keyword
https://read.qxmd.com/read/38432069/igf-2-mediated-hypoglycemia-a-case-series-and-review-of-the-medical-therapies-for-refractory-hypoglycemia
#21
JOURNAL ARTICLE
Albert Vu, Constance Chik, Sarah Kwong
SUMMARY: Non-islet cell tumour hypoglycemia (NICTH), typically mediated by insulin-like growth factor 2 (IGF-2), is a rare but highly morbid paraneoplastic syndrome associated with tumours of mesenchymal or epithelial origin. Outside of dextrose administration and dietary modification which provide transient relief of hypoglycemia, resection of the underlying tumour is the only known cure for NICTH. Available medical therapies to manage hypoglycemia include glucocorticoids, recombinant growth hormone, and pasireotide...
January 1, 2024: Endocrinology, Diabetes & Metabolism Case Reports
https://read.qxmd.com/read/38427811/beckwith-wiedemann-syndrome-mimicking-the-classical-form-of-congenital-adrenal-hyperplasia-in-newborn-screening
#22
Jéssica Mallmann Erbes Schaefer Martins, Barbara Leitao Braga, Klevia Nunes Feitosa Sampaio, Tamires de Souza Garcia, Juliana Van de Sande Lee, Edson Cechinel, Genoir Simoni, Marilza Leal Nascimento, Paulo Cesar Alves da Silva, Maria C V Fragoso, Tania A A S Bachega, Mirian Y Nishi, Berenice B Mendonca
Beckwith-Wiedemann syndrome (BWS) is a common genetic congenital disease characterized by somatic overgrowth and its broad clinical spectrum includes pre- and post-natal macrosomia, macroglossia, visceromegaly, increased risk of neonatal hypoglycemia, and development of embryonic tumors. BWS occurs due to genetic/epigenetic changes involving growth-regulating genes, located on region 11p15, with an important genotype-phenotype correlation. Congenital adrenal hyperplasia (CAH) comprises a spectrum of autosomal recessive diseases presenting a variety of clinical manifestations due to a deficiency in one of the enzymes involved in cortisol secretion...
February 29, 2024: Archives of Endocrinology and Metabolism
https://read.qxmd.com/read/38423900/current-status-and-future-perspectives-of-fgf21-analogues-in-clinical-trials
#23
REVIEW
Zara Siu Wa Chui, Qing Shen, Aimin Xu
Recent advances in fibroblast growth factor 21 (FGF21) biology and pharmacology have led to the development of several long-acting FGF21 analogues and antibody-based mimetics now in various phases of clinical trials for the treatment of obesity-related metabolic comorbidities. The efficacy of these FGF21 analogues/mimetics on glycaemic control and weight loss is rather mild and inconsistent; nevertheless, several promising therapeutic benefits have been reproducibly observed in most clinical studies, including amelioration of dyslipidaemia (particularly hypertriglyceridaemia) and hepatic steatosis, reduction of biomarkers of liver fibrosis and injury, and resolution of metabolic dysfunction-associated steatohepatitis (MASH)...
February 28, 2024: Trends in Endocrinology and Metabolism: TEM
https://read.qxmd.com/read/38416754/dietary-regimens-appear-to-possess-significant-effects-on-the-development-of-combined-antiretroviral-therapy-cart-associated-metabolic-syndrome
#24
JOURNAL ARTICLE
Boniface M Chege, Peter W Mwangi, Charles G Githinji, Frederick Bukachi
INTRODUCTION: This study investigated the interactions between a low protein high calorie (LPHC) diet and an integrase inhibitor-containing antiretroviral drug regimen (INI-CR)in light of evidence suggesting that the initiation of cART in patients with poor nutritional status is a predictor of mortality independent of immune status. METHODS: Freshly weaned Sprague Dawley rats (120) were randomized into the standard, LPHC and normal protein high calorie (NPHC) diet groups (n = 40/group) initially for 15 weeks...
2024: PloS One
https://read.qxmd.com/read/38413286/the-clinical-and-biochemical-spectrum-of-ectopic-acromegaly
#25
REVIEW
Patricia Fainstein-Day, Tamara Estefanía Ullmann, Mercedes Corina Liliana Dalurzo, Gustavo Emilio Sevlever, David Eduardo Smith
Ectopic acromegaly is a rare condition caused by extrapituitary central or peripheral neuroendocrine tumours (NET) that hypersecrete GH or, more commonly, GHRH. It affects less than 1% of acromegaly patients and a misdiagnosis of classic acromegaly can lead to an inappropriate pituitary surgery. Four types of ectopic acromegaly have been described: 1) Central ectopic GH-secretion: Careful cross-sectional imaging is required to exclude ectopic pituitary adenomas. 2) Peripheral GH secretion: Extremely rare. 3) Central ectopic GHRH secretion: Sellar gangliocytomas immunohistochemically positive for GHRH are found after pituitary surgery...
February 14, 2024: Best Practice & Research. Clinical Endocrinology & Metabolism
https://read.qxmd.com/read/38411716/fgd1-related-aarskog-scott-syndrome-identification-of-four-novel-variations-and-a-literature-review-of-clinical-and-molecular-aspects
#26
JOURNAL ARTICLE
Sujuan Li, Anran Tian, Yu Wen, Wei Gu, Wei Li, Xiaohong Qiao, Cai Zhang, Xiaoping Luo
Patients with Aarskog-Scott syndrome (AAS) have short stature, facial anomalies, skeletal deformities, and genitourinary malformations. FYVE, RhoGEF, and PH domain-containing 1 (FGD1) is the only known causative gene of AAS. However, the diagnosis of AAS remains difficult, and specific treatments are still absent. Patients suspected with AAS were recruited, and clinical information was collected. Genetic testing and functional analysis were carried out for the diagnosis. By literature review, we summarized the clinical and genetic characteristics of FGD1-related AAS and analyzed the genotype-phenotype correlation...
February 27, 2024: European Journal of Pediatrics
https://read.qxmd.com/read/38404048/positive-effects-of-rutin-on-female-reproduction
#27
REVIEW
Alexander V Sirotkin
This article reviews the source and properties of rutin (vitamin P), its general physiological and medicinal effects and their mechanisms, but the main subject of it is the currently available knowledge concerning the character and mechanisms of action of rutin on female reproductive processes. The available data demonstrate the stimulatory action of rutin on female reproductive processes: it can promote ovarian follicles development and ovulation, ovarian cyclicity, and viability of ovarian cells. On the other hand, it can suppress ovarian cancer cell and tumour development by inhibition of cell proliferation and growth and activation of their apoptosis and death...
February 2024: Reproduction in Domestic Animals
https://read.qxmd.com/read/38403003/the-rhizomes-of-atractylodes-macrocephala-koidz-improve-gastrointestinal-health-and-pregnancy-outcomes-in-pregnant-mice-via-modulating-intestinal-barrier-and-water-fluid-metabolism
#28
JOURNAL ARTICLE
Wang Chenxing, Su Jie, Tian Yajuan, Li Ting, Zhong Yuying, Chen Suhong, Lv Guiyuan
ETHNOPHARMACOLOGICAL RELEVANCE: Baizhu (BZ) is the dried rhizome of Atractylodes macrocephala Koidz (Compositae), which invigorates the spleen, improves vital energy, stabilizes the fetus, and is widely used for treating spleen deficiency syndrome. However, the impact of BZ on gastrointestinal function during pregnancy remains unexplored. AIM OF THE STUDY: This study elucidated the ameliorative effects of BZ on gastrointestinal health and pregnancy outcomes in pregnant mice with spleen deficiency diarrhea (SDD)...
February 24, 2024: Journal of Ethnopharmacology
https://read.qxmd.com/read/38397994/hypoglycemia-unawareness-a-review-on-pathophysiology-and-clinical-implications
#29
REVIEW
Laura Hölzen, Bernd Schultes, Sebastian M Meyhöfer, Svenja Meyhöfer
Hypoglycemia is a particular problem in people with diabetes while it can also occur in other clinical circumstances. Hypoglycemia unawareness describes a condition in which autonomic and neuroglycopenic symptoms of hypoglycemia decrease and hence are hardly perceivable. A failure to recognize hypoglycemia in time can lead to unconsciousness, seizure, and even death. The risk factors include intensive glycemic control, prior episodes of severe hypoglycemia, long duration of diabetes, alcohol consumption, exercise, renal failure, and sepsis...
February 8, 2024: Biomedicines
https://read.qxmd.com/read/38397072/changes-in-cells-associated-with-insulin-resistance
#30
REVIEW
Leszek Szablewski
Insulin is a polypeptide hormone synthesized and secreted by pancreatic β-cells. It plays an important role as a metabolic hormone. Insulin influences the metabolism of glucose, regulating plasma glucose levels and stimulating glucose storage in organs such as the liver, muscles and adipose tissue. It is involved in fat metabolism, increasing the storage of triglycerides and decreasing lipolysis. Ketone body metabolism also depends on insulin action, as insulin reduces ketone body concentrations and influences protein metabolism...
February 18, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38392036/the-growing-understanding-of-the-pituitary-implication-in-the-pathogenesis-of-long-covid-19-syndrome-a-narrative-review
#31
REVIEW
Ach Taieb, Ben Haj Slama Nassim, Gorchane Asma, Methnani Jabeur, Saad Ghada, Ben Abdelkrim Asma
Long COVID-19, also known as post-acute sequelae of SARS-CoV-2 infection, is a condition where individuals who have recovered from the acute phase of COVID-19 continue to experience a range of symptoms for weeks or even months afterward. While it was initially thought to primarily affect the respiratory system, it has become clear that Long COVID-19 can involve various organs and systems, including the endocrine system, which includes the pituitary gland. In the context of Long COVID-19, there is a growing understanding of the potential implications for the pituitary gland...
February 14, 2024: Advances in Respiratory Medicine
https://read.qxmd.com/read/38390278/a-case-report-of-a-child-with-pulmonary-hypertension-associated-with-sars-cov-2-infection
#32
Kentaro Okunushi, Hironobu Kobayashi, Yuri Yoh, Masaya Kunimatsu, Tadashi Shiohama, Tomozumi Takatani, Hiromichi Hamada
We encountered a pediatric case of pulmonary hypertension triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. A 14-year-old girl was brought to the emergency department of our hospital with fever, respiratory distress, and impaired consciousness. She tested positive for SARS-CoV-2 upon a polymerase chain reaction examination and had prolonged hypoxemia without pneumonia. An echocardiography revealed elevated right ventricular pressure. She was diagnosed with pilocytic astrocytoma at the age of 10 years and underwent a resection of a pituitary tumor...
2024: Frontiers in Pediatrics
https://read.qxmd.com/read/38380275/diabetes-mellitus-and-thyroid-cancers-risky-correlation-underlying-mechanisms-and-clinical-prevention
#33
REVIEW
Rongqian Wu, Junping Zhang, Guilin Zou, Shanshan Li, Jinying Wang, Xiaoxinlei Li, Jixiong Xu
The incidences of thyroid cancer and diabetes are rapidly increasing worldwide. The relationship between thyroid cancer and diabetes is a popular topic in medicine. Increasing evidence has shown that diabetes increases the risk of thyroid cancer to a certain extent. This mechanism may be related to genetic factors, abnormal thyroid-stimulating hormone secretion, oxidative stress injury, hyperinsulinemia, elevated insulin-like growth factor-1 levels, abnormal secretion of adipocytokines, and increased secretion of inflammatory factors and chemokines...
2024: Diabetes, Metabolic Syndrome and Obesity
https://read.qxmd.com/read/38379862/serum-afamin-and-its-implications-in-adult-growth-hormone-deficiency-a-prospective-gh-withdrawal-study
#34
JOURNAL ARTICLE
Balázs Ratku, Hajnalka Lőrincz, Sára Csiha, Veronika Sebestyén, Eszter Berta, Miklós Bodor, Endre V Nagy, Zoltán Szabó, Mariann Harangi, Sándor Somodi
INTRODUCTION: Adult growth hormone deficiency (AGHD) is associated with a high prevalence of metabolic syndrome (MS), which contributes to the unfavorable cardiovascular risk profile in these patients. Insulin like growth factor-1 (IGF-1) is a widely used biomarker, however it does not always reflect the cardiometabolic risk and has a poor relationship with clinical efficacy endpoints. Consequently, there is an unmet need for biomarkers to monitor responses to GH-replacement. Afamin is a hormone-like glycoprotein, expressed in the liver...
2024: Frontiers in Endocrinology
https://read.qxmd.com/read/38379171/the-effect-of-metformin-treatment-on-disease-control-in-patients-with-acromegaly
#35
JOURNAL ARTICLE
Humeyra Rekali Sahin, Serdar Sahin, Betul Sarac, Cem Sulu, Pinar Kadioglu, Hande Mefkure Ozkaya
BACKGROUND: The increase in portal insulin levels has been shown to upregulate growth hormone receptor expression in the liver, leading to increased insulin-like growth hormone- 1 levels. Metformin inhibits hepatic gluconeogenesis and reduces fasting insulin. OBJECTIVE: We evaluated the effect of metformin treatment in patients with acromegaly on growth hormone, insulin-like growth hormone-1, and pituitary adenoma size. METHODS: Patients who were followed up with the diagnosis of acromegaly in Istanbul University- Cerrahpaşa, Cerrahpaşa Medical Faculty were evaluated...
2024: Endocrine, Metabolic & Immune Disorders Drug Targets
https://read.qxmd.com/read/38365697/clinical-genetic-profile-and-therapy-evaluation-of-11-chinese-pediatric-patients-with-fanconi-bickel-syndrome
#36
JOURNAL ARTICLE
Taozi Du, Yu Xia, Chengkai Sun, Zhuwen Gong, Lili Liang, Zizhen Gong, Ruifang Wang, Deyun Lu, Kaichuang Zhang, Yi Yang, Yuning Sun, Manqing Sun, Yu Sun, Bing Xiao, Wenjuan Qiu
BACKGROUND: Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder characterized by impaired glucose and galactose utilization as well as proximal renal tubular dysfunction. METHODS: Clinical, biochemical, genetic, treatment, and follow-up data for 11 pediatric patients with FBS were retrospectively analysed. RESULTS: Hepatomegaly (10/11), short stature (10/11) and hypophosphataemic rickets (7/11) were the most common initial symptoms...
February 16, 2024: Orphanet Journal of Rare Diseases
https://read.qxmd.com/read/38355440/orthopedic-manifestations-in-children-with-prader-willi-syndrome
#37
JOURNAL ARTICLE
Miao Miao, Guo-Qiang Zhao, Qiong Zhou, Yun-Qi Chao, Chao-Chun Zou
BACKGROUND: Prader-Willi syndrome (PWS) is a rare genetic disease often associated with bone problems, mainly scoliosis and hip dysplasia (HD). This study aimed to analyze the clinical characteristics of orthopedic deformities in patients with PWS. METHODS: A retrospective study was conducted on 175 patients up to March 2023. The Cobb angle(CA) of the spine, the alpha angle of the hip joint, and the acetabular index (AI) were measured. This study aimed to evaluate the relationship between demographic parameters and bone deformities...
February 14, 2024: BMC Pediatrics
https://read.qxmd.com/read/38332670/growth-during-pubertal-induction-in-girls-with-turner-syndrome-a-retrospective-cohort-analysis
#38
JOURNAL ARTICLE
Joshua Nicholls, Mark Harris, Ian Hughes, Tony Huynh, Sarah K McMahon
CONTEXT: Patients with Turner Syndrome often present with short stature and ovarian insufficiency. The optimal method of pubertal induction to maximize adult height (AH) is unknown. OBJECTIVE: To identify variables related to pubertal induction that are associated with growth and AH. DESIGN & SETTING: Retrospective cohort analysis of patients attending a specialized Turner Syndrome clinic at a quaternary children's hospital. PATIENTS: Patients with Turner Syndrome (n=107) who attended the clinic between 2015 and 2021...
February 9, 2024: Journal of Clinical Endocrinology and Metabolism
https://read.qxmd.com/read/38326058/-effect-of-growth-hormone-supplementation-on-liver-and-lung-function-in-patients-with-hypopituitarism
#39
JOURNAL ARTICLE
J F Mao, H L Xu, Z X Liu, X Wang, B Q Yu, Y Y Zhu, W Ji, J Y Zhang, M Nie, X Y Wu
To analyze the clinical features of patients with anterior hypopituitarism (HP) complicated with cirrhosis, and to explore the effects of growth hormone supplementation on liver and lung function. A total of 11 patients with HP complicated with cirrhosis admitted to Peking Union Medical College Hospital from January 2016 to December 2022 were included in the study, including 8 males and 3 females, aged [ M ( Q 1 , Q 3 )]31 (20, 37) years. There were 6 patients with pituitary stalk interruption syndrome, 4 patients after craniopharyngioma resection, and 1 patient after germinal cell tumor chemoradiotherapy...
February 6, 2024: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://read.qxmd.com/read/38321911/mutational-analysis-of-consanguineous-families-and-their-targeted-therapy-against-dwarfism
#40
JOURNAL ARTICLE
Feroz Khan, Sarmir Khan, Nehal Rana, Tariq Rahim, Abida Arshad, Imtiaz Khan, Hanan A Ogaly, Dalia Abd El Moneim Ahmed, Ayed A Dera, Sumera Zaib
Dwarfism is a medical term used to describe individuals with a height-vertex measurement that falls below two standard deviations (-2SD) or the third percentile for their gender and age. Normal development of growth is a complicated dynamic procedure that depends upon the coordination of different aspects involving diet, genetics, and biological aspects like hormones in equilibrium. Any severe or acute pathologic procedure may disturb the individual's normal rate of growth. In this research, we examined four (A-D) Pakistani consanguineous families that exhibited syndromic dwarfism, which was inherited in an autosomal recessive pattern...
February 7, 2024: Journal of Biomolecular Structure & Dynamics
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