Xanthomatous Hypophysitis Presenting in an Adolescent Girl: A Long-Term Follow-Up of a Rare Case and Review of the Literature.

OBJECTIVE: Primary hypophysitis refers to the isolated inflammation of the pituitary gland not associated with other secondary causes. Among its histopathologic subtypes, xanthomatous is the rarest. METHODS: We describe a 22-year-old woman with xanthomatous hypophysitis (XH), its clinical progression over 8 years as well as the treatment effects of prednisolone and azathioprine. Our patient was first referred for severe short stature and delayed puberty at the age of 14 years. RESULTS: Investigations revealed multiple pituitary deficiencies. Magnetic resonance imaging showed a pituitary mass whereby a partial resection was performed. A full resection was not feasible due to the location of the mass. The histopathologic analysis of the tissue was consistent with XH. The results of secondary workout for neoplasm, infection, autoimmune, and inflammatory disorders were negative. After surgery, a progressive enlargement of the mass was observed. Two courses of prednisolone were administered with a significant reduction in the mass size. Azathioprine was added due to the unsustained effects of prednisolone when tapered off and the concern of steroid toxicity with continued use. No further increase in the mass size was noted after 6 months on azathioprine. CONCLUSION: Glucocorticoid and immunotherapy are treatment options for XH; however, more cases are needed to better understand its pathogenesis and clinical progression.

Reference intervals for thyroid-stimulating hormone (TSH) and free thyroxine (FT4) in infants' day 14-30 of life and a comparison with other studies.

Objectives Established reference intervals of thyroid function in neonates are important; however, studies often consist of a small sample size or lack of clinical information. We aim to define reference intervals for thyroid-stimulating hormone (TSH) and free thyroxine (FT4) for infants aged 14-30 days. We also reviewed follow-up TSH for infants with initial values 10-20 mIU/L. Methods Venous TSH and FT4 of term babies aged 14-30 days with breast milk jaundice that had thyroid function test performed as part of a prolonged jaundice workout from September 2016 to March 2017 were analyzed. Electronic medical records were reviewed to ensure only well babies with no pathological causes of jaundice or conditions that may affect thyroid function were included. TSH and FT4 were analyzed using immunoassay analyzer Dxl 800, Beckman Coulter. Results There were no correlations between FT4 and TSH with gender, birth weight and ethnicity. Correlation coefficient between FT4 and total bilirubin was weak at 0.138 (p=0.001). No association was found between TSH and bilirubin levels. Mean FT4 was higher in the younger age group day 14-21 (p<0.01). There was no significant difference in TSH values between the age groups. Infants with mildly elevated TSH 10-20 mIU/L had normalized values on follow-up (mean, 11.41 vs. 4.42 mIU/L; p<0.01; 95%CI, 5.88-8.09). The following reference intervals (2.5-97.5th percentile) were derived: FT4 day 14-21 (n=513): 11.59-21.00 pmoL/L; FT4 day 22-30 (n=66): 10.14-19.60 pmoL/L; TSH day 14-30 (n=579): 1.90-10.34 mIU/L. Comparison between studies showed variations of reference intervals with different manufacturer assays, age and methodology. Conclusions Our reference intervals would be useful in the clinical setting. Infants with mildly elevated TSH could be monitored first instead of immediate treatment.

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.