Endocrine dysfunction in adrenoleukodystrophy

2021 ◽  
pp. 257-267
Author(s):  
Marc Engelen ◽  
Stephan Kemp ◽  
Florian Eichler
Keyword(s):  
Endocrine Dysfunction

scholarly journals Endocrine Dysfunction in Children with Zika-Related Microcephaly Who Were Born during the 2015 Epidemic in the State of Pernambuco, Brazil

Viruses ◽  
2020 ◽  
Vol 13 (1) ◽  
pp. 1
Author(s):  
Andréia Veras Gonçalves ◽  
Demócrito de B. Miranda-Filho ◽  
Líbia Cristina Rocha Vilela ◽  
Regina Coeli Ferreira Ramos ◽  
Thalia V. B. de Araújo ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Viral Infections ◽  
Case Series ◽  
Optic Nerve Hypoplasia ◽  
Endocrine Dysfunction ◽  
Careful Monitoring ◽  
Early Puberty ◽  
Growth Impairment ◽  
Appropriate Treatment ◽  
The Brain

Congenital viral infections and the occurrence of septo-optic dysplasia, which is a combination of optic nerve hypoplasia, abnormal formation of structures along the midline of the brain, and pituitary hypofunction, support the biological plausibility of endocrine dysfunction in Zika-related microcephaly. In this case series we ascertained the presence and describe endocrine dysfunction in 30 children with severe Zika-related microcephaly from the MERG Pediatric Cohort, referred for endocrinological evaluation between February and August 2019. Of the 30 children, 97% had severe microcephaly. The average age at the endocrinological consultation was 41 months and 53% were female. The most frequently observed endocrine dysfunctions comprised short stature, hypothyroidism, obesity and variants early puberty. These dysfunctions occurred alone 57% or in combination 43%. We found optic nerve hypoplasia (6/21) and corpus callosum hypoplasia (20/21). Seizure crises were reported in 86% of the children. The most common—and clinically important—endocrine dysfunctions were pubertal dysfunctions, thyroid disease, growth impairment, and obesity. These dysfunctions require careful monitoring and signal the need for endocrinological evaluation in children with Zika-related microcephaly, in order to make early diagnoses and implement appropriate treatment when necessary.

scholarly journals Endocrine Challenges in Patients with Continuous-Flow Left Ventricular Assist Devices

Nutrients ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 861
Author(s):  
Gennaro Martucci ◽  
Federico Pappalardo ◽  
Harikesh Subramanian ◽  
Giulia Ingoglia ◽  
Elena Conoscenti ◽  
...  
Keyword(s):  
Continuous Flow ◽  
Left Ventricular ◽  
Endocrine Function ◽  
Ventricular Assist Devices ◽  
Circulatory Support ◽  
Endocrine Dysfunction ◽  
Left Ventricular Assist Devices ◽  
Ventricular Assist ◽  
Assist Devices ◽  
Left Ventricular Assist

Heart failure (HF) remains a leading cause of morbidity, hospitalization, and mortality worldwide. Advancement of mechanical circulatory support technology has led to the use of continuous-flow left ventricular assist devices (LVADs), reducing hospitalizations, and improving quality of life and outcomes in advanced HF. Recent studies have highlighted how metabolic and endocrine dysfunction may be a consequence of, or associated with, HF, and may represent a novel (still neglected) therapeutic target in the treatment of HF. On the other hand, it is not clear whether LVAD support, may impact the outcome by also improving organ perfusion as well as improving the neuro-hormonal state of the patients, reducing the endocrine dysfunction. Moreover, endocrine function is likely a major determinant of human homeostasis, and is a key issue in the recovery from critical illness. Care of the endocrine function may contribute to improving cardiac contractility, immune function, as well as infection control, and rehabilitation during and after a LVAD placement. In this review, data on endocrine challenges in patients carrying an LVAD are gathered to highlight pathophysiological states relevant to this setting of patients, and to summarize the current therapeutic suggestions in the treatment of thyroid dysfunction, and vitamin D, erythropoietin and testosterone administration.

scholarly journals Post-haemorrhagic hydrocephalus is associated with poorer surgical and neurodevelopmental sequelae than other causes of infant hydrocephalus

2021 ◽  
Author(s):  
Malak Mohamed ◽  
Saniya Mediratta ◽  
Aswin Chari ◽  
Cristine Sortica da Costa ◽  
Greg James ◽  
...  
Keyword(s):  
Gestational Age ◽  
Cox Regression ◽  
Severe Disabilities ◽  
Median Number ◽  
Shunt Revision ◽  
Endocrine Dysfunction ◽  
Neurodevelopmental Outcomes ◽  
Shunt Insertion ◽  
Street Hospital ◽  
Shunt Survival

Abstract Purpose This retrospective cohort study aimed to investigate the surgical and neurodevelopmental outcomes (NDO) of infant hydrocephalus. We also sought to determine whether these outcomes are disproportionately poorer in post-haemorrhagic hydrocephalus (PHH) compared to other causes of infant hydrocephalus. Methods A review of all infants with hydrocephalus who had ventriculoperitoneal (VP) shunts inserted at Great Ormond Street Hospital (GOSH) from 2008 to 2018 was performed. Demographic, surgical, neurodevelopmental, and other clinical data extracted from electronic patient notes were analysed by aetiology. Shunt survival, NDO, cerebral palsy (CP), epilepsy, speech delay, education, behavioural disorders, endocrine dysfunction, and mortality were evaluated. Results A total of 323 infants with median gestational age of 37.0 (23.29–42.14) weeks and birthweight of 2640 g (525–4684 g) were evaluated. PHH was the most common aetiology (31.9%) and was associated with significantly higher 5-year shunt revision rates, revisions beyond a year, and median number of revisions than congenital or “other” hydrocephalus (all p < 0.02). Cox regression demonstrated poorest shunt survival in PHH, related to gestational age at birth and corrected age at shunt insertion. PHH also had the highest rate of severe disabilities, increasing with age to 65.0% at 10 years, as well as the highest CP rate; only genetic hydrocephalus had significantly higher endocrine dysfunction (p = 0.01) and mortality rates (p = 0.04). Conclusions Infants with PHH have poorer surgical and NDO compared to all other aetiologies, except genetic hydrocephalus. Research into measures of reducing neurodisability following PHH is urgently required. Long-term follow-up is essential to optimise support and outcomes.

Endocrine Dysfunction following Traumatic Brain Injury in Children

2010 ◽  
Vol 157 (6) ◽  
pp. 894-899 ◽  
Author(s):  
Anne-Marie D. Kaulfers ◽  
Philippe F. Backeljauw ◽  
Kent Reifschneider ◽  
Samantha Blum ◽  
Linda Michaud ◽  
...  
Keyword(s):  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Endocrine Dysfunction

scholarly journals A tale of two pancreases: exocrine pathology and endocrine dysfunction

Diabetologia ◽  
2020 ◽  
Vol 63 (10) ◽  
pp. 2030-2039
Author(s):  
Michael R. Rickels ◽  
Andrew W. Norris ◽  
Rebecca L. Hull
Keyword(s):  
Endocrine Dysfunction

Ocular Changes Related to Endocrine Dysfunction*

1960 ◽  
Vol 50 (1) ◽  
pp. 26-33 ◽  
Author(s):  
Richard O. Schultz ◽  
Henry E. Hamilton ◽  
Alson E. Braley
Keyword(s):  
Endocrine Dysfunction ◽  
Ocular Changes

scholarly journals Antioxidant Therapy in Pancreatitis

Antioxidants ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 657
Author(s):  
Lourdes Swentek ◽  
Dean Chung ◽  
Hirohito Ichii
Keyword(s):  
Oxidative Stress ◽  
Chronic Pancreatitis ◽  
Total Pancreatectomy ◽  
Current Status ◽  
Related Factor ◽  
Endocrine Dysfunction ◽  
Deep Dive ◽  
Pathway Activation ◽  
Advanced Therapies ◽  
And Oxidative Stress

Pancreatitis is pathologic inflammation of the pancreas characterized by acinar cell destruction and oxidative stress. Repeated pancreatic insults can result in the development of chronic pancreatitis, characterized by irreversible fibrosis of the pancreas and many secondary sequelae, ultimately leading to the loss of this important organ. We review acute pancreatitis, chronic pancreatitis, and pancreatitis-related complications. We take a close look at the pathophysiology with a focus on oxidative stress and how it contributes to the complications of the disease. We also take a deep dive into the evolution and current status of advanced therapies for management including dietary modification, antioxidant supplementation, and nuclear factor erythroid-2-related factor 2-Kelch-like ECH-associated protein 1(Nrf2-keap1) pathway activation. In addition, we discuss the surgeries aimed at managing pain and preventing further endocrine dysfunction, such as total pancreatectomy with islet auto-transplantation.

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