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Gland Surgery Jul 2019This chapter describes the use of fluorescence via indocyanine green (ICG) in minimally invasive adrenal surgery (laparoscopic and robotic). ICG is a non-toxic dye that... (Review)
Review
This chapter describes the use of fluorescence via indocyanine green (ICG) in minimally invasive adrenal surgery (laparoscopic and robotic). ICG is a non-toxic dye that can aid identification of vascular structures and parenchymal tissue planes in real time. The primary utility of ICG fluorescence in adrenal surgery is to help delineate the margins of resection, to guide a more precise operation. In particular, for patients with bilateral adrenal disease or a heredity associated with high risk of recurrence (e.g., VHL, MEN2a) this may facilitate subtotal adrenal resection (e.g., cortical sparing adrenalectomy), obviating the incidence of iatrogenic adrenal insufficiency and its numerous sequelae including lifelong hormone supplementation, osteoporosis and risk of Addisonian crisis.
PubMed: 31404180
DOI: 10.21037/gs.2019.03.01 -
Neuro-ophthalmology (Aeolus Press) 2021A 3-month-old boy was referred for assessment and management of apparently absent ocular globes. Ocular examination showed small orbits with apparently absent globes,...
A 3-month-old boy was referred for assessment and management of apparently absent ocular globes. Ocular examination showed small orbits with apparently absent globes, small conjunctival cul-de-sac, shallow fornices, microblepharon and sunken eyelids. Magnetic resonance imaging of the orbit and brain revealed bilateral extreme microphthalmia, replacement of the optic nerves by disorganised rudimentary tissue tufts, hypoplastic orbits and extraocular muscles, an absent septum pellucidum and an absent corpus callosum. A pituitary hormonal essay showed decreased adrenocorticotropic hormone and thyroid-stimulating hormone. Septo-optic dysplasia has been rarely reported to be associated with microphthalmia. Timely treatment with hydrocortisone and levothyroxine is essential to prevent Addisonian crisis from the stress and pain that may accompany insertion of socket expanders.
PubMed: 34483412
DOI: 10.1080/01658107.2020.1791910 -
Gland Surgery Feb 2020Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that secrete excess catecholamines leading to secondary hypertension and cardiovascular... (Review)
Review
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that secrete excess catecholamines leading to secondary hypertension and cardiovascular morbidity. Once biochemical testing with either 24-hour urinary fractioned metanephrines or plasma free metanephrines confirms the diagnosis, patients are optimized with adequate hydration to maintain their intravascular volume and the appropriate antihypertensive medications are initiated for optimal blood pressure control. Genetic testing and imaging is performed to determine the extent of adrenalectomy and the optimal surgical approach. Surgical approaches include transabdominal or retroperitoneal minimally invasive approaches, and transabdominal open approaches. Factors that influence the surgical approach include germline genetic test results, the size of the tumor, body mass index, surgeon experience, and the likelihood of malignancy. The extent of adrenalectomy is based on germline genetic findings. Patients with syndromes such as von Hippel Lindau (VHL) or multiple endocrine neoplasia 2 (MEN 2) benefit from cortical-sparing adrenalectomy to avoid chronic steroid replacement and the risk of Addisonian crisis. Postoperative management includes hemodynamic monitoring and assessment for signs of hypoglycemia. Outcomes after surgery show improved blood pressure control in most patients and normalization of blood pressure in about a third of patients. Long-term follow-up is required for all patients to assess for recurrence.
PubMed: 32206597
DOI: 10.21037/gs.2019.10.20 -
The Canadian Veterinary Journal = La... May 2023Primary objectives of this study were to determine presenting complaints, physical examination, clinicopathologic findings, and hospitalization time of dogs with...
OBJECTIVE
Primary objectives of this study were to determine presenting complaints, physical examination, clinicopathologic findings, and hospitalization time of dogs with spontaneous hypoadrenocorticism presenting with critical disease; and to compare those end points to dogs with a more stable presentation. Secondary objectives were to evaluate the shock index and to identify precipitating stressors.
ANIMALS
Eighty-four dogs at the Western College of Veterinary Medicine between 1998 and 2018 were included.
PROCEDURE
Data were retrieved from the medical records.
RESULTS
Collapse and depression were more common among critically ill dogs. Hyperlactatemia was rare despite a diagnosis of hypovolemic shock, and a shock index was ineffective in this patient subset. Isosthenuria, total hypocalcemia, and more severe acidosis were more common ( < 0.05) in critical dogs. Owner separation was the most common precipitating stressor.
CONCLUSION AND CLINICAL RELEVANCE
We concluded that the critical Addisonian dog has unique characteristics that may aid in early disease identification.
Topics: Dogs; Animals; Dog Diseases; Adrenal Insufficiency; Acidosis
PubMed: 37138712
DOI: No ID Found -
Frontiers in Veterinary Science 2021To determine whether administration of intravenous hydrocortisone is a safe and effective alternative treatment in comparison to the traditional treatment with...
Comparison of Hydrocortisone Continuous Rate Infusion and Prednisolone or Dexamethasone Administration for Treatment of Acute Hypoadrenocortical (Addisonian) Crisis in Dogs.
OBJECTIVES
To determine whether administration of intravenous hydrocortisone is a safe and effective alternative treatment in comparison to the traditional treatment with prednisolone/dexamethasone in dogs presenting with Addisonian crisis; and to assess if there is any advantage of the former over the latter in normalisation of electrolyte imbalances and in hospitalisation length in these dogs.
METHODS
Medical records of client-owned dogs with hypoadrenocorticism were retrospectively reviewed. Time until normalisation of sodium and potassium concentration, intravenous fluid needs over the first 24 h and hospitalisation length were compared between hydrocortisone and prednisolone/dexamethasone treated dogs.
RESULTS
Twenty-five dogs met the inclusion criteria; 13 received hydrocortisone and 12 prednisolone/dexamethasone. Intravenous hydrocortisone was well-tolerated but failed to prove superiority in terms of time to normalisation of sodium and potassium concentration. Interestingly, potassium normalised in all dogs prior to discharge, but sodium did not in 1/11 hydrocortisone and 5/9 prednisolone/dexamethasone treated dogs with initial hyponatraemia ( = 0.05). Hydrocortisone treated dogs, however, had more electrolyte re-checks [hydrocortisone treated dogs, median (range): 4 (2-16); prednisolone/dexamethasone treated dogs: 2 (0-6); = 0.001]. There was no difference in intravenous fluid needs over the first 24 h but hydrocortisone treated dogs had longer hospitalisation [hydrocortisone: 81 (45-309) h; prednisolone/dexamethasone: 52 (22-138) h; = 0.01].
CLINICAL SIGNIFICANCE
Intravenous hydrocortisone is well-tolerated and safe, but no clear additional benefit over traditional glucocorticoid replacement could be identified. Also, it might result in longer hospitalisation time and more intensive monitoring.
PubMed: 35146018
DOI: 10.3389/fvets.2021.818515 -
Frontiers in Endocrinology 2023Acute adrenal crisis classically presents with vomiting, altered sensorium, and hypotension. We describe a unique case manifesting with severe hypercalcemia. Addisonian... (Review)
Review
Acute adrenal crisis classically presents with vomiting, altered sensorium, and hypotension. We describe a unique case manifesting with severe hypercalcemia. Addisonian crisis was unusually precipitated by fluconazole use. We reviewed other reported cases and discuss the possible mechanisms of hypercalcemia in adrenal insufficiency. This 67-year-old man presented with fever, cough, and vomiting for 1 week and with anorexia and confusion for 3 weeks. He was hypotensive and clinically dehydrated. Investigations revealed left-sided lung consolidation, acute renal failure, and severe non-parathyroid hormone (PTH)-mediated hypercalcemia (calcium, 3.55mol/L; PTH, 0.81pmol/L). Initial impression was pneumonia complicated by septic shock and hypercalcemia secondary to possible malignancy. He received mechanical ventilation; treatment with intravenous fluids, inotropes, and hydrocortisone for septic shock; and continuous renal replacement therapy with low-calcium dialysate. Although hypercalcemia resolved and he was weaned off inotropes, dialysis, and hydrocortisone, his confusion persisted. When hypercalcemia recurred on day 19 of admission, early morning cortisol was <8 nmol/L, with low ACTH level (3.2 ng/L). Other pituitary hormones were normal. Hypercalcemia resolved 3 days after reinstating stress doses of hydrocortisone, and his mentation normalized. On further questioning, he recently received fluconazole for a forearm abscess. He previously consumed traditional medications but stopped several years ago, which may have contained glucocorticoids. He was discharged on oral hydrocortisone. Cortisol levels improved gradually, and glucocorticoid replacement was ceased after 8 years, without any recurrence of hypercalcemia or Addisonian crisis. Both hypercalcemia and adrenal insufficiency may present with similar non-specific symptoms. It is important to consider adrenal insufficiency in hypercalcemia of unclear etiology.
Topics: Humans; Male; Hypercalcemia; Fluconazole; Adrenal Insufficiency; Hydrocortisone; Aged; Shock, Septic; Treatment Outcome
PubMed: 37274338
DOI: 10.3389/fendo.2023.1168797 -
Frontiers in Endocrinology 2022Congenital Adrenal Hyperplasia (CAH) due to CYP11B1 is a rare autosomal recessive adrenal disorder that causes a decrease in cortisol production and accumulation of...
BACKGROUND
Congenital Adrenal Hyperplasia (CAH) due to CYP11B1 is a rare autosomal recessive adrenal disorder that causes a decrease in cortisol production and accumulation of adrenal androgens and steroid precursors with mineralocorticoid activity. Clinical manifestations include cortisol deficiency, ambiguous genitalia in females (differences of sex development (DSD)), and hypertension. Medical treatment recommendations are well defined, consisting of glucocorticoid treatment to substitute glucocorticoid deficiency and consequently normalize adrenal androgen and precursors levels. Current guidelines also emphasize the need for specialized multidisciplinary DSD teams and psychosocial support. In many developing countries, care for DSD patients, especially when caused by an adrenal disease, is challenging due to the lack of infrastructure, knowledge, and medication.
OBJECTIVE
The study aims to report the conflicting decision-making process of medical treatment and sex assignment in late-identified CAH patients in developing countries.
METHODS
We describe the clinical and biochemical findings and the psychological assessment of five affected but untreated family members with CAH due to CYP11B1 deficiency.
RESULTS
All patients had a 46,XX karyotype, ambiguous genitalia, low cortisol levels, and hypertension. Two identified as males, two as females, and one had undecided gender. The patients were counselled that refusing treatment will lead to infertility and the potential risk of developing Addisonian crisis and severe hypertension. However, all 46,XX CAH males refused treatment with glucocorticoids due to the expected lowering of adrenal androgens as their main source of testosterone. None of the patients developed Addisonian crisis, probably due to some residual cortisol activity and glucocorticoid activity of elevated adrenal steroid precursors.
CONCLUSION
Medical treatment and sex assignment in late-identified 46,XX CAH patients in Indonesia may often depend on local and cultural factors. The management of DSD conditions may have to be individualized and integrated into the psychological and social context of the affected family.
Topics: Female; Humans; Male; Adrenal Hyperplasia, Congenital; Androgens; Developing Countries; Disorders of Sex Development; Glucocorticoids; Hydrocortisone; Hypertension; Steroid 11-beta-Hydroxylase; Steroids
PubMed: 36589846
DOI: 10.3389/fendo.2022.1015973 -
Cureus Mar 2023Autoimmune rheumatic diseases (ARD) present unique challenges in clinical practice. Many of them present in medical emergencies in an unstable state and need immediate...
BACKGROUND
Autoimmune rheumatic diseases (ARD) present unique challenges in clinical practice. Many of them present in medical emergencies in an unstable state and need immediate evaluation for further plans of action. The clinical conundrum is to distinguish between sepsis, disease flare, or Addisonian crisis (AC) (secondary to steroid withdrawal). This may be further complicated by overlapping clinical features like shock/fever and the coexistence of a combination of the above pathophysiologic mechanisms (e.g. AC with sepsis or AC with disease flare). The known biomarkers may not perform optimally to distinguish them and additional supportive investigations like imaging, cultures, autoimmune serological markers, etc. are needed. Ultimately the boundaries between "the art of medicine" and "the science of medicine" may get blurred, as the established literature evidence falls short and the expert opinion is needed in a time-sensitive manner. In this pragmatic study, researchers have attempted to explore the presentation of rheumatologic emergencies on the above three differentials (sepsis, disease flare, and AC).
MATERIALS AND METHODS
In this hospital-based cross-sectional study, adult patients (age >18 years) with ARD who had unplanned hospital admission due to acute worsening were enrolled. This study was conducted over one year, after getting the Institutional Human Ethics Committee's approval. All relevant hematological, immunological, and hormonal parameters (specifically morning cortisol) were collected and analyzed. The aim was to find the individual and combined prevalence of sepsis, disease flare, or AC in this study group.
RESULTS
Forty-one patients were analyzed, with females in the majority (95%) and the dominant age group being 26-49 years (56.1%). A majority had a diagnosis of rheumatoid arthritis (RA) (56.1%) or systemic lupus erythematosus (SLE) (31.7%); the rest were other connective tissue diseases (12.2%). High-risk Quick Sequential Organ Failure Assessment score (qSOFA) score 2-3 was present in 29.3% while the rest had low-risk scores (qSOFA score 0-1). Thirty-two percent had severe disease activity, 46% had mild to moderate disease activity, and 22% of patients had no disease activity. While 78% of patients had low procalcitonin (PCT) values <0.5 microgm/L (low risk of sepsis), 15% had <20 microgm/L, and 7% percentage of patients had serum levels >20 microgm/L (high risk of sepsis). A total of 73.2% of patients had no evidence of infection while 26.8% had either microbiological/radiological evidence of infection. Only 7% of all patients had the presence of an AC. qSOFA scores didn't statistically correlate with a diagnosis of infection or AC but positively correlated with PCT and C-reactive protein (CRP) values. Serum PCT didn't correlate with the presence of infection with statistically significance (p-value 0.217).
CONCLUSION
Infections and sepsis are the most important considerations in the emergency presentations of ARDs. Disease flare and AC are also important differentials. Current inflammatory biomarkers like serum CRP and PCT may be less valuable for discriminating between infectious and non-infectious sepsis, especially in chronic inflammatory diseases like ARDs. qSOFA scores may have a prognostic role with less discriminant value. Management of ARD emergencies needs better biomarkers and more research is warranted.
PubMed: 37050995
DOI: 10.7759/cureus.35916