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American Journal of Hematology Dec 2014A 21 year old male student presented in 1980 as an Olympic athlete with a 12 year history of jaundice, pallor, and darkened urine induced by the atraumatic exercise of...
A 21 year old male student presented in 1980 as an Olympic athlete with a 12 year history of jaundice, pallor, and darkened urine induced by the atraumatic exercise of swimming (1). Physical examination at that time was remarkable only for moderate scleral icterus without hepatosplenomegaly. Hematological examination revealed moderate macrocytosis (MCV 102 fL) without anemia (Hct 50%, Hb 17 g/dL, 9% reticulocytes). The peripheral blood smear showed occasional target cells. Red cell osmotic fragility was decreased. Red cell Na content was increased and K content was decreased, with reduced total monovalent ion content. Passive red cell permeability of both Na and K were increased. A supervised 2.5 hr swimming workout increased free plasma Hb from <5 to 45 mg/dL and decreased serum haptoglobin from 25 to 6 mg/dL. The post-exercise urine sediment was remarkable for hemosiderin-laden tubular epithelial cells, without frank hemoglobinuria. The circulating 15 day erythrocyte half-life measured after 6 days without exercise was further shortened to 12 days after resumption of twice-per-day swimming workouts for 1 week. The patient’s red cells were hypersensitive to shear stress applied by cone-plate viscometer.
Topics: Adolescent; Adult; Adult Children; Anemia, Hemolytic, Congenital; Erythrocytes; Glucuronosyltransferase; Hemochromatosis Protein; Hepatocytes; Heterozygote; Histocompatibility Antigens Class I; Humans; Hydrops Fetalis; Ion Channels; Liver; Male; Membrane Proteins; Middle Aged; Mutation; Osmotic Fragility; Pedigree
PubMed: 25044010
DOI: 10.1002/ajh.23799 -
Current Opinion in Rheumatology Jul 2017The Assessment of Spondyloarthritis International Society (ASAS) axial spondyloarthritis (axSpA) classification criteria marked a major step forward in SpA research,... (Review)
Review
PURPOSE OF REVIEW
The Assessment of Spondyloarthritis International Society (ASAS) axial spondyloarthritis (axSpA) classification criteria marked a major step forward in SpA research, distinguishing axial from peripheral disease, and allowing earlier identification through MRI. This facilitated all aspects of research including epidemiology, therapeutics and patient outcomes.
RECENT FINDINGS
The ASAS axSpA classification criteria have been applied broadly in research, and were validated in a recent meta-analysis of international studies. Concerns arose because of clinical differences between the clinical and imaging arms, which imply different risk for radiographic progression, and perform differently in validation studies. Low specificity of the MRI finding of sacroiliac joint bone marrow edema may lead to misclassification in populations with low axSpA prevalence. We suggest methodology to improve upon the criteria, including rigorous assessment of potential candidate criteria sets, discrete choice experiments to allow consideration of feature weights, and validation. Separately, assessment of structural and inflammatory MRI abnormalities should be performed to refine the MRI definition of sacroiliitis.
SUMMARY
The debate regarding the validation and modification of the ASAS axSpA classification criteria should lead to international efforts to build upon the gains made by these criteria, to further refine the axSpA population definitions for research and ultimately improve patient outcomes.
Topics: Bone Marrow; Disease Progression; Edema; Humans; Magnetic Resonance Imaging; Prevalence; Sacroiliac Joint; Sacroiliitis; Sensitivity and Specificity; Societies, Medical; Spondylarthritis; Spondylarthropathies; Spondylitis, Ankylosing
PubMed: 28376062
DOI: 10.1097/BOR.0000000000000402 -
The Journal of Pharmacology and... Jan 2021Pharmacological openers of ATP-sensitive potassium (K) channels are effective antihypertensive agents, but off-target effects, including severe peripheral edema, limit...
Pharmacological openers of ATP-sensitive potassium (K) channels are effective antihypertensive agents, but off-target effects, including severe peripheral edema, limit their clinical usefulness. It is presumed that the arterial dilation induced by K channel openers (KCOs) increases capillary pressure to promote filtration edema. However, K channels also are expressed by lymphatic muscle cells (LMCs), raising the possibility that KCOs also attenuate lymph flow to increase interstitial fluid. The present study explored the effect of KCOs on lymphatic contractile function and lymph flow. In isolated rat mesenteric lymph vessels (LVs), the prototypic K channel opener cromakalim (0.01-3 µmol/l) progressively inhibited rhythmic contractions and calculated intraluminal flow. Minoxidil sulfate and diazoxide (0.01-100 µmol/l) had similar effects at clinically relevant plasma concentrations. High-speed in vivo imaging of the rat mesenteric lymphatic circulation revealed that superfusion of LVs with cromakalim and minoxidil sulfate (0.01-10 µmol/l) maximally decreased lymph flow in vivo by 38.4% and 27.4%, respectively. Real-time polymerase chain reaction and flow cytometry identified the abundant K channel subunits in LMCs as the pore-forming Kir6.1/6.2 and regulatory sulfonylurea receptor 2 subunits. Patch-clamp studies detected cromakalim-elicited unitary K currents in cell-attached patches of LMCs with a single-channel conductance of 46.4 pS, which is a property consistent with Kir6.1/6.2 tetrameric channels. Addition of minoxidil sulfate and diazoxide elicited unitary currents of similar amplitude. Collectively, our findings indicate that KCOs attenuate lymph flow at clinically relevant plasma concentrations as a potential contributing mechanism to peripheral edema. SIGNIFICANCE STATEMENT: ATP-sensitive potassium (K) channel openers (KCOs) are potent antihypertensive medications, but off-target effects, including severe peripheral edema, limit their clinical use. Here, we demonstrate that KCOs impair the rhythmic contractions of lymph vessels and attenuate lymph flow, which may promote edema formation. Our finding that the K channels in lymphatic muscle cells may be unique from their counterparts in arterial muscle implies that designing arterial-selective KCOs may avoid activation of lymphatic K channels and peripheral edema.
Topics: Action Potentials; Animals; Cells, Cultured; Cromakalim; Diazoxide; Edema; KATP Channels; Lymphatic Vessels; Male; Minoxidil; Muscle Contraction; Myocytes, Smooth Muscle; Potassium; Rats; Rats, Sprague-Dawley
PubMed: 33100270
DOI: 10.1124/jpet.120.000121 -
Medicine Sep 2022To elucidate the relationship between peripheral edema and programmed cell death-1/programmed cell death ligand 1 (PD-1/PD-L1) inhibitors, the meta-analysis was... (Meta-Analysis)
Meta-Analysis
PURPOSE
To elucidate the relationship between peripheral edema and programmed cell death-1/programmed cell death ligand 1 (PD-1/PD-L1) inhibitors, the meta-analysis was performed.
METHOD
Following the guidelines of Preferred Reporting Items for Systematic Reviews and Meta-analyses, all-grade and grade 3-5 of peripheral edema data extracted from clinical trials were taken into account for the final comprehensive assessments.
RESULTS
Twenty-seven PD-1/PD-L1-related clinical trials with peripheral edema data were collected. Compared with chemotherapy (PD-1/PD-L1 vs chemotherapy), the risk of developing peripheral edema for all-grade was much lower (odds ratio [OR] = 0.36, 95% confidence interval [CI]: [0.23, 0.56], Z = 4.55 [P < .00001]). When PD-1/PD-L1 plus chemotherapy were compared with chemotherapy, no significant analysis results for all-grade was found (OR = 1.15, 95% CI:[0.93, 1.44], I2 = 25%, Z = 1.27 [P = .20]). Similar risk trends could also be found when the incidence risk of peripheral edema for grade 3-5 was evaluated. No obvious publication bias was identified throughout the total analysis process.
CONCLUSION
The effect of PD-1/PD-L1 inhibitor on the risk of developing peripheral edema was weaker than that of chemotherapy, and the combination with chemotherapy slightly increased the incidence risk of developing peripheral edema without statistical significance.
Topics: B7-H1 Antigen; Edema; Humans; Immune Checkpoint Inhibitors; Incidence; Neoplasms; Programmed Cell Death 1 Receptor
PubMed: 36086680
DOI: 10.1097/MD.0000000000030151 -
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 -
Postgraduate Medical Journal Jul 1986A 35 year old markedly underweight woman presented with uncontrolled diabetes. Following insulin therapy she developed gross fluid retention with extensive peripheral...
A 35 year old markedly underweight woman presented with uncontrolled diabetes. Following insulin therapy she developed gross fluid retention with extensive peripheral oedema, bilateral pleural effusions and weight gain of 18.8 kg in 22 days, accompanied by a fall in plasma albumin. She responded well to treatment with diuretics and salt-poor albumin, losing 10.3 kg in 6 days without recurrence of oedema. Severe insulin oedema is an uncommon complication of insulin therapy and may be due to effects of insulin on both vascular permeability and the renal tubule.
Topics: Adult; Edema; Female; Humans; Insulin
PubMed: 3529068
DOI: 10.1136/pgmj.62.729.665 -
Clinical Cardiology Jan 2006The purpose of this study was to determine whether baseline physical examination and history are useful in identifying patients with cardiac edema as defined by...
BACKGROUND
The purpose of this study was to determine whether baseline physical examination and history are useful in identifying patients with cardiac edema as defined by echocardiography, and to compare survival for patients with cardiac and noncardiac causes of edema.
HYPOTHESIS
Physical examination and history data can help to identify patients with edema who have significant cardiac disease.
METHODS
We reviewed the medical records of 278 consecutive patients undergoing echocardiography for evaluation of peripheral edema. We classified cardiac edema as the presence of any of the following: left ventricular ejection fraction < 45%, systolic pulmonary artery pressure > 45 mmHg, reduced right ventricular function, enlarged right ventricle, and a dilated inferior vena cava.
RESULTS
The mean age of the 243 included patients was 67 +/- 12 years and 92% were male. A cardiac cause of edema was found in 56 (23%). Independent predictors of a cardiac cause of edema included chronic obstructive pulmonary disease (COPD, odds ratio [OR] 1.74, 95% confidence interval [CI] 1.14-2.60) and crackles (OR 1.98, 95% CI 1.26-3.10). The specificity for a cardiac cause of edema was high (91% for COPD, 93% for crackles); however, the sensitivity was quite low (27% for COPD, for 24% crackles). Compared with patients without a cardiac cause of edema, those with a cardiac cause had increased mortality (25 vs. 8% at 2 years, p < 0.01), even after adjustment for other characteristics (hazard ratio 1.55, 95% CI 1.08-2.24).
CONCLUSIONS
A cardiac cause of edema is difficult to predict based on history and examination and is associated with high mortality.
Topics: Aged; Chi-Square Distribution; Echocardiography; Edema; Female; Heart Diseases; Humans; Logistic Models; Male; Predictive Value of Tests; Proportional Hazards Models; Survival Analysis
PubMed: 16477775
DOI: 10.1002/clc.4960290108 -
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 -
European Radiology May 2023To evaluate extensor carpi ulnaris (ECU) tendon pathology and ulnar styloid process bone marrow edema (BME) as diagnostic MRI markers for peripheral triangular...
Extensor carpi ulnaris tendon pathology and ulnar styloid bone marrow edema as diagnostic markers of peripheral triangular fibrocartilage complex tears on wrist MRI: a case-control study.
OBJECTIVES
To evaluate extensor carpi ulnaris (ECU) tendon pathology and ulnar styloid process bone marrow edema (BME) as diagnostic MRI markers for peripheral triangular fibrocartilage complex (TFCC) tears.
METHODS
One hundred thirty-three patients (age range 21-75, 68 females) with wrist 1.5-T MRI and arthroscopy were included in this retrospective case-control study. The presence of TFCC tears (no tear, central perforation, or peripheral tear), ECU pathology (tenosynovitis, tendinosis, tear or subluxation), and BME at the ulnar styloid process were determined on MRI and correlated with arthroscopy. Cross-tabulation with chi-square tests, binary logistic regression with odds ratios (OR), and sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were used to describe diagnostic efficacy.
RESULTS
On arthroscopy, 46 cases with no TFCC tear, 34 cases with central perforations, and 53 cases with peripheral TFCC tears were identified. ECU pathology was seen in 19.6% (9/46) of patients with no TFCC tears, in 11.8% (4/34) with central perforations and in 84.9% (45/53) with peripheral TFCC tears (p < 0.001); the respective numbers for BME were 21.7% (10/46), 23.5% (8/34), and 88.7% (47/53) (p < 0.001). Binary regression analysis showed additional value from ECU pathology and BME in predicting peripheral TFCC tears. The combined approach with direct MRI evaluation and both ECU pathology and BME yielded a 100% positive predictive value for peripheral TFCC tear as compared to 89% with direct evaluation alone.
CONCLUSIONS
ECU pathology and ulnar styloid BME are highly associated with peripheral TFCC tears and can be used as secondary signs to diagnose tears.
KEY POINTS
• ECU pathology and ulnar styloid BME are highly associated with peripheral TFCC tears and can be used as secondary signs to confirm the presence of TFCC tears. • If there is a peripheral TFCC tear on direct MRI evaluation and in addition both ECU pathology and BME on MRI, the positive predictive value is 100% that there will be a tear on arthroscopy compared to 89% with direct evaluation alone. • If there is no peripheral TFCC tear on direct evaluation and neither ECU pathology nor BME on MRI, the negative predictive value is 98% that there will be no tear on arthroscopy compared to 94% with direct evaluation alone.
Topics: Tendons; Radius; Bone Marrow Diseases; Magnetic Resonance Imaging; Edema; Biomarkers; Triangular Fibrocartilage; Case-Control Studies; Wrist Injuries; Wrist Joint; Humans; Male; Female; Young Adult; Adult; Middle Aged; Aged; Sensitivity and Specificity; Reproducibility of Results; Retrospective Studies; Rupture
PubMed: 36809434
DOI: 10.1007/s00330-023-09446-x -
Prilozi (Makedonska Akademija Na... Jul 2023: Insulin induced edema (IIE) is a rare condition, usually found in newly diagnosed diabetes patients, either after insulin treatment initiation or after dose increment....
: Insulin induced edema (IIE) is a rare condition, usually found in newly diagnosed diabetes patients, either after insulin treatment initiation or after dose increment. It is a self-limited process, rarely associated with serosal effusions. Teenage girls with type 1 diabetes (T1DM) are most commonly affected. : A 12-year-old girl was diagnosed with ketoacidosis (DKA). Seven days after initiation of the insulin treatment, at a stable total daily dose of insulin (TDDI) of 0.55 IU/kg, she came with two kilograms weight gain in only two days and edema of the feet and calves. Ultrasound of the heart found a 7 mm pericardial effusion. The diagnostic workout included clinical examination, biochemical, hormonal, allergen analyses and imaging which excluded other known causes of swelling. : We describe an adolescent girl with newly diagnosed T1DM and a rare association of peripheral insulin-induced edema and pericardial effusion. Short-term diuretic treatment and salt restriction resolved this rare complication of insulin treatment.
Topics: Female; Adolescent; Humans; Child; Insulin; Diabetes Mellitus, Type 1; Pericardial Effusion; Edema; Weight Gain
PubMed: 37453114
DOI: 10.2478/prilozi-2023-0037