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Cureus Nov 2023This case report presents the management and diagnosis of an 80-year-old gentleman who presented to the Accident and Emergency Department with refractory hypotension,...
This case report presents the management and diagnosis of an 80-year-old gentleman who presented to the Accident and Emergency Department with refractory hypotension, hypoglycemia, and hyponatremia. Initially diagnosed with sepsis and cellulitis, it was later discovered that the patient was suffering from an Addisonian crisis due to adrenal insufficiency induced by long-term steroid use for rheumatoid arthritis. Prompt administration of intravenous hydrocortisone and fluid resuscitation led to a significant improvement in the patient's condition. This case emphasizes the importance of considering adrenal insufficiency in patients with similar clinical presentations and highlights the critical role of early diagnosis and treatment.
PubMed: 38024080
DOI: 10.7759/cureus.49424 -
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 Jul 2023This case report underscores the significance of maintaining a broad list of differential diagnoses, including adrenal insufficiency, when evaluating patients who...
This case report underscores the significance of maintaining a broad list of differential diagnoses, including adrenal insufficiency, when evaluating patients who present with recurring episodes of hypotension and generalized fatigue. It further underscores that T3 thyrotoxicosis can manifest as the initial and sole presenting feature of Graves' disease. Finally, it emphasizes the critical importance of employing a multidisciplinary approach to discharge high-risk patients from the hospital to minimize the risk of acute decompensation.
PubMed: 37551210
DOI: 10.7759/cureus.41528 -
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 -
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 -
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 -
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 -
BMC Pediatrics Dec 2022We report a novel presentation of deficit in anterior pituitary function with variable immune deficiency (DAVID) syndrome in a healthy young girl presenting in...
BACKGROUND
We report a novel presentation of deficit in anterior pituitary function with variable immune deficiency (DAVID) syndrome in a healthy young girl presenting in Addisonian crisis with raised intracranial pressure. Nearly all cases of DAVID syndrome described in the literature have presented with recurrent infections and variable immunodeficiency. Pseudotumour cerebri has not been reported in DAVID syndrome to date.
CASE PRESENTATION
A four-year-old girl represented to hospital with vomiting, confusion and diplopia after ten days of tiredness, neck and abdominal pain, and headache. Her cranial nerve examination demonstrated a right abducens nerve palsy and papilloedema, and she was found to have ketotic hypoglycaemia and hypocortisolaemia secondary to adrenocorticotrophic hormone (ACTH) deficiency. Her neuroimaging was consistent with pseudotumour cerebri, and her lumbar puncture opening pressure confirmed raised intracranial pressure (30-40 cmHO). Cerebrospinal fluid analysis was normal. The patient's symptoms improved with hydrocortisone replacement and acetazolamide, but the raised intracranial pressure recurred after acetazolamide was discontinued. She was subsequently found to have panhypogammaglobulinaemia, and DAVID syndrome was diagnosed. Genetic testing demonstrated a truncating mutation in the NFKB2 gene c.2557C > T, p.(Arg853*).
CONCLUSIONS
This case demonstrates pseudotumour cerebri as a novel neurological presentation of DAVID syndrome, highlights the rare association between adrenal insufficiency and intracranial hypertension, and shows the challenges in diagnosing isolated ACTH deficiency. We emphasise that cortisol should be checked in pre-pubertal children with pseudotumour cerebri and a diagnosis of DAVID syndrome considered in those presenting with low cortisol and neurological symptoms.
Topics: Child; Female; Humans; Child, Preschool; Pseudotumor Cerebri; Acetazolamide; Spinal Puncture; Syndrome; Hydrocortisone; Adrenocorticotropic Hormone
PubMed: 36494638
DOI: 10.1186/s12887-022-03760-x