Pilomatrixoma imitating infantile hemangioma

Many lesions can masquerade as infantile hemangioma, the most common tumor of infancy. We describe an infant with pilomatrixoma mimicking hemangioma. The patient presented at 8 months of age with an 1.2-cm, red, asymptomatic lesion of the right ear. The mass was noted at 4 months of age and subsequently enlarged. Ultrasonography demonstrated hypervascularity "consistent with infantile hemangioma," and the infant was observed. At 12 months of age, the lesion continued to expand and became ulcerated; it was excised, and histopathology showed pilomatrixoma. Diagnostic confusion was caused by atypical features of this pilomatrixoma that overlapped with infantile hemangioma: onset in infancy, ulceration, red color, and fast flow on imaging. Deviation from the predictable clinical features of an infantile hemangioma should prompt consideration for other types of pediatric lesions, including pilomatrixoma.     

Lymphaticovenous bypass of the thoracic duct for the treatment of chylous leak in central conducting lymphatic anomalies

Background

Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.

Effect of physical activity on levels of homocysteine,folate, and vitamin B12 in the elderly

Objectives: Homocysteine (Hcy) has emerged as a risk factor for cardiovascular diseases (CVD). However the relationship of physical activity (PA) with Hcy levels in the elderly is controversial. Accordingly, the current study examined the effect of low and high participation in PA on serum Hcy in young (n = 77; 18–50 years) and old (n = 207; > 65 years) males (n = 141) and females (n = 142). Methods: Level of PA was obtained in a 1-to-1 interview and participants divided into low and high groups. Serum Hcy, folate, and vitamin B₁₂ were obtained after 12 hour fast drawn by venipuncture. Results: Levels of Hcy correlated with folate (r = ?0. 5; p = 0.000) and vitamin B₁₂ (r = ?0.3; p = 0.000). The ANOVA revealed a main effect of PA for Hcy (p = 0.04) but not for folate (p = 0.2) and vitamin B₁₂ (p = 0.2). Main effects were found also of age for Hcy (p = 0.000) and folate (p = 0.005) as well as of gender for Hcy (p = 0.000) and vitamin B₁₂ (p = 0.000). Subsequent ANCOVA showed lower levels of Hcy in the participants with greater versus lower PA even after controlling for B₁₂ vitamin. Conclusion: These results confirm the importance of the vitamins for regulating Hcy levels. Additionally, the data suggests that PA affects Hcy levels without affecting and independent of B vitamins in the elderly.     

Periodontal diseases and the risk of coronary heart and cerebrovascular diseases: a meta-analysis

BACKGROUND: This meta-analysis was conducted to examine the relationship between periodontal diseases and coronary heart diseases (CHD) and cerebrovascular diseases (CVD) in observational studies. METHODS: This study was based on seven cohort studies and four studies of other designs that met prestated inclusion criteria. Information on study design, year of publication, study location, sample size, study population, participant characteristics, measurement of risk factors, exposure and outcome measures, matching, controlling for confounders, and risk estimates was abstracted independently by two investigators using a standard protocol. RESULTS: Subjects with periodontitis had an overall adjusted risk of CHD that was 1.15 times (95% confidence interval [CI]: 1.06 to 1.25; P = 0.001) the risk for healthy subjects. There was no heterogeneity among the studies in the overall relative risk estimate (P = 0.472). As compared to healthy subjects, those with periodontitis had an overall adjusted relative risk of CVD of 1.13 (95% CI: 1.01 to 1.27; P = 0.032). CONCLUSIONS: Findings indicated that periodontal infection increases the risk of CHD and CVD. However, this meta-analysis provided no evidence for the existence of strong associations between periodontitis and CHD and CVD. Larger and better-controlled studies involving socially homogeneous populations and measuring specific periodontal pathogens are required to identify a definite association between periodontal disease and the risk of coronary heart disease and cerebrovascular disease.     

Therapeutic plasma exchange in patients with life-threatening COVID-19: a randomised controlled clinical trial

Assessment of efficacy of therapeutic plasma exchange (TPE) following life-threatening COVID-19. This was an open-label, randomised clinical trial of ICU patients with life-threatening COVID-19 (positive RT-qPCR plus ARDS, sepsis, organ failure, hyperinflammation). Study was terminated after 87/120 patients enrolled. Standard treatment plus TPE (n = 43) versus standard treatment (n = 44), and stratified by PaO₂/FiO₂ ratio (>150 vs. ≤150), were compared. Primary outcomes were 35-day mortality and TPE safety. Secondary outcomes were association between TPE and mortality, improvement in SOFA score, change in inflammatory biomarkers, days on mechanical ventilation (MV), and ICU length of stay (LOS). Eighty-seven patients [median age 49 (IQR 34–63) years; 82.8% male] were randomised (44 standard care; 43 standard care plus TPE). Days on MV (P = 0.007) and ICU LOS (P = 0.02) were lower in the TPE group. 35-Day mortality was non-significantly lower in the TPE group (20.9% vs. 34.1%; Kaplan-Meier, P = 0.582). TPE was associated with increased lymphocytes and ADAMTS-13 activity and decreased serum lactate, lactate dehydrogenase, ferritin, d-dimers and interleukin-6. Multivariable regression analysis provided several predictors of 35-day mortality: PaO₂/FiO₂ ratio (HR, 0.98, 95% CI 0.96–1.00; P = 0.02]; ADAMTS-13 activity (HR, 0.89, 95% CI 0.82–0.98; P = 0.01); pulmonary embolism (HR, 3.57, 95% CI 1.43–8.92; P = 0.007). Post-hoc analysis revealed a significant reduction in SOFA score for TPE patients (P < 0.05). In critically-ill COVID-19 patients, addition of TPE to standard ICU therapy was associated with faster clinical recovery and no increased 35-day mortality.     

Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry

Purpose

Hepatic hemangiomas, though histologically benign, may be associated with significant morbidity and mortality in afflicted infants. The literature presents much confusion regarding the natural history and treatment options for hepatic hemangiomas. Clinical manifestations range from asymptomatic self-limiting lesions to congestive heart failure associated with high-volume vascular shunting to fulminant hepatic failure with hypothyroidism, abdominal compartment syndrome, and death. There has been little rationale to choose among observation, corticosteroid, other pharmacologic agents, arterial embolization, hepatic artery ligation, resection, or liver transplantation for any given patient.

Methods

We analyzed several recent retrospective radiologic analyses and pathologic studies to determine whether hepatic hemangiomas could be categorized, allowing prediction of their natural history and rational choice of therapies based upon their clinical presentation and radiographic appearance.

Results

We propose that hepatic hemangiomas do not represent a single entity but, rather, 3 principle categories of lesions: focal, multifocal, and diffuse. Because these 2 categories represent different anatomical and physiologic variants, so, too, may they respond differently to previously anecdotally applied treatment regimens. With input from international multidisciplinary authorities on hemangiomas, we developed and proposed a clinical practice algorithm for the evaluation and management of hepatic hemangiomas. Toward that end, we propose a plan to institute a web-based international hepatic hemangioma registry. Participants in the registry could obtain no-cost centralized review of imaging studies (and histology if available) and guidance regarding the management algorithm from an established multidisciplinary team. In exchange, the registry will facilitate the acquisition of systematic clinical and imaging information.

Conclusion

Longitudinal observation of response to more directed treatment protocols may contribute greatly to the understanding of these potentially fatal tumors.     

Angiographic demonstration of an aberrant abdominal umbilical artery in a patient with penoscrotal transposition and caudal regression

Penoscrotal transposition (PST) is a rare congenital anomaly characterized by caudal location of the penis with respect to the scrotum. PST may be seen in isolation or associated with caudal regression syndrome. We present a case of an infant born with penoscrotal transposition, bladder agenesis, a solitary dysplastic kidney, and caudal regression. The patient developed anuria and was referred for angiography for preoperative planning for renal transplantation and genital reconstruction. Angiography demonstrated an aberrant abdominal umbilical artery, an anomaly classically associated with sirenomelia but also has been described in caudal regression.