Should we routinely assess hypothalamic-pituitary-adrenal axis in pediatric patients with Prader-Willi syndrome?

Background: It has been reported that central adrenal insufficiency (CAI) in pediatric patients (pts) with Prader-Willi syndrome (PWS) may be a potential cause of their sudden death. In addition, the risk of CAI may increase during treatment with recombinant human growth hormone (rhGH).

Objective: To prevent both over- and undertreatment with hydrocortisone, we evaluated the prevalence of CAI in a large multicenter cohort of pediatric pts with PWS analyzing adrenal response in the low-dose ACTH test (LDAT) and/or the glucagon stimulation test (GST) and reviewing the literature.

Long-term ultrasound follow-up of intrathyroidal ectopic thymus in children.

Objective: To present the sonographic follow-up of intrathyroidal ectopic thymus (IET) in children and adolescent patients.

Patients: Out of the 507 children referred to FNAB between 2006 and 2018, 30 (5.9%) pediatric patients (10 females), mean age 5.

Cushing disease in children and adolescents - assessment of the clinical course, diagnostic process, and effects of the treatment - experience from a single paediatric centre.

Introduction: Adrenocorticotropic hormone-dependent Cushing syndrome, known as Cushing disease (CD), is a rare disease in paediatric patients, the signs and symptoms of which differ from those seen in adult patients.

Aim Of The Study: The objective of the study was to present the diagnostics and treatment results of CD in children and adolescents.

Material And Methods: We included four consequent patients in the study, 7-15 years old, with CD confirmed by laboratory tests and finally by histology ex-aminations after surgery.

Cyclic Cushing's Disease in the Prepubertal Period-A Case Report and Review of Literature.

Cyclic Cushing's disease (CD) has been described in about 15% of adult patients with CD. In the pediatric population, diagnosis of CD is rare and cyclic presentations of the disease are not adequately understood or described. Moreover, prepubertal patients usually do not present with the typical signs and symptoms of CD, which can obscure or delay diagnosis.

Amiodarone-induced thyroid dysfunction in the developmental period: prenatally, in childhood, and adolescence - case reports and a review of the literature.

Introduction: Amiodarone is an important antiarrhythmic drug used in paediatric practice, mainly in children with complex congenital cardiac diseases and/or severe arrhythmias. One of the side effects of amiodarone therapy is thyroid dysfunction, which is observed in about 20% of patients. The thyroid dysfunction may present with various forms: from subclinical changes in hormone levels to amiodaroneinduced thyrotoxicosis (AIT) and amiodarone-induced hypothyroidism (AIH).

Novel insights in ultrasound evaluation of thyroid gland in children with papillary thyroid carcinoma.

Background: The coincidence of autoimmune thyroiditis (AIT) in patients with papillary thyroid carcinoma (PTC) is ranging between 10 and 58% in the general population.

Material And Methods: In the present study retrospective ultrasound, clinical and autoimmune assessment of 24 patients diagnosed with papillary thyroid carcinoma between 2000-2016 was performed.

Results: The coexistence of PTC and AIT was found in 50% of patients with PTC.

The first description of metyrapone use in severe Cushing Syndrome due to ectopic ACTH secretion in an infant with immature sacrococcygeal teratoma. Case Report.

Cushing syndrome due to ectopic secretion of ACTH in infants is rare. The treatment of choice is radical resection of the tumour in combination with pre-operative chemotherapy using steroidogenesis inhibitors if necessary. If radical surgery is not possible, palliative treatment of hypercortisolemia is recommended.

Cerebral salt wasting in a postoperative period.

Cerebral salt wasting syndrome (CSW-cerebral salt wasting) was first described in 1950 by Peters. This syndrome can occur in patients who have sustained damage to the central nervous system (e.g.

Atypical Clinical Presentation of ACTH-Dependent Cushing's Syndrome in a Patient Treated with Retinoic Acid.

Objective: The leading signs and symptoms of Cushing's syndrome (CS) in adolescents, which depend on the duration and the severity of hypercortisolemia, are: a decrease in growth velocity with an increase in body weight, redistribution of fat tissue (round face), and less commonly, acne due to hyperandrogenization. A widely used antiacne drug, retinoic acid, can change the clinical presentation of CS and delay the diagnosis.

Methods: We report an atypical presentation of adrenocorticotropic hormone (ACTH)-dependent CS in a patient treated with retinoic acid due to severe acne.

[Hypertension in obese children and adolescents].

Background: The frequency of arterial hypertension (AHT) in children is around 3.2-4.5% nowadays, however 3-4 decades ago it was below 1.

A decrease in fasting FGF19 levels is associated with the development of non-alcoholic fatty liver disease in obese adolescents.

Aim: Fibroblast growth factor 19 (FGF19) is a hormone released from the small intestine; recently, it has emerged as an endocrine regulator of glucose and lipid metabolism. The aim of this study was to investigate the role of FGF19 in the development of nonalcoholic fatty liver disease (NAFLD).

Patients: This study included 23 (17 boys) obese adolescents (mean age of 14.

[Disorders of lipid metabolism in adolescents with simple obesity].

Background: Obesity affects approximately 45 millions of children worldwide. Some of them present with secondary dyslipidemia that leads to premature atherosclerosis.

Aim Of The Study: 1) Assessment of the frequency and type of dyslipidemia in obese adolescents.

[Neurofibromatosis type I--the pediatric endocrinologist's point of view].

Aim Of The Study: A retrospective analysis of endocrine disorders in patients with neurofibromatosis type I consulted in the Children's University Hospital of Krakow in the period 2007-2010.

Material And Methods: The analysis included 60 patients (33 girls, and 27 boys) aged 1.2-32 years, mean 11.

[Long-term endocrine complications after brain tumor treatment--own experience].

Background: Long-term endocrine complications affect approximately 40% of childhood cancer survivors.

The Aim: The retrospective analysis of parameters of the endocrine system function up to 10 years after head radiotherapy (RT) and chemotherapy (CT) due to malignant solid tumor of the central nervous system.

Material And Methods: The analysis included 30 patients (15 girls; 15 boys) followed in Endocrine Outpatient Department, University Children's Hospital of Krakow for 1-10 years (mean 5.

[Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL].

Introduction: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children.

Material And Methods: The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children.

[Thyroid peroxidase antibodies and thyroid diseases in children and adolescents with type 1 diabetes mellitus from Southeast Poland].

The authors evaluated the prevalence of TPO Ab and thyroid disorders in 219 children and adolescents (119/54% girls) with type 1 diabetes from southeast Poland aged 3.2-22.3 years (mean age-13.

Suprasellar arachnoidal cyst as a cause of precocious puberty--report of three patients and literature overview.

The authors present three boys--3 years old, 5.8 years old and 10.4 years old--who were diagnosed with isosexual precocious puberty (IPP) triggered by a rare developmental disorder of suprasellar arachnoid cyst (SAC) accompanied by corpus callosum and fornix dysgenesis as well as anterior commissura magna agenesis (patient 1) and empty sella (patients 2, 3).

[Thyroid peroxidase antibodies and thyroid diseases in children and adolescents with newly diagnosed type I diabetes].

The objective of the authors was to evaluate the prevalence of TPO Ab and thyroid diseases in children with newly diagnosed type 1 diabetes. The examination included 153 patients (85/55.6% girls) from southeast Poland aged 11 months to do 17.

[Sporadic generalized insensitivity of peripheral tissue receptors to thyroid hormone in a 15 year old boy].

Sporadic generalized form of insensitivity to thyroid hormones of peripheral tissue receptor was proposed as diagnosis in a clinically euthyroid boy 15.5 years old with goiter, having elevated levels of blood serum triiodothyronine (T3) and thyroxine (T4) associated with absence of TSH suppression. Other possible causes of biochemical hyperthyreosis were excluded by differential diagnosis.