Beyond the bias! Sex distribution in paediatric growth hormone deficiency reexamined.

Objectives: Various biases pertaining to stature account for a male sex predominance in growth hormone deficiency (GHD) cases diagnosed by endocrinology clinics. This manuscript will assess the sex distribution when biases are minimised.

Methods: Retrospective chart review was conducted on patients diagnosed with GHD between 3 and 16 years of age.

Approach to the Patient: Case Studies in Pediatric Growth Hormone Deficiency and Their Management.

Context: Pathologies attributed to perturbations of the GH/IGF-I axis are among the most common referrals received by pediatric endocrinologists.

Aim: In this article, distinctive cased-based presentations are used to provide a practical and pragmatic approach to the management of pediatric growth hormone deficiency (GHD).

Cases: We present 4 case vignettes based on actual patients that illustrate (1) congenital GHD, (2) childhood GHD presenting as failure to thrive, (3) childhood GHD presenting in adolescence as growth deceleration, and (4) childhood-onset GHD manifesting as metabolic complications in adolescence.

Growth hormone after CNS tumor diagnosis: the fundamentals, fears, facts, and future directions.

Growth hormone deficiency (GHD) may occur in pediatric patients with central nervous system (CNS) tumors at initial tumor presentation or later as treatment-related sequelae. While it is well recognized that growth hormone (GH) has beneficial effects on growth and endocrinopathies, there's often hesitancy by clinicians to initiate GH therapy for GHD after CNS tumor diagnosis due to the perceived increased risk of tumor recurrence. The available data is described here and based on this review, there is no evidence of increased risk of tumor recurrence or secondary malignancy in patients treated with GH after CNS tumor diagnosis.

The utility of a random cortisol level in determining neonatal central adrenal insufficiency.

Objectives: While it has been established within the first 4 months of life that there is no circadian rhythm, what is unclear is the usefulness of a random serum cortisol (rSC) level in determining neonatal central adrenal insufficiency (CAI). The objective of the study is to determine the utility of using rSC in infants less than 4 months old in the evaluation of CAI.

Design And Patients: Retrospective chart review of infants who underwent a low dose cosyntropin stimulation test ≤4 months of life with rSC taken as baseline cortisol before stimulation.

Macimorelin Acetate for the Diagnosis of Childhood-onset Growth Hormone Deficiency.

Growth hormone provocation testing forms the cornerstone of the diagnosis of childhood growth hormone deficiency in clinical practice. Despite the widespread use of these tests, various criticisms have been levelled against them, such as the labour-intensive nature of the tests, their potential for serious adverse effects and their questionable reproducibility. Macimorelin acetate, a ghrelin mimetic approved for the diagnosis of adult growth hormone deficiency, could serve an unmet need in the diagnosis of childhood-onset growth hormone deficiency based on its good tolerability and benign side effect profile.

The necessity of magnetic resonance imaging in the evaluation of pediatric growth hormone deficiency: Lessons from a large academic center.

Background: Current guidelines indiscriminately recommend magnetic resonance imaging (MRI) of the pituitary gland in pediatric growth hormone deficiency (GHD). The relationship between abnormal MRI, most importantly a tumor, and peak GH levels is not well known.

Methods: In this retrospective chart review, pituitary MRI results of children, ages of 3-16 years with GHD were collected and divided into 3 groups according to peak stimulated GH levels; ≤5, 5-7.

When They're Done Growing, Don't Forget They May Still Need Growth Hormone.

The effect of the growth hormone (GH) in promoting linear growth is well known; however, less recognized by practitioners especially pediatric, are its metabolic properties. This may be because the deleterious effects of improperly treated or untreated growth hormone deficiency (GHD) can present beyond the pediatric years. In addition, clinicians may lack familiarity with the potential issues that can arise due to inadequately treated GHD.

The utility of a random growth hormone level in determining neonatal growth hormone sufficiency.

Background: Random growth hormone (GH) levels have been used in the neonate to investigate congenital growth hormone deficiency (GHD). The cut-off value for use in this diagnosis is yet to be established.

Methods: This is a retrospective chart review of all random GH levels obtained in neonates ≤28 days of age.

Childhood growth hormone deficiency, a diagnosis in evolution: The intersection of growth hormone history and ethics.

In 1958 the first recorded case of a patient treated with human growth hormone for growth hormone deficiency was published. Since that time, the source and availability of human growth hormone have changed. With the increased availability of growth hormone, there has been an uptrend in the level below which childhood growth hormone deficiency is diagnosed based on provocative GH stimulation testing.

For Debate: The Two Paths of Growth Hormone (Excess and Deficiency): Both Roads Uniquely Lead to Diabetes Mellitus.

Glucose dysregulation (GD), a feature of growth hormone excess, in the setting of acromegaly is well known by clinicians. However, less well known is that GD may be a feature of growth hormone deficiency. The fact that either ends of the spectrum of growth hormone (excess or deficiency) can be associated with diabetes mellitus is unique and clinicians should especially recognize that impaired glucose tolerance and even diabetes mellitus may develop in individuals predisposed to having growth hormone deficiency.

Type 2 Diabetes Mellitus, a Sequel of Untreated Childhood Onset Growth Hormone Deficiency Developing in a 17-Year-Old Patient.

In a seminal report, a 17-year-old boy with panhypopituitarism had fatty liver (FL) amelioration with growth hormone (GH). By extension, since hepatic insulin resistance (IR) is key to FL and type 2 diabetes mellitus (T2DM), GH then may ameliorate the IR of T2DM. We present a 17-year-old nonobese female with untreated childhood onset growth hormone deficiency (CO-GHD) who developed type 2 diabetes mellitus (T2DM) and steatohepatitis with bridging fibrosis.

Growth Hormone Deficiency and Nonalcoholic Fatty Liver Disease with Insights from Humans and Animals: Pediatric Implications.

In addition to its growth promoting role, growth hormone (GH) has a significant effect on intermediary metabolism in the well state. Despite the latter fact, pediatric practitioners are usually focused on the growth promoting aspects of GH as opposed to those metabolic. In recent years various animal and human studies (in adults mainly) and clinical reports in children have repeatedly shown the association of GH deficiency (GHD) and fatty liver disease.

Myxedema Coma due to Hashimoto Thyroiditis: A Rare but Real Presentation of Failure to Thrive in Infancy.

Background: Hashimoto thyroiditis (HT) is uncommon in infancy, and myxedema coma (MC) is even less common. While prior reports have documented these entities separately, to our knowledge, MC in combination with HT has not been reported before in this age group.

Methods/results: A 10-month-old female presented with ptosis, lethargy, dysphagia, and failure to thrive (FTT).

In iodine-induced thyrotoxicosis, steroid therapy today could keep the surgical knife at bay.

Background: Amiodarone-induced thyrotoxicosis (AIT) type 2, characterized as a destructive thyroiditis, is well described in the medical literature; however, iodine-induced thyrotoxicosis (IIT) is not, though the latter has similar features and can be managed similarly.

Case Presentation: We present a 17-year-old female who presented with a history of an intermittent goiter with thyroid function tests (TFTs): thyroid-stimulating hormone (TSH)<0.015 (0.

Severe hypertriglyceridemia at new onset type 1 diabetes mellitus.

Background: Severe hypertriglyceridemia (HTG) as well as diabetic ketoacidosis (DKA) are complications of type 1 diabetes (T1DM). HTG is an exceedingly rare complication in the pediatric population and herein we report a case of HTG at new-onset T1DM in DKA and discuss management and potential complications.

Case Presentation: An 11-year-old previously well patient with a history of fatigue and weight loss presented with: glucose >600 mg/dL, venous blood gas: pH 7.

Hypercalcemia due to Milk-Alkali Syndrome and Fracture-Induced Immobilization in an Adolescent Boy with Hypoparathyroidism.

Background: Hypercalcemia of immobilization, while rare, may occur in adolescent boys after fracture. Although not fully understood, the mechanism appears to be related to bone turnover uncoupling, in part mediated by upregulation of RANKL. Animal studies suggest that parathyroidectomy suppresses RANKL-stimulated osteoclastogenesis in immobilized bone.

17p13.3 microduplication including CRK leads to overgrowth and elevated growth factors: A case report.

17p13.3 microduplications classified as class I duplications involving YWHAE but not PAFAH1B1 (formerly LIS1) and class II duplications which extend to involve PAFAH1B1, are associated with diverse phenotypes including intellectual disability and structural brain malformations. We report a girl with an approximately 1.

Myxedema Coma Secondary to Central Hypothyroidism: A Rare but Real Cause of Altered Mental Status in Pediatrics.

Background: Myxedema coma (MC), a medical emergency defined as severe hypothyroidism leading to altered mental status, is more common in older women with hypothyroidism.

Methods/results: A 7-year-old Caucasian male with chromosome 1q deletion presented with altered mental status preceded by milestone regression. His presenting labs results were: thyroid-stimulating hormone (TSH) 0.

Prenatal risk factors for childhood CKD.

Development of CKD may be programmed prenatally. We sought to determine the association of childhood CKD with prenatal risk factors, including birth weight, maternal diabetes mellitus (DM), and maternal overweight/obesity. We conducted a population-based, case-control study with 1994 patients with childhood CKD (<21 years of age at diagnosis) and 20,032 controls in Washington state.

Cushing's syndrome secondary to isolated micronodular adrenocortical disease (iMAD) associated with rapid onset weight gain and negative abdominal MRI findings in a 3 year old male.

Cushing's syndrome (CS) is uncommon in childhood. CS may be either dependent or independent of adrenocorticotrophic hormone (ACTH). ACTH independent micronodular adrenocortical (MAD) disease may present in the second to third decade of life or between ages 2-3 years.