Browsing by Author "Alradadi, Rasha"

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    SUN-401 New Right Adrenal Nodule and Beckwith-Wiedemann Syndromein a Girl with Left Adrenocortical Tumor Resection
    (Oxford University Press, 2019-04-15) Alradadi, Rasha; Sanchez, Juan; Pediatrics, School of Medicine
    Background: The adrenocortical tumors have been reported in patients with Beckwith-Wiedemann syndrome, but the optimal way of management is not well studied. We report a case of left adrenocortical adenoma status post left adrenalectomy who subsequently diagnosed with Beckwith-Wiedemann syndrome, thereafter found to have a growing right adrenal nodule. Clinical Case: A 9-year-old female who was initially seen in Endocrinology clinic when she was seven months old. At that time, she was referred for pubic hair that was preceded by acne around the age of 5 months. Screening labs showed elevated DHEA 137 mcg/dl and testosterone 161 ng/d. MRI revealed a 2.5 cm well defined rounded homogenously enhancing soft tissue mass located at the anteroinferior to the lateral limb of the left adrenal gland. She had left adrenalectomy at seven months of age. Pathology of the mass revealed adrenocortical adenoma. She was referred to hematology and oncology. Very mild hemi-hypertrophy started to appear as patient got older, so further work- up revealed loss of methylation of DNA at the DMR2 which is consistent with Beckwith Wiedemann Syndrome. All symptoms had resolved, and she had multiple screening including Ultrasounds and AFP. All her images and AFP remained normal. Then at the age of 5 years, she started to have new pubic hair and acne. CT scan revealed right adrenal thickening and nodularity with a 5 mm nodule. It has been monitored with frequent MRI alternated with ultrasounds. The nodule has increased in size gradually over the years to 15.79 mm x 8.72 mm. PET scan showed focal increased FDG activity within the 1.1 cm x 0.7 cm right adrenal nodule. Bone age was normal. After consultations of multiple national centers, there was no consistency about how to manage adrenal tumors in patient with BWS. Therefore parents were given choices of total right adrenalectomy vs partial adrenalectomy vs observations. Parent chose watching waiting, so decision was made to follow up with MRI every three months. Conclusions: Although patients with BWS get screened with abdominal U/S frequently for adrenal tumors as the part of the tumor surveillance, there is no consensus on management options if such tumors are detected.This case raises the need for uniform guidelines to outline the optimal approach of management of adrenocortical tumor in patient with BWS.
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    What Are the Common Characteristics of Pediatric Patients With Antibody Negative Primary Hypothyroidism?
    (Oxford University Press, 2021) Alradadi, Rasha; Eugster, Erica A.; Pediatrics, School of Medicine
    Background: The most common cause of acquired primary hypothyroidism is autoimmune thyroiditis which is typically associated with positive anti-thyroid peroxidase and/or anti-thyroglobulin antibodies. However, some children present with primary hypothyroidism and negative antibodies. Whether there are differences between patients with acquired primary hypothyroidism who have positive vs negative anti-thyroid antibodies has not been systematically examined. Aim:To define the characteristics of patients with primary hypothyroidism and negative antibodies. Methods: A retrospective chart review of patients with hypothyroidism seen in the pediatric endocrine clinic at Riley Hospital for Children in Indianapolis, Indiana from August 2016 until December 2019 was performed. Variables examined included age at diagnosis, signs and symptoms at presentation, height, weight, BMI, TSH, FT4, T4, thyroid peroxidase and anti-thyroglobulin antibodies, family history of thyroid disease, physical exam at diagnosis, and associated diseases. Results: Of 173 patients aged 10.6 ± 3.9 years, 128 (74%) had positive antibodies and 44 (26%) had negative antibodies. Of those with positive antibodies, 80 % were female and 20% were male. Of those with negative antibodies, 53% were female and 47% were male. No differences were seen in the incidence of obesity or Down syndrome in patients with positive antibodies compared with those who had negative antibodies. A positive family history of thyroid disease was present in 45% of those with positive antibodies and in 22% of those with negative antibodies, P=0.006 Fifty-eight patients (45%) with positive antibodies reported excessive fatigue and 40 (31%) had a goiter. In contrast, 10 (22.7%) who had negative antibodies reported mild intermittent fatigue, P=0.006 and 7 (15.9 %) had a goiter, P=0.04 The average TSH in the antibody positive group was 129± 230 mcu/ml compared with 48 ± 131 mcu/ml in those with negative antibodies, p=0.04. A trend was also noted for a lower FT4 in those with positive antibodies (0.68±0.37 vs 0.85±0.27, p=0.050) No other differences in baseline characteristics were seen between patients with negative vs positive antibodies. Conclusion: Patients with positive anti-thyroid antibodies had more severe hypothyroidism and were more likely to report extreme fatigue than those with negative antibodies. It is unknown why some children with acquired primary hypothyroidism presumed due to autoimmune thyroid disease have negative antibodies. Long-term follow-up will be needed to determine whether the natural history of thyroid disease in children with primary hypothyroidism is associated with antibody status.