Oakland Museum of California

BackgroundPrune belly syndrome (PBS), also known as Eagle‐Barrett syndrome (EGBRS), is a rare congenital disease characterized by deficiency or absence of abdominal wall muscles, urological abnormalities, and bilateral cryptorchidism.Types of Studies ReviewedA review of literature was done using four search engines (PubMed, Google Scholar, Scopus, Science Direct) and keywords (individually and in combinations): prune belly syndrome, PBS, Eagle‐Barrett syndrome, dental manifestation, clinical manifestation, and psychological aspects. The search was run with no language restrictions and covered the 1965–2021 time period.ResultsThe search yielded a large number of articles. The vast majority were dealing with a variety of treatments. PBS is a multisystem disease with a variable spectrum ranging from mild cases to infant mortality. Comorbidities of PBS (63% gastrointestinal, 65% orthopedic, and 49% cardiopulmonary) present challenges for treatment. PBS affects quality of life of patients and caregivers. We selected and summarized published information that is relevant to oral health and dental care.Conclusions and Practical ImplicationsProviding information to dental practitioners will improve their understanding of PBS. It will help them to better treat patients with PBS and it will encourage more dental providers to welcome patients with PBS into their dental clinics.

Physicians must sometimes learn medicine the hard way. I had prescribed terazosin for decades, so during a December office visit with my own internist, I was comfortable when she suggested this agent as first-line therapy for my prostatism. Meanwhile, I continued training for an upcoming marathon. My limited endurance in early January was expected because of holiday disruptions, but it seemed to get worse rather than better as the month progressed. I was shamefully slow to suspect the increasing doses of my new prostate medication, but when I did, the drug monograph's adverse effects list gave me no concerns. I pushed myself through another grueling week of running, then increased the terazosin to 15 mg. Two days and a dozen miserable miles later, I finally peeked into PubMed and found a 1994 cross-over study of doxazosin in hypertensive athletes that convincingly demonstrated impaired performance in 5000 meter runs.1 A 1995 study found that doxazosin significantly increased 2-mile running time in non-elite athletes.2 Within 2 days of stopping terazosin, I was again running well. Could I have figured out my alpha blocker problem sooner? The terazosin package insert and drug monographs do mention asthenia among the adverse effects, most commonly with a 7%–11% incidence. And yes, I knew that asthenia refers to "weakness, tiredness, lassitude and fatigue." But reading that a small number of study participants reported asthenia from terazosin (4–7 percentage points higher than placebo) was not the same as learning that alpha blockers predictably impair athletic performance. I experienced no asthenia-like side effects except when running. A recent review of hypertension3 cites the 1994 study and warns against the use of alpha blockers in athletes, but the American Urological Association guideline on benign prostatic hyperplasia4 does not. The take-home lesson is that we need our guidelines and point-of-care tools to compensate for the inherent limitations in our drug labeling system. Post-marketing spontaneous surveillance depends upon our reporting adverse drug events, a task that we find challenging for multiple reasons.5 But even when reports are filed and/or new studies performed, the financial and logistical barriers to relabeling are generally insurmountable for most drugs. Neither the Food and Drug Administration's efforts to update labels (for oncology drugs only)6 nor calls for active surveillance rather than spontaneous reporting (for dementia drugs)7 are likely to be a harbinger of relabeling for alpha blockers or other commonplace drugs. Many of us are literally running around quite well with our hypertrophied prostates. We need guidelines, updated point-of-care tools, and a much higher index of suspicion to keep doing so. Dr. Hill was solely responsible for this submission. The author declares no conflicts of interest. None.

We reviewed five models for modifying the natural log mean and within-event standard deviation of ground-motion models (GMMs) to account for directivity effects in the near-fault environment. We found broad consistency for strike–slip ruptures, with positive and negative directivity effects for cases of rupture towards and away from a site of interest, respectively. We found substantial divergence among directivity models for reverse slip, with some providing maximum directivity for sites positioned to experience the peak alignment of rupture direction with the fault-slip direction (this occurs in the up–dip direction), while others optimize directivity based on the amount of fault rupture towards the site (even if the azimuth of rupture propagation does not align with the fault-slip direction). We found four of five NGA-West2 GMMs to be centered on a condition of null directivity. Therefore, we consider those GMMs suitable for use in combination with similarly centered directivity models. We present two deterministic methods for adjusting ground-motion hazard results for the effects of directivity: one modifies ground motions for a specified hazard level based on location-specific (relative to fault) changes in mean and standard deviation; and the second produces a directivity-compatible conditional mean spectra. We also provide recommendations for incorporating directivity effects into calculations within the hazard integral by either (1) modifying the mean and standard deviation of ground motion to approximately account for the effect of variable hypocenter location; (2) integrating over a location-specific distribution of the directivity parameter, which also indirectly accounts for variable hypocenter location; or (3) integrating directly over alternate hypocenter locations. Review Panel: Jonathan D. Bray, Stephen A. Mahin, I. M. Idriss, Robert W. Graves and Tom Shantz.

The peptide hormone relaxin has numerous roles both within and independent of pregnancy and is often thought of as a "pleiotropic hormone." Relaxin targets several tissues throughout the body, and has many functions associated with extracellular matrix remodeling and the vasculature. This review considers the potential therapeutic applications of relaxin in cervical ripening, in vitro fertilization, preeclampsia, acute heart failure, ischemia-reperfusion, and cirrhosis. We first outline the animal models used in preclinical studies to progress relaxin into clinical trials and then discuss the findings from these studies. In many cases, the positive outcomes from preclinical animal studies were not replicated in human clinical trials. Therefore, the focus of this review is to evaluate the various animal models used to develop relaxin as a potential therapeutic and consider the limitations that must be addressed in future studies. These include the use of human relaxin in animals, duration of relaxin treatment, and the appropriateness of the clinical conditions being considered for relaxin therapy.