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BMJ Case Reports Jun 2021A previously well 37-year-old woman attended the emergency assessment unit with symptoms of lethargy, breathlessness and peripheral oedema, whereby initial basic...
A previously well 37-year-old woman attended the emergency assessment unit with symptoms of lethargy, breathlessness and peripheral oedema, whereby initial basic investigations revealed an iron deficiency anaemia and serum hypoalbuminaemia. The patient subsequently had multiple admissions to secondary care over a 2-year period due to worsening peripheral and central oedema. Investigations ruled out non-gastrointestinal causes of serum hypoalbuminaemia, such as renal, cardiac and hepatic failures. Gastrointestinal investigations later revealed raised faecal alpha-1 antitrypsin and small bowel ulceration on capsule endoscopy, with a histological diagnosis of Crohn's disease made after a small bowel wedge resection. This case describes the unusual presentation of Crohn's disease displaying symptoms primarily of protein-losing enteropathy, an uncommon and under-recognised consequence of inflammatory bowel disease. A review of current literature and the underlying pathophysiology for this rare condition are discussed, particularly in relation to Crohn's disease.
Topics: Adult; Capsule Endoscopy; Crohn Disease; Digestive System Surgical Procedures; Female; Humans; Intestine, Small; Protein-Losing Enteropathies
PubMed: 34108157
DOI: 10.1136/bcr-2021-242703 -
Ophthalmologica. Journal International... 2020In the last decades, significant changes have been taking place regarding the pathogenesis of diabetic retinopathy (DR) and the complex mechanisms that eventually lead... (Review)
Review
In the last decades, significant changes have been taking place regarding the pathogenesis of diabetic retinopathy (DR) and the complex mechanisms that eventually lead to the various manifestations of the disease, including diabetic macular edema (DME). DR was first considered a pure microvascular disease, due to the evident capillary structural changes (microaneurysms), fluid extravasation, and lipid exudation. With the advent of fundus fluorescein angiography, the concept of ischemia and the correlation between peripheral nonperfusion and neovascularization has been introduced, which was eventually followed by the advent of new therapeutic strategies, such as peripheral photocoagulation. Nowadays, thanks to more advanced imaging techniques, namely optical coherence tomography (OCT), OCT angiography, and wide-field imaging (imaging up to 200° of the retina in a single shot), it became clear that other elements participate in the occurrence of DR and DME, including inflammation and neurodegeneration. In the future, integration of standard investigations with new diagnostic devices would allow the prompt recognition of DR even before clinical signs of the disease are ophthalmoscopically evident, and the development of personalized treatment for both retinopathy and DME will be available.
Topics: Diabetic Retinopathy; Fluorescein Angiography; History, 18th Century; History, 19th Century; History, 20th Century; Humans; Tomography, Optical Coherence
PubMed: 32015239
DOI: 10.1159/000506312 -
European Journal of Case Reports in... 2022Protein-losing enteropathy (PLGE) is an uncommon condition with a multifactorial origin, that is characterized by excessive loss of serum proteins into the...
UNLABELLED
Protein-losing enteropathy (PLGE) is an uncommon condition with a multifactorial origin, that is characterized by excessive loss of serum proteins into the gastrointestinal tract, resulting in hypoproteinaemia and oedema. The authors present the case of a 24-year-old man admitted to hospital for a 2-month history of lower extremity oedema and diarrhoea with a secretory pattern. Blood analysis revealed hypoalbuminaemia and iron deficiency anaemia. Liver disease and severe proteinuria were excluded as possible aetiologies. Upper gastrointestinal endoscopy revealed signs of chronic gastritis. After completion of eradication, the patient had complete resolution of clinical and laboratory abnormalities. The results suggest the need to consider less frequent aetiologies for peripheral oedema and hypoproteinaemia, such as PLGE, especially those caused by prevalent bacterial agents like .
LEARNING POINTS
Protein-losing enteropathy may be related to infection.Protein-losing enteropathy and its associated symptoms may be resolved by eradication.
PubMed: 35774737
DOI: 10.12890/2022_003312 -
Acta Informatica Medica : AIM : Journal... Dec 2022Retinitis pigmentosa (RP) is a set of inherited rod-cone degenerative diseases that clinically presents with similar signs and symptoms. Mutations in one of more than 70... (Review)
Review
BACKGROUND
Retinitis pigmentosa (RP) is a set of inherited rod-cone degenerative diseases that clinically presents with similar signs and symptoms. Mutations in one of more than 70 genes are involved. Patients will commonly present with bone-spicule pigment formation, waxy optic nerve pallor, and attenuated blood vessels in the posterior pole.Symptoms often begin with progressive night blindness, mid-peripheral visual field defects, and eventual tunnel vision. Central vision loss will ultimately occur following loss of rod function. Complete blindness is uncommon.
OBJECTIVE
The aim of this article is to present two cases of retinitis pigmentosa (mother and daughter) trough optalmologic exams in our clinic. The next aim it to show how to menage a low vision service and to treat cystoid macular oedema as a complication of retinitis pigmentosa.
METHODS
All medical reports are shown in this article. Every diagnostic tool as well as report is a part from our archived history of the patients and has been throughly analysed. We also reviewed available literature using the key words retinitis pigmentosa, cystoid macular oedema, gene therapy.
CASE PRESENTATION
A 38 year old female patient for a low vision consultation. The patient was legally blind secondary to retinitis pigmentosa, which was diagnosed in her late 20s. She reported gradually progressive hazy central vision and decreasing peripheral vision in both eyes as well as severe night blindness. Other than the diagnosis of retinitis pigmentosa in both eyes,the patient had no other remarkable ocular conditions. Findings at that visit included unaided distance visual acuities VOD: 0,04 VOS: 0,06. Pupils were round with brisk responses. Extraocular muscle motility was full in both eyes. Confrontation methode visual fields were noted as temporal loss in the right eye and superior and temporal loss in the left eye. The perimetry test could not be performed due to the lack of correspondece of the patient even after a couple repetitions of the perimetry. She had normal ocular adnexa and quiet lids, conjunctiva, and sclera in both eyes. Corneas in both eyes were noted as clear epithelium, clear stroma, and clear endothelium. Anterior chambers had normal depth, iris with no pathological findings in both eyes; lens incipient sclerotic. Intraocular pressures were noted as 22 mmHg in both eyes with Icare, 21mmHg and 19 mmHg with aplanation tonometry; pahimetry corretional factor was +1 on both eyes. The vitreous was clear in both eyes. Both optic nerves were measured as 0.4 cup-to-disc ratios with no disc edema, disc hemorrhages, notching, or thinning noted.Waxy disc pallor and attenuated blood vessels were observed in both eyes. The macula in both eyes had retinal pigment epithelium (RPE) changes with no edema or hemorrhages. Bone spicule changes were noted 360 in the periphery of both eyes with no holes or tears(Figure 1a+1b+1c+1d).
CONCLUSION
We presented two cases of retinitis pigmentosa - the mother with diagnosed RP more than 15 years ago in need for low vision rehabilitation service and the daughter that got diagnosed after our initial examination and with complications in visual impairment through cystoid macular oedema.
PubMed: 36467319
DOI: 10.5455/aim.2022.30.329-333 -
Clinical Journal of the American... Apr 2016Although venous congestion has been linked to renal dysfunction in heart failure, its significance in a broader context has not been investigated.
BACKGROUND AND OBJECTIVES
Although venous congestion has been linked to renal dysfunction in heart failure, its significance in a broader context has not been investigated.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS
Using an inception cohort of 12,778 critically ill adult patients admitted to an urban tertiary medical center between 2001 and 2008, we examined whether the presence of peripheral edema on admission physical examination was associated with an increased risk of AKI within the first 7 days of critical illness. In addition, in those with admission central venous pressure (CVP) measurements, we examined the association of CVPs with subsequent AKI. AKI was defined using the Kidney Disease Improving Global Outcomes criteria.
RESULTS
Of the 18% (n=2338) of patients with peripheral edema on admission, 27% (n=631) developed AKI, compared with 16% (n=1713) of those without peripheral edema. In a model that included adjustment for comorbidities, severity of illness, and the presence of pulmonary edema, peripheral edema was associated with a 30% higher risk of AKI (95% confidence interval [95% CI], 1.15 to 1.46; P<0.001), whereas pulmonary edema was not significantly related to risk. Peripheral edema was also associated with a 13% higher adjusted risk of a higher AKI stage (95% CI, 1.07 to 1.20; P<0.001). Furthermore, levels of trace, 1+, 2+, and 3+ edema were associated with 34% (95% CI, 1.10 to 1.65), 17% (95% CI, 0.96 to 1.14), 47% (95% CI, 1.18 to 1.83), and 57% (95% CI, 1.07 to 2.31) higher adjusted risk of AKI, respectively, compared with edema-free patients. In the 4761 patients with admission CVP measurements, each 1 cm H2O higher CVP was associated with a 2% higher adjusted risk of AKI (95% CI, 1.00 to 1.03; P=0.02).
CONCLUSIONS
Venous congestion, as manifested as either peripheral edema or increased CVP, is directly associated with AKI in critically ill patients. Whether treatment of venous congestion with diuretics can modify this risk will require further study.
Topics: Acute Kidney Injury; Aged; Central Venous Pressure; Critical Illness; Edema; Female; Humans; Male; Middle Aged; Risk Assessment
PubMed: 26787777
DOI: 10.2215/CJN.08080715 -
Frontiers in Cellular Neuroscience 2021Brain edema is a severe stroke complication that is associated with prolonged hospitalization and poor outcomes. Swollen tissues in the brain compromise cerebral... (Review)
Review
Brain edema is a severe stroke complication that is associated with prolonged hospitalization and poor outcomes. Swollen tissues in the brain compromise cerebral perfusion and may also result in transtentorial herniation. As a physical and biochemical barrier between the peripheral circulation and the central nervous system (CNS), the blood-brain barrier (BBB) plays a vital role in maintaining the stable microenvironment of the CNS. Under pathological conditions, such as ischemic stroke, the dysfunction of the BBB results in increased paracellular permeability, directly contributing to the extravasation of blood components into the brain and causing cerebral vasogenic edema. Recent studies have led to the discovery of the glymphatic system and meningeal lymphatic vessels, which provide a channel for cerebrospinal fluid (CSF) to enter the brain and drain to nearby lymph nodes and communicate with the peripheral immune system, modulating immune surveillance and brain responses. A deeper understanding of the function of the cerebral lymphatic system calls into question the known mechanisms of cerebral edema after stroke. In this review, we first discuss how BBB disruption after stroke can cause or contribute to cerebral edema from the perspective of molecular and cellular pathophysiology. Finally, we discuss how the cerebral lymphatic system participates in the formation of cerebral edema after stroke and summarize the pathophysiological process of cerebral edema formation after stroke from the two directions of the BBB and cerebral lymphatic system.
PubMed: 34483842
DOI: 10.3389/fncel.2021.716825 -
Annals of the American Thoracic Society May 2016The clinical significance of peripheral edema has not been well described in critical illness.
RATIONALE
The clinical significance of peripheral edema has not been well described in critical illness.
OBJECTIVES
To assess the clinical significance of peripheral edema detected on physical examination at the time of hospital admission for patients who were treated in an intensive care unit (ICU).
METHODS
Using a large inception cohort of critically ill patients, we examined the association of peripheral edema, as documented on hospital admission physical examination, with hospital and 1-year survival.
MEASUREMENTS AND MAIN RESULTS
Of 12,778 patients admitted to an ICU at a teaching hospital in Boston, Massachusetts, 2,338 (18%) had peripheral edema. Adjusting for severity of illness and comorbidities, including pulmonary edema, admission peripheral edema was associated with a 26% (95% confidence interval [CI] = 1.11-1.44, P < 0.001) higher risk of hospital mortality. In those patients whose peripheral edema could be graded, trace, 1+, 2+, and 3+ admission peripheral edema was associated with a 2% (95% CI = 0.80-1.31, P = 0.89), 17% (95% CI = 1.00-1.56, P = 0.05), 60% (95% CI = 1.26-2.04, P < 0.001), and 54% (95% CI = 1.04-2.29, P = 0.03) higher adjusted risk of hospital mortality, respectively, compared with patients without edema. The association was consistent across strata of patients with diabetes, congestive heart failure, sepsis, and premorbid diuretic or calcium channel blocker use. In a subset of patients with central venous pressures measurements obtained within 6 hours of ICU admission, the highest central venous pressure quartile (>13 cm H2O) was similarly associated with a 35% (95% CI = 1.05-1.75, P = 0.02) higher adjusted risk of hospital mortality compared with the lowest quartile (≤7 cm H2O).
CONCLUSIONS
Peripheral edema, as detected on physical examination at the time of hospital admission, is a poor prognostic indicator in critical illness. Whether peripheral edema simply reflects underlying pathophysiology, or has an independent pathogenic role, will require further interventional studies.
Topics: Aged; Aged, 80 and over; Central Venous Pressure; Critical Illness; Edema; Female; Heart Failure; Hospital Mortality; Hospitals, Teaching; Humans; Intensive Care Units; Logistic Models; Male; Massachusetts; Middle Aged; Monitoring, Physiologic; Patient Admission; Prognosis; Pulmonary Edema; Retrospective Studies; Risk Factors; Sepsis; Severity of Illness Index
PubMed: 26966784
DOI: 10.1513/AnnalsATS.201511-737OC -
Mediterranean Journal of Hematology and... 2023Gilteritinib (XOSPATA®, Astellas) is a type I oral FLT3 inhibitor, a tyrosine kinase AXL inhibitor, involved in both c-Kit and FMS-like tyrosine kinase 3 (FLT3)...
BACKGROUND AND OBJECTIVES
Gilteritinib (XOSPATA®, Astellas) is a type I oral FLT3 inhibitor, a tyrosine kinase AXL inhibitor, involved in both c-Kit and FMS-like tyrosine kinase 3 (FLT3) resistance. In the phase 3 ADMIRAL trial, gilteritinib was compared with the standard of care in (R/R) acute myeloid leukemia (AML) patients who harbored any FLT3 mutation and showed superior efficacy with regard to response and survival.
OBJECTIVES
This research aimed to investigate the real-life efficacy and safety of gilteritinib in FLT3-positive R/R AML patients who were treated as a part of an early access program held in Turkey in April 2020 (NCT03409081).
RESULTS
The research included 17 R/R AML patients who had received gilteritinib from seven centers. The overall response rate was 100%. The most common adverse events were anemia and hypokalemia (7 patients, 41.2%). Grade 4 thrombocytopenia was observed in one patient only (5.9%), leading to permanent treatment discontinuation. Patients with peripheral edema had a 10.47 (95% CI: 1.64-66.82) times higher risk of death than those without peripheral edema (p<0.05).
CONCLUSION
This research showed that patients with febrile neutropenia and peripheral edema were at a high risk of death when compared to patients without febrile neutropenia and peripheral edema.
PubMed: 37180209
DOI: 10.4084/MJHID.2023.031 -
Journal of Neurology Dec 2020Classifying and diagnosing peripheral vestibular disorders based on their symptoms is challenging due to possible symptom overlap or atypical clinical presentation. To... (Review)
Review
BACKGROUND
Classifying and diagnosing peripheral vestibular disorders based on their symptoms is challenging due to possible symptom overlap or atypical clinical presentation. To improve the diagnostic trajectory, gadolinium-based contrast-enhanced magnetic resonance imaging of the inner ear is nowadays frequently used for the in vivo confirmation of endolymphatic hydrops in humans. However, hydrops is visualized in both healthy subjects and patients with vestibular disorders, which might make the clinical value of hydrops detection on MRI questionable.
OBJECTIVE
To investigate the diagnostic value of clinical and radiological features, including the in vivo visualization of endolymphatic hydrops, for the classification and diagnosis of vestibular disorders.
METHODS
A literature search was performed in February and March 2019 to estimate the prevalence of various features in healthy subjects and in common vestibular disorders to make a graphical comparison between healthy and abnormal.
RESULTS
Of the features studied, hydrops was found to be a highly prevalent feature in Menière's disease (99.4%). Though, hydrops has also a relatively high prevalence in patients with vestibular schwannoma (48.2%) and in healthy temporal bones (12.5%) as well. In patients diagnosed with (definite or probable) Menière's disease, hydrops is less frequently diagnosed by magnetic resonance imaging compared to the histological confirmation (82.3% versus 99.4%). The mean prevalence of radiologically diagnosed hydrops was 31% in healthy subjects, 28.1% in patients with vestibular migraine, and 25.9% in patients with vestibular schwannoma. An interesting finding was an absolute difference in hydrops prevalence between the two diagnostic techniques (histology and radiology) of 25.2% in patients with Menière's disease and 29% in patients with vestibular schwannoma.
CONCLUSIONS
Although the visualization of hydrops has a high diagnostic value in patients with definite Menière's disease, it is important to appreciate the relatively high prevalence of hydrops in healthy populations and other vestibular disorders. Endolymphatic hydrops is not a pathognomic phenomenon, and detecting hydrops should not directly indicate a diagnosis of Menière's disease. Both symptom-driven and hydrops-based classification systems have disadvantages. Therefore, it might be worth to explore features "beyond" hydrops. New analysis techniques, such as Radiomics, might play an essential role in (re)classifying vestibular disorders in the future.
Topics: Ear, Inner; Edema; Endolymphatic Hydrops; Gadolinium; Humans; Magnetic Resonance Imaging; Meniere Disease
PubMed: 33201310
DOI: 10.1007/s00415-020-10278-8 -
Cureus Oct 2023Heparin, a mixture of sulfated polymorphic polysaccharides (glycosaminoglycan) chains of variable lengths and weights and a natural anticoagulant, is widely used in... (Review)
Review
Heparin, a mixture of sulfated polymorphic polysaccharides (glycosaminoglycan) chains of variable lengths and weights and a natural anticoagulant, is widely used in medical practice to prevent intravascular blood coagulation. Heparin has demonstrated antithrombotic and anti-inflammatory activity, and it is mostly administered systemically (intravenously or subcutaneously) for primary or secondary prevention of venous thromboembolism after surgical interventions, or immobilized patients, or on short-term antithrombotic therapy of patients with atrial fibrillation who must undergo treatment. However, since systemic administration of heparin could be, in certain cases, linked to an increased risk of bleeding, topical heparin is widely used for the prevention and treatment of local symptoms of peripheral vascular disorders, such as venous insufficiency, varicose veins, or superficial thrombophlebitis. This review summarizes the main safety and efficacy characteristics of the topical formulation of Heparin in Gel form (1000 International Units of Heparin/g Gel) currently in use, which has demonstrated an excellent efficacy and tolerability profile in reducing signs and symptoms of peripheral vascular disease, e.g., varicose syndromes and their complications, phlebothrombosis, thrombophlebitis, superficial periphlebitis, varicose ulcers, for post-operative varicophlebitis, sequelae of saphenectomy, for traumas and contusions, local edemas and infiltrates, subcutaneous hematoma and for traumatic affections of musculotendinous and capsuloligamentous apparatuses.
PubMed: 38022089
DOI: 10.7759/cureus.47418