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Abstracts of the Pediatric Endocrinology Nursing Society Conference 2014

(930 umol/L), alkaline phosphatase 429 (120280 unit/L), AST


86 (980 unit/L), GGT 124 (17126 unit/L), albumin 3.9 (3.55.0
gm/dL), total bilirubin 7.1 (0.21.3 mg/dL), direct bilirubin 4.5
(0.00.6 mg/dL), cortisol b 1.0 (0.919.4 ug/dL). Pituitary MRI
revealed an ectopic posterior pituitary, small anterior pituitary, and
absent pituitary stalk. ACTH was 14 (2080 pg/mL). T4 was 5.2
(6.113.7 ug/dL). Free T4 was 0.8 (1.01.8 ng/dL). TSH was 1.57
(1.79.1 uIU/mL). Critical sample during hypoglycemia (50 mg/dL)
revealed growth hormone level 4.3 (N 10 ng/mL), cortisol 1.1
(0.919.4 ug/dL [range for random sample]), and insulin b 2 (626 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 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, caf-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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Abstracts of the Pediatric Endocrinology Nursing Society Conference 2014

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 Childrens 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 0.03.3 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 4070 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

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