Management in Neonatal Priapism: Case and Review.
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ABSTRACT:
SUBMITTER: Kuwano AY
PROVIDER: S-EPMC5522373 | biostudies-other | 2017 Sep
REPOSITORIES: biostudies-other
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Project description:Malignant priapism, a rare disease with only about 500 reported cases to date, consists of persistent erection secondary to invasion or metastasis of a primary neoplasm. While treatment guidelines for priapism in non-malignant cases have been established, there is currently no guideline for treating malignant priapism. Herein, we describe three cases of malignant priapism and suggest a step-by-step approach for clinical management. This study reports three cases of malignant priapism resulting from advanced genitourinary cancers. All patients experienced a sub-acute progression of penile pain and ultimately underwent palliative penectomy, resulting in sustained symptom relief. Treatment of malignant priapism needs to be individualized to the needs of the patient. No matter the primary or secondary nature of the disease, current data suggest that malignant priapism is associated with poor outcomes and emphasis should be put on palliative care. Similar to previous cases, our cases died shortly after the diagnosis of malignant priapism. Conventional procedures such as shunting may not necessarily provide symptom relief in these patients. Although new radiation techniques have shown favorable outcomes, penectomy should be considered the last resort in clinical management. Revisions to the existing management guidelines for priapism are necessary to address its occurrence in malignant contexts.
| S-EPMC10643380 | biostudies-literature
Project description:Priapism is defined as a prolonged and persistent erection of the penis without sexual stimulation. This is a poorly understood disease process with little information on the pathophysiology of this erectile disorder. Complications from this disorder are devastating due to the irreversible erectile damage and resultant erectile dysfunction (ED). Stuttering priapism, though relatively rare, affects a high prevalence of men with sickle-cell disease (SCD) and presents a challenging problem with guidelines for treatment lacking or resulting in permanent ED. The mechanisms involved in the development of priapism in this cohort are poorly characterized; therefore, medical management of priapism represents a therapeutic challenge to urologists. Additional research is warranted, so we can effectively target treatments for these patients with prevention as the goal. This review gives an introduction to stuttering priapism and its clinical significance, specifically with regards to the patient with SCD. Additionally, the proposed mechanisms behind its pathophysiology and a summary of the current and future targets for medical management are discussed.
| S-EPMC3753435 | biostudies-other
Project description:Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16/491 (3.2%) boys (median age 13.5 years, range 4-18) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months (n = 1), over 6 weeks (n = 1), over 1-2 weeks (n = 2), over several days (n = 2), or 24 h (n = 1), while the remaining 4 boys reported continuous erection lasting over 11-12 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000/µL, range 236,700-899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids ± heparin (n = 2), penile puncture (n = 5) ± injection of sympathomimetics (n = 4) ± intracavernous shunt operation (n = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19-25) responded to a questionnaire. All had maintained full erectile function; however, 5/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted <12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients' lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions ± cytotoxic chemotherapy.
| S-EPMC10380995 | biostudies-literature
Project description:IntroductionIschemic priapism is a urologic emergency managed by both urologic and nonurologic providers in the community. Given ischemic priapism's rare occurrence and the time-sensitive nature of treatment, effective provider education on management of this entity is imperative. We sought to develop a low-cost effective simulation model and curriculum to enhance trainee education.MethodsA comprehensive didactic curriculum based on national urologic guidelines was developed, along with a low-cost, easily reproducible priapism simulator using hot dogs and Red Vines candy. The simulators cost $1.25 each, and assembly took 10 minutes. All materials were reviewed by three urology faculty members. The curriculum was piloted with two andrology fellowship-trained urology faculty among eight urology residents (PGY2-PGY4/U1-U2) and one medical student. Participants provided feedback regarding the overall course as well as the face and content validity of the simulator.ResultsCognitive test scores significantly improved on average by 15.0% (p = .002), and confidence improved from baseline somewhat or very much among 88.9% of participants after completion of the curriculum. The task trainer was rated easy to use (average score: 4.78 out of 5), and 77.8% of participants though it was somewhat or very useful for training (average score: 4.00 out of 5). Additionally, 77.8% recommended its incorporation into resident training (average score: 4.00 out of 5).DiscussionThis simulation curriculum is effective, inexpensive, and easily reproducible, making it ideal for groups with limited resources. Expanding access to simulation-based curricula on priapism management may improve education of both urologic and nonurologic trainees.
| S-EPMC6342429 | biostudies-literature
Project description:Priapism is a rare condition that involves persistent penile erection for greater than 4 h. Distinct variants exist, each with unique characteristics. Ischemic priapism is a painful medical emergency that may occur as a result of veno-occlusion leading to hypoxia and tissue death. Recurrent bouts of ischemic priapism, or stuttering priapism, require treatment for individual attacks as well as long-term prevention. Non-ischemic priapism is associated with trauma and may be managed conservatively. Recent advances into the pathophysiology of priapism have allowed the development of treatment algorithms that specifically target the mechanisms involved. In this review, we outline the basics of smooth muscle contraction and describe how derangement of these pathways results in priapism. A pathophysiological approach to the treatment of priapism is proposed with duration-based algorithms presented to assist in management.
| S-EPMC3739130 | biostudies-literature
Project description:Priapism, a rare but well-known adverse reaction of first and second generation anti-psychotics, has been hypothesized to be associated with blockade of alpha 1 receptors. However, genetic abnormalities or heritability of affected cytochrome P450 alleles has not been ruled out as a causal mechanism. A case is presented with three episodes of priapism within 17 days while taking standard FDA (Food and Drug Administration) approved doses of quetiapine. Cytochrome P450 3A4 genotype testing was performed and resulted with normal enzymatic activity. This case further eliminates enzymatic metabolism as a possible cause of priapism, thus strengthening the alpha one receptor blockade hypothesis.
| S-EPMC4714272 | biostudies-literature
Project description:Priapism, an unwanted, painful, prolonged erection that is unrelated to sexual stimulation, is a common complication of sickle cell disease (SCD). Priapic events in SCD are stuttering, meaning they occur repeatedly with intervening periods of detumescence. Without health care intervention, repeated episodes can lead to erectile dysfunction. There are limited treatment options for SCD-related priapism and no approved targeted therapies. Crizanlizumab is a monoclonal antibody that binds to P-selectin and is used to reduce the frequency of vaso-occlusive crises in patients with SCD. Here, we report the cases of 3 patients with SCD-related priapism who were treated with crizanlizumab. All patients were African American men who experienced numerous priapic episodes that interfered with their daily lives. Upon treatment with crizanlizumab, priapic events were reduced in all 3 patients. These successful cases suggest a potential role for crizanlizumab in the prevention of SCD-related priapism.
| S-EPMC10515560 | biostudies-literature
Project description:INTRODUCTION:India contributes to the highest neonatal deaths globally. Case management is said to be the cornerstone of pneumonia control. Much of the published evidence focuses on children aged 1 to 59 months. This scoping review, thus, aims to identify the treatment options for and barriers to case management of neonatal pneumonia in India. METHODS AND ANALYSIS:This protocol is part of a series of three reviews on neonatal pneumonia in India. Studies addressing treatment of or barriers to case management of neonatal pneumonia in Indian context, published in English in peer-reviewed and indexed journals will be eligible for inclusion. Electronic search will be conducted on nine databases. Hand searching and snowballing will be done for published and grey literature. Selection of studies will be done in title, abstract and full-text stages. A narrative summary will be performed to summarise the details of evidence. ETHICS AND DISSEMINATION:As this is a review involving analysis of secondary data which is available in the public domain and does not involve human participants, ethical approval was not required. The findings of the study will be shared with all stakeholders of this research. Knowledge dissemination workshops will be conducted with relevant stakeholders to ultimately transfer the evidence tailored to the stakeholder (eg, policy briefs, publications, information booklets and so on). PROSPERO 2016: CRD42016045449.
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Project description:A case study of a neonatal meningitis case
| PRJEB81278 | ENA
Project description:The study's purpose is to investigate the clinical characteristics and research progress of PURA syndrome. It will also provide new ideas and methods for the diagnosis of neonatal hypotonia etiology. A case of PURA syndrome admitted to Shenzhen Hospital of Peking University was analyzed retrospectively. The keywords "PURA", "PURα", "PURA syndrome", and "5q31" were used to search the Chinese periodical full-text database and Wanfang database. The keywords "PURA", "PURα", "Pur-alpha", "PURA syndrome", and "5q31" were used to search the biomedical literature database (PubMed). The Web of Science database and Proquest database were used to find works of literature from the establishment of the database to November 10, 2019. By analyzing the 72 cases of PURA syndrome reported in ten Chinese and international studies, it was found that 57% (21/37) of the patients had a gestational age greater than 41 weeks. Neonatal patients exhibited hypotonia (82%, 59/72), feeding difficulties (97%, 64/66), apnea or primary hypoventilation (57%, 41/72), intrauterine excessive hiccupping (55%, 6/11), and drowsiness (51%, 24/47). After the neonatal period, the pediatric patients demonstrated moderate to severe mental retardation (100%), epilepsy (54%, 29/54), progressive hip dysplasia (17%, 7/42), scoliosis (48%, 11/23), dysphagia and salivation (69%, 25/36), and constipation (60%, 21/35). The clinical manifestations of the present case were consistent with those in the literature reports. It was the first confirmed case at Shenzhen Hospital in the neonatal period and had a de novo mutation. It was difficult to diagnose PURA syndrome in the neonatal period, which might affect multiple systems. In newborns with obvious hypotonia, the evaluation should be expanded to consider other symptoms. Additionally, targeted gene detection should be completed to achieve early diagnosis and intervention, improve the prognosis, and perform genetic counseling.
| S-EPMC7882292 | biostudies-literature