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Pediatrics endocrinology

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https://www.readbyqxmd.com/read/29148123/donohue-syndrome-a-review-of-literature-case-series-and-anesthetic-considerations
#1
REVIEW
Alana Kirkwood, Grant Stuart, Louise Harding
BACKGROUND: Donohue syndrome is a rare autosomal recessive disorder of insulin resistance, causing a functional defect in insulin receptor function, and affecting the ability of the insulin to bind the receptor. Features include severe hyperinsulinism and fasting hypoglycemia, along with severe failure to thrive despite feeding. An accelerated fasting state results in muscle wasting, decreased subcutaneous fat, and an excess of thick skin. A reduced thoracic diameter is accentuated by increased abdominal distension, which impacts on respiratory reserve...
November 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29127766/etiologies-of-short-stature-in-a-pediatric-endocrine-clinic-in-southern-thailand
#2
Tansit Saengkaew, Edward McNeil, Somchit Jaruratanasirikul
BACKGROUND: Short stature is one of the common disorders referred for investigation of an endocrine disorder. The etiologies of short stature vary and are commonly grouped into pathological and non-pathological disorders. The objective of the study was to determine the etiologies and describe the characteristics of short stature patients who attended the Pediatric Endocrinology Clinic and to compare factors between normal variant short stature (NVSS) and growth hormone deficiency (GHD)...
November 11, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29125446/suprasellar-and-recurrent-pediatric-craniopharyngiomas-expanding-indications-for-the-extended-endoscopic-transsphenoidal-approach
#3
Andrew F Alalade, Elizabeth Ogando-Rivas, Jerome Boatey, Mark M Souweidane, Vijay K Anand, Jeffrey P Greenfield, Theodore H Schwartz
OBJECTIVE The expanded endonasal endoscopic transsphenoidal approach has become increasingly used for craniopharyngioma surgery in the pediatric population, but questions still persist regarding its utility in younger children, in recurrent and irradiated tumors, and in masses primarily located in the suprasellar region. The narrow corridor, incomplete pneumatization, and fear of hypothalamic injury have traditionally relegated this approach to application in older children with mostly cystic craniopharyngiomas centered in the sella...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29121256/malignant-testicular-germ-cell-tumors-in-postpubertal-individuals-with-androgen-insensitivity-prevalence-pathology-and-relevance-of-single-nucleotide-polymorphism-based-susceptibility-profiling
#4
M Cools, K P Wolffenbuttel, R Hersmus, B B Mendonca, J Kaprová, S L S Drop, H Stoop, A J M Gillis, J W Oosterhuis, E M F Costa, S Domenice, M Y Nishi, L Wunsch, C A Quigley, G T'Sjoen, L H J Looijenga
STUDY QUESTION: What is the prevalence of malignant testicular germ cell tumors (TGCT) and its precursors, (pre-) germ cell neoplasia in situ (GCNIS), in late teenagers and adults who have androgen insensitivity syndrome (AIS) and the impact of an individual's genetic susceptibility to development of TGCT? SUMMARY ANSWER: No GCNIS or TGCT was diagnosed, but pre-GCNIS was identified in 14 and 10% of complete and partial AIS patients, respectively, and was associated with a higher genetic susceptibility score (GSS), with special attention for KITLG (rs995030) and ATFZIP (rs2900333)...
November 7, 2017: Human Reproduction
https://www.readbyqxmd.com/read/29083441/measuring-active-and-healthy-ageing-applying-a-generic-interdisciplinary-assessment-model-incorporating-icf
#5
P Stute, N Bitterlich, J Bousquet, F Meissner, M von Wolff, D Poethig
OBJECTIVES: In this study we compared the chronological and bio-functional age between two German speaking cohorts 30 years apart applying a comprehensive and generic Active and Healthy Aging (AHA) assessment model incorporating ICF. METHODS: Single-centre, cross-sectional, observational, non-interventional, non-randomized trial at an University based women's hospital, division of Gynecological Endocrinology and Reproductive Medicine. All participants followed a standardized, holistic battery of biopsychosocial assessments consisting of bio-functional status (BFS), bio-functional age (BFA) and additional validated psychometric questionnaires...
2017: Journal of Nutrition, Health & Aging
https://www.readbyqxmd.com/read/29077292/-scientific-publication-output-of-spanish-emergency-physicians-from-2005-to-2014-a-comparative-study
#6
Inés María Fernández-Guerrero, Francisco Javier Martín-Sánchez, Guillermo Burillo-Putze, Òscar Miró Rafat Sellarés
OBJECTIVES: To analyze the research output of Spanish emergency physicians between 2005 and 2014 and to compare it to their output in the previous 10-year period (1995-2004) as well as to that of emergency physicians in other countries and Spanish physicians in other specialties. MATERIAL AND METHODS: Original articles indexed in the Science Citation Index Expanded of the Web of Science were included. Documents from Spanish emergency physicians were identified by combining the word Spain and any other search term identifying an emergency service or unit in Spain...
October 2017: Emergencias
https://www.readbyqxmd.com/read/29062584/adrenal-insufficiency-in-pediatric-eosinophilic-esophagitis-patients-treated-with-swallowed-topical-steroids
#7
Stephanie Hsu, Colleen Wood, Zhaoxing Pan, Haseeb Rahat, Philip Zeitler, David Fleischer, Calies Menard-Katcher, Glenn T Furuta, Dan Atkins
Swallowed topical steroids (STS) are the only effective pharmacological therapy for eosinophilic esophagitis (EoE). Thus far, studies of small populations of EoE patients have reported conflicting results in relation to adrenal insufficiency (AI). We sought to measure AI in a clinical setting in children taking STS for EoE. We performed a quality improvement study of pediatric EoE patients seen in a multidisciplinary clinic, who were treated with STS for at least 3 months. Two hundred twenty-five patients completed questionnaires to assess for signs of AI...
September 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29055783/prenatal-organochlorine-pesticide-exposure-and-the-disruption-of-steroids-and-reproductive-hormones-in-cord-blood-the-hokkaido-study
#8
Atsuko Araki, Chihiro Miyashita, Takahiko Mitsui, Houman Goudarzi, Futoshi Mizutani, Youichi Chisaki, Sachiko Itoh, Seiko Sasaki, Kazutoshi Cho, Kimihiko Moriya, Nobuo Shinohara, Katsuya Nonomura, Reiko Kishi
Certain organochlorine pesticides (OCPs) are designated as persistent organic pollutants and are regulated in many countries. The effects of OCPs on pediatric endocrinology are a concern; however, only limited data exist from human studies on maternal OCP exposure and its effects on infants' hormone levels. This study was conducted as part of the Hokkaido Study Sapporo Cohort, a prospective birth cohort study in Japan. Participants included 514 women who enrolled at 23-35weeks of gestation between 2002 and 2005; maternal blood samples were collected in late pregnancy, and 29 OCPs were measured...
January 2018: Environment International
https://www.readbyqxmd.com/read/29050119/-feasibility-of-domestic-18-f-dopa-pet-ct-scanning-in-the-differential-diagnosis-of-pancreatic-lesions-in-children-with-hyperinsulinemic-hypoglycemia
#9
M Y Zhang, J J Ge, Z Pei, K R Dong, L Chen, X Wang, Z W Zhang, X J Li, L Xi, R Q Cheng, G P Lu, Y H Guan, F H Luo
Objective: To study the feasibility of (18)F-fluoro-L-dihydroxyphenylalanine positron emission tomography/Computed tomography ((18)F-DOPA PET/CT) scanning in the localization and differential diagnosing of focal versus diffuse form of pancreas lesions in patients with hyperinsulinemic hypoglycemia (HH). Method: Twenty-four patients were diagnosed with HH between January, 2016 and February, 2017 in the Department of Pediatric Endocrinology and Inherited Metabolic Diseases, Children's Hospital of Fudan University using an integrated clinical and biochemical diagnostic protocol, domestic (18)F-DOPA PET/CT imaging technique were applied after MRI and ultrasound failed to detect pancreas lesions...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29039345/validation-of-transition-readiness-assessment-questionaire-in-turkish-adolescents-with-diabetes
#10
Evrim Kızıler, Dilek Yıldız, Berna Eren Fidancı
BACKGROUND: Today, more than 90% of adolescents with chronic conditions are surviving into adulthood and move pediatric to adult care with their chronic illness. It is important to gain autonomy and ensure readiness for adolescents/ young adults to use the adult health care system prior to the transfer of care. However, the lack of the transition readiness assessment tool which is validated, patient-centered and appropriate to developmental differences in pediatric care is seen a major obstacle for transition of care from pediatric to adult services...
October 17, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/29026269/clinical-characteristics-of-septo-optic-dysplasia-accompanied-by-congenital-central-hypothyroidism-in-japan
#11
Keisuke Nagasaki, Takuo Kubota, Hironori Kobayashi, Hirotake Sawada, Chikahiko Numakura, Shohei Harada, Kei Takasawa, Kanshi Minamitani, Tomohiro Ishii, Satoshi Okada, Hotaka Kamasaki, Shigetaka Sugihara, Masanori Adachi, Toshihiro Tajima
Septo-optic dysplasia (SOD) is a congenital anomaly in which agenesis of the septum pellucidum and optic nerve hypoplasia are accompanied by hypopituitarism. Typically, the symptoms develop in 3 organs, the brain, eyes, and pituitary, and approximately one third of the patients present with all of the three cardinal features. The diagnostic criteria for SOD were established in Japan in 2015. The purpose of this study is to review clinical features regarding SOD patients with hypopituitarism in Japan. In this study, 21 patients with SOD were identified by a questionnaire survey for congenital central hypothyroidism...
2017: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/29025202/clinical-manifestations-of-rathke-s-cleft-cysts-and-their-natural-progression-during-2-years-in-children-and-adolescents
#12
Jo Eun Jung, Juhyun Jin, Mo Kyung Jung, Ahreum Kwon, Hyun Wook Chae, Duk Hee Kim, Ho-Seong Kim
PURPOSE: Rathke's cleft cyst (RCC) is an asymptomatic benign lesion. With increased interest in pediatric endocrinology, the prevalence of RCCs in children is also increasing. However, the clinical relevance and proper management of RCC is not well defined in children. Therefore, we investigated the clinical manifestations and radiologic features of RCC in children and adolescents, as well as the natural progression of RCC. METHODS: We retrospectively reviewed the medical records of 91 children and adolescents with RCC diagnosed with magnetic resonance imaging (MRI) in Severance Children's Hospital from January 2006 to December 2015...
September 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29023255/adolescent-perceptions-of-peer-responses-to-diabetes-self-management-a-qualitative-study
#13
Pei-Yun Yang, Meei-Fang Lou, Angela Shin-Yu Lien, Bih-Shya Gau
BACKGROUND: Type 1 diabetes (T1D) is more prevalent in adolescents than in adults, and the self-management of insulin-dependent diabetes is complex. T1D requires injections of insulin, self-management of blood testing, regular physical activity, and diet monitoring, which are challenging for growing and developing adolescents. Adolescents are often more concerned with how they are perceived by their peers than how they perceive themselves. Positive peer responses influence the self-care management of adolescents with T1D in school settings...
October 12, 2017: Journal of Nursing Research: JNR
https://www.readbyqxmd.com/read/28986152/vocal-evaluation-of-children-with-congenital-hypothyroidism
#14
Ana Paula Dassie-Leite, Mara Behlau, Suzana Nesi-França, Monica Nunes Lima, Luiz de Lacerda
OBJECTIVE: To evaluate the vocal characteristics of a group of children with congenital hypothyroidism (CH) and the association of these characteristics with the children's clinical, laboratory, and therapeutic profiles. MATHERIAL AND METHODS: Observational, analytical, cross-sectional study including 200 prepubertal children, of whom 100 had CH (study group [SG]) and 100 had no CH (control group [CG]). The following parameters were evaluated: 1) history (identification, complaints, and interfering variables), 2) auditory-perceptual and acoustic evaluation (samples analyzed by a group of specialists, and objectively by a computer program), 3) self-assessment scores in the Pediatric Voice-Related Quality-of-Life (PVRQoL) survey, 4) laryngological evaluation (presence or absence of laryngeal lesions and data regarding glottal closure), and 5) medical records (CH etiology, age at treatment initiation, disease severity at diagnosis, treatment quality, and thyroid function tests on the day of the examination)...
October 3, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28982637/pediatric-chronic-patients-at-outpatient-clinics-a-study-in-a-latin-american-university-hospital
#15
Renata A Alveno, Caroline V Miranda, Caroline G Passone, Aurora R Waetge, Elza S Hojo, Sylvia C L Farhat, Vicente Odone-Filho, Uenis Tannuri, Werther B Carvalho, Magda Carneiro-Sampaio, Clovis A Silva
OBJECTIVE: To describe the characteristics of children and adolescentes with chronic diseases of outpatient clinics at a tertiary university hospital. METHODS: A cross-sectional study was performed with 16,237 patients with chronic diseases followed-up in one year. The data were collected through the electronic system, according to the number of physician appointments in 23 pediatric specialties. Patients were divided in two groups: children (0-9 years) and adolescents (10-19 years)...
October 2, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28968603/10th-individual-abstracts-for-international-meeting-of-pediatric-endocrinology-free-communication-and-poster-sessions-abstracts
#16
(no author information available yet)
No abstract text is available yet for this article.
2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28940497/scrotal-hair-in-infancy-a-case-series
#17
Zeynep Uzan Tatlı, Ulku Gul, Nihal Hatipoglu, Selim Kurtoglu
Scrotal hair is a clinical condition that occurs rarely in infancy. Its prevalence is not known. We present a retrospective analysis of six patients referred to our pediatric endocrinology clinic. Except for scrotal hair development, all physical examinations were normal. Underlying pathologic hyperandrogenism was excluded in each case. Clinical regression was observed in all four infants with documented follow-up visits. Scrotal hair in infancy is not well known to most pediatricians and dermatologists and can cause parental anxiety...
September 21, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28940011/guidelines-for-the-diagnosis-and-management-of-critical-illness-related-corticosteroid-insufficiency-circi-in-critically-ill-patients-part-i-society-of-critical-care-medicine-sccm-and-european-society-of-intensive-care-medicine-esicm-2017
#18
Djillali Annane, Stephen M Pastores, Bram Rochwerg, Wiebke Arlt, Robert A Balk, Albertus Beishuizen, Josef Briegel, Joseph Carcillo, Mirjam Christ-Crain, Mark S Cooper, Paul E Marik, Gianfranco Umberto Meduri, Keith M Olsen, Sophia Rodgers, James A Russell, Greet Van den Berghe
OBJECTIVE: To update the 2008 consensus statements for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in adult and pediatric patients. PARTICIPANTS: A multispecialty task force of 16 international experts in Critical Care Medicine, endocrinology, and guideline methods, all of them members of the Society of Critical Care Medicine and/or the European Society of Intensive Care Medicine. DESIGN/METHODS: The recommendations were based on the summarized evidence from the 2008 document in addition to more recent findings from an updated systematic review of relevant studies from 2008 to 2017 and were formulated using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology...
September 21, 2017: Intensive Care Medicine
https://www.readbyqxmd.com/read/28938253/guidelines-for-the-diagnosis-and-management-of-critical-illness-related-corticosteroid-insufficiency-circi-in-critically-ill-patients-part-i-society-of-critical-care-medicine-sccm-and-european-society-of-intensive-care-medicine-esicm-2017
#19
Djillali Annane, Stephen M Pastores, Bram Rochwerg, Wiebke Arlt, Robert A Balk, Albertus Beishuizen, Josef Briegel, Joseph Carcillo, Mirjam Christ-Crain, Mark S Cooper, Paul E Marik, Gianfranco Umberto Meduri, Keith M Olsen, Sophia C Rodgers, James A Russell, Greet Van den Berghe
OBJECTIVE: To update the 2008 consensus statements for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in adult and pediatric patients. PARTICIPANTS: A multispecialty task force of 16 international experts in critical care medicine, endocrinology, and guideline methods, all of them members of the Society of Critical Care Medicine and/or the European Society of Intensive Care Medicine. DESIGN/METHODS: The recommendations were based on the summarized evidence from the 2008 document in addition to more recent findings from an updated systematic review of relevant studies from 2008 to 2017 and were formulated using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology...
December 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28917084/evaluation-of-neurodevelopment-of-children-with-congenital-hypothyroidism-by-the-denver-developmental-screening-test
#20
Ayşe Derya Buluş, Esra Tiftik
BACKGROUND: Thyroid hormones are essential for growth and brain development in childhood. Although congenital hypothyroidism (CH) is the most common reason for mental retardation, normal neurological development can be achieved through early and effective treatment. The aim of the present study was to evaluate the neurological development of CH patients aged 24-56 months. METHODS: The study included a total of 116 healthy control subjects and 112 patients aged 24-56 months who were diagnosed with CH during the neonatal period and were being followed up at the Pediatric Endocrinology Department, Keçiören Training and Research Hospital, between 2012 and 2015...
October 26, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
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