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1.
Childs Nerv Syst ; 39(1): 35-40, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36198892

RESUMO

BACKGROUND: Congenital unilateral lower lip palsy (CULLP), also referred to as congenital asymmetric crying facies (ACF), is a rare condition that causes pronounced depression of the unaffected lower lip when crying, despite symmetric appearance of the mouth and lips at rest. Unlike the acquired form of ACF, CULLP is idiopathic and often involves permanent defect. METHODS AND RESULTS: We present a case-based review of CULLP, including a thorough analysis of the relevant literature and a discussion of the exemplary case of 5-year-old patient presenting with unilateral facial asymmetry resulting from left-sided facial weakness. The patient was diagnosed with ACF at birth, and documentation from a previous neurologic consultation specifies the root cause of the asymmetry as developmental aplasia of the left depressor anguli oris muscle (DAOM). However, there is no record of electrodiagnostic testing or B-scan ultrasound imaging that would support this conclusion, and the patient's dysarthric speech may suggest lower motor neuron involvement. Botox chemodenervation of the right, unaffected side was recommended to deanimate the contralateral lower lip and achieve facial symmetry, in addition to potentially resolving some of the patient's speech difficulties. CONCLUSIONS: There are several approaches, both surgical and non-surgical, to the management and correction of CULLP. These include weakening the muscles of the contralateral side or increasing muscular tension on the ipsilateral side, referred to as deanimation and reanimation procedures, respectively.


Assuntos
Paralisia Facial , Lábio , Recém-Nascido , Humanos , Pré-Escolar , Músculos Faciais/anormalidades , Ultrassonografia
2.
Front Oncol ; 14: 1294331, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38800408

RESUMO

While typically low-risk, cutaneous squamous cell carcinoma (cSCC) can infrequently progress to metastatic disease with in-transit lesions, localized to the dermis or subcutaneous tissue between the primary tumor and draining regional lymph nodes. These lesions are associated with poor prognostic values, including decreased survival rates and increased risk of recurrence. We present the case of a 75-year-old male with cSCC and in-transit metastases on his scalp treated with the immune checkpoint inhibitor (ICI) pembrolizumab in conjunction with diphencyprone (DPCP), a topical hapten that induces a delayed-type hypersensitivity reaction in the skin. The patient was enrolled in a clinical trial (NCT05481658) that involved the twice-weekly application of DPCP 0.04% ointment to four of the in-transit metastases on his frontal scalp, concurrent with pembrolizumab 300 mg administered every three weeks. Following effective sensitization and a twelve-week treatment course, complete clearance of all lesions, DPCP-treated and non-DPCP treated, was achieved, with no adverse events. The immunologic profiles of the post-treatment biopsies were analyzed by TaqMan Low Density Array quantitative real-time polymerase chain reaction to measure immune marker gene expression. Relative to the non-DPCP-treated lesion, the DPCP-treated lesion demonstrated increased pro-inflammatory genetic markers and T-cell activation. This case represents the first reported instance of in-transit metastases of cSCC successfully treated with DPCP and an ICI. It highlights the potential safety and efficacy of DPCP with systemic immunotherapy for the management of in-transit metastases of cSCC in patients for whom surgery and radiation may be contraindicated.

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