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339 results on '"Liang, Marilyn"'

1. Multicenter Study of Long-Term Outcomes and Quality of Life in PHACE Syndrome after Age 10

Author

Braun, Mitchell, Frieden, Ilona J., Siegel, Dawn H., George, Elizabeth, Hess, Christopher P., Fox, Christine K., Chamlin, Sarah L., Drolet, Beth A., Metry, Denise, Pope, Elena, Powell, Julie, Holland, Kristen, Ulschmid, Caden, Liang, Marilyn G., Barry, Kelly K., Ho, Tina, Cotter, Chantal, Baselga, Eulalia, Bosquez, David, Jain, Surabhi Neerendranath, Bui, Jordan K., Lara-Corrales, Irene, Funk, Tracy, Small, Alison, Baghoomian, Wenelia, Yan, Albert C., Treat, James R., Hogrogian, Griffin Stockton, Huang, Charles, Haggstrom, Anita, List, Mary, McCuaig, Catherine C., Barrio, Victoria, Mancini, Anthony J., Lawley, Leslie P., Grunnet-Satcher, Kerrie, Horii, Kimberly A., Newell, Brandon, Nopper, Amy, Garzon, Maria C., Scollan, Margaret E., and Mathes, Erin F.

Published
2024
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5. ACR Appropriateness Criteria® Soft Tissue Vascular Anomalies: Vascular Malformations and Infantile Vascular Tumors (Non-CNS)-Child.

Author

Bardo, Dianna M.E., Gill, Anne E., Iyer, Ramesh S., Chan, Sherwin S., Cooper, Matthew L., Dasgupta, Roshni A., Guimaraes, Carolina V., Hammer, Matthew R., Krowchuk, Daniel P., Levin, Terry L., Liang, Marilyn G., Meyers, Mariana L., Samet, Jonathan D., Sammer, Marla B.K., Schooler, Gary R., Squires, Judy H., Sura, Amit S., Trout, Andrew T., and Pruthi, Sumit

Abstract

Soft tissue vascular anomalies may be composed of arterial, venous, and/or lymphatic elements, and diagnosed prenatally or later in childhood or adulthood. They are divided into categories of vascular malformations and vascular tumors. Vascular malformations are further divided into low-flow and fast-flow lesions. A low-flow lesion is most common, with a prevalence of 70%. Vascular tumors may behave in a benign, locally aggressive, borderline, or malignant manner. Infantile hemangioma is a vascular tumor that presents in the neonatal period and then regresses. The presence or multiple skin lesions in an infant can signal underlying visceral vascular anomalies, and complex anomalies may be associated with overgrowth syndromes. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation. [ABSTRACT FROM AUTHOR]

Published
2024
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7. Stigmatization and Mental Health Impact of Chronic Pediatric Skin Disorders

Author

Paller, Amy S., Rangel, Stephanie M., Chamlin, Sarah L., Hajek, Aleena, Phan, Sheshanna, Hogeling, Marcia, Castelo-Soccio, Leslie, Lara-Corrales, Irene, Arkin, Lisa, Lawley, Leslie P., Funk, Tracy, Castro Porto Silva Lopes, Fabiana, Antaya, Richard J., Ramien, Michele L., Vivar, Karina L., Teng, Joyce, Coughlin, Carrie C., Rehmus, Wingfield, Gupta, Deepti, Bercovitch, Lionel, Stein, Sarah L., Boull, Christina, Tom, Wynnis L., Liang, Marilyn G., Hunt, Raegan, Luu, Minnelly, Holland, Kristen E., Schoch, Jennifer J., Cella, David, Lai, Jin-Shei, and Griffith, James W.

Abstract

IMPORTANCE: Chronic skin disorders in children frequently are visible and can cause stigmatization. However, the extent of stigmatization from chronic skin disease and association with mental health needs further study. OBJECTIVE: To examine the extent of stigma, dependence on disease visibility and severity, and association with mental health and quality of life (QOL) in chronic pediatric skin disease. DESIGN, SETTING, AND PARTICIPANTS: A cross-sectional, single-visit study was conducted at 32 pediatric dermatology centers in the US and Canada from November 14, 2018, to November 17, 2021. Participants included patients aged 8 to 17 years with chronic skin disease and 1 parent. MAIN OUTCOMES AND MEASURES: Using the Patient-Reported Outcomes Measurement Instrumentation System (PROMIS) Stigma-Skin, the extent of stigma with child-, caregiver-, and physician-assessed disease visibility (primary outcome) and severity was compared, as well as reduced QOL (assessed by Skindex-Teen), depression, anxiety, and poor peer relationships (PROMIS child and proxy tools) (secondary outcomes). RESULTS: The study included 1671 children (57.9% female; mean [SD] age, 13.7 [2.7] years). A total of 56.4% participants had self-reported high disease visibility and 50.5% had moderate disease severity. Stigma scores significantly differed by level of physician-assessed and child/proxy-assessed disease visibility and severity. Among children with chronic skin disorders, predominantly acne, atopic dermatitis, alopecia areata, and vitiligo, only 27.0% had T scores less than 40 (minimal or no stigma) and 43.8% had at least moderate stigma (T score ≥45) compared with children with a range of chronic diseases. Stigma scores correlated strongly with reduced QOL (Spearman ρ = 0.73), depression (ρ = 0.61), anxiety (ρ = 0.54), and poor peer relationships (ρ = −0.49). Overall, 29.4% of parents were aware of bullying of their child, which was strongly associated with stigma (Cohen d = −0.79, with children who were not bullied experiencing lower levels of stigma). Girls reported more stigma than boys (Cohen d = 0.26). Children with hyperhidrosis and hidradenitis suppurativa were most likely to have increased depression and anxiety. CONCLUSIONS AND RELEVANCE: The findings of this study suggest that physician assessment of disease severity and visibility is insufficient to evaluate the disease impact in the patient/caregiver. Identifying stigmatization, including bullying, and tracking improvement through medical and psychosocial interventions may be a key role for practitioners.

Published
2024
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8. Verrucous Venous Malformation—Subcutaneous Variant

Author

Schmidt, Birgitta A.R., El Zein, Sophie, Cuoto, Javier, Al-Ibraheemi, Alyaa, Liang, Marilyn G., Paltiel, Harriet J., Anderson, Megan E., Labow, Brian I., Upton, Joseph, Fishman, Steven J., Mulliken, John B., Greene, Arin K., Warman, Mathew L., and Kozakewich, Harry

Published
2021
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9. Non‐vascular intracranial lesions in three children with PHACE association.

Author

Mologousis, Mia A., Balkin, Daniel M., Smith, Edward R., Lidov, Hart G. W., Li, Alice M., Yang, Edward, and Liang, Marilyn G.

Subjects
CHILD patients, NEUROCUTANEOUS disorders, HEMANGIOMAS, HUMAN abnormalities
Abstract

PHACE (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac anomalies, eye anomalies) association has many recognized clinical features. A link between PHACE and non‐vascular intracranial lesions has not been well‐described. We report three pediatric patients with PHACE and non‐vascular intracranial lesions. [ABSTRACT FROM AUTHOR]

Published
2024
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10. Next generation sequencing aids diagnosis and management in a case of encephalocraniocutaneous lipomatosis.

Author

Barry, Kelly K., Liang, Marilyn G., Balkin, Daniel M., Srivastava, Siddharth, Church, Alanna J., and Eng, Whitney

Subjects
*NUCLEOTIDE sequencing, *LIPOMATOSIS, *BRAIN tumors, *GENETIC testing, *NEUROCUTANEOUS disorders, *MOLECULAR diagnosis
Abstract

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous disorder caused by somatic FGFR1 and KRAS variants. It shares significant phenotypic overlap with several closely related disorders caused by mutations in the RAS‐MAPK pathway (mosaic RASopathies). We report a diagnostically challenging case of ECCL in which next‐generation sequencing of affected tissue identified a pathologic FGFR1 p.K656E variant, thereby establishing a molecular diagnosis. Patients with FGFR1‐associated ECCL carry a risk of developing malignant brain tumors; thus, genetic testing of patients with suspected ECCL has important management implications. [ABSTRACT FROM AUTHOR]

Published
2024
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16. Paradoxical Psoriasiform Eruptions in Children Receiving Tumor Necrosis Factor α Inhibitors

Author

Eickstaedt, Joshua, primary, Paller, Amy S., additional, Lund, Emily, additional, Murphrey, Morgan, additional, Brandling-Bennett, Heather, additional, Maurano, Megan, additional, Fernandez Faith, Esteban, additional, Holland, Kristen E., additional, Ibler, Erin, additional, Liang, Marilyn G., additional, Todd, Patricia S., additional, Siegfried, Elaine, additional, Igelman, Sean, additional, Cordoro, Kelly M., additional, and Tollefson, Megha M., additional

Published
2023
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17. What Syndrome Is This?

Author

Kim, Caroline Choi, Liang, Marilyn G, Pfendner, Ellen, and Kimonis, Virginia E

Subjects
Paediatrics, Biomedical and Clinical Sciences, Clinical Sciences, Child, Epidermolysis Bullosa, Female, Frameshift Mutation, Granulation Tissue, Humans, Hyperpigmentation, Laminin, Laryngostenosis, Nails, Malformed, Pakistan, Palate, Hard, Syndrome, Paediatrics and Reproductive Medicine, Dermatology & Venereal Diseases, Clinical sciences
Published
2007

18. PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations

Author

Garzon, Maria C., Epstein, Leon G., Heyer, Geoffrey L., Frommelt, Peter C., Orbach, Darren B., Baylis, Adriane L., Blei, Francine, Burrows, Patricia E., Chamlin, Sarah L., Chun, Robert H., Hess, Christopher P., Joachim, Shawna, Johnson, Katherine, Kim, Wendy, Liang, Marilyn G., Maheshwari, Mohit, McCoy, Garrett N., Metry, Denise W., Monrad, Priya A., Pope, Elena, Powell, Julie, Shwayder, Tor A., Siegel, Dawn H., Tollefson, Megha M., Vadivelu, Sudhakar, Lew, Sean M., Frieden, Ilona J., and Drolet, Beth A.

Published
2016
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27. Kaposiform Lymphangiomatosis

Author

Perez-Atayde, Antonio R., primary, Debelenko, Larisa, additional, Al-Ibraheemi, Alyaa, additional, Eng, Whitney, additional, Ruiz-Gutierrez, Melisa, additional, O’Hare, Meghan, additional, Croteau, Stacy E., additional, Trenor, Cameron C., additional, Boyer, Debra, additional, Balkin, Daniel M., additional, Barclay, Sarah F., additional, Hsi Dickie, Belinda, additional, Liang, Marilyn G., additional, Chaudry, Gulraiz, additional, Alomari, Ahmad I., additional, Mulliken, John B., additional, Adams, Denise M., additional, Kurek, Kyle C., additional, Fishman, Steven J., additional, and Kozakewich, Harry P.W., additional

Published
2022
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28. Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson–Gilford progeria syndrome

Author

Gordon, Leslie B., Kleinman, Monica E., Miller, David T., Neuberg, Donna S., Giobbie-Hurder, Anita, Gerhard-Herman, Marie, Smoot, Leslie B., Gordon, Catherine M., Cleveland, Robert, Snyder, Brian D., Fligor, Brian, Bishop, W. Robert, Statkevich, Paul, Regen, Amy, Sonis, Andrew, Riley, Susan, Ploski, Christine, Correia, Annette, Quinn, Nicolle, Ullrich, Nicole J., Nazarian, Ara, Liang, Marilyn G., Huh, Susanna Y., Schwartzman, Armin, and Kieran, Mark W.

Published
2012

29. Paraneoplastic pemphigus associated with Castleman disease: A multicenter case series.

Author

Barry, Kelly K., Plumptre, Isabella, Bazewicz, Christopher G., Green, Brian P., Treat, James R., Hughes, Meagan, Morel, Kimberly D., Wiss, Karen, and Liang, Marilyn G.

Subjects
CASTLEMAN'S disease, MUCOUS membrane diseases, BRONCHIOLITIS obliterans, CHILD patients, MYASTHENIA gravis, PEMPHIGUS, THYMOMA
Abstract

Introduction: Paraneoplastic pemphigus (PNP) is a rare, often fatal, autoimmune blistering disease of the skin and mucous membranes. In children, PNP is frequently associated with Castleman disease (CD). This series describes five cases of PNP associated with CD. Methods: Data were collected retrospectively from the medical records of patients with a diagnosis of PNP and CD from January 2013 to June 2022. Patients ≤22 years old with clinical and immunopathologic evidence of PNP were included; CD was diagnosed histopathologically. Results: Two children, two adolescents, and one young adult (two males, three females) were included. The average age at disease presentation was 11.8 years (range: 7–22 years). Oral (n = 5) and anogenital (n = 3) mucositis were common. Four patients had "unicentric" CD (UCD); one patient had "multicentric" CD (MCD). Castleman tumors were in the retroperitoneum (n = 4) or axilla (n = 1). One patient had myasthenia gravis without thymoma. Three patients had bronchiolitis obliterans (BO). Three patients had complete resection of their CD; two had partial resection. Three patients remain alive with a median follow‐up of 13 months (range: 12 months to 13 years); two are clinically stable with resolution of mucocutaneous lesions; one has persistent BO requiring ongoing ventilatory support. Patients who remain alive had UCD with complete resection; all deceased patients had partial resection and BO. Conclusion: Most patients had UCD, and the retroperitoneum was the most common location. Patients with MCD, incomplete resection, and BO died; patients with UCD and complete resection remain alive, even in the setting of BO. Consideration of PNP is critical when pediatric patients present with mucositis as PNP may be clinically indistinguishable from more common causes of mucositis. [ABSTRACT FROM AUTHOR]

Published
2023
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36. Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.

Author

Perez-Atayde, Antonio R., Debelenko, Larisa, Al-Ibraheemi, Alyaa, Eng, Whitney, Ruiz-Gutierrez, Melisa, O'Hare, Meghan, Croteau, Stacy E., Trenor III, Cameron C., Boyer, Debra, Balkin, Daniel M., Barclay, Sarah F., Hsi Dickie, Belinda, Liang, Marilyn G., Chaudry, Gulraiz, Alomari, Ahmad I., Mulliken, John B., Adams, Denise M., Kurek, Kyle C., Fishman, Steven J., and Kozakewich, Harry P.W.

Published
2022
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44. Cutaneovisceral Angiomatosis With Thrombocytopenia

Author

Prasad, Vinay, Fishman, Steven J., Mulliken, John B., Fox, Victor L., Liang, Marilyn G., Klement, Giannoula, Kieran, Mark W., Burrows, Patricia E., Waltz, David A., Powell, Julie, Dubois, Josée, Levy, Moise L., Perez-Atayde, Antonio R., and Kozakewich, Harry P.W.

Published
2005
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