-
The Canadian Veterinary Journal = La... Feb 2010Dogs with chronic, vague gastrointestinal signs and those with signs and laboratory abnormalities suggestive of an Addisonian crisis should be tested for...
Dogs with chronic, vague gastrointestinal signs and those with signs and laboratory abnormalities suggestive of an Addisonian crisis should be tested for hypoadrenocorticism. A previous article (Part I; Can Vet J 2009;50:63-69) discussed the etiology, pathophysiology, clinical signs, and diagnostic abnormalities found in these patients. The present article discusses definitive diagnosis and treatment for both the acute and the chronic Addisonian patient. Expedient treatment remains the cornerstone of management for these patients, particularly those in the former category. The long-term prognosis is excellent for these patients, given well-educated, committed, and vigilant owners.
Topics: Addison Disease; Adrenal Cortex Diseases; Adrenal Insufficiency; Adrenocorticotropic Hormone; Animals; Diagnosis, Differential; Dog Diseases; Dogs; Human-Animal Bond; Humans; Hydrocortisone; Prognosis; Reference Values; Shock
PubMed: 20436864
DOI: No ID Found -
Clinical and Experimental Immunology Jul 1970A case of primary hypothyroidism, idiopathic Addison's disease, idiopathic hypoparathyroidism (with preceding moniliasis), Addisonian pernicious anaemia and primary...
A case of primary hypothyroidism, idiopathic Addison's disease, idiopathic hypoparathyroidism (with preceding moniliasis), Addisonian pernicious anaemia and primary ovarian failure is described. She died at the age of 24 years following an illness compatible with adrenal crisis. At post-mortem there was no recognizable adrenal or ovarian tissue; there was only a minute portion of probable parathyroid tissue and the uterus was infantile. Her serum contained antibodies reactive with adrenal cortex, steroid-producing cells in the gonads, placental trophoblasts and thyroid epithelial cytoplasm and intrinsic factor. Her brother, who was known to have gluten enteropathy, died aged 11 years following an illness compatible with adrenal crisis. His adrenal glands were grossly atrophic at autopsy. The parents were consanguinous and both showed either clinical or serological evidence of organ specific autoimmune disease.
Topics: Addison Disease; Adrenal Glands; Adult; Anemia, Pernicious; Autoantibodies; Autoimmune Diseases; Autopsy; Candidiasis; Celiac Disease; Child; Consanguinity; Female; Glutens; Humans; Hyperthyroidism; Hypoparathyroidism; Male; Ovarian Diseases; Ovary; Parathyroid Glands; Uterus
PubMed: 5202743
DOI: No ID Found -
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 -
Primary Care Respiratory Journal :... Jun 2007Inhaled corticosteroids remain the most important therapy for chronic asthma in both adults and children. As all inhaled corticosteroids act by binding to a common... (Review)
Review
Inhaled corticosteroids remain the most important therapy for chronic asthma in both adults and children. As all inhaled corticosteroids act by binding to a common glucocorticoid receptor there is little evidence of any real difference in clinical efficacy between the different inhaled corticosteroids. The main potential differences are in their propensity to cause side effects. Local side effects such as a hoarse voice do occur in a proportion of adults and there is some limited evidence that ciclesonide may cause less local side effects. In adults there is little evidence for clinically important systemic side effects from doses of inhaled steroids below 800 mcg/day (beclomethasone equivalent). Above this dose a proportion of patients may show some adrenocortical suppression, though it is unlikely to be of clinical importance. Data on bone mineral density and fracture rates is discrepant, but an overview would suggest that below 800 mcg/day there is no increase in fracture risk whereas above this dose there might be an increased fracture risk. The properties of ciclesonide would suggest that it has less propensity for systemic side effects, but large long term studies are needed to confirm this. In children using inhaled steroids at above-licensed doses reductions in short-term growth can occur, but there is little evidence for reductions in long-term growth at normal doses. At above-licensed doses, biochemical adrenocortical suppression can occur with some unusual but documented cases of clinical Addisonian crisis. Limited evidence in paediatric age groups would suggest that ciclesonide may have some advantage although it is not as yet licensed in all countries for paediatric use. Data on differences in side effects between normal and asthmatic patients, and between asthmatic patients with near-normal lung function compared to those with impaired lung function, indicate that inhaled corticosteroids (particularly fluticasone) are absorbed more in those with normal lung function; this strongly supports stepping down the inhaled steroid dose when asthma is controlled - as is recommended in asthma guidelines.
Topics: Administration, Inhalation; Adrenal Glands; Anti-Asthmatic Agents; Asthma; Beclomethasone; Bone and Bones; Dose-Response Relationship, Drug; Glucocorticoids; Growth; Hoarseness; Humans; Nebulizers and Vaporizers; Practice Guidelines as Topic; Pregnenediones
PubMed: 17530144
DOI: 10.3132/pcrj.2007.00038 -
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 -
Journal of Clinical Medicine May 2015We report a case of a previously undiagnosed panhypopituitarism initially presenting as a full-blown Addisonian crisis with hypoglycemia, hyponatremia, hypotension and...
We report a case of a previously undiagnosed panhypopituitarism initially presenting as a full-blown Addisonian crisis with hypoglycemia, hyponatremia, hypotension and neuropsychological symptoms, more than 30 years after a severe traumatic brain injury (TBI). The patient also displayed clearly visible pathognomonic clinical signs of long-standing pituitary dysfunction. The case highlights the importance of being aware of endocrine sequelae even decades after serious TBI.
PubMed: 26239458
DOI: 10.3390/jcm4050965 -
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 -
A qualitative analysis of the impact of canine hypoadrenocorticism on the quality of life of owners.BMC Veterinary Research Sep 2023Canine hypoadrenocorticism is a rare chronic disease, which demands intense dog-owner interaction, as its treatment requires to be individualised. The aim of this study...
BACKGROUND
Canine hypoadrenocorticism is a rare chronic disease, which demands intense dog-owner interaction, as its treatment requires to be individualised. The aim of this study was a qualitative analysis of the challenges owners face when dealing with the disease, especially regarding its management and how this affects quality of life. By promoting an online discussion between owners, we transcribed and summarised their experiential knowledge in dealing with the disease.
METHODS
Owners were recruited for the online seminars via social media. After a theoretical introduction, participants were free to share experiences and ask questions. The recorded events were retrospectively analysed.
RESULTS
Twenty-four owners of 22 Addisonian dogs took part in four events. Owners felt most "traumatised" when experiencing their dog's acute adrenal crisis. The initial adjustment phase and distinguishing the non-specific symptoms of hypoadrenocorticism from those of other diseases were also challenging. Overall, owners were well informed on the disease and committed to its long-term adjustment.
CONCLUSIONS
Adrenal crisis and the initial adjustment phase may be more burdening to owners than expected. Understanding what their clients' concerns are, can help veterinarians provide better care and reduce the negative impacts of canine hypoadrenocorticism. Promoting peer to peer support, as well as providing a framework for participative communication might also help.
Topics: Animals; Dogs; Humans; Quality of Life; Retrospective Studies; Communication; Records; Veterinarians
PubMed: 37689678
DOI: 10.1186/s12917-023-03716-y -
Cureus Jun 2022Autoimmune polyendocrine syndrome type 2, also known as Schmidt's syndrome, is a rare autosomal dominant life-threatening syndrome. It is defined by the presence of...
Autoimmune polyendocrine syndrome type 2, also known as Schmidt's syndrome, is a rare autosomal dominant life-threatening syndrome. It is defined by the presence of Addison's disease in combination with at least one of the known autoimmune diseases: thyroid autoimmune disease, type 1 diabetes, and hypogonadism. It is more common in middle-aged females and is treatable if diagnosed early. However, in this case, we report Schmidt's syndrome in a young male without a family history. A 20-year-old male with a past medical history of hypothyroidism, adrenal insufficiency, and type 1 diabetes presented to the emergency department (ED) feeling lethargic, somnolent, and diaphoretic. Laboratory blood tests showed elevated thyroid-stimulating hormone, hyperkalemia of 6.4 mmol/L, and hyponatremia of 131 mmol/l indicating an Addisonian crisis. The patient had low blood glucose (at home: 60 mg/dL, and at ED: 85 mg/dL), hypotensive blood pressure of approximately 85/55 mmHg, and a peaked T-wave on EKG, which were consistent with the diagnosis of Schmidt's syndrome. Based on the laboratory findings and history, the patient was diagnosed with polyendocrine syndrome Type 2 (Schmidt's syndrome). The patient was treated for adrenal insufficiency first followed by thyroid insufficiency. Schmidt's syndrome is a rare disease and difficult to diagnose because the presentation depends on which gland is initially involved. A few cases have been reported in the literature of atypical presentations of Schmidt's syndrome. Therefore, this case report can contribute to the medical literature on Schmidt's syndrome, which can help in early diagnosis and improve patient outcomes.
PubMed: 35911265
DOI: 10.7759/cureus.26322 -
Genes Apr 2022Nicotinamide nucleotide transhydrogenase (NNT) deficiency causes primary adrenal insufficiency (PAI) and possibly some extra-adrenal manifestations. A limited number of...
Nicotinamide nucleotide transhydrogenase (NNT) deficiency causes primary adrenal insufficiency (PAI) and possibly some extra-adrenal manifestations. A limited number of these patients were previously described. We present the clinical and genetic characteristics of three family members with a biallelic novel pathogenic variant in the gene. The patients were followed until the ages of 21.6, 20.2, and 4.2 years. PAI was diagnosed in the eldest two brothers after an Addisonian crisis and the third was diagnosed at the age of 4.5 months in the asymptomatic stage due to the genetic screening of family members. Whole exome sequencing with a targeted interpretation of variants in genes related to PAI was performed in all the patients. The urinary steroid metabolome was determined by gas chromatography-mass spectrometry in the asymptomatic patient. The three patients, who were homozygous for c.1575dup in the gene, developed isolated glucocorticoid deficiency. The urinary steroid metabolome showed normal excretion of cortisol metabolites. The adolescent patients had slow pubertal progression with low-normal testicular volume, while testicular endocrine function was normal. Bone mineral density was in the range for osteopenia in both grown-up siblings. Echocardiography revealed no structural or functional heart abnormalities. This article is among the first with a comprehensive and chronologically-detailed description of patients with NNT deficiency.
Topics: Addison Disease; Adolescent; Child, Preschool; Follow-Up Studies; Humans; Infant; Male; Mitochondrial Proteins; NADP Transhydrogenase, AB-Specific; NADP Transhydrogenases; Siblings; Steroids; Young Adult
PubMed: 35627102
DOI: 10.3390/genes13050717