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Clinical Case Reports May 2024This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the...
This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the priapism but induced a significant vaso-occlusive crisis during the second infusion. Encouragingly, no subsequent vaso-occlusive crises occurred. However, the potential for infusion-related adverse events warrants close supervision. Further research is necessary to explore its full benefits on priapism management.
PubMed: 38736574
DOI: 10.1002/ccr3.8585 -
Pediatric Research Feb 2024
Topics: Child; Humans; United States; Quality of Health Care; Delivery of Health Care; Prisoners
PubMed: 37468718
DOI: 10.1038/s41390-023-02739-3 -
Cureus Feb 2024The case presented is of a 39-year-old male with severe right groin pain and perineal pain the morning after sexual intercourse with the use of sildenafil without a...
The case presented is of a 39-year-old male with severe right groin pain and perineal pain the morning after sexual intercourse with the use of sildenafil without a diagnosis of erectile dysfunction. Partial segmental thrombosis of the corpus cavernosum (PSTCC) was diagnosed using magnetic resonance imaging and treated with direct oral anticoagulation without complications. Sildenafil use has been noted as an inciting factor for PSTCC in only two other cases of less than 60 cases reported in the literature and has even been used successfully as a component of therapeutic management of PSTCC in another previous case.
PubMed: 38435230
DOI: 10.7759/cureus.53462 -
Blood Advances Feb 2024Preliminary evidence from a series of 4 adults with sickle cell disease (SCD) suggests that hematopoietic stem cell transplant (HSCT) improves cerebral hemodynamics....
Preliminary evidence from a series of 4 adults with sickle cell disease (SCD) suggests that hematopoietic stem cell transplant (HSCT) improves cerebral hemodynamics. HSCT largely normalizes cerebral hemodynamics in children with SCD. We tested the hypothesis in adults with SCD that cerebral blood flow (CBF), oxygen extraction fraction (OEF), and cerebral metabolic rate of oxygen (CMRO2) measured using magnetic resonance imaging, normalized to healthy values, comparing measurements from ∼1 month before to 12 to 24 months after HSCT (n = 11; age, 33.3 ± 8.9 years; 389 ± 150 days after HSCT) with age-, race- and sex-matched values from healthy adults without sickle trait (n = 28; age, 30.2 ± 5.6 years). Before transplant, 7 patients had neurological indications for transplant (eg, overt stroke) and 4 had nonneurological reasons for haploidentical bone marrow transplant (haplo-BMT). All received haplo-BMT from first-degree relatives (parent, sibling, or child donor) with reduced-intensity preparation and maintained engraftment. Before transplant, CBF was elevated (CBF, 69.11 ± 24.7 mL/100 g/min) compared with that of controls (P = .004). Mean CBF declined significantly after haplo-BMT (posttransplant CBF, 48.2 ± 13.9 mL/100 g/min; P = .003). OEF was not different from that of controls at baseline and did not change significantly after haplo-BMT (pretransplant, 43.1 ± 6.7%; posttransplant, 39.6 ± 7.0%; P = .34). After transplant, CBF and OEF were not significantly different from controls (CBF, 48.2 ± 13.4 mL/100 g/min; P = .78; and OEF, 39.6 ± 7.0%; P > .99). CMRO2 did not change significantly after haplo-BMT (pretransplant, 3.18 ± 0.87 mL O2/100 g/min; posttransplant, 2.95 ± 0.83; P = .56). Major complications of haplo-BMT included 1 infection-related death and 1 severe chronic graft-versus-host disease. Haplo-BMT in adults with SCD reduces CBF to that of control values and maintains OEF and CMRO2 on average at levels observed in healthy adult controls. The trial was registered at www.clinicaltrials.gov as #NCT01850108.
Topics: Adult; Child; Humans; Young Adult; Bone Marrow Transplantation; Anemia, Sickle Cell; Hematopoietic Stem Cell Transplantation; Hemodynamics; Oxygen
PubMed: 37883803
DOI: 10.1182/bloodadvances.2023010717 -
Urology Case Reports May 2024Necrotizing penile infection following ischemic priapism takedown is a rare complication. A 60-year-old man presented with 1-week history of green and pink purulent...
Necrotizing penile infection following ischemic priapism takedown is a rare complication. A 60-year-old man presented with 1-week history of green and pink purulent discharge, penile swelling, and inability to void. Computed tomography demonstrated gas and fluid accumulation within the corporal bodies, suggestive for bilateral penile necrotizing corporal infection with possibility of intra-corporal abscesses. The patient underwent extensive serial surgical debridement of the penile and corporal tissues which was complicated with poly-microbial tissue cultures, growing and . Despite maximal penile drainage, glans penis gangrene was developed to level of mid penile shaft necessitating a partial penectomy.
PubMed: 38711672
DOI: 10.1016/j.eucr.2024.102745 -
BMC Nutrition Jan 2024Malnutrition and sickle cell anemia (SCA) result in high childhood mortality rates. Although maternal depression is an established risk factor for malnutrition in...
BACKGROUND
Malnutrition and sickle cell anemia (SCA) result in high childhood mortality rates. Although maternal depression is an established risk factor for malnutrition in younger children, little is known about its impact on treatment response in children with malnutrition. We aimed to determine the relationship, if any, between maternal depression scores and malnutrition treatment outcomes in older children with SCA.
METHODS
We conducted a planned ancillary study to our randomized controlled feasibility trial for managing severe acute malnutrition in children aged 5-12 with SCA in northern Nigeria (NCT03634488). Mothers of participants completed a depression screen using the Patient Health Questionnaire (PHQ-9).We used a multivariable linear regression model to describe the relationship between the baseline maternal PHQ-9 score and the trial participant's final body mass index (BMI) z-score.
RESULTS
Out of 108 mother-child dyads, 101 with maternal baseline PHQ-9 scores were eligible for inclusion in this analysis. At baseline, 25.7% of mothers (26 of 101) screened positive for at least mild depression (PHQ-9 score of 5 or above). The baseline maternal PHQ-9 score was negatively associated with the child's BMI z-score after 12 weeks of malnutrition treatment (β=-0.045, p = 0.041).
CONCLUSIONS
Maternal depressive symptoms has an impact on malnutrition treatment outcomes. Treatment of malnutrition in older children with sickle cell anemia should include screening for maternal depression and, if indicated, appropriate maternal referral for depression evaluation and treatment.
TRIAL REGISTRATION
The trial was registered at clinicaltrials.gov (#NCT03634488) on January 30, 2018, https://clinicaltrials.gov/study/NCT03634488 .
PubMed: 38268013
DOI: 10.1186/s40795-024-00826-0 -
Urology Case Reports Mar 2024Refractory ischemic priapism is a difficult to treat clinical entity for which there are a host of shunt procedures identified, but no singular agreed upon technique for...
Refractory ischemic priapism is a difficult to treat clinical entity for which there are a host of shunt procedures identified, but no singular agreed upon technique for surgical therapy. Recent literature describes success using a penoscrotal decompression technique that uses a similar dissection of a penoscrotal penile prosthesis placement. We demonstrate that this technique is easily applicable in the private practice setting as it uses a familiar setup to most general urologists in our case report.
PubMed: 38420333
DOI: 10.1016/j.eucr.2024.102681