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Hypopituitarism can result in potentially life-threatening complications including .... that the diagnosis of gender identify disorder (GID) be made by a mental health ... pediatric endocrine nurse be able to support the transgender patient through the ... polydipsia, she presented with DKA and started insulin with subsequent ...
Abstracts of the Pediatric Endocrinology Nursing Society Conference 2014 (9–30 umol/L), alkaline phosphatase 429 (120–280 unit/L), AST 86 (9–80 unit/L), GGT 124 (17–126 unit/L), albumin 3.9 (3.5–5.0 gm/dL), total bilirubin 7.1 (0.2–1.3 mg/dL), direct bilirubin 4.5 (0.0–0.6 mg/dL), cortisol b 1.0 (0.9–19.4 ug/dL). Pituitary MRI revealed an ectopic posterior pituitary, small anterior pituitary, and absent pituitary stalk. ACTH was 14 (20–80 pg/mL). T4 was 5.2 (6.1–13.7 ug/dL). Free T4 was 0.8 (1.0–1.8 ng/dL). TSH was 1.57 (1.7–9.1 uIU/mL). Critical sample during hypoglycemia (50 mg/dL) revealed growth hormone level 4.3 (N 10 ng/mL), cortisol 1.1 (0.9–19.4 ug/dL [range for random sample]), and insulin b 2 (6–26 uU/mL [range for random sample]). Weight was 3.4 kg (b 3%), and length was 56.0 cm (3%). Liver biopsy demonstrated no evidence of biliary atresia, and hyperbilirubinemia was attributed to growth hormone deficiency. Ophthalmologic evaluation revealed minimal bilateral optic nerve hypoplasia. Interventions: Hormone replacement therapy was initiated with hydrocortisone 2.5 mg TID (25 mg/m2/day), followed by levothyroxine 25 mcg daily and growth hormone 0.1 mg daily (0.3 mg/kg/ week). Glucose level was normalized, and feeding was improved although slow weight gain persisted into the present period (7% at 3 years 8 months). Jaundice resolved by 3 ½ months. Discussion/Recommendations: Low growth hormone and cortisol levels, hypoglycemia in an infant, and abnormal MRI support the diagnosis of hypopituitarism. Hyperbilirubinemia associated with hypopituitarism in an infant is uncommon although it has been reported. Hypopituitarism can result in potentially life-threatening complications including severe hypoglycemia, electrolyte imbalance, and shock. Hypopituitarism should be included in the evaluation of infants with jaundice and failure to thrive. http://dx.doi.org/10.1016/j.pedn.2014.03.008 006 – Systemic Reaction Following Histrelin Subdermal Implant in a Female With Central Precocious Puberty Cynthia Gordner MSN, RN, Paul Mueller MD Penn State Hershey Medical Center, Hershey, PA, USA

Patient Demographics: Ten-year 2-month-old Caucasian female. Clinical Presentation: RD presented to the endocrinology office approximately 10 days post-histrelin subdermal implant insertion. She complained of severe incisional pain, rash, hives, and itching beginning at the area of the adhesive bandage covering the insertion site approximately 1 week after a second yearly implant. The rash extended to include the face, arms, and legs. She reported tightness and itching in her throat. She had multiple emergency department visits over the previous week for recurrence of these symptoms. The symptoms began with a local reaction and progressed to a systemic reaction. Past History: RD was diagnosed with central precocious puberty at age 8 years 9 months: height velocity 10 cm/year, height N 97%, tanner 4 breast development, café-au-lait spots on left side, bone age consistent with chronologic age, stimulated LH 25.6 mIU/mL, normal MRI. She was treated with histrelin subdermal implant. Evaluation: RD received evaluations from multiple specialty providers: pediatric surgery, endocrinology, dermatology, and allergy and immunology. Thorough history to potential allergens (foods, skin products, detergents) did not reveal any new exposures. Patch testing to the adhesive bandage did not produce an allergic reaction.

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Interventions: Hydrocodone and acetaminophen were prescribed for pain relief. Triamcinolone cream was ordered for topical treatment of rash, hives and itch. Diphenhydramine, hydroxyzine, and prednisone were prescribed for the systemic reaction without improvement. Histrelin implant was surgically removed 2 weeks following insertion, with resolution of allergic symptoms within 3 days. Additional patch testing with the hydrogel polymer reservoir used to house histrelin and RD's own implant were planned, but never completed as RD was lost to follow-up. Discussion/Recommendations: Histrelin subdermal implant is generally well-tolerated. Site reactions reported during clinical studies include bruising, pain, soreness, erythema, and swelling. One other systemic reaction has been reported to date in postmarketing surveillance. RD had previously received a histrelin implant without incident. Although allergic reactions to histrelin subdermal implant are rare, pediatric endocrinology nurses should have an awareness of the potential for allergic reactions, and should educate parents to report any allergic symptoms. http://dx.doi.org/10.1016/j.pedn.2014.03.009 007 – Thyroxine Malabsorption in Pediatric Patient With Helicobacter Pylori Infection Autoimmune Gastritis Pamela Jennings DNP, RN, PNP, Bassem Dekelbab MD, Robert Truding MD, Melissa Jennings MS1 Beaumont Hospital, Royal Oak, MI, USA

Patient Demographics: Twelve-year-old Caucasian female. Clinical Presentation: During the 15 months after diagnosis of autoimmune hypothyroidism, she required increasing doses of levothyroxine that were unexplained. Parents were asked to supervise medication administration. Past History: Referred for delayed growth and puberty. She had a small goiter. Height and weight were at the 3rd percentile. No family history of hypothyroidism or autoimmune disease. Evaluation: Celiac screen, sedimentation rate, complete blood count, stool occult blood, and vitamin B-12 level were completed to rule out a malabsorption problem. Studies were normal except for mild macrocytic anemia. She had no gastrointestinal symptoms. Over the next 2 years she had significantly increased TSH values (200s mclU/L) interspersed with normal values. Levothyroxine loading test with 1000 mcg PO showed a normal increase in serum FT4 level after 6 hours, indicating good absorption. Levothyroxine was changed to Synthroid. Endoscopy showed normal colonoscopy and positive H. pylori gastritis which was treated. She continued to have erratic TSH elevation (up to 947.60 mclU/L) despite a high dose of Synthroid (5.4 mcg/kg/day). A C-14 urea breath test was positive indicating recurrence of H. pylori infection. Interventions: She was treated again for H. pylori. TSH decreased to 1.38 mclU/L 1 month after treatment. TSH levels were stable for 1 year with subsequent increased value of 102.35 mcIU/L. H.pylori antigen in stool was negative. Repeated endoscopic biopsies were consistent with autoimmune gastritis and no indication of H.pylori infection. Intrinsic factor blocking antibody was negative with positive anti-parietal antibody. Due to B-12 and iron deficiency, she was started on oral supplementation of both. She is currently taking Synthroid 4 mcg/kg/d. Discussion/Recommendations: Recent reports of increased thyroxine dose in adult patients with impaired gastric acid secretion

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highlight the role of the stomach in subsequent intestinal T4 absorption. Gastric acid producing machinery is compromised in atrophic gastritis, use of proton pump inhibitors, and H. pylori infection. There are no known previous reports in the pediatric population. An unexplained high dose requirement of thyroxine or erratic thyroid function studies should trigger workup for malabsorption including causes of decreased gastric acidity. Patients with acquired hypothyroidism should be screened for autoimmune diseases. http://dx.doi.org/10.1016/j.pedn.2014.03.010 008 – A Transgender Youth With Diabetes Rebecca Marks BSN, RN Randall Children's Diabetes and Endocrine Center, Portland, OR, USA

Patient Demographics: Twenty-year-old affirmed Caucasian female. Clinical Presentation: She was diagnosed with type 1 diabetes mellitus 8 years ago. As she progressed through puberty, her family and clinic staff noticed increasing withdrawal and depression and the negative effects it had on her diabetes control. Past History: For 6 years her diabetes was well controlled. Over time, her testing was less frequent, HbA1C levels increased, grades decreased, and she had increased social anxiety. She acknowledged depression and through counseling with the social worker, she shared that she was transgender. Evaluation: Nursing staff discussed several local options for counseling; she was was referred and subsequently followed by a local psychologist specializing in transgender youth. It is imperative that the diagnosis of gender identify disorder (GID) be made by a mental health professional, with training in adolescent developmental psychotherapy. Nursing staff scheduled appointment with the clinic's pediatric endocrinologist who specializes in transgender youth and agreed with the GID diagnosis and prescribing crosshormone treatment. Interventions: With clinic staff support as well as strong family support, she was able to start cross-hormone therapy. Leuprolide acetate depot was authorized, and she was taught how to selfadminister. Cross-hormone medications were ordered; nursing staff reviewed medications and possible side effects. It is crucial that the pediatric endocrine nurse be able to support the transgender patient through the intricate maze of the health care system. This can include understanding their underlying mental and physical needs, knowing the local resources/organizations that can support the transgender youth, and understanding their legal rights in healthcare, schools, and the community. Discussion/Recommendations: Pediatric endocrine nurses play a unique role in the emotional and physical well being of transgender children. Many emotionally suffer as they experience the disconnect between their biological sex, which is their anatomy, and their gender, which includes behaviors, roles, and activities. It is well documented that both suicidal ideation and attempts are higher in this population. Given that it is now accepted and often routine practice for transgender children to be followed in pediatric endocrine clinics, this gives the pediatric endocrine nurse a new and essential role. http://dx.doi.org/10.1016/j.pedn.2014.03.011

009 – Hypoglycemia Without Insulin in Early Type 1 Diabetes Joanne T. Moser MSN, CRNP, David R. Langdon MD The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

Patient Demographics: Thirty-three-month-old Caucasian female. Clinical Presentation: At 24 months, after 10 days of polyuria and polydipsia, she presented with DKA and started insulin with subsequent hypoglycemia. Past History: There was no hypoglycemia prior to diagnosis of type 1 diabetes mellitus (T1DM). At 22 months, she had a febrile seizure with prolonged fever treated with prednisone × 3 days. At 23 months, she was treated with amoxicillin for otitis media. After developing increased thirst and saturated diapers, the antibiotic was discontinued on day 8. Evaluation: Initial labs: glucose 566 mg/dL, 2 + ketonuria, bicarb 20 mmol/L, HgA1c 9.1%, insulin b 2 uU/ml, C-peptide 0.21 ng/ml (range 0.0–3.3 ng/ml), and low IgA level. Initial autoantibodies were undetectable, including GAD, IAA, ICA512, anti-TTg, and anti-TPO. Basal/bolus insulin regimen was initiated at total daily dose 0.4 u/kg/day. After discharge, insulin was discontinued within a week due to hypoglycemia. Hypoglycemia persisted after insulin was discontinued. No severe symptoms were reported. Blood glucoses, typically 40–70 mg/dL, caused mild irritability that resolved with food. An ordinary but pronounced honeymoon was presumed. Two months later, diabetes antibodies including zinc transporter antibody were strongly positive, IgG anti-TTG weakly positive, and antiadrenal antibody negative. Over the next 3 months, hypoglycemia continued, and she was readmitted. No insulin was given. At a glucose level of 46 mg/dL, insulin was 2.6 uU/ml. A mixed meal tolerance test found: (1) mildly low but asymptomatic fasting and basal glucoses,(2) ongoing post prandial hyperglycemia, and (3) shortened fast duration of 17 hours. Findings were suggestive of mild hyperinsulinemic hypoglycemia. Interventions: Nutritional counseling included avoidance of high carbohydrate meals except for symptomatic hypoglycemia. CGMS was initiated for safety and confirmation. The documented pattern described above continued. During an illness, blood glucose rose requiring rapid acting insulin 0.5 unit doses sporadically. Telephone support is ongoing given the high level of parental anxiety. Discussion/Recommendations: This is an unusual course of T1DM. Because of mild hypoglycemia and apparent onset after insulin treatment, hyperinsulinism was thought to be a manifestation of her autoimmune beta cell dysregulation. No evidence of inappropriate exogenous insulin use was seen. Close follow up and further genetic testing are warranted. http://dx.doi.org/10.1016/j.pedn.2014.03.012 010 – No Pancreas, No Problem! A Case of Mild Diabetes After Total Pancreatectomy With Islet Autotransplantation Linda J. Steinkrauss MSN, CPNP, Daniel A. Doyle MD Nemours A.I. duPont Hospital for Children, Wilmington, DE, USA

Patient Demographics: Five-year-old African American female. Clinical Presentation: JC presented to endocrine clinic with the diagnosis of post-pancreatectomy diabetes mellitus. She was euglycemic on 0.4 units/kg/day of insulin. Past History: JC has a history of a recessively inherited, genetic form of chronic pancreatitis. JC's genetic testing demonstrated