A Case of Primary Hyperparathyroidism Secondary to Parathyroid Adenoma in a Pediatric Patient.

Background/Objective: Although common in adults, primary hyperparathyroidism (PHPT) is a rare condition in children with the most common etiology being solitary parathyroid adenoma (PTA). The typical presentation is symptomatic hypercalcemia. Management of PHTP secondary to PTA requires excision of the adenoma. Case Report: A 13-year-old adolescent boy presented because of orbital cellulitis and was noted to have hypercalcemia. Despite this, the patient was curiously asymptomatic. Further investigations yielded an elevated parathyroid hormone (PTH) level and a normal urine calcium-to-creatinine ratio making the most likely cause of hypercalcemia PHTP secondary to PTA. Imaging demonstrated PTA. The patient underwent parathyroidectomy with the pathology demonstrating PTA. Postoperatively, the PTH levels were undetectable; hence, the patient was treated with calcitriol and calcium supplementation for 1 month and 4 months, respectively. Genetic work-up for multiple endocrine neoplasia 1 and rearranged during transfection mutations was negative. Discussion: Solitary PTA is the most common cause of PHPT. Adenomas are mostly sporadic or may be a manifestation of an inheritable syndrome, such as multiple endocrine neoplasia. Although symptomatic disease is more common in children, our patient denied any hypercalcemia symptoms. The distinguishing biochemical feature of PHPT because of PTA is high or inappropriately normal PTH level in the context of high-normal or elevated serum calcium levels. Urinary calcium excretion is usually normal or high. PTAs are localized by ultrasound and Tc-99m-Sestamibi scintigraphy. Management includes parathyroidectomy and monitoring for postoperative hypocalcemia. Conclusion: In a child or adolescent presenting with hypercalcemia and elevated PTH levels, it is important to consider PHPT secondary to PTA, because an early diagnosis will aid in preventing complications from hypercalcemia.

Impact of Weight on Severity of Hospital Course in Children Admitted With COVID-19.

Objective. To describe the impact of weight on length of stay (LOS) and oxygen requirement among hospitalized children with COVID-19. Methods. This is a retrospective review of 153 children admitted for COVID-19 from March 2020 to October 2021. Body mass index (BMI) percentile and weight-for-age (WFA) percentile were used to determine weight status for children ≥2 years and <2 years respectively. Results. We found 2 distinct patterns for patients <2 years and ≥2 years; The likelihood of needing oxygen and LOS ≥ 5 days was higher for children ≥2 years with BMI ≥ 85th percentile (P = .0415 and P = .0197). Among those <2 years, mean WFA percentile decreased with increasing oxygen need (P = .0325). There was a negative correlation between LOS and WFA percentile (r = -.31, P = .0123). Conclusion. It is important to stratify patients' risk according to their age, BMI and WFA percentile during hospitalization for COVID 19.

Unusual Endocrinopathies in 18q Deletion Syndrome: Pseudoparathyroidism and Hyper-/Hypo-Thyroidism.

OBJECTIVE: To describe new and unusual endocrinopathies in children with de novo 18q deletion (18q-) syndrome. METHODS: We describe 2 patients who have atypical thyroid conditions and 1 who also developed symptomatic hypocalcemia. RESULTS: The first patient developed hyperthyroidism at the age of 3 years, with a free thyroxine level of 3.9 (range, 0.8-1.8) ng/dL. Thyroid peroxidase antibodies were 262 (range, 0-32) IU/mL, and thyroid-stimulating immunoglobulin antibodies were 384% (range, 0-139%). On low-dose methimazole treatment, she developed hypothyroidism. Thyroid-stimulating hormone (TSH) level was 163 (range, 0.4-4.5) mIU/mL. Moreover, she later developed growth hormone deficiency. The second patient developed hypothyroidism at the age of 4 years, with a TSH level of 46 mIU/mL. However, TSH remained elevated at levels of 10 to 24 mIU/mL for 3 years, despite appropriate treatment, suggesting TSH resistance. She then developed hypocalcemic seizures and was diagnosed with pseudohypoparathyroidism. Her total calcium level was 6.6 (range, 8.5-10.5) mg/dL and parathyroid hormone level was 432 (range, 15-65) pg/dL. CONCLUSION: The first patient had a mixed picture of autoimmune hypothyroidism and hyperthyroidism, requiring a combination of methimazole and levothyroxine to achieve a euthyroid state. For the second patient, the mild TSH resistance was possibly the early suggestion of a parathyroid hormone resistant state. Although growth hormone deficiency and hypothyroidism are common in patients with 18q- syndrome, the occurrence of hyperthyroidism due to Graves' disease with the coexistence of Hashimoto's hypothyroidism is rare. Pseudohypoparathyroidism has not yet been reported in patients with 18q- syndrome.

Effect of Covid-19 quarantine on diabetes Care in Children.

BACKGROUND: With the onset of the COVID-19 pandemic and state-mandated school closures in the spring of 2020, the management of type 1 diabetes in children underwent significant changes. The aim of our study was to assess the effect of stay-at-home orders on glycemic control in children. METHODS: We conducted a retrospective review of 238 children with type 1 and type 2 diabetes who were seen in the Pediatric Endocrinology Clinic at the University of South Alabama. Average Hemoglobin A1c (A1c) levels in the year prior to stay-at home orders (May 2019-April 2020) were compared with A1c values during the quarantine period (May 2020-July 2020) using a paired t-test. We also analyzed the change of A1c level with respect to sex, race, type of diabetes, type of insurance, and mode of insulin administration, using a 2-sample t-test. RESULTS: The average A1c significantly increased from 9.2% during the previous year to 9.5% during the quarantine period (p = 0.0097). The increase of A1c was significantly higher in public insurance patients (0.49% increase) compared to private insurance patients (0.03% increase), (p = 0.0137). We also observed a significant association between the direction of change and type of insurance. Forty-eight percent of public insurance patients had an A1c increase of > 0.5% while 54% of private insurance patients had no change or decrease in A1c (p = 0.0079). CONCLUSIONS: The COVID-19 pandemic resulted in worsening glycemic control in children with type 1 diabetes, with those on public insurance affected in greater proportion than those with private insurance.

Legg-Calve-Perthes disease in an 8-year old girl with Acrodysostosis type 1 on growth hormone therapy: case report.

BACKGROUND: Acrodyostosis type 1 (ACRDYS1) is a rare skeletal dysplasia, and sometimes it can be misdiagnosed as pseudohypoparathyroidism type 1A (PHP1A), a subtype of Albright hereditary osteodystrophy (AHO), due to overlapping features. Growth hormone releasing hormone (GHRH) resistance with severe short stature is common in both ACRDYS1 and PHP1A (Emily L. Germain-Lee, et al. J Clin Endocrinol Metab, 88:4059-4069, 2003). Whereas growth hormone (GH) treatment has been studied in patients with PHP1a, the same is not true for the rarer ACRDYS1. Here in we report an adverse orthopedic outcome in a patient with ACRDYS1 with severe short stature treated with growth hormone. Our experience could have implications for the treatment of other patients with this disorder. CASE PRESENTATION: We report a case of Legg-Calve-Perthes Disease (LCPD) in an 8-year old female with ACRDYS1 treated with GH. She initially presented with marked short stature (height Z-score - 3.46) with a low normal insulin like growth factor-1 (IGF1) level, and had biochemical evidence of thyrotropin and parathyroid hormone resistance. GH therapy was initiated at 0.35 mg/kg/week leading to increased growth velocity. After 7 months on GH, she developed right knee pain. Radiographic images revealed flattening of her right femoral head consistent with LCPD. GH was discontinued. Six weeks later, radiographs revealed further collapse of the entire femoral head. Her lesion stabilized after 8 months with conservative management and she never resumed GH. Her final adult height is 4'2″ (128 cm). CONCLUSION: Patients with ACRDYS1 on GH therapy may be at increased risk of LCPD. This has not been reported in patients with PHP1A treated with GH. Clinicians and families need to be aware of this potential complication when counseling about GH treatment.