Cerebral and cervical arterial abnormalities are the most common non-cutaneous anomaly in PHACE syndrome, but the location and type of arterial lesions that occur have not been systematically ...assessed in a large cohort. Our aim was to characterize the phenotypic spectrum of arteriopathy, assess the frequency with which different arteries are involved, and evaluate spatial relationships between arteriopathy, brain structural lesions, and hemangiomas in PHACE syndrome.
Intracranial MRA and/or CTA images from 70 children and accompanying brain MR images in 59 patients with arteriopathy and PHACE syndrome were reviewed to identify the type and location of arterial lesions and brain abnormalities. Five categories of arteriopathy were identified and used for classification: dysgenesis, narrowing, nonvisualization, primitive embryonic carotid-vertebrobasilar connections, and anomalous arterial course or origin. Univariate logistic regression analyses were performed to test for associations between arteriopathy location, hemangiomas, and brain abnormalities.
By study design, all patients had arterial abnormalities, and 57% had >1 form of arteriopathy. Dysgenesis was the most common abnormality (56%), followed by anomalous course and/or origin (47%), narrowing (39%), and nonvisualization (20%). Primitive embryonic carotid-vertebrobasilar connections were present in 20% of children. Hemangiomas were ipsilateral to arteriopathy in all but 1 case. The frontotemporal and/or mandibular facial segments were involved in 97% of cases, but no other specific associations between arteriopathy location and hemangioma sites were detected. All cases with posterior fossa anomalies had either ICA anomalies or persistent embryonic carotid-basilar connections.
The arteriopathy of PHACE syndrome commonly involves the ICA and its embryonic branches, ipsilateral to the cutaneous hemangioma, with dysgenesis and abnormal arterial course the most commonly noted abnormalities. Brain abnormalities are also typically ipsilateral.
Large facial infantile hemangiomas have higher rates of complications than small localized hemangiomas, more often require treatment, and can be associated with neurological, ophthalmologic, and ...cardiac anomalies (PHACE syndrome). The anatomic patterns of these hemangiomas are often referred to as "segmental" despite a lack of precise anatomic definitions. Our study aims to define "segmental" hemangiomas based on clinically observed patterns. Our secondary goal is to relate the observed patterns to currently accepted developmental patterns to gain insight into hemangioma pathogenesis and craniofacial development.
Photographic data were extracted from a large cohort of patients with infantile hemangiomas. We mapped 294 hemangiomas and recorded common morphologic patterns. Anatomic descriptions of the most common patterns were described and compared with accepted concepts of craniofacial development.
Four primary segments were identified (Seg1-Seg4). Seg2 and Seg3 correspond with the previously recognized maxillary and mandibular prominences. Seg1 and Seg4 differ from standard human embryology texts. The frontotemporal segment, Seg1, encompasses the lateral forehead, anterior temporal scalp, and lateral frontal scalp. The segment Seg4, encompassing the medial frontal scalp, nasal bridge, nasal tip, ala, and philtrum, is substantially narrower on the forehead than the previously described frontonasal prominence.
The patterns provide new clues regarding facial development. The observed patterns resemble previously described facial developmental units on the lower face but are distinctly different on the upper face. The patterns suggest that neural crest derivatives may play a role in the development of facial hemangiomas. Finally, these patterns (Seg1-Seg4) help standardize the nomenclature of facial segmental hemangiomas to analyze more effectively hemangioma risks and behavior.
Background/Objectives
Infantile hemangiomas (IHs) are commonly encountered in primary care and most often remain asymptomatic, resolving without sequelae. Certain characteristics are associated with ...a greater risk of complications, associated anomalies, and disfigurement. The heterogeneous presentation poses a clinical challenge for physicians in determining the need for treatment and subspecialty referral. This study aims to evaluate the utility of the previously published Hemangioma Severity Scale (HSS) to predict the need for treatment.
Methods
This retrospective study included 106 patients with IHs seen in the Indiana University Dermatology Clinic in 2011. Data from electronic medical records and clinical photographs taken at patients' initial visits were used to score the hemangiomas using the HSS. Treatments used over 9 to 14 months of follow‐up were recorded.
Results
Four HSS score subgroups were identified. Higher HSS scores correlated with the need for treatment; 98% of patients with HSS scores of 10 or greater received local or systemic therapy. Higher HSS scores also correlated with greater frequency of complications and risks of associated structural anomalies and permanent disfigurement. Scores did not correlate with sex, age at initial presentation, history of bleeding or pain, or IH size.
Conclusions
The HSS may be a useful tool for primary care physicians in identifying high‐risk IHs that may benefit from therapy. This easy‐to‐use scale can improve clinical outcomes by identifying which patients need intervention to minimize complications. IHs with total HSS scores of 6 or greater should be referred for subspecialty evaluation.
Inhibitors of mammalian target of rapamycin function to downregulate cell growth and proliferation and have off‐label use in pediatrics for vascular malformations. Hypertriglyceridemia is a known ...side effect of mammalian target of rapamycin (mTOR) inhibitors. Further studies to better understand the incidence and treatment of hypertriglyceridemia in infants and neonates are warranted.
Infantile hemangiomas often are inapparent at birth and have a period of rapid growth during early infancy followed by gradual involution. More precise information on growth could help predict ...short-term outcomes and make decisions about when referral or intervention, if needed, should be initiated. The objective of this study was to describe growth characteristics of infantile hemangioma and compare growth with infantile hemangioma referral patterns.
A prospective cohort study involving 7 tertiary care pediatric dermatology practices was conducted. Growth data were available for a subset of 526 infantile hemangiomas in 433 patients from a cohort study of 1096 children. Inclusion criteria were age younger than 18 months at time of enrollment and presence of at least 1 infantile hemangioma. Growth stage and rate were compared with clinical characteristics and timing of referrals.
Eighty percent of hemangioma size was reached during the early proliferative stage at a mean age of 3 months. Differences in growth between hemangioma subtypes included that deep hemangiomas tend to grow later and longer than superficial hemangiomas and that segmental hemangiomas tended to exhibit more continued growth after 3 months of age. The mean age of first visit was 5 months. Factors that predicted need for follow-up included ongoing proliferation, larger size, deep component, and segmental and indeterminate morphologic subtypes.
Most infantile hemangioma growth occurs before 5 months, yet 5 months was also the mean age at first visit to a specialist. Recognition of growth characteristics and factors that predict the need for follow-up could help aid in clinical decision-making. The first few weeks to months of life are a critical time in hemangioma growth. Infants with hemangiomas need close observation during this period, and those who need specialty care should be referred and seen as early as possible within this critical growth period.
Objectives To characterize demographic, prenatal, and perinatal features of patients with infantile hemangiomas and to determine the importance of these factors in predicting rates of complication ...and treatment. Study design We conducted a prospective study at 7 U.S. pediatric dermatology clinics. A consecutive sample of 1058 children, aged 12 years and younger, with infantile hemangiomas was enrolled between September 2002 and October 2003. A standardized questionnaire was used to collect demographic, prenatal, perinatal, and hemangioma-specific data. National Vital Statistic System Data (NVSS) was used to compare demographic variables and relevant rates of prenatal events. Results In comparison with the 2002 United States National Vital Statistics System birth data, we found that infants with hemangiomas were more likely to be female, white non-Hispanic, premature ( P < .0001) and the product of a multiple gestation (10.6% versus 3.1%; P < .001). Maternal age was significantly higher ( P < .0001), and placenta previa (3.1%) and pre-eclampsia (11.8%) were more common. Conclusions Infants with hemangiomas are more likely to be female, white non-Hispanic, premature, and products of multiple gestations. Prenatal associations include older maternal age, placenta previa, and pre-eclampsia. No demographic, prenatal, and perinatal factors predicted higher rates of complications or need for treatment.
Background
The COVID-19 pandemic has highlighted the inability of health systems to leverage existing system infrastructure in order to rapidly develop and apply broad analytical tools that could ...inform state- and national-level policymaking, as well as patient care delivery in hospital settings. The COVID-19 pandemic has also led to highlighted systemic disparities in health outcomes and access to care based on race or ethnicity, gender, income-level, and urban-rural divide. Although the United States seems to be recovering from the COVID-19 pandemic owing to widespread vaccination efforts and increased public awareness, there is an urgent need to address the aforementioned challenges.
Objective
This study aims to inform the feasibility of leveraging broad, statewide datasets for population health–driven decision-making by developing robust analytical models that predict COVID-19–related health care resource utilization across patients served by Indiana’s statewide Health Information Exchange.
Methods
We leveraged comprehensive datasets obtained from the Indiana Network for Patient Care to train decision forest-based models that can predict patient-level need of health care resource utilization. To assess these models for potential biases, we tested model performance against subpopulations stratified by age, race or ethnicity, gender, and residence (urban vs rural).
Results
For model development, we identified a cohort of 96,026 patients from across 957 zip codes in Indiana, United States. We trained the decision models that predicted health care resource utilization by using approximately 100 of the most impactful features from a total of 1172 features created. Each model and stratified subpopulation under test reported precision scores >70%, accuracy and area under the receiver operating curve scores >80%, and sensitivity scores approximately >90%. We noted statistically significant variations in model performance across stratified subpopulations identified by age, race or ethnicity, gender, and residence (urban vs rural).
Conclusions
This study presents the possibility of developing decision models capable of predicting patient-level health care resource utilization across a broad, statewide region with considerable predictive performance. However, our models present statistically significant variations in performance across stratified subpopulations of interest. Further efforts are necessary to identify root causes of these biases and to rectify them.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
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