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PloS One 2023Sickle cell disease (SCD) is a genetic disorder that has been associated with priapism. The role of hydroxyurea, a common SCD therapy, in influencing the nitric oxide...
Sickle cell disease (SCD) is a genetic disorder that has been associated with priapism. The role of hydroxyurea, a common SCD therapy, in influencing the nitric oxide (NO)-cGMP pathway and its effect on priapism is unclear. To investigate the effect of hydroxyurea treatment on smooth muscle relaxation of corpus cavernosum induced by stimulation of the NO-cGMP pathway in SCD transgenic mice and endothelial NO synthase gene-deficient (eNOS-/-) mice, which are used as model of priapism associated with the low bioavailability of endothelial NO. Four-month-old wild-type (WT, C57BL/6), SCD transgenic, and eNOS-/- male mice were treated with hydroxyurea (100 mg/Kg/day) or its vehicle (saline) daily for three weeks via intraperitoneal injections. Concentration-response curves for acetylcholine (ACh), sodium nitroprusside (SNP), and electrical field stimulation (EFS) were generated using strips of mice corpus cavernosum. The SCD mice demonstrated an amplified CC relaxation response triggered by ACh, EFS, and SNP. The corpus cavernosum relaxation responses to SNP and EFS were found to be heightened in the eNOS-/- group. However, the hydroxyurea treatment did not alter these escalated relaxation responses to ACh, EFS, and SNP in the corpus cavernosum of the SCD group, nor the relaxation responses to EFS and SNP in the eNOS-/- group. In conclusion, hydroxyurea is not effective in treating priapism associated with SCD. It is likely that excess plasma hemoglobin and reactive oxygen species, which are reported in SCD, are reacting with NO before it binds to GCs in the smooth muscle of the corpus cavernosum, thus preventing the restoration of baseline NO/cGMP levels. Furthermore, the downregulation of eNOS in the penis may impair the pharmacological action of hydroxyurea at the endothelial level in SCD mice. This study emphasize the urgency for exploring alternative therapeutic avenues for priapism in SCD that are not hindered by high plasma hemoglobin and ROS levels.
Topics: Humans; Mice; Male; Animals; Priapism; Nitric Oxide; Hydroxyurea; Mice, Inbred C57BL; Penis; Nitroprusside; Anemia, Sickle Cell; Mice, Transgenic; Muscle Relaxation; Acetylcholine; Phenotype; Hemoglobins
PubMed: 37812620
DOI: 10.1371/journal.pone.0292706 -
Clinical Case Reports Oct 2023High-flow priapism in pediatric population is rare, yet comprehensive clinical evaluation, along with penile Doppler ultrasound, and cavernosal blood analysis are...
KEY CLINICAL MESSAGE
High-flow priapism in pediatric population is rare, yet comprehensive clinical evaluation, along with penile Doppler ultrasound, and cavernosal blood analysis are crucial for accurate diagnosis. Conservative therapy is effective as an initial treatment.
ABSTRACT
High-flow priapism is considerably rare in the pediatric age group. We report a four-month-old infant presented with a prolonged penile erection. Diagnostic confirmation was achieved through the utilization of Doppler ultrasound and cavernous blood gas analysis. We also review published data on the management of this condition.
PubMed: 37780917
DOI: 10.1002/ccr3.7985 -
Frontiers in Pediatrics 2023Priapism is a urologic emergency requiring prompt management. There are three types of priapism: stuttering (intermittent), non-ischemic (high-flow/arterial), and...
Priapism is a urologic emergency requiring prompt management. There are three types of priapism: stuttering (intermittent), non-ischemic (high-flow/arterial), and ischemic (low-flow/veno-occlusive). Here, we present the first case of an infant with recurrent non-ischemic priapism as the first sign of severe hypertension. An 11-month-old infant was admitted to the hospital for high-flow priapism. On admission, he was found to have severe hypertension that required a combination of five antihypertensive drugs; abdominal ultrasound showed polycystic kidneys, splenomegaly, and a parenchymal liver lesion. The priapism resolved spontaneously and did not recur again after the initiation of antihypertensive treatment. Genetic analysis confirmed autosomal recessive polycystic kidney disease (ARPKD). We found no other explanation for the priapism, such as genital trauma, hematologic disease, or anything else. Decreased nitric oxide (NO) bioavailability seen in patients with hypertension seems to be the principal mechanism of hypertension causing priapism. This hypothesis is supported by animal models of genetically modified mice lacking nitric oxide synthase. The same mechanism is thought to be the genesis of priapism and other complications, such as pulmonary hypertension, in patients with sickle cell disease. We present a case of severe hypertension-associated priapism in a child with unrecognized ARPKD. The endothelial dysfunction with decreased NO bioavailability seen in patients with hypertension may be the principal pathogenic mechanism.
PubMed: 37780053
DOI: 10.3389/fped.2023.1216239 -
Blood Advances Nov 2023Previously, we demonstrated that older children with sickle cell anemia (SCA) living in Nigeria are at increased risk of death if they are underweight (weight-for-age z... (Comparative Study)
Comparative Study
Previously, we demonstrated that older children with sickle cell anemia (SCA) living in Nigeria are at increased risk of death if they are underweight (weight-for-age z score < -1). We now conducted a cross-sectional study in low- and high-income settings to determine the risk factors for being underweight a in children aged 5 to 12 years with SCA. The children from low- and high-income settings were eligible participants for the Primary Prevention of Stroke in Children with Sickle Cell Disease in Nigeria (SPRING; N = 928) and the Silent Cerebral Infarct (SIT, North America/Europe; N = 1093) trials, respectively. The median age in the SPRING and SIT cohorts was 8.1 and 8.5 years, respectively (P < .001). A total of 87.9% (n = 816) of participants in the SPRING trial (low-income) met the study criteria for being underweight (weight-for-age z score < -1), and 22.7% (n = 211) for severely underweight (weight-for-age z score < -3), significantly higher than the SIT (high-income) cohort at 25.7% underweight (n = 281) and 0.7% severely underweight (n = 8; P < .001 for both comparisons). In the combined cohort, older age (odds ratio [OR], 1.24; P < .001) and lower hemoglobin level (OR, 0.67; P < .001) were associated with being underweight. Age and hemoglobin level remained statistically significant in separate models for the SPRING and SIT cohorts. Older age and lower hemoglobin levels in children aged 5 to 12 years with SCA are associated with being underweight in low- and high-income settings.
Topics: Adolescent; Child; Humans; Anemia, Sickle Cell; Cross-Sectional Studies; Developed Countries; Hemoglobins; Risk Factors; Thinness
PubMed: 37756514
DOI: 10.1182/bloodadvances.2023009711 -
Cureus Sep 2023Purpose The purpose of this research is to examine the impact of syringic acid on ischemia-reperfusion injury in cavernosal tissue, utilizing a rat model of induced...
Purpose The purpose of this research is to examine the impact of syringic acid on ischemia-reperfusion injury in cavernosal tissue, utilizing a rat model of induced priapism. Materials and methods A total of 24 rats were allocated into three groups. Group 1 was designated as the control group, while Group 2 underwent ischemia-reperfusion injury assessment using the priapism model. Group 3 underwent the same procedures as Group 2, with the addition of intraperitoneal administration of syringic acid (100 mg/kg) 60 min after priapism initiation. All rats underwent penectomy, and sufficient blood samples were collected. Histopathological assessment of penile cavernosal tissue involved grading tissue damage, inflammation, vasocongestion, desquamation, and edema on a scale of 0-3 (0: normal, 1: mild, 2: moderate, 3: severe). Result Significant differences were observed among the three groups in terms of IL-1 beta and TNF-alpha levels (p=0.001 and p<0.001, respectively). IL-1 beta and TNF-alpha levels in Group 2 were found to be significantly higher than Group 3 (p=0.003 and p=0.004). There was also a significant difference among the three groups in terms of median MDA levels (p<0.001). Furthermore, the median MDA level in Group 2 was found to be significantly higher than that in Group 3 (p<0.001). While significant differences were observed among the three groups in terms of median SOD and GSH-px levels, no significant difference was found among the groups in terms of median PC levels (p=0.004, p= 0.048, and p=0.159, respectively). In direct microscopic examination, a significant improvement in pathological scores was noted in Group 3 compared to Group 2 (p<0.001). Conclusion Syringic acid demonstrated protective properties against ischemia-reperfusion injury caused by priapism in cavernosal tissue.
PubMed: 37745750
DOI: 10.7759/cureus.45475 -
Methods and Protocols Sep 2023In pregnancies complicated by sickle cell disease (SCD), the maternal-fetal dyad is at high risk for mortality and morbidity. In healthy pregnancies, maternal...
Epidemiology of Maternal Nutritional Status and Risk of Adverse Birth Outcomes in Undernourished Mothers with Sickle Cell Disease: A Systematic Review and Meta-Analysis Protocol.
In pregnancies complicated by sickle cell disease (SCD), the maternal-fetal dyad is at high risk for mortality and morbidity. In healthy pregnancies, maternal nutritional status is a critical factor for the healthy growth and development of the fetus. However, there are no reviews of the current research on the nutritional status of pregnant women with SCD and pregnancy outcomes. First, we aim to assess the burden of malnutrition in pregnant women with SCD. Next, we aim to systematically evaluate if pregnant women with SCD who have poor nutritional status are at increased risk for adverse birth outcomes compared to pregnant women with sickle cell disease and normal nutritional status. We will systematically search multiple electronic databases. Our exposure is pregnant women with SCD and poor nutritional status. The primary outcomes of interest include low birth weight (categorical) and birth weight z-scores (continuous). We will also evaluate maternal and perinatal outcomes as secondary outcomes. We will evaluate the risk of bias and overall certainty of evidence with Risk of Bias in Non-randomized Studies-of Interventions (ROBINS-I), and the overall evidence will be assessed using Grading of Recommendation Assessment, Development, and Evaluation (GRADE) criteria. We will pool findings with a meta-analysis if sufficient homogeneity exists among studies. Findings will be published in a peer-reviewed journal and disseminated to SCD advocacy groups. PROSPERO registration number: 429412.
PubMed: 37736971
DOI: 10.3390/mps6050088 -
Urology Case Reports Sep 2023Malignant priapism (MP) is defined as a condition of persistent erection of the penile without sexual stimulation due to malignant cell invasion to the cavernous sinus...
Malignant priapism (MP) is defined as a condition of persistent erection of the penile without sexual stimulation due to malignant cell invasion to the cavernous sinus and the efferent veins. We present a case of a man, 63 years old, with previous history of high-grade renal carcinoma pT3N0M0 had been through radical nephrectomy, diagnosed with MP secondary from metastatic renal carcinoma. The management of this case was aspiration of corpora cavernosa and distal shunting with the Al-Ghorab procedure, then continued to total penectomy and perineostomy.
PubMed: 37719182
DOI: 10.1016/j.eucr.2023.102496 -
Urology Case Reports Nov 2023Corpus cavernosum abscess is a rare condition that can lead to permanent and debilitating consequences. This case reports a 58-year-old man who developed erectile...
Corpus cavernosum abscess is a rare condition that can lead to permanent and debilitating consequences. This case reports a 58-year-old man who developed erectile dysfunction with no response to oral and intracavernous medications after the surgical treatment of a penile abscess.
PubMed: 37701420
DOI: 10.1016/j.eucr.2023.102545 -
Cureus Aug 2023Sickle cell anemia is a hemoglobinopathy that causes complications such as Vaso-Occlusive Crisis (VOC), stroke, priapism, Acute Chest Syndromes (ACS), and bone infarcts... (Review)
Review
Sickle cell anemia is a hemoglobinopathy that causes complications such as Vaso-Occlusive Crisis (VOC), stroke, priapism, Acute Chest Syndromes (ACS), and bone infarcts due to blood vessel occlusion, resulting in hypoxia, ischemia, and inflammation. Preventing these incidents improves the quality of life and lowers mortality rates in Sickle Cell Disease (SCD) patients. This systematic review aims to describe the drugs, their mechanisms of action, dosages, changes in hemoglobin parameters, decrease in VOCs, delay the time for the next VOC, decrease in the length of hospital stay, and side effects associated with these drugs. This review adheres to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) 2020 guidelines. For this review, we searched the PubMed, Google Scholar, and Cochrane databases and screened them for full free texts published in English and studied in humans in the last five years beginning in 2018. Randomized clinical trials (RCT), observational studies, meta-analyses, systemic reviews, and traditional reviews were all included in the search. According to the type of study, quality assessment tools are used, and eight papers are chosen. Full-text articles from these papers are studied, analyzed, and tabulated. We discussed seven interventions that are used to treat sickle cell disease. Voxelotor, crizanlizumab, L-glutamate, long-term blood transfusions, Zinc (Zn), Niprisan®, and Ciklavit* were found to reduce the number and severity of VOC. We discovered that VOCs containing L -glutamate reduced the length of hospitalization. Magnesium (Mg) did not affect the number and severity of VOCs. This review includes a few articles for the study. Future papers on this subject should include a large sample size and many papers. More clinical trials are required to evaluate the dosages and outcomes of using these drugs in combination to prevent VOCs.
PubMed: 37664256
DOI: 10.7759/cureus.42785