scholarly journals Using cyproterone acetate to treat recurrent ischemic priapism in a patient with sickle cell anemia as a comorbidity: a case report

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Ali Alshahrani
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cyproterone Acetate ◽  
Structural Damage ◽  
Successful Management ◽  
Case Presentation ◽  
Ischemic Priapism ◽  
Preventative Strategy ◽  
History Of ◽  
Contemporary Practice

Abstract Introduction The management of recurrent ischemic priapism is unclear in contemporary practice. Yet, if left untreated, the condition may evolve into an acute ischemic priapism and in some cases result in erectile dysfunction. This report documents the results of successful management of recurrent ischemic priapism using cyproterone acetate in a 30-year-old Saudi man with sickle cell anemia as a comorbidity. Case presentation A 30-year-old Saudi man denoted visited the emergency room with a painful erection which had lasted for more than four hours. The patient has sickle cell anemia and a family history of sickle cell disease. He is married and has two children. His first priapism case occurred when he was 7 years old. At the age of 15, the condition recurred, and the patient’s doctor prescribed cyproterone acetate 50 mg twice daily for 5 days. The doctor had told him that whenever he was experiencing priapism, he should adhere to this regimen for 5 days. The doctor could not find any guidelines for the prescription of cyproterone acetate. Conclusion Priapism cases represent a significant challenge in therapeutic management because of the elevated risk of structural damage to the penis. The fact that there lacks a clinically approved standard approach to managing the condition make it difficult for physicians to effectively manage the condition. Management of the condition is further complicated by existence of comorbidities such as sickle cell anemia. This patient’s case demonstrates that cyproterone acetate prescription is a great preventative strategy that limits priapism recurrences.

scholarly journals Bilateral Hydronephrosis with Grade 3 To 4 Vesicoureteral Reflux

2021 ◽  
pp. 121-124
Author(s):  
Mayur B. Wanjari ◽  
Hina Rodge ◽  
Deeplata Mendhe ◽  
Pratibha Wankhede ◽  
Sampada Late
Keyword(s):  
Developmental Delay ◽  
Vesicoureteral Reflux ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Renal Pelvis ◽  
Female Child ◽  
Case Presentation ◽  
Bilateral Hydronephrosis ◽  
Grade 3

Introduction: Bilateral hydronephrosis is the enlargement of the parts of the kidney that collect urine. Bilateral means both sides. Bilateral hydronephrosis occurs when urine is unable to drain from the kidney into the bladder. Hydronephrosis is not itself a disease. It occurs as a result of a problem that prevents urine from draining out of the kidneys, ureters, and bladder. VUR grade 3 is similar to grade 2 where urine travels up the ureter and enters the part of the kidney where urine is collected before it drains to the ureter (renal pelvis). However, in grade 3 the ureters and renal pelvis appear abnormal in size or shape. Case Presentation: A 3 Years old female child is a known case of bilateral vesicoureteric reflex with bilateral hydroureteronephrosis with the developmental delay with sickle cell anemia came to the hospital for further management. As narrated by mother-child was apparently alright till 6 months of age after the child developed excessive passing of urine and in increased more times. Conclusion: After taking treatment for the bilateral vesicoureteric reflex with bilateral hydroureteronephrosis patient was hemodynamically stable hence the patient is being discharged.

Hydroxyurea corrects the dysregulated L-selectin expression and increased H2O2 production of polymorphonuclear neutrophils from patients with sickle cell anemia

Blood ◽  
2002 ◽  
Vol 99 (7) ◽  
pp. 2297-2303 ◽  
Author(s):  
Malika Benkerrou ◽  
Charlotte Delarche ◽  
Lamia Brahimi ◽  
Michèle Fay ◽  
Etienne Vilmer ◽  
...  
Keyword(s):  
Adhesion Molecules ◽  
Sickle Cell ◽  
Adhesion Molecule ◽  
Sickle Cell Anemia ◽  
Plasma Levels ◽  
Polymorphonuclear Neutrophil ◽  
H2o2 Production ◽  
Soluble Adhesion Molecules ◽  
Hydroxyurea Treatment ◽  
History Of

Impaired polymorphonuclear neutrophil (PMN) functions during sickle cell anemia (SCA) may have a pathogenic role in the onset of vasoocclusive events. We used flow cytometry to study, in whole blood, the adhesion molecule expression and respiratory burst of PMNs from children with SCA. Three different clinical groups were studied: (1) patients with no history of vasoocclusive events (n = 15); (2) patients with a history of vasoocclusive events (n = 17); and (3) patients receiving hydroxyurea therapy for severe vasoocclusive events (n = 9). Unstimulated PMNs showed decreased L selectin expression and increased H2O2 production whatever the severity of the disease, reflecting PMN activation. This could contribute to endothelial activation reflected by abnormal plasma levels of soluble adhesion molecules (soluble intercellular adhesion molecule-1, sE selectin, and sL selectin). After stimulation with bacterial N-formyl peptides (N-formyl-methionyl-leucyl-phenylalanine [fMLP]), PMNs from untreated patients with a history of vasoocclusive events showed dysregulated L selectin shedding and increased H2O2 production. Furthermore, in these patients, tumor necrosis factor priming followed by fMLP stimulation induced an H2O2 production significantly higher than in the other patient groups and controls. These impairments could immobilize PMNs on the endothelium, thereby inducing reduced blood flow and fostering microvascular occlusion and vascular damage. In contrast, children treated with hydroxyurea showed near-normal basal and poststimulation H2O2 production as well as normal L selectin shedding after stimulation but no change in plasma levels of soluble adhesion molecules. To our knowledge, this is the first report showing major qualitative changes of PMN abnormalities upon hydroxyurea treatment in SCA patients. This strongly suggests that PMNs are a primary target of this drug.

scholarly journals Sickle Cell Anemia Mediates Carotid Artery Expansive Remodeling That Can Be Prevented by Inhibition of JNK (c-Jun N-Terminal Kinase)

2020 ◽  
Vol 40 (5) ◽  
pp. 1220-1230 ◽  
Author(s):  
Hannah Song ◽  
Philip M. Keegan ◽  
Suhaas Anbazhakan ◽  
Christian P. Rivera ◽  
Yundi Feng ◽  
...  
Keyword(s):  
Carotid Artery ◽  
Mouse Model ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Structural Damage ◽  
Proteolytic Enzymes ◽  
Arterial Compliance ◽  
Cathepsin K ◽  
Large Artery ◽  
Elastic Lamina

Objective: Sickle cell anemia (SCA) causes chronic inflammation and multiorgan damage. Less understood are the arterial complications, most evident by increased strokes among children. Proteolytic mechanisms, biomechanical consequences, and pharmaceutical inhibitory strategies were studied in a mouse model to provide a platform for mechanistic and intervention studies of large artery damage due to sickle cell disease. Approach and Results: Townes humanized transgenic mouse model of SCA was used to test the hypothesis that elastic lamina and structural damage in carotid arteries increased with age and was accelerated in mice homozygous for SCA (sickle cell anemia homozygous genotype [SS]) due to inflammatory signaling pathways activating proteolytic enzymes. Elastic lamina fragmentation observed by 1 month in SS mice compared with heterozygous littermate controls (sickle cell trait heterozygous genotype [AS]). Positive immunostaining for cathepsin K, a powerful collagenase and elastase, confirmed accelerated proteolytic activity in SS carotids. Larger cross-sectional areas were quantified by magnetic resonance angiography and increased arterial compliance in SS carotids were also measured. Inhibiting JNK (c-jun N-terminal kinase) signaling with SP600125 significantly reduced cathepsin K expression, elastin fragmentation, and carotid artery perimeters in SS mice. By 5 months of age, continued medial thinning and collagen degradation was mitigated by treatment of SS mice with JNK inhibitor. Conclusions: Arterial remodeling due to SCA is mediated by JNK signaling, cathepsin proteolytic upregulation, and degradation of elastin and collagen. Demonstration in Townes mice establishes their utility for mechanistic studies of arterial vasculopathy, related complications, and therapeutic interventions for large artery damage due to SCA.

scholarly journals Subacute Thyroiditis from COVID-19 Infection: A Case Report and Review of Literature

2020 ◽  
pp. 1-5
Author(s):  
Akshay Khatri ◽  
Esti Charlap ◽  
Angela Kim
Keyword(s):  
Clinical Features ◽  
Clinical Laboratory ◽  
Early Recognition ◽  
Subacute Thyroiditis ◽  
The Novel ◽  
Review Of Literature ◽  
Successful Management ◽  
Case Presentation ◽  
Radiologic Findings ◽  
History Of

<b><i>Introduction:</i></b> The novel severe-acute-respiratory-syndrome-coronavirus-2 (SARS-CoV-2) virus has led to the ongoing Coronavirus disease 2019 (COVID-19) disease pandemic. There are increasing reports of extrapulmonary clinical features of COVID-19, either as initial presentations or sequelae of disease. We report a patient diagnosed with subacute thyroiditis precipitated by COVID-19 infection, as well as review the literature of similar cases. <b><i>Case Presentation:</i></b> A 41-year-old female with no significant personal or family history of endocrinologic disorders presented with clinical features of thyroiditis that began after COVID-19 infection. Clinical, laboratory, and radiologic findings were indicative of subacute thyroiditis. Workup for potential triggers other than SARS-CoV-2 was negative. <b><i>Discussion/Conclusion:</i></b> We compared the clinical and diagnostic findings of our patient with other well-documented cases of subacute thyroiditis presumed to be triggered by SARS-CoV-2 viral infection. We also reviewed the literature related to the potential mechanisms leading to thyroiditis. Clinicians must be aware of the possibility of thyroid dysfunction after COVID-19 infection. Early recognition and timely anti-inflammatory therapy help in successful management.

scholarly journals Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies

Anemia ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Genevieve M. Crane ◽  
Nelson E. Bennett
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Treatment Strategies ◽  
Vascular Complications ◽  
Cell Disease ◽  
Nitric Oxide Signaling ◽  
Risk Of Death ◽  
Ischemic Priapism

Sickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunction. The severity of sickle cell disease manifestations is variable dependent on a number of contributing genetic factors; however, priapism tends to cluster with other severe vascular complications including pulmonary hypertension, leg ulceration, and overall risk of death. The mechanisms underlying priapism in sickle cell disease have begun to be elucidated including hemolysis-mediated dysregulation of the nitric oxide signaling pathway and dysregulation of adenosine-mediated vasodilation. A better understanding of these mechanisms is leading toward novel preventative strategies. This paper will focus on the mechanisms underlying development of ischemic priapism in sickle cell disease, current acute and preventative treatment strategies, and future directions for improved management of this disorder.

Correlation of Iron Levels with Asthma and Lung Function in Pediatric Patients with Sickle Cell Anemia

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 12-12
Author(s):  
Yusra D Shaikh ◽  
Nataly Apollonsky ◽  
Bruce Bernstein
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Serum Iron ◽  
Pediatric Patients ◽  
Pulmonary Complications ◽  
Acute Chest Syndrome ◽  
Iron Level ◽  
Obstructive Sleep ◽  
History Of ◽  
Hb S

Introduction:Significant morbidity and mortality in patients with sickle cell disease (SCD) is attributed to the pulmonary sequalae of the disease. Patients with SCD often suffer airway hyper-reactivity, acute chest syndrome (ACS), chronic lung disease, pulmonary hypertension (PHTN), and obstructive sleep apnea (OSA). Recent literature has provided evidence supporting the strong association between asthma and airway hyper-reactivity in SCD. One of the factors linked to chronic inflammation and asthma is iron status. The present study examined whether iron levels are associated with pulmonary complications in pediatric patients with SCD. Method:Through retrospective review of electronic medical records (EMR) we evaluated patients with diagnosis of asthma and SCD. All patients with available PFT (3/21/2013-3/11/2020) and iron studies were included in the analysis. Chi square and ANOVA tests were used to explore relationships of respiratory conditions with lab data and relevant medical history. Results:The analysis reviewed information of 100 patients with SCD -- 56 males and 44 females The sample population had the following genotypes: 63% Hemoglobin (Hb) SS, 23% Hb SC, 2% Hb S Beta Zero Thalassemia, and 12% Hb S Beta Thalassemia. 38% of these patients were receiving treatment via hydroxyurea. The results generated found that patients with a large airway obstruction (LAO) had a marginally statistically significantly higher serum iron level than those with no LAO (p=0.067.) Patients with homozygous Hb S disease were four times as likely to have a history of ACS (p=0.004) than those without and were marginally significantly more likely to be SS and SB0Thal (p=0.052). Patients with history of ACS had a significantly higher mean iron saturation and lower total iron binding capacity (TIBC.) Patients with PHTN had significantly higher serum iron levels (p=0.029). Conclusion:Our findings reveal that while iron might play a more significant role in the development of PHTN and ACS in patients with SCD, the role in asthma is borderline in our sample. These findings, although of borderline statistical significance p=0.067, are clinically noteworthy. These results may open a new window for therapy targeted at maintaining iron in normal physiologic ranges to decrease pulmonary complications in patients with sickle cell anemia. Further studies with larger samples are necessary to clarify the meaning of our marginally significant findings. Disclosures No relevant conflicts of interest to declare.

Transcranial Doppler in Sickle Cell Anemia Adult Patients: Brazilian Experience.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3736-3736
Author(s):  
Gisele S. Silva ◽  
Maria S. Figueiredo ◽  
Perla Vicari ◽  
Airton R. Massaro ◽  
Adauto Castelo Filho ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Stroke Risk ◽  
Arterial Disease ◽  
Neurological Complications ◽  
Adult Patients ◽  
History Of ◽  
The Mean

Abstract Sickle cell anemia (SCA) may cause a variety of neurological complications, including stroke and headaches. Stroke occurs in up to 9% of children with SCA, and transcranial Doppler (TCD) studies have demonstrated that increased velocities are related to higher stroke risk. Throbbing headache occurs in SCA but its cause, frequency, and relationship to TCD velocities have received little attention. On the other hand, there are few TCD studies in adult patients. Our aims were: 1) to describe the main features of TCD in adult SCA patients, and 2) to investigate if there were correlation between TCD features and presence of headache. TCD was performed in 56 adult SCA patients (≥ 16 years old) and in 56 healthy individuals (HI), matched by age and race. There were 6 patients with a remote history of stroke but none were on chronic transfusion. The SCA group was submitted to a neurological evaluation and specifically asked about the occurrence of headache and its characteristics. The highest flow velocity (maxFV) recorded for each artery was considered the most representative. We analyzed the frequency of FV asymmetry (side-to-side difference > 20%) and focal FV changes. The mean maxFV was significantly higher in patients (117.7 ± 21.6 cm/s) than in HI (72.45 ± 11.48 cm/s) (p<0.005). Only one patient had maxFV higher than 170 cm/s. The frequencies of asymmetry and of focal FV changes were significantly higher in SCA. Forty-one patients (73.2%) reported having headaches. Twenty-eight patients (50%) had severe (= 5 for pain intensity at a 1–10 scale) and frequent headaches (at least once a month). This group of patients presented TCD velocities significantly higher than patients without or with milder headaches (p=0.035). In conclusion, TCD maxFV was significantly higher in adult patients with SCA than HI, however, only one patient was considered at risk of stroke according to TCD criteria described in children. FV asymmetry and focal FV changes may be markers for arterial disease in adult SCA patients, and need to be further confirmed by neuroimaging and clinical follow up studies. The patients with severe headaches presented TCD velocities significantly higher than patients without or with milder headaches, but this finding needs to be confirmed by more and larger studies.

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