Large, ulcerated infantile hemangioma of the chest wall complicated by life-threatening hemorrhage: Case report and literature review.

While ulceration is one of the most common infantile hemangioma (IH) complications, severe bleeding is a rare consequence, with a paucity of patients reported. We report a 5-month-old girl with a very large, mixed, partial segmental IH of the upper chest wall who, despite medical intervention, developed severe ulceration and multiple episodes of life-threatening bleeding that ultimately led to hemorrhagic shock. Experience in our patient and a review of six previous reports shows that severe bleeding is a risk when ulceration extends directly into an arterial feeding vessel that is often visible clinically. Other potential predictors for severe bleeding include large to very large IH size with extension of the tumor into underlying structures, segmental or partial segmental patterning, mixed and bulky morphology, and white discoloration as a sign of impending or worsening ulceration.

Sporadic inclusion body myositis and primary Sjogren's syndrome: an overlooked diagnosis.

Sporadic inclusion body myositis (sIBM) has been reported to occur in association with autoimmune diseases and in particular, primary Sjogren's syndrome (pSS). This brief report describes patients identified with a positive SSA antibody and diagnosis of sIBM at a large academic medical center over a 13.5-year period. A cohort identification tool was used to identify patients with positive SSA antibody and a diagnosis of sIBM between January 1, 2006 and June 1, 2019. All cases of sIBM had diagnostic confirmation by a neuromuscular specialist. Demographics, clinical features, autoantibodies, MRI and EMG findings, and muscle biopsy features were reviewed for each identified case. Eight patients were found to carry the diagnosis of pSS and sIBM. Two additional sIBM patients were SSA antibody positive without other pSS features. The mean time from initial symptom onset of muscle weakness to diagnosis was 5.4 years (range 1-15 years). All patients had alternative diagnoses offered for their myopathic symptoms prior to sIBM identification. The NT5c1A antibody was positive in 7 of 8 patients tested. No patient had a durable response to immunosuppressive therapy. The diagnosis of sIBM went unrecognized for over 5 years in our cohort of SSA antibody-positive patients with myopathy. The patients in this cohort were treated with a variety of immunosuppressive agents prior to diagnosis without benefit. Recognizing the clinical features of sIBM in patients with pSS is crucial in instituting appropriate therapy and avoiding unnecessary immunosuppression. Key Points • Sporadic inclusion body myositis (sIBM) can be associated with Sjogren's syndrome. • In this case series, prevalence of the NT5c1A antibody was higher among patients with associated Sjogren's syndrome compared to the cited prevalence of the NT5c1A antibody in patients with isolated sIBM. • It is crucial to consider sIBM in patients with Sjogren's syndrome presenting with motor weakness in order to avoid unnecessary immunosuppression and institute appropriate therapy.

Polymyalgia Rheumatica: a Common Disease in Seniors.

PURPOSE OF THE REVIEW: Polymyalgia rheumatica (PMR) is one of the most common inflammatory rheumatologic condition occurring in older adults. It is characterized by proximal pain and stiffness in the shoulders, neck, and/or pelvic girdle in individuals over 50 years of age along with evidence of an intense systemic inflammatory response. Although the above clinical symptoms are very characteristic for the condition, it can be mimicked by other autoimmune, infectious, malignant, and endocrine disorders chief among which are giant cell arteritis (GCA) and elderly-onset rheumatoid arthritis (EORA). Recently, PMR was reported in relation to treatment with immune checkpoint inhibitors. Current treatment of PMR consists of low-to-medium doses of glucocorticosteroids (GC) with variable response rates and disease recurrence estimated to occur in 50% of patients while tapering down GC doses. In addition, GC-based regimens cause much of the morbidity associated with PMR in older adults, requiring close monitoring for GC-induced toxicity during therapy and highlighting the need for novel therapeutic strategies. Here, we review the latest findings in the field regarding specific etiologic factors, genetic associations, diagnostic methods, and advancements in treatment strategies and disease monitoring indices. RECENT FINDINGS: Recent discoveries involving novel therapeutic targets in GCA have accelerated the study of PMR pathophysiology and have advanced treatment strategies in PMR management leading to current trials in IL-6 blocking agents. PMR remains an enigmatic inflammatory condition affecting older adults, with current treatment approach causing much morbidity in this patient population. Advancements in our understanding of novel immunopathologic targets can serve as a solid foundation for future treatment strategies in the field.

A multidisciplinary approach to VA ECMO cannulation in children.

PURPOSE: Extracorporeal membrane oxygenation (ECMO) supports gas exchange and circulation in critically ill patients. This study describes a multidisciplinary approach to ECMO cannulation using the expertise of pediatric surgery (PS) and interventional radiology (IR). MATERIAL AND METHODS: Pediatric patients (<18 years) undergoing percutaneous cannulation for peripheral veno-arterial (VA) ECMO by PS and IR from April 2017 to May 2018 were included. Cardiac patients and children cannulated by PS alone were excluded. RESULTS: Five patients were included in the series. Median age was 16 [12.5-17] years and 3 were female. Median ECMO arterial and venous catheter sizes were 19 [17-22] Fr and 25 [25-28] Fr, respectively. Both catheters were placed in the common femoral vessels. A 6Fr antegrade distal perfusion cannula (DPC) was also placed in the superficial femoral artery by IR at the time of cannulation. The median time from admission to procedure start was 10 [7-50] hours and the children were on ECMO for a median length of 3.2 [2.3-4.8] days. There were two episodes of bleeding. No patients had loss of limb circulation. CONCLUSION: A multidisciplinary approach to peripheral VA ECMO cannulation is feasible and safe. Maintenance of limb perfusion by percutaneous placement and removal of DPC may be an advantage of this collaborative approach. LEVEL OF EVIDENCE: IV. TYPE OF RESEARCH: Case series.

Percutaneous Lung Biopsy in Immunocompromised Pediatric Patients.

PURPOSE: To determine the diagnostic yield and safety of image-guided lung biopsies in immunocompromised pediatric patients. MATERIALS AND METHODS: This was a retrospective pediatric cohort study conducted from June 2000 to April 2017. Subjects were 0-17 years of age (median, 10 years of age). There were 46 males (48%). A total of 73 consecutive image-guided lung biopsies were performed in 68 patients (weight range, 4.9-97.3 kg [median, 25.3 kg]). The indication for biopsy was to isolate an organism to tailor medical therapy. All patients were immunocompromised with an underlying history of bone marrow transplantation (n = 50), primary immunodeficiency (n = 14), and solid organ transplantation (n = 4). Patient and technical factors were analyzed for rates of complication. RESULTS: Overall diagnostic yield was 43 of 73 patients (60%). There were 14 minor (19%) and 8 major (11%) complications. Major complications included pneumothorax or hemoptysis requiring intervention (n = 6), and death (n = 2). The histological diagnosis was an infectious cause in 5 of 8 major complications (63%). There were statistically significant differences between the rates of complications with the imaging modality used (P = .02) and the use of fine needle aspiration (P = .02). CONCLUSIONS: Image-guided percutaneous lung biopsy can be helpful in isolating an organism to tailor therapy. Biopsies performed in immunosuppressed patients result in an elevated complication risk of up to 30% and demonstrate lower diagnostic yield and increased mortality, which should warrant detailed discussion with the primary team and family.

Ovarian steroids alter dopamine receptor populations in the medial preoptic area of female rats: implications for sexual motivation, desire, and behaviour.

Dopamine (DA) transmission in the medial preoptic area (mPOA) plays a critical role in the control of appetitive sexual behaviour in the female rat. We have shown previously that a DA D1 receptor (D1R)-mediated excitatory state appears to occur in females primed with estradiol benzoate (EB) and progesterone (P), whereas a DA D2 receptor (D2R)-mediated inhibitory state appears to occur in females primed only with EB. The present experiment employed three techniques to better understand what changes occur to DA receptors (DARs) in the mPOA under different hormonal profiles. Ovariectomized females were randomly assigned to one of three steroid treatment groups: EB + P (10 and 500 µg, respectively), EB + Oil, or the control (Oil + Oil), with hormone injections administered at 48 and 4 h prior to euthanizing. First, the number of neurons in the mPOA that contained D1R or D2R was assessed using immunohistochemistry. Second, the mPOA and two control areas (the prelimbic cortex and caudate putamen) were analysed for DAR protein levels using western blot, and DAR functional binding levels using autoradiography. Ovarian steroid hormones affected the two DAR subtypes in opposite ways in the mPOA. All three techniques supported previous behavioural findings that females primed with EB have a lower D1R : D2R ratio, and thus a D2R-mediated system, and females primed with EB + P have a higher D1R : D2R ratio, and thus a D1R-mediated system. This provides strong evidence for a DA-driven pathway of female sexual motivation, desire, and behaviour that is modified by different hormone priming regimens.

Food restriction-induced augmentation of heroin seeking in female rats: manipulations of ovarian hormones.

RATIONALE: Food restriction augments heroin seeking in chronically food-restricted male rats under withdrawal, an effect not yet examined in female rats. Importantly, women and female rats possess an increased vulnerability to drugs of abuse, which may be mediated by fluctuations in ovarian hormones. OBJECTIVES: We investigated the role of estradiol and progesterone in augmented heroin seeking in chronically food-restricted female rats, under withdrawal. METHODS: Female rats self-administered heroin for 10-12 days and were then allowed unrestricted (sated) or restricted access to food (FDR; ∼10 % reduction in body weight) for 14 days. On day 14, rats underwent a heroin-seeking test. Exp. 1: Rats underwent ovariectomy or sham surgery and were treated with a low dose of estradiol (5.0 % in cholesterol; subcutaneous capsule). Exp. 2: Rats underwent ovariectomy and were administered with a high dose of estradiol (0.5 mg/kg; subcutaneous) for 8 days before testing. Exp. 3: Progesterone injections (2.0 mg/kg; subcutaneous) were administered 24 h and 2 h before testing. RESULTS: Food restriction resulted in augmented heroin seeking, compared to sated controls. While ovariectomy had no effect, estradiol replacement attenuated the food restriction effect. Injections of progesterone had no effect on heroin seeking in either the sated or FDR groups. CONCLUSIONS: The effect of food restriction on heroin seeking in female rats under withdrawal is as robust as previously found in males. Interestingly, estradiol replacement, but not progesterone, attenuates the food restriction effect in the ovariectomized rats, possibly due to its anorexic properties.

Vaginocervical stimulation attenuates the sensitization of appetitive sexual behaviors by estradiol benzoate in the ovariectomized rat.

The acute administration of estradiol benzoate (EB) to the ovariectomized (OVX) rat induces low levels of lordosis while sexually appetitive behaviors (e.g., hops, darts, solicitations) are absent, yet the repeated administration of EB results in a behavioral sensitization in which lordosis is potentiated and sexually appetitive behaviors are induced. We have shown that repeated copulation attenuates the sensitization of appetitive sexual behaviors. Here, we assessed which component of male stimulation during copulation is involved in the attenuation. On 8 occasions, sexually experienced OVX Long-Evans rats were treated with 10µgEB and 48h later assigned to one of six groups that differed in their experience on intermediates tests (2-7). One was given repeated access to a male (EB/Male), and another was placed in the copulation chamber alone (EB/Alone) on intermediate tests. Three groups were given one of three somatosensory stimuli by the experimenter: manual flank stimulation (FLS), clitoral stimulation (CLS), or vaginocervical stimulation (VCS). Finally, the control group was left undisturbed in the animal care facility (ACF). Sexual behaviors were measured on Tests 1 and 8. VCS received from the experimenter (VCS) or from the male during copulation (EB/Male) attenuated the magnitude of the sensitization of appetitive sexual behaviors compared with those that were not brought to the testing rooms (ACF), and the effect was most pronounced on sexual solicitations. These results suggest that VCS received during penile intromission inhibits the sensitization of sexually appetitive behaviors by repeated administration of EB. As such, repeated administration of EB may oppose those mechanisms that induce estrous termination, perhaps by sensitizing inhibitory processes within the ventromedial hypothalamus that typically prevent the display of sexual behaviors (i.e., by facilitating disinhibition).

RU486 facilitates or disrupts the sensitization of sexual behaviors by estradiol in the ovariectomized Long-Evans rat: Effect of timecourse.

An acute injection of estradiol benzoate (EB) to the ovariectomized (OVX) rat activates low levels of lordosis, and subsequent progesterone (P) administration augments lordosis and recruits a complete pattern of sexual behavior including appetitive behaviors (e.g., hops/darts and solicitations). However, repeated injections of 5µg or 10µg EB (but not 2µg EB), administered every 4days to sexually-experienced OVX rats results in a behavioral sensitization, such that lordosis quotients (LQs) and appetitive behaviors progressively increase. We have shown that adrenal P does not play a critical role because behavioral sensitization to EB is not prevented by adrenalectomy. Here we tested whether P receptors play a role by examining the effect of chronic administration of the P receptor antagonist RU486 at a dose that reliably inhibits sexual behavior in fully primed OVX rats. Females were treated with EB (5 or 10µg), and 5mg RU486 dissolved in 0.4mL vehicle (VEH; 80% sesame oil, 15% benzyl benzoate, 5% benzyl alcohol) 48h and 5h prior to each of 7 tests, respectively, occurring at 4-day intervals in unilevel 4-hole pacing chambers. Control animals were treated with 2, 5, or 10µg EB+VEH. As expected, sensitization did not occur in females treated with 2µg EB+VEH, and those females received fewer intromissions and ejaculations than all other groups. RU486 did not prevent the sensitization of LQ, moderate and high lordosis magnitudes (LM2 and LM3) or appetitive sexual behaviors on early tests, and in fact potentiated appetitive behaviors, LQ, LM2 and LM3, consistent with its facilitative actions in females treated with EB-alone, as we and others have reported previously. However, despite the initial facilitation, blocking P receptors by chronic administration of RU486 inhibited the maintenance of behavioral sensitization to EB.

Quantitative and morphologic change associated with breast cancer-related lymphedema. Comparison of 3.0T MRI to external measures.

UNLABELLED: Abstract Introduction: Lymphedema is a chronic disease of increasing importance to cancer survivors. A tape measurement tool used for lymphedema relies on indirect volume calculations based on external circumference, which may not reflect the true extent of abnormal fluid accumulation accurately. Fluid-sensitive MRI sequences may be able to delineate the severity of this condition more precisely and thus also monitor response to therapy. METHODS AND RESULTS: Eight patients being followed by physical therapy for clinically diagnosed breast cancer-related lymphedema were recruited to participate in this study. External measurements and upper extremity MRI were performed on all subjects. Arm circumference, arm volume, and lymphedema volumes were calculated for each method. MR imaging detected lymphedema in all study subjects. Correlation was found between external circumferential measurements and with the 3.0T MRI (r=0.9368). There was poor correlation between lymphedema volumes calculated from clinical measurements and MR imaging (r=0.5539). CONCLUSIONS: External measurements were not found to be an accurate measure of lymphedema volume associated with breast cancer lymphedema. MRI is a reliable means to obtain upper extremity circumferential and volume measurements. MRI is able to evaluate morphologic change associated with breast cancer-related lymphedema. Lymphedema research requires integrated use of tools to further describe the disease process over time, quantitate the distribution of tissue changes, and improve the sensitivity and specificity of the measurements.

Lipid-associated rheumatologic syndromes.

Rheumatologic manifestations of hyperlipidemia and lipid-associated arthritis are rarely seen in the rheumatologist's office. On the other hand, a rheumatologist may be the clinician who identifies and initiates proper therapy for disorders related to hyperlipidemia when the musculoskeletal manifestations of these syndromes are recognized. In this article both the joint and tendon manifestations are reviewed, including the lesser known lipid liquid crystal form of arthritis. The relationship between gout and hyperuricemia is briefly discussed, as are the autoimmune manifestations of lipid-lowering therapy.

A survey of arthrocentesis and soft-tissue injection procedures performed in primary care practice: effect of resident training and using data to shape curriculum.

OBJECTIVE: The purpose of this study is (1) to survey graduates of our internal medicine program for use of musculoskeletal (MSK) procedures in primary care practice and assess the influence of participating in a first-year resident arthrocentesis and soft-tissue injection training course on their MSK procedure comfort/utilization; and (2) to use the results to modify our MSK procedure curriculum. METHODS: A questionnaire designed to assess numbers of, comfort with, and effect of resident training on MSK procedures in the preceding year was sent to 2002-2006 graduates of the internal medicine training program in outpatient primary care (OPC). Graduates practicing hospital medicine (HM) also received the questionnaire and served as a comparison group. RESULTS: There were 52 responses from this group of 84 graduates (64% response rate). OPC graduates (N = 32) were more comfortable doing procedures than those practicing HM exclusively (N = 20), and performed significantly more procedures in the preceding year (32.9 procedures per OPC/year vs 2.2 for HM). The most common procedures performed were knee joint, subacromial bursa, and trochanteric bursa, comprising > 75% of all procedures performed. A structured resident course in MSK procedures had a significant effect on the OPC physicians. Course participants (N = 17) performed almost twice the number of procedures/year as the nonparticipants (N = 15), were more comfortable with the procedures, and were significantly less likely to refer procedures to other clinicians. Written comments by respondents suggest additional MSK procedure training during and after residency is needed. CONCLUSION: Our results suggest a structured resident course in MSK procedures has a longterm influence. A progressively more focused approach to training is needed.

Impact of educational intervention on confidence and competence in the performance of a simple surgical task.

BACKGROUND: Many complex new procedures involve a learning curve, and patients treated by individuals who are new to a procedure may have more complications than those treated by a practitioner who has performed the intervention more frequently. Still, at some point on the learning curve, each individual must decide that he or she is qualified to perform a procedure, presumably on the basis of his or her level of confidence, background, education, and skill. To evaluate the interrelationship of these factors, we designed a study in which we assessed the performance of a simulated knee joint injection. METHODS: Ninety-three practitioners attending a continuing medical education session on knee injection were randomized to receive skills instruction through the use of a printed manual, a video, or hands-on instruction; each performed one injection before and after instruction. The participants completed pre-instruction and post-instruction questionnaires gauging confidence and also provided self-assessments of their performances of injections before and after instruction. Self-assessments were compared with objective performance standards measured by custom-designed knee models with electronic sensors that detected correct needle placement. RESULTS: Before instruction, the participants' confidence was significantly but inversely related to competent performance (r = -0.253, p = 0.02); that is, greater confidence correlated with poorer performance. Both men and physician-practitioners displayed higher pre-instruction confidence (p < 0.01), which was not correlated with better performance. After instruction, performance improved significantly in all three training groups (p < 0.001), with no significant differences in efficacy detected among the three groups (p = 0.99). After instruction, confidence correlated with objective competence in all groups (r = 0.24, p = 0.04); however, this correlation was weaker than the correlation between the participants' confidence and their self-assessment of performance (r = 0.72, p = 0.001). CONCLUSIONS: Even low-intensity forms of instruction improve individuals' confidence, competence, and self-assessment of their skill in performing the fairly straightforward psychomotor task of simulated knee injection. However, men and physicians disproportionately overestimated their skills both before and after training, a finding that worsened as confidence increased. The inverse relationship between confidence and competence that we observed before the educational intervention as well as the demographic differences that we noted should raise questions about how complex new procedures should be introduced and when self-trained practitioners should begin to perform them.