosis: T1DM vs. T2DM
Mohd Fyzal Bahrudin ; Chin Voon Tong ; Raja Nurazni Raja Azwan ; Adilah Zulaikha Abd Latib ; idayatil Alimi Keya Nordin ; Qin Zhi Lee ; Kean Heng Lim ; Jia Ling Low ; Syaza Izhar Hisham ; Liang Wei Wong ; Jia Whey Jacelyn Ong ; Lisa Mohamed Nor ; Zanariah Hussein
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):39-40
Introduction:
The rising incidence of diabetic ketoacidosis (DKA) in type 2
diabetes mellitus (T2DM) represents a paradigm shift from
its traditional recognition as a hallmark complication of
type 1 diabetes mellitus (T1DM). However, contemporary
data comparing clinical presentation, precipitating factors,
and outcomes between these two populations remain
limited.
Methodology:
In this retrospective observational study, all adult DKA
admissions with T1DM or T2DM at a tertiary centre between
2001 and 2025 were studied. DKA was defined according
to standard biochemical criteria. Electronic medical records were reviewed to extract demographic data, biochemical
parameters, precipitating factors, management details, and
clinical outcomes of all adult DKA admission in T1DM and
T2DM. Patients aged <18 years or those with incomplete
data were excluded.
Results:
A total of 601 DKA episodes were analyzed, comprising
130 (21.6%) in patients with T1DM and 471 (78.4%) in those
with T2DM. Mean age was 26.9 ± 0.71 years for T1DM and
52.1 ± 0.7 years for T2DM. Gender distribution was balanced
(male 48.3%, female 51.7%). Mean hemoglobin A1c (HbA1c)
was 11.3 ± 0.3% in T1DM and 12.1 ± 0.2% in T2DM. Infection
was the most common precipitating factor overall (69.0%),
occurring more frequently in T2DM than in T1DM (74.7%
vs. 56.2%), followed by medication non-adherence (62.4%
vs. 43.8%). Significant differences were observed between
groups in admission pH, bicarbonate (both p <0.001), blood
ketones (p = 0.028), and HbA1c (p = 0.010), whereas anion
gap (p = 0.056) and blood glucose levels (p = 0.755) did not
differ significantly. Clinical outcomes were comparable
with respect to intensive care unit (ICU) admission rates
(40.0% in T1DM vs. 39.0% in T2DM, p = 0.779) and median
resolution time (p = 0.462). However, the median length of
hospital stay was significantly longer in T2DM (8.2 ± 0.32
vs. 5.4 ± 0.4 days; p <0.001). Overall mortality was 6.0%,
with substantially higher mortality in T2DM compared to
T1DM (7.4% vs. 0.8%, p = 0.013).
Conclusion
In this large regional series of adult DKA, most episodes
occurred in T2DM. Despite similar ICU admission rates
and time to resolution, T2DM was associated with more
frequent infection-related precipitants, longer hospital
stays, and higher mortality, underscoring the need for
targeted preventive strategies in this population.
Adult
;
Diabetes Mellitus, Type 1
;
Diabetic Ketoacidosis
;
Diabetes Mellitus, Type 2
4.Diabetic Ketoacidosis in Pregnancy: Clinical Triggers, Outcomes, and Missed Opportunities—A Case Series
Jia Whey Jacelyn Ong ; Chin Voon Tong ; Raja Nurazni binti Raja Azwan ; Adilah Zulaikha binti Abd Latib ; Hidayatil Alimi bin Keya Nordin ; Qin Zhi Lee ; Kean Heng Lim ; Jia Ling Low ; Mohd Fyzal bin Bahrudin ; Syaza binti Izhar Hisham ; Liang Wei Wong ; Lisa Mohamed Nor ; Nurain Mohd Noorr
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):48-49
Introduction:
Diabetic ketoacidosis (DKA) in pregnancy is an uncommon
yet life-threatening emergency, with disproportionate risks
to both mother and fetus. Pregnancy-specific physiological changes predispose patients to rapid metabolic decompensation, often with atypical presentations. Despite
this, local data remain limited. We describe the clinical
profile, precipitating factors, and outcomes of DKA in
pregnancy in a tertiary centre, with emphasis on potentially
preventable triggers.
Cases:
Nine pregnant patients with DKA were identified from a
retrospective review of all cases admitted for DKA from
2002 to 2025. Mean age was 31.67 ± 5.20 years; all were
Malay. The majority had type 2 diabetes mellitus (55.6%),
followed by type 1 diabetes (33.3%) and latent autoimmune
diabetes in adults (11.1%). The mean period of amenorrhea
was 19.67 ± 12.62 weeks.
Infection was the leading precipitant (44.4%), with
additional triggers including insulin omission (22.2%),
hyperemesis gravidarum, preterm labor, steroid exposure,
and perioperative fasting. Most diagnoses were made in
the emergency department (55.6%).
Biochemical parameters reflected significant severity (mean
bicarbonate 7.89 ± 2.98 mmol/L; anion gap 25.00 ± 5.81),
with 88.9% classified as severe DKA. Intensive Care Unit
(ICU) care was required in 77.8% of cases. The majority
(77.8%) were admitted to the ICU unit, with a median time
to resolution of 13.00 ± 12.00 hours (interquartile range
[IQR]), and the median hospital length of stay was 7.00 ±
5.00 days (IQR).
Complications during treatment included hypokalemia
(33.3%), acute kidney injury (22.2%), and hypoglycemia
(11.1%). Rebound DKA occurred in one-third of patients.
All patients were discharged clinically stable. Outcome
data demonstrated pregnancy loss in three cases and one
preterm birth.
Conclusion
DKA in pregnancy remains a severe and resource-intensive
condition. This series highlights missed opportunities in
prevention, with modifiable precipitants such as infection
and insulin omission commonly identified. The high
severity at presentation suggests delays in recognition.
Early detection, optimized metabolic care, and targeted
preventive strategies are crucial to improving maternal
and fetal outcomes.
Female
;
Pregnancy
;
Diabetic Ketoacidosis
5.Carrier screening and prenatal diagnosis for Spinal muscular atrophy in 17 926 women of reproductive age in Chongqing.
Xia CHEN ; Yang GAO ; Wenhong CHEN ; Xing LUO ; Keya TONG
Chinese Journal of Medical Genetics 2025;42(2):180-186
OBJECTIVE:
To assess the carrier frequency of spinal muscular atrophy (SMA) in women of childbearing age in Chongqing and to evaluate prenatal diagnostic outcomes in high-risk couples.
METHODS:
A total of 17 926 women of childbearing age attending Chongqing Health Center for Women and Children between May 2021 and November 2023 were enrolled, including 3 398 pre-pregnant women and 14 528 pregnant women, all of whom had no clinical phenotype or family history of SMA or related neuromuscular disorders. Real-time quantitative PCR (RT-qPCR) was used to determine the copy number variations in exons 7 and 8 (E7, E8) of the SMN1 gene. High-risk carriers were identified based on the genetic screening results. Multiplex ligation-dependent probe amplification (MLPA) was employed for prenatal diagnosis of fetuses from high-risk couples. This study was approved by the Medical Ethics Committee of Chongqing Health Center for Women and Children (Ethics No.2021-RGI-02).
RESULTS:
Among the 17 926 women of childbearing age, 298 (1.66%) were identified as heterozygous carriers, including 278 (1.55%) with concurrent deletions of E7 and E8, and 20 (0.11%) with isolated deletions of E7. Seven high-risk couples were identified, six of whom were prenatal couples. Of the two fetuses from these high-risk pregnancies, both exhibited heterozygous deletions of E7 and E8 in the SMN1 gene, while four fetuses showed no abnormalities.
CONCLUSION
This study provides a comprehensive assessment of the carrier frequency of SMA among women of childbearing age in Chongqing, offering valuable data for the primary and secondary prevention of SMA-related birth defects in the region.
Humans
;
Female
;
Muscular Atrophy, Spinal/diagnosis*
;
Pregnancy
;
Prenatal Diagnosis/methods*
;
Adult
;
Survival of Motor Neuron 1 Protein/genetics*
;
Genetic Carrier Screening/methods*
;
DNA Copy Number Variations/genetics*
;
China
;
Genetic Testing
;
Heterozygote
6.Carrier screening and prenatal diagnosis for Spinal muscular atrophy in 17 926 women of reproductive age in Chongqing
Xia CHEN ; Yang GAO ; Wenhong CHEN ; Xing LUO ; Keya TONG
Chinese Journal of Medical Genetics 2025;42(2):180-186
Objective:To assess the carrier frequency of spinal muscular atrophy (SMA) in women of childbearing age in Chongqing and to evaluate prenatal diagnostic outcomes in high-risk couples.Methods:A total of 17 926 women of childbearing age attending Chongqing Health Center for Women and Children between May 2021 and November 2023 were enrolled, including 3 398 pre-pregnant women and 14 528 pregnant women, all of whom had no clinical phenotype or family history of SMA or related neuromuscular disorders. Real-time quantitative PCR (RT-qPCR) was used to determine the copy number variations in exons 7 and 8 (E7, E8) of the SMN1 gene. High-risk carriers were identified based on the genetic screening results. Multiplex ligation-dependent probe amplification (MLPA) was employed for prenatal diagnosis of fetuses from high-risk couples. This study was approved by the Medical Ethics Committee of Chongqing Health Center for Women and Children (Ethics No.2021-RGI-02). Results:① Among the 17 926 women of childbearing age, 298 (1.66%) were identified as heterozygous carriers, including 278 (1.55%) with concurrent deletions of E7 and E8, and 20 (0.11%) with isolated deletions of E7. ② Seven high-risk couples were identified, six of whom were prenatal couples. Of the two fetuses from these high-risk pregnancies, both exhibited heterozygous deletions of E7 and E8 in the SMN1 gene, while four fetuses showed no abnormalities. Conclusion:This study provides a comprehensive assessment of the carrier frequency of SMA among women of childbearing age in Chongqing, offering valuable data for the primary and secondary prevention of SMA-related birth defects in the region.
7.Carrier screening and prenatal diagnosis for Spinal muscular atrophy in 17 926 women of reproductive age in Chongqing
Xia CHEN ; Yang GAO ; Wenhong CHEN ; Xing LUO ; Keya TONG
Chinese Journal of Medical Genetics 2025;42(2):180-186
Objective:To assess the carrier frequency of spinal muscular atrophy (SMA) in women of childbearing age in Chongqing and to evaluate prenatal diagnostic outcomes in high-risk couples.Methods:A total of 17 926 women of childbearing age attending Chongqing Health Center for Women and Children between May 2021 and November 2023 were enrolled, including 3 398 pre-pregnant women and 14 528 pregnant women, all of whom had no clinical phenotype or family history of SMA or related neuromuscular disorders. Real-time quantitative PCR (RT-qPCR) was used to determine the copy number variations in exons 7 and 8 (E7, E8) of the SMN1 gene. High-risk carriers were identified based on the genetic screening results. Multiplex ligation-dependent probe amplification (MLPA) was employed for prenatal diagnosis of fetuses from high-risk couples. This study was approved by the Medical Ethics Committee of Chongqing Health Center for Women and Children (Ethics No.2021-RGI-02). Results:① Among the 17 926 women of childbearing age, 298 (1.66%) were identified as heterozygous carriers, including 278 (1.55%) with concurrent deletions of E7 and E8, and 20 (0.11%) with isolated deletions of E7. ② Seven high-risk couples were identified, six of whom were prenatal couples. Of the two fetuses from these high-risk pregnancies, both exhibited heterozygous deletions of E7 and E8 in the SMN1 gene, while four fetuses showed no abnormalities. Conclusion:This study provides a comprehensive assessment of the carrier frequency of SMA among women of childbearing age in Chongqing, offering valuable data for the primary and secondary prevention of SMA-related birth defects in the region.
8.Live born monochorionic dizygotic twins after in vitro fertilization: a case report
Xueqi LI ; Keya TONG ; Jianqing WEN ; Dongyun LIU
Chinese Journal of Perinatal Medicine 2019;22(6):420-423
This paper reported a pair of twin babies who were diagnosed as monochorionic (MC) twins (MC) in the first-trimester ultrasound scan after in vitro fertilization.Fluorescence in situ hybridization and chromosomal karyotype analysis of amniotic fluid that was randomly collected from one of the twins were performed in the second trimester,but no abnormality was detected.Chromosome karyotype analysis of peripheral blood of the twins at the age of two days and three years showed that both were chi 47,XY,+21/46,XX.And the single nucleotide polymorphism microarray analysis of oral mucosa cells of the twins at three years old showed that the boy was 47,XY,+21 and the girl was 46,XX.Therefore,we confirmed that they are MC dizygotic twins presenting with blood chimerism but no tissue chimerism.Simply the boy presented with facial features of Down syndrome.


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