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APPLICATION OF PHARMACEUTICAL KNOWLEDGE TO THE UNCONVENTIONAL USE OF A RARELY USED ANAESTHETIC AGENT.

CASE SUMMARY: A 6 yr old 45 kg child with severe Cushinoid features was admitted to PICU with probable hypertensive encephalopathy. She pres … enal suppression and optimise cortisol levels to 200-800 nmol/L. Mineralocorticoid replacement with fludrocortisone became necessary, together with significant electrolyte replacement therapy. Surgery was delayed due to sepsis, and block and replace therapy was continued for a period of 3 weeks. During this time she experienced minimal sedative effects from the e …

Pregnancy after liver transplantation with tacrolimus immunosuppression: a single center's experience update at 13 years.

BACKGROUND: Chronic liver disease often leads to amenorrhea in women of childbearing age. There are several reports of successful pregnancy after liver transplantation (LTx) with cyclosporine A immunosuppression. Tacrolimus has been increasingly used in solid-organ transplantation, and the effect of the drug on pregnancy is still of interest to clinicians. This study updates our single-center experience. METHODS: All pregnancies after LTx with tacrolimus immunosuppression were followed prospectively. Patients' clinical courses during pregnancy and labor along with gestational period and birth

[Syncope - a systematic overview of classification, pathogenesis, diagnosis and management].

Syncope is defined as a temporary interruption of cerebral perfusion with a sudden and transient loss of consciousness and spontaneous recov … ic tolerance. Among the drugs recommended for pharmacologic treatment are mineralocorticoids (e. g. fludrocortisone), vasoconstrictor agents (e. g. ephedrine, midodrine), adenosine receptor blockers (theophylline) and beta2-blockers (propanolol), anticholinergic agents, e. g. scopolamine or disopyramide, and negative cardiac inotropes, e. g. beta1-adrenergic bloc …

X-linked adrenal hypoplasia in a large Greenlandic family. Detection of a missense mutation (N4401) in the DAX-1 gene; implication for genetic counselling and carrier diagnosis.

X-linked congenital adrenal hypoplasia (AHC) is a developmental disorder of the human adrenal gland that results in profound hormonal deficiencies, which are lethal if untreated. Hypogonadotropic hypogonadism (HHG) is frequently associated with this disorder. The gene (DAX-1) responsible for the disease has recently been isolated. It encodes a protein with large similarity to members of the nuclear hormone receptor superfamily. Several different mutations in this gene have been found in patients suffering from AHC. We have identified a missense mutation (N440I) in three patients with AHC and H

The Movement Disorder Society Evidence-Based Medicine Review Update: Treatments for the non-motor symptoms of Parkinson's disease.

The Movement Disorder Society (MDS) Task Force on Evidence-Based Medicine (EBM) Review of Treatments for Parkinson's Disease (PD) was first … alantamine, and memantine for the treatment of dementia; quetiapine for the treatment of psychosis; fludrocortisone and domperidone for the treatment of orthostatic hypotension; sildenafil for the treatment of erectile dysfunction, ipratropium bromide spray for the treatment of sialorrhea; levodopa/carbidopa controlled release (CR), pergolide, eszopiclone, melato …

Targeted disruption of the mouse gene encoding steroidogenic acute regulatory protein provides insights into congenital lipoid adrenal hyperplasia.

An essential component of regulated steroidogenesis is the translocation of cholesterol from the cytoplasm to the inner mitochondrial membrane where the cholesterol side-chain cleavage enzyme carries out the first committed step in steroidogenesis. Recent studies showed that a 30-kDa mitochondrial phosphoprotein, designated steroidogenic acute regulatory protein (StAR), is essential for this translocation. To allow us to explore the roles of StAR in a system amenable to experimental manipulation and to develop an animal model for the human disorder lipoid congenital adrenal hyperplasia (lipoid

Cardiac Amyloidosis: A Rare TTR Mutation Found in an Asian Female.

BACKGROUND: Transthyretin cardiac amyloidosis (ATTR) is a life-threatening, debilitating disease caused by abnormal formation and deposit of transthyretin (TTR) protein in multiple tissues, including myocardial extracellular matrix. It can be challenging to diagnose due to the myriad of presenting signs and symptoms. Additionally, numerous TTR mutations exist in certain ethnicities. Interestingly, our patient was discovered to have a very rare Gly67Ala TTR mutation typically not found in individuals of Asian descent. Recent advances in cardiovascular imaging techniques have allowed for earlier

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… Midodrine and fludrocortisone were started, as well as antiemetics and proton pump inhibitors for symptom relief. With improvement in her hemodynamic status, she was able to be discharged and followed up in our cardiomyopathy clinic for further management. 2.6. … Her orthostatic hypotension was treated with 2.5 mg midodrine three times daily and 0.1 mg fludrocortisone daily. She was sent to a neurologist for further evaluation of her peripheral neuropathy. …

Dysautonomia: A Forgotten Condition - Part 1.

Dysautonomia covers a range of clinical conditions with different characteristics and prognoses. They are classified as Reflex Syndromes, Po … the water are important treatment steps. Various drugs can be used for symptomatic nOH, especially fludrocortisone, midodrine and droxidopa, the latter not available in Brazil. The risk of exacerbation or triggering supine hypertension should be considered. Chronic Fatigue Syndrome represents a form of Dysautonomia and has been renamed as a systemic disease of e …

Drug Repurposing for the Management of Depression: Where Do We Stand Currently?

A slow rate of new drug discovery and higher costs of new drug development attracted the attention of scientists and physicians for the repurposing and repositioning of old medications. Experimental studies and off-label use of drugs have helped drive data for further studies of approving these medications. A deeper understanding of the pathogenesis of depression encourages novel discoveries through drug repurposing and drug repositioning to treat depression. In addition to reducing neurotransmitters like epinephrine and serotonin, other mechanisms such as inflammation, insufficient blood supp

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… [ 129 ] | Experimental: minocycline and standard antidepressant treatment Placebo comparator: placebo and standard antidepressant treatment | 168/NA/NA MDD | R, PG, TB | NA | NA | NA Cycloserine Not applicable/2017 [ 130 , 131 ] | Group A: placebo + placebo: placebo 8 mm pill, single dose Group B: fludrocortisone … + placebo: fludrocortisone Astonin H 0, 1 gm, single-dose + placebo 8 mm pill, single dose Group C: D-cycloserine + placebo: cycloserine 250 mg capsule, single-dose + placebo 8 mm pill, single dose Group D: fludrocortisone + D-Cycloserine: fludrocortisone Astonin H 0, 1 gm, single-dose + cycloserine …

State of the Art of Cardiac Amyloidosis.

Cardiac amyloidosis is defined by extracellular deposition of misfolded proteins in the heart. The most frequent cases of cardiac amyloidosis are caused by transthyretin and light chain amyloidosis. This condition is underdiagnosed, and its incidence has been continuously rising in recent studies because of the aging of the population and the development of noninvasive multimodal diagnostic tools. Amyloid infiltration affects all cardiac tunics and causes heart failure with preserved ejection fraction, aortic stenosis, arrythmia, and conductive disorder. Innovative, specific therapeutic approa

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… Last but not least, synthetic mineralocorticoids like fludrocortisone can be used to increase renal sodium and water retention [ 59 ]. 6.2. Specific Treatment of Cardiac Amyloidosis 6.2.1. … Fludrocortisone Is Associated With a Higher Risk of All-Cause Hospitalizations Compared With Midodrine in Patients With Orthostatic Hypotension. J. Am. Heart Assoc. 2017;6:e006848. doi: 10.1161/JAHA.117.006848. 60. …

Staged Hand-Assisted Bilateral Native Nephrectomy for Management of Posttransplant Polyuria in an Adult with Dent's Disease.

Polyuria after kidney transplantation causes graft dysfunction and increased thrombotic risk. We present a case of a polyuric adult with Dent's disease who underwent staged bilateral native nephrectomies, the first operation before transplant and the second four months after transplant. This led to improved allograft function maintained during four years of follow-up. The retroperitoneal laparoscopic approach was well tolerated and allowed continuation of peritoneal dialysis before transplantation. A staged approach helps regulate fluid balance perioperatively and may be tailored to individual

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… He remained polyuric, which was resistant to treatment with oral fludrocortisone (100 mcg once daily) and high dose oral desmopressin (300 mcg three times daily). …

Помимо обычных подозреваемых: клинические и патологоанатомические ощущения из жестокого случая амилоидоза у филиппинского пациента.

Систематическая световая цепочка (AL) амилоидоз представляет собой редкое, угрожающее жизни расстройство, вызванное неправильной завязкой и осаждением моноклонных иммуноглобулиновых световых цепей в виде амилоидных фибрилов в различных органах, что приводит к постепенной дисфункции. Мы сообщаем о случае 58-year-old Филиппинский мужчина, представлявший four- месячная история болезненной парестезии, прогрессивной нейропатии, значительной потери веса и автономных симптомов, включая ортостатическую гипотенцию и желудочно-кишечную дисмотивацию. Первоначальный анализ показал, что аксональная и демие

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… Autonomic symptoms, such as orthostatic hypotension, can be managed with dietary modifications (high salt intake), fludrocortisone, or compression stockings. In cases of cardiomyopathy, midodrine - an alpha-adrenergic agonist - may be utilized [ 7 , 8 , 10 ]. …

Treating Primary Aldosteronism-Induced Hypertension: Novel Approaches and Future Outlooks.

Primary aldosteronism (PA) is the most common cause of secondary hypertension and is associated with increased morbidity and mortality when compared with blood pressure-matched cases of primary hypertension. Current limitations in patient care stem from delayed recognition of the condition, limited access to key diagnostic procedures, and lack of a definitive therapy option for nonsurgical candidates. However, several recent advances have the potential to address these barriers to optimal care. From a diagnostic perspective, machine-learning algorithms have shown promise in the prediction of P

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… Similarly, the gold standard status of confirmatory testing has recently been called in to question in a comprehensive meta-analysis (55 studies; 7357 patients) of the 4 commonly used investigations (SIT, salt-loading test, fludrocortisone suppression test, and captopril challenge test) ( 63 ). … Acknowledging the limitations highlighted within this metanalysis, the fludrocortisone challenge test demonstrated the best performance as a confirmatory test, followed by the recumbent SIT and the captopril challenge test. …

Pressures do not equal volumes: implications for heart failure management in patients with CardioMEMS.

Pulmonary arterial pressures are often used as surrogates for intravascular blood volume. However, the relationship between pressure and volume remains controversial. To contextualize and provide concrete examples and impact on clinical management, we report two cases of heart failure (one in heart failure with reduced ejection fraction and one in heart failure with preserved ejection fraction) where pressure-based measurements did not accurately represent the intravascular status and affected clinical management.

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… She remained on fludrocortisone 0.1 mg BID for adrenal insufficiency and midodrine 5 mg TID for her low blood pressures and low‐dose HF GDMT (metoprolol succinate 25 mg daily, lisinopril 2.5 mg daily, spironolactone 12.5 mg daily). …

Systemic amyloidosis: an aggressive evolution in a patient with relapsing polychondritis and monoclonal gammopathy of undetermined significance (MGUS) undergoing peritoneal dialysis.

Herein, we report the case of primary amyloidosis with multi-organ involvement in a female patient in her 50s. The patient had a history of relapsing polychondritis, chronic kidney disease, and monoclonal gammopathy of undetermined significance (MGUS). The clinical manifestations included neuropathic pain, sensorimotor polyneuropathy, intrahepatic cholestatic liver injury, gastrointestinal symptoms, dysautonomia, and myocardial thickening. Initial histologic evaluations of the abdominal fat pad aspirate and bone marrow biopsy were negative for amyloid deposition. However, due to a high index o

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… The generalized progressive autonomic failure was managed with pharmacological agents that increase intravascular volume, in this case, fludrocortisone. …

A Comprehensive Review of the Management of Light-Chain (AL) and Transthyretin (ATTR) Cardiac Amyloidosis.

Cardiac amyloidosis, once considered a rare and untreatable disorder, is now increasingly recognized as a significant cause of heart failure, particularly in older adults. The two most clinically relevant subtypes of cardiac amyloidosis-immunoglobulin light-chain amyloidosis (AL) and transthyretin-related amyloidosis (ATTR)-differ in pathogenesis, natural history, and management strategies, thereby necessitating a tailored approach to diagnosis and therapy. Advances in multimodality cardiac imaging, including echocardiography, cardiac magnetic resonance, and nuclear scintigraphy, have enabled

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… Fludrocortisone is another option that increases water retention by the kidneys but is associated with increased hospitalization when compared to midodrine [ 134 , 135 ]. 9. … Midodrine remains the first-line agent; droxidopa can be added in refractory cases, and fludrocortisone may be cautiously used to augment intravascular volume. AS frequently coexists with ATTR amyloidosis, particularly in older men with low-flow, low-gradient physiology. …

Between Charybdis and Scylla-an Odyssey in AL amyloidosis: insights and learnings from a narrative review and case report series.

Being "between Scylla and Charybdis" is an idiom derived from Greek mythology to mean "between a rock and a hard place" and clinicians managing amyloid light-chain (AL) amyloidosis often find themselves in this predicament. AL amyloidosis is caused by monoclonal light chains, most commonly produced by CD-38 positive plasma cells in target organs. The disease usually involves significant cardiac and/or renal involvement, but the systemic nature of the disease often leads to variable and non-specific manifestations that can critically delay early diagnosis and treatment. Here, we present a case

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… Importantly, the patient had recently initiated fludrocortisone 0.1 mg OD for hypotension. The patient’s orthostatic hypotension and gastrointestinal symptoms, alongside symptoms indicative of autonomic and peripheral neuropathy, were considered disproportionate to his diabetes. … A complete response occurred after cycle 1; after cycle 2, appetite, diarrhea, and dizziness improved; after cycle 3, fludrocortisone was stopped, and blood pressure started to stabilize. …

Renal calcification in children with renal tubular acidosis: What a paediatrician ‎should ‎know‎.

Renal tubular acidosis (RTA) can lead to renal calcification in children, which can cause various complications and impair renal function. This review provides pediatricians with a comprehensive understanding of the relationship between RTA and renal calcification, highlighting essential aspects for clinical management. The article analyzed relevant studies to explore the prevalence, risk factors, underlying mechanisms, and clinical implications of renal calcification in children with RTA. Results show that distal RTA (type 1) is particularly associated with nephrocalcinosis, which presents a

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… Daily intake of 0.1 mg of fludrocortisone can effectively manage hyperaldosteronism-associated hyperkalemia. However, this is not generally recommended due to the high risk of hypertension, edema, and heart failure. … Fludrocortisone therapy for persistent hyperkalaemia. Diabet Med. 2017;34:1005–1008. doi: 10.1111/dme.13359. 93. Cho SG, Yi JH, Han SW, Kim HJ. Electrolyte imbalances and nephrocalcinosis in acute phosphate poisoning on chronic type 1 renal tubular acidosis due to Sjögren's syndrome. …

Patient with AL amyloidosis with recurrent syncope as the first symptom: case report and literature review.

BACKGROUND: Immunoglobulin light-chain (AL) amyloidosis is a rare systemic disease caused by the extracellular deposition of misfolded light chains. Nonspecific manifestations, especially those dominated by severe autonomic neuropathy, frequently delay diagnosis. METHODS AND RESULTS: A 68-year-old woman was admitted after five months of recurrent syncope. The clinical picture was dominated by refractory orthostatic hypotension, nausea and vomiting alternating with diarrhea and constipation, stocking-glove paresthesias, and proteinuria. Bone marrow examination showed the presence of clonal pla

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… subcutaneous fat | YES | Disorientation, ataxia | Sortezomib | NA P3 ( 6 ) | 68/M | Endoneurial blood vessels | YES | Hypotension | Large dose of dexamethasone | Survived P4 ( 7 ) | 73/F | Hyperemic gastric antrum pointed out perivascular deposits | YES | Refractory hypotension, diarrhea, dysphagia | Fludrocortisone …