Bladder Repair With Irrigation and Debridement and Open Reduction Internal Fixation of the Anterior Pelvic Ring Is Safe and Decreases Risk of Infection in Pelvic Ring Injuries With Extraperitoneal Bladder Ruptures.

OBJECTIVES: To determine the incidence of infection in nonoperative versus operative management of extraperitoneal bladder ruptures in patients with pelvic ring injuries. DESIGN: A retrospective cohort study of 2 prospectively collected trauma registries. SETTING: Two Level 1 trauma centers. PATIENT SELECTION CRITERIA: Patients with operative pelvic ring injuries, 68 (6%) had extraperitoneal bladder ruptures. OUTCOME MEASURES AND COMPARISONS: The primary outcome was the incidence and associated risk factors of deep pelvic infection requiring return to OR for surgical debridement. Secondary outcomes included quality of reduction, other complications, and radiographic union. Comparisons were made based on the status of any associated bladder injury. RESULTS: Of 1127 patients with operative pelvic ring injuries, 68 patients had extraperitoneal bladder ruptures, 55 had bladder repair and 13 did not. Of those 13 without repair, none had ORIF of the anterior pelvic ring. Patients without bladder repair had an increased odds of infection 17-fold compared to patients who did have a repair performed (OR 16.9, 95% CI 1.75 - 164, P = 0.01). Other associated factors for deep pelvic infection included use of suprapubic catheter ( p < 0.02) and a closed reduction of the anterior ring ( p < 0.01). Patients undergoing anterior ring ORIF and bladder repair had improved reductions and no increased infection risk. CONCLUSIONS: Operative repair of extraperitoneal bladder ruptures decreases risk of infection in patients with pelvic ring injuries. Additionally, ORIF of the anterior pelvic ring does not increase the risk of infection and results in better reductions compared to closed reduction. Treatment algorithms for these combined injuries should consider recommending early bladder repair and anterior pelvic ORIF. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Surgical prophylaxis in pediatric orthopedic patients with penicillin allergy: a multicentered retrospective prognostic study.

Up to 20% of orthopedic surgeons still avoid the use of cephalosporins in patients with penicillin allergies despite its reported safety in the adult and general surgery pediatric population. The primary objective is to determine the incidence of adverse effects and allergic reactions when using cephalosporins in pediatric orthopedic patients labeled as penicillin-allergic as compared to those without previously reported penicillin allergy. A multicenter retrospective chart review was performed across three level 1 trauma centers from January 2013 to February 2020 to identify penicillin-allergic as well as non-penicillin-allergic pediatric patients treated for orthopedic injuries. Data were collected regarding patient demographics, antibiotic administered, timing of antibiotic administration, reported drug allergy, and described allergic reaction. Postoperative or intraoperative allergic reactions to antibiotics, surgical site infections, and complications were recorded. A total of 2289 surgeries performed by four fellowship-trained surgeons were evaluated. Eighty-five patients diagnosed with penicillin allergy were identified and underwent 95 surgeries and 95 patients without previously reported penicillin allergy underwent 95 surgeries. One patient, with a documented history of anaphylaxis to cefazolin, sustained an anaphylactic reaction intraoperatively to cefazolin. There were no other reported reactions, surgical site infections, or complications. There was no statistically significant difference in rate of allergic reaction in patients with previously reported penicillin allergy treated with cefazolin and those with no previous reported reaction (P > 0.05). Prophylaxis with cephalosporins is not associated with increased risk for allergic reaction. Cephalosporins can be safely administered to pediatric patients with penicillin allergy undergoing orthopedic intervention. Level of evidence: Level II, Multicenter Retrospective Prognostic Study.

Delayed Presentation of Berardinelli-Siep Lipodystrophy in an Adolescent Female.

Berardinelli-Siep syndrome (BSS) is a form of congenital generalized lipodystrophy that disrupts the pathways of lipid metabolism. It presents with physical exam findings, including muscular hypertrophy and lipoatrophy as well as serious metabolic consequences such as diabetes mellitus, hypertriglyceridemia, acute pancreatitis, hepatomegaly, and hepatic steatosis. Diagnosis generally occurs soon after birth or in childhood. The case presented is significant for a delayed diagnosis of suspected BSS Type 1 which is rather uncommon in a developed country. Due to the detrimental complications of BSS, such as hypertrophic cardiomyopathy, pancreatitis, and liver disease, early diagnosis and intervention are crucial. Pediatric providers must be knowledgeable about physical features of BSS and common presentations such as new onset diabetes, hypertriglyceridemia, or pancreatitis throughout early childhood and adolescence in order to avoid delayed diagnoses.

DHCR24, a Key Enzyme of Cholesterol Synthesis, Serves as a Marker Gene of the Mouse Adrenal Gland Inner Cortex.

Steroid hormones are synthesized through enzymatic reactions using cholesterol as the substrate. In steroidogenic cells, the required cholesterol for steroidogenesis can be obtained from blood circulation or synthesized de novo from acetate. One of the key enzymes that control cholesterol synthesis is 24-dehydrocholesterol reductase (encoded by DHCR24). In humans and rats, DHCR24 is highly expressed in the adrenal gland, especially in the zona fasciculata. We recently reported that DHCR24 was expressed in the mouse adrenal gland's inner cortex and also found that thyroid hormone treatment significantly upregulated the expression of Dhcr24 in the mouse adrenal gland. In the present study, we showed the cellular expression of DHCR24 in mouse adrenal glands in early postnatal stages. We found that the expression pattern of DHCR24 was similar to the X-zone marker gene 20αHSD in most developmental stages. This finding indicates that most steroidogenic adrenocortical cells in the mouse adrenal gland do not synthesize cholesterol locally. Unlike the 20αHSD-positive X-zone regresses during pregnancy, some DHCR24-positive cells remain present in parous females. Conditional knockout mice showed that the removal of Dhcr24 in steroidogenic cells did not affect the overall development of the adrenal gland or the secretion of corticosterone under acute stress. Whether DHCR24 plays a role in conditions where a continuous high amount of corticosterone production is needed requires further investigation.

Peripheral Levels of Renin-Angiotensin System Components Are Associated With Cognitive Performance in Huntington's Disease.

The renin-angiotensin system (RAS) has proven to be involved in the pathophysiology of neurodegenerative diseases, such as Parkinson's disease (PD) and Alzheimer's disease (AD), serving as a potential therapeutic target and a disease burden marker. Studies have associated negative clinical outcomes with the activation of the classical RAS arm composed of the angiotensin-converting enzyme (ACE) and angiotensin (Ang) II, while suggested positive outcomes with the activation of the counter-regulatory RAS arm involving ACE2 and Ang-(1-7). Huntington's disease (HD) shares many pathological and clinical outcomes with AD and PD, but the evidence of direct involvement of RAS components in the pathophysiology of HD is still limited and needs further investigation. Herein, we investigated peripheral levels of the RAS components Ang II, Ang-(1-7), ACE, and ACE2 in controls, premanifest, and manifest HD gene carriers and their relationship with clinical outcomes. Peripheral blood samples were collected via phlebotomy, and plasma concentrations of RAS components were measured by Enzyme-Linked Immunosorbent Assay. Clinical evaluation included a questionnaire about socio-demographic characteristics, motor, and cognitive assessments. Results showed (1) no significant group differences in plasma concentrations of RAS components; (2) positive correlations between ACE2 and Verbal Fluency Test (VFT) scores; and (3) negative correlations between Ang II and Mini-Mental State Examination scores. These results corroborate the proposed balance between the classical (ACE/Ang II) and the counter-regulatory [ACE2/Ang-(1-7)] arms of the RAS, with the former associated with negative clinical outcomes and the latter with positive effects in HD.