Precocious puberty in children: A review of imaging findings.

OBJECTIVES: This review was aimed at determining the imaging findings in patients with precocious puberty. RESULTS: Within a period of 8 years (from 2002 to 2010) there were 53 patients diagnosed with precocious puberty. Out of the 53 patients, 37 had undergone diagnostic imaging to detect the possible organic causes of precocious puberty. Imaging findings were positive in 31 patients and out of that, 3 patients had 2 findings each (34 abnormalities). Of the patients with positive imaging findings, central precocious puberty (gonadotrophin-dependent) was more common (81%; 25/31) and the causes included: tuber cinereum hamartoma (n = 10), glioma (n = 6), pineal gland tumour (n = 4), hydrocephalous (n = 3), arachnoid cyst (n = 2) and others (n = 3). Peripheral precocious puberty (gonadotrophin-independent) causes included: testicular adrenal rest tumour (n = 3), adrenal carcinoma (n = 1), ovarian granulosa thecal cell tumour (n = 1), and tuberous sclerosis (n = 1). CONCLUSION: Positive imaging findings were observed in 84% (31/37) of the subjects. Hypothalamic hamartoma was the most common imaging finding in central precocious puberty while testicular adrenal rest tumour was the most common imaging finding in peripheral precocious puberty.

The Third National Health and Morbidity Survey: prevalence of obesity, and abdominal obesity among the Malaysian elderly population.

Obesity is an emerging public health threat in the elderly population in developing countries. Hence, the Third National Health and Morbidity Survey has assessed 4746 individuals aged 60 years and older recruited through a household survey to determine the prevalence of adiposity using body mass index and waist circumference. The national's prevalence of overweight and obesity in men was 29.2% (95% confidence interval [CI] = 27.2-31.3) and 7.4% (95% CI = 6.4-8.6), respectively. However, the prevalence decreased with age. The figures in women were 30.3% (95% CI = 28.5-32.1) and 13.8% (95% CI = 12.5-15.2), respectively. The prevalence of abdominal obesity was 21.4% (95%CI = 20.2-22.6), with 7.7% (95% CI = 6.7-9.0) in men and 33.4% (95% CI = 31.4-35.3) in women. Predictors of adiposity include the following: Malay and Indian ethnicity, higher education level, higher household income, from urban area, and being married. In conclusion, adiposity affects about one third of the Malaysian elderly population, especially those of the younger age group, women, and those with higher socioeconomic status.

Precocious puberty in children: A review of imaging findings

Objectives: This review was aimed at determining the imaging findings in patients with precocious puberty. Results: Within a period of 8 years (from 2002 to 2010) there were 53 patients diagnosed with precocious puberty. Out of the 53 patients, 37 had undergone diagnostic imaging to detect the possible organic causes of precocious puberty. Imaging findings were positive in 31 patients and out of that, 3 patients had 2 findings each (34 abnormalities). Of the patients with positive imaging findings, central precocious puberty (gonadotrophin-dependent) was more common (81%; 25/31) and the causes included: tuber cinereum hamartoma (n = 10), glioma (n = 6), pineal gland tumour (n = 4), hydrocephalous (n = 3), arachnoid cyst (n = 2) and others (n = 3). Peripheral precocious puberty (gonadotrophin-independent) causes included: testicular adrenal rest tumour (n = 3), adrenal carcinoma (n = 1), ovarian granulosa thecal cell tumour (n = 1), and tuberous sclerosis (n = 1). Conclusion: Positive imaging findings were observed in 84% (31/37) of the subjects. Hypothalamic hamartoma was the most common imaging finding in central precocious puberty while testicular adrenal rest tumour was the most common imaging finding in peripheral precocious puberty

Sensitivity and specificity of waist circumference as a single screening tool for identification of overweight and obesity among Malaysian adults.

Generalised obesity and central obesity are risk factors for Type II diabetes mellitus and cardiovascular diseases. Waist circumference (WC) has been suggested as a single screening tool for identification of overweight or obese subjects in lieu of the body mass index (BMI) for weight management in public health program. Currently, the recommended waist circumference cut-off points of > or = 94cm for men and > or =80cm for women (waist action level 1) and > or = 102cm for men and > or = 88cm for women (waist action level 2) used for identification of overweight and obesity are based on studies in Caucasian populations. The objective of this study was to assess the sensitivity and specificity of the recommended waist action levels, and to determine optimal WC cut-off points for identification of overweight or obesity with central fat distribution based on BMI for Malaysian adults. Data from 32,773 subjects (14,982 men and 17,791 women) aged 18 and above who participated in the Third National Health Morbidity Survey in 2006 were analysed. Sensitivity and specificity of WC at waist action level 1 were 48.3% and 97.5% for men; and 84.2% and 80.6% for women when compared to the cut-off points based on BMI > or = 25kg/m2. At waist action level 2, sensitivity and specificity were 52.4% and 98.0% for men, and 79.2% and 85.4% for women when compared with the cut-off points based on BMI (> or = 30 kg/m2). Receiver operating characteristic analyses showed that the appropriatescreening cut-off points for WC to identify subjects with overweight (> or = 25kg/m2) was 86.0cm (sensitivity=83.6%, specificity=82.5%) for men, and 79.1cm (sensitivity=85.0%, specificity=79.5%) for women. Waist circumference cut-off points to identify obese subjects (BMI > or = 30 kg/m2) was 93.2cm (sensitivity=86.5%, specificity=85.7%) for men and 85.2cm (sensitivity=77.9%, specificity=78.0%) for women. Our findings demonstrated that the current recommended waist circumference cut-off points have low sensitivity for identification of overweight and obesity in men. We suggest that these newly identified cut-off points be considered.

Reliability, technical error of measurements and validity of length and weight measurements for children under two years old in Malaysia.

The National Health and Morbidity Survey III 2006 wanted to perform anthropometric measurements (length and weight) for children in their survey. However there is limited literature on the reliability, technical error of measurement (TEM) and validity of these two measurements. This study assessed the above properties of length (LT) and weight (WT) measurements in 130 children age below two years, from the Hospital Universiti Kebangsaan Malaysia (HUKM) paediatric outpatient clinics, during the period of December 2005 to January 2006. Two trained nurses measured WT using Tanita digital infant scale model 1583, Japan (0.01kg) and Seca beam scale, Germany (0.01 kg) and LT using Seca measuring mat, Germany (0.1cm) and Sensormedics stadiometer model 2130 (0.1cm). Findings showed high inter and intra-examiner reliability using 'change in the mean' and 'intraclass correlation' (ICC) for WT and LT. However, LT was found to be less reliable using the 'Bland and Altman plot'. This was also true using Relative TEMs, where the TEM value of LT was slightly more than the acceptable limit. The test instruments were highly valid for WT using 'change in the mean' and 'ICC' but was less valid for LT measurement. In spite of this we concluded that, WT and LT measurements in children below two years old using the test instruments were reliable and valid for a community survey such as NHMS III within the limits of their error. We recommend that LT measurements be given special attention to improve its reliability and validity.

Reliability, technical error of measurements and validity of instruments for nutritional status assessment of adults in Malaysia.

INTRODUCTION: The Third National Health and Morbidity Survey Malaysia 2006 includes a nutritional status assessment of children. This study aimed to assess the inter- and intra-examiner reliability, the technical error of measurement and the validity of instruments for measuring weight, height and waist circumference. METHODS: A convenience sample of 130 adults working in a selected office setting was chosen to participate in the study, subject to the inclusion and exclusion study criteria. Two public health nurses, trained to follow a standard protocol, obtained the weight, height and waist circumference measurements. The weight was measured using the Tanita HD-318 digital weighing scale to the nearest 0.1 kg, and Seca Beam Scale to the nearest 0.01 kg. The height was measured using the Seca Bodymeter 206 and Stadiometer, both to the nearest 0.1 cm. The waist circumference was measured using the Seca circumference measuring tape S 201, to the nearest 0.1 cm. RESULTS: The intra-examiner reliability in descending order was weight and height followed by waist circumference. The height measurement, on average, using the test instrument, reported a recording of 0.4 cm higher than the reference instrument, with the upper and lower limits at 2.5 cm and 1.6 cm, respectively. The technical error of measurement and coefficient of variation of weight and height for both inter-examiner and intra-examiner measurements were all within acceptable limits (below five percent). CONCLUSION: The findings of this study suggest that weight, height and waist circumference measured in adults aged 18 years and above, using the respective above mentioned instruments, are reliable and valid for use in a community survey. Limiting the number of examiners, especially for waist circumference measurements, would yield a higher degree of reliability and validity.

Nutritional Status of Children below Five Years in Malaysia: Anthropometric Analyses from the Third National Health and Morbidity Survey III (NHMS, 2006).

The Third National Health and Morbidity Survey (NHMS III) was conducted in 2006 on a nationally representative sample of population in Malaysia. Over 21,000 children aged 0-17.9 years were measured for body weight and stature according to the protocol of the World Health Organization. This article describes the nutritional status of children aged 0-59.9 months. Mean z score for weightfor-age (WAZ), height-for-age (HAZ) and BMI-for-age were compared with the z-scores tables of the WHO standards. The overall prevalence of underweight and stunting of the children were 12.9% and 17.2% respectively. These levels included 2.4% severe underweight and 6.0% severe stunting. In terms of z scores, the age group of 0-5.9 months showed the best nutritional status with mean WAZ of -0.33 (95%CI: -0.52, 0.15) and -0.40 (-0.57, 0.24) for boys and girls respectively, while mean HAZ was 0.64 (0.38, 0.89) for boys and 0.76 (0.54, 0.98) for girls. Mean HAZ and WAZ status was least satisfactory after about 6 months, suggesting a faltering in growth rate at an age that coincides with dependence on complementary feeding. Prevalence of overweight based on BMI-for-age for the sexes combined was 6.4%, while that based on WAZ was 3.4%. The NHMS III results indicate that Malaysian children have better nutritional status compared to children under 5 years in neighbouring countries. In order to meet the targets set in the National Plan of Nutrition (2006-2015), more effective intervention programmes are needed to accelerate the reduction of underweight and stunting, and to arrest the rise of overweight in young children.

Nutritional Status of Children below Five Years in Malaysia: Anthropometric Analyses from the Third National Health and Morbidity Survey III (NHMS, 2006)

The Third National Health and Morbidity Survey (NHMS III) was conducted in 2006 on a nationally representative sample of population in Malaysia. Over 21,000 children aged 0-17.9 years were measured for body weight and stature according to the protocol of the World Health Organization. This article describes the nutritional status of children aged 0-59.9 months. Mean z score for weightfor-age (WAZ), height-for-age (HAZ) and BMI-for-age were compared with the z-scores tables of the WHO standards. The overall prevalence of underweight and stunting of the children were 12.9% and 17.2% respectively. These levels included 2.4% severe underweight and 6.0% severe stunting. In terms of z scores, the age group of 0–5.9 months showed the best nutritional status with mean WAZ of -0.33 (95%CI: -0.52, 0.15) and -0.40 (-0.57, 0.24) for boys and girls respectively, while mean HAZ was 0.64 (0.38, 0.89) for boys and 0.76 (0.54, 0.98) for girls. Mean HAZ and WAZ status was least satisfactory after about 6 months, suggesting a faltering in growth rate at an age that coincides with dependence on complementary feeding. Prevalence of overweight based on BMI-for-age for the sexes combined was 6.4%, while that based on WAZ was 3.4%. The NHMS III results indicate that Malaysian children have better nutritional status compared to children under 5 years in neighbouring countries. In order to meet the targets set in the National Plan of Nutrition (2006-2015), more effective intervention programmes are needed to accelerate the reduction of underweight and stunting, and to arrest the rise of overweight in young children.

Audit of newborn screening programme for congenital hypothyroidism.

Newborn screening for congenital hypothyroidism (CH) was implemented in Hospital UKM in December 2004 using cord blood sample. From the audit over a period of 25 months, a total of 13,875 newborn babies were screened with a coverage of 98.8%. From this cohort, the mean recall rate was 0.32%; unfortunately the mean percentage of recalled babies that came for retesting was only 79.5%. In addition, the mean sample rejection rate was high, i.e. 2.2%. Two babies were diagnosed to have CH. These findings implied that whilst the coverage of screening was good, there is a need for regular surveillance of performance of both clinical and laboratory personnel. In addition, a more concerted effort should be carried out to promote community awareness of such a programme.

A national database on children and adolescent with diabetes (e-DiCARE): results from April 2006 to June 2007.

In Malaysia, Diabetes in Children and Adolescents Registry (DiCARE) was launched nationwide in August 2006 to determine and monitor the number, the time trend of diabetes mellitus (DM) patients, their socio-demographic profiles, outcome of intervention and facilitate research using this registry. This is an on going real time register of diabetic patients < or = 20 years old via the e-DiCARE, an online registration system. To date were 240 patients notified from various states in Malaysia. The mean age was 12.51 years (1.08-19.75) and 46.4% were boys. The mean age at diagnosis was 8.31 +/- 4.13 years old with an estimated duration of diabetes of 4.32 +/- 3.55 years. A total of 166/240 (69.2%) have T1DM, 42/240 (17.5%) have T2DM and 18/240 (7.5%) have other types of DM. Basis of diagnosis was known in 162 patients with T1DM and 41 patients with T2DM. In T1DM patients, 6.0% of the girls and 19.1% boys were overweight or obese. As for T2DM, 64.3% had their BMI reported: 66.7% girls and 91.6% boys were overweight or obese. Most patients (80.4%) practiced home blood glucose monitoring. Patients were seen by dietitian (66.7%), diabetes educator (50.0%), and optometrist or ophthalmologist (45.0%). Only 10.8% attended diabetic camps. In the annual census of 117 patients, the mean HbAlc level was 10.0% + 2.2 (range 5.2 to 17.0%). The early results of DiCARE served as a starting point to improve the standard of care of DM among the young in the country.

Abdominal Obesity in Malaysian Adults: National Health and Morbidity Survey III (NHMS III, 2006).

Abdominal obesity (AO) is an independent risk factor for cardiovascular disease, hypertension and diabetes mellitus in adults. There is a lack of data on the magnitude and socio-demographic profile of AO among Malaysian adults at the national level. In the Third National Health and Morbidity Survey (NHMS III) conducted in 2006, AO of adults aged 18 years and above was determined based on the waist circumference as part of the nutritional status assessment. This article reports the prevalence of AO in relation to socio-economic factors and demographic characteristics of adult subjects. Out of a total of 33,465 eligible individuals 18 years and above, waist circumference was measured in 32,900 (98.3%) individuals. The prevalence of AO was assessed using the cut-off points recommended by World Health Organization. The mean waist circumference in men and women was 84.0cm [95% confidence interval (95% CI): 83.8, 84.3] and 80.3cm (95% CI: 80.1, 80.6) respectively. The national prevalence of AO was 17.4% (95% CI: 16.9, 17.9). The identified risks of AO were women (OR: 4.2, 95% CI: 3.8, 4.6), aged 50-59 years (OR: 5.6, 95% CI: 4.0, 7.7), Indians (OR: 3.0, 95% CI: 2.4, 3.8), housewives (OR: 1.4, 95% CI: 1.1, 1.7), subjects with primary education (OR: 1.3, 95% CI: 1.1, 1.5) and ever married (OR: 1.4, 95% CI: 1.2, 1.6). Being the largest population-based study on AO among Malaysians, these findings have important public health implications. There is an urgent need to revise public health policies and programmes aimed at prevention of abdominal obesity especially in the groups at risk.

Reliability and Technical Error of Calf Circumference and Mid-half Arm Span Measurements for Nutritional Status Assessment of Elderly Persons in Malaysia.

This study sought to examine the reliability of two measurements; Calf Circumference (CC) and Mid-half Arm Span (MHAS). A sample of 130 elderly persons aged 60 years and above seen consecutively in the Kuala Lumpur Hospital outpatient clinic during the period of December 2005 to January 2006, upon consent, were recruited to the study. There was a high degree of reliability for both inter- and intra-examiner (r close to 1). For inter-examiner, on average the CC measurements taken by the first examiner were 0.3 cm lower than that of the second examiner. The upper and lower limit of the differences were +0.4 to -0.9 cm respectively. Inter-examiner MHAS measurements on average by the first examiner were 0.2 cm lower than that of the second examiner. The limits were +1.7 to -2.1 cm. By comparison, the inter-examiner CC measurements were more reliable than the MHAS measurements. For intra-examiner, on average the CC measurements at Time 1 were consistent with Time 2 (mean difference=0) with limits of the difference at + 0.5 cm. MHAS measurements at Time 1 were on average 0.1 cm less than at Time 2 with limits at +1.7 and -1.8 cm. The technical error of measurement (TEM) and coefficient of variation of CC and MHAS for both interexaminer and intra-examiner measurements were within acceptable limits with the exception of MHAS TEM. This study suggests that CC and MHAS measured in elderly persons 60 years and above, using Seca Circumference Tape ® 206, Germany (0.05 cm) are reliable and can be used in a community survey.

Abdominal Obesity in Malaysian Adults: National Health and Morbidity Survey III (NHMS III, 2006)

Abdominal obesity (AO) is an independent risk factor for cardiovascular disease, hypertension and diabetes mellitus in adults. There is a lack of data on the magnitude and socio-demographic profile of AO among Malaysian adults at the national level. In the Third National Health and Morbidity Survey (NHMS III) conducted in 2006, AO of adults aged 18 years and above was determined based on the waist circumference as part of the nutritional status assessment. This article reports the prevalence of AO in relation to socio-economic factors and demographic characteristics of adult subjects. Out of a total of 33,465 eligible individuals 18 years and above, waist circumference was measured in 32,900 (98.3%) individuals. The prevalence of AO was assessed using the cut-off points recommended by World Health Organization. The mean waist circumference in men and women was 84.0cm [95% confidence interval (95% CI): 83.8, 84.3] and 80.3cm (95% CI: 80.1, 80.6) respectively. The national prevalence of AO was 17.4% (95% CI: 16.9, 17.9). The identified risks of AO were women (OR: 4.2, 95% CI: 3.8, 4.6), aged 50-59 years (OR: 5.6, 95% CI: 4.0, 7.7), Indians (OR: 3.0, 95% CI:2.4, 3.8), housewives (OR: 1.4, 95% CI: 1.1, 1.7), subjects with primary education (OR: 1.3, 95% CI: 1.1, 1.5) and ever married (OR: 1.4, 95% CI: 1.2, 1.6). Being the largest population-based study on AO among Malaysians, these findings have important public health implications. There is an urgent need to revise public health policies and programmes aimed at prevention of abdominal obesity especially in the groups at risk.

Bilateral adrenal cysts and ectopic pancreatic tissue in Beckwith-Wiedemann syndrome: is a conservative approach acceptable?

Beckwith-Wiedemann syndrome is a common overgrowth syndrome associated with an increased risk of neoplasias which might be explained by the nature and localization of the genetic defect. While malignant tumors are often associated with hemihypertrophy, benign tumors are also found. We report a patient with the typical features of Beckwith-Wiedemann syndrome with two histologically different abdominal tumors, bilateral cystic adrenals and ectopic pancreatic tissue present at birth. In both tumors no malignancy could be detected. Ectopic pancreatic tissue is rarely seen and has been described in Beckwith-Wiedemann syndrome only once. After extirpation of the ectopic pancreatic tissue the cystic adrenals were left in situ since macroscopically no normal adrenal tissue could be identified and separated. Regular ultrasound examinations revealed complete resolution of the cystic adrenals within 24 months. Thus it seems that a conservative approach in selected tumors associated with the Beckwith-Wiedemann syndrome might be acceptable.

Short stature and truncal shortening in transfusion dependent thalassemia patients: results from a thalassemia center in Malaysia.

One of the major complications in patients with transfusion dependent thalassemia is growth impairment secondary to iron overload. We studied the growth status in 66 patients with beta-thalassemia major and HbE-beta thalassemia who were transfusion dependent, aged from 2 to 24 years, and 66 controls matched for sex and age. The prevalence of short stature in transfusion-dependent thalassemics was 54.5% compared to 4.5% in control group (p<0.001). Short stature was more prevalent in those above the age of 10 years in this study group (83.3% vs 16.7%). Transfusion dependent thalassemics with short stature were found to have significantly lower mean standing height standard deviation scores (SDS), sitting height SDS and subischial leg length SDS values (p<0.001). There was also a significant difference between the mean sitting height SDS and the mean subischial leg length SDS in our thalassemics with short stature, suggesting that the short stature was due to disproportionate truncal shortening. Serum ferritin levels were significantly higher in transfusion dependent thalassemics who were short compared to those who were of normal height (p = 0.002). However, the mean pre-transfusion hemoglobin levels did not differ significantly between patients with short stature and those with normal height (p = 0.216). The prevalence of short stature also did not differ significantly between those with beta-thalassemia major and those with HbE-beta thalassemia (p = 0.32). This study highlighted the importance of providing optimal treatment in these patients, including monitoring of growth parameters and optimizing iron chelation therapy.

Management and short-term outcome of persistent hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis).

BACKGROUND/AIM OF STUDY: Persistent hyperinsulinaemic hypoglycaemia is a rare metabolic disorder of glucose regulation. It is however the most common cause of persistent hypoglycaemia in the neonatal period. Various drugs have been used with generally poor results, but diazoxide and a long-acting somatostatin analogue, octreotide, have been found to be rather successful. When medical therapy fails, early pancreatectomy is recommended to maintain euglycaemia. Since pancreatectomy seems to carry the long-term risk of diabetes mellitus, some authors recommend long-term medical therapy as an alternative to surgery. The outcome of treatment seems to correlate with neurological status prior to surgery. Even in early recognised and treated patients, publications suggest that a subtle neurological deficit may be present despite apparently normal intelligence. In view of the varying recommendations on treatment and the variations in outcome, we reviewed our experience over a period of three years (1992-1995) to determine whether we could formulate a rational approach to the management. METHODS: From our records, we identified 8 children who fullfilled the criteria for the diagnosis of persistent hyperinsulinaemic hypoglycaemia of infancy and retrospectively reviewed their documents. We also included 2 more who presented to us during the study period. RESULTS: Two out of the 10 were born premature and four were considered large for gestational age, mean birth weight was 3679 gms (range 2580-4400 gms). All except three were symptomatic by day two of life. All except one were given hydrocortisone prior to transfer to our care at a mean age of 22 days (range 8-52 days). Our regime included a trial of diazoxide and octreotide. Near total pancreatectomy was performed in nine patients, seven following a short trial of octreotide. Our two most recent cases were given a longer trial of medical therapy of 9 and 6 months respectively prior to pancreatectomy. Our two early cases in the series had recurrence of hypoglycaemia within a week post-pancreatectomy. One still needed insulin therapy 5 months post-surgery. Seven were available for outcome assessment; while longitudinal growth is normal in all, three were developmentally delayed. CONCLUSION: Based on our experience with short and prolonged course of somatostation analogue, we conclude that early pancreatectomy should be performed on those with inadequate maintenance of euglycaemia while prolonged course of medical therapy is feasible only in selected cases.