Urogenital presentation of a male patient with focal dermal hypoplasia.

Focal dermal hypoplasia (FDH) is a rare X-linked dominant syndrome characterized by streaky cutaneous atrophy in a blaschkoid distribution, skeletal dysplasias, and ocular abnormalities. Here, we report hypospadias and chordee identified in a male patient with molecularly confirmed FDH. This report highlights a new clinical manifestation of male patients with FDH.

Single-Center Analysis of Characteristics and Outcomes of De Novo, Concurrent, and Transformed Diffuse Large B-Cell Lymphoma.

Outcomes of diffuse large B-cell lymphoma (DLBCL), either concurrent with or transformed from indolent lymphoma, treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone are described in retrospective studies. The efficacy of other regimens in transformed or concurrent DLBCL is largely unknown. In this single-center retrospective study, we present characteristics of concurrent and transformed DLBCL and outcomes after dose-adjusted etoposide, vincristine, cyclophosphamide, prednisone, and doxorubicin with rituximab (DA-EPOCH-R) comparative with de novo DLBCL. Of 170 patients with DLBCL, 136 were de novo, 17 were concurrent, and 17 were transformed. Transformed DLBCL had significantly lower complete response rates and progression-free survival (PFS) but similar overall survival (OS) compared with de novo counterpart. There was no significant difference between de novo and concurrent DLBCL regarding response rates, PFS, and OS. DA-EPOCH-R was associated with inferior OS. Thus, intensified treatment with DA-EPOCH-R might not improve outcomes of transformed DLBCL.

Clearance of Hepatitis C Virus (HCV) Is Associated With Improved Outcomes in HCV-Associated Lymphoma.

BACKGROUND: Improved hepatitis C virus (HCV) clearance due to directly acting antiviral agents has led to remarkably improved outcomes of indolent HCV-associated non-Hodgkin lymphoma (NHL). The impact of directly acting antivirals on the outcomes of aggressive NHL is still under investigation. Characteristics of HCV-associated NHL in black patients are not well characterized. We report outcomes of HCV-associated NHL compared to their HCV-negative counterparts in a predominantly black population. PATIENTS AND METHODS: Patients with lymphoma between January 2007 and December 2017 were retrospectively studied. Depending on presence or absence of HCV RNA, patients were grouped into HCV positive (HCV+) and HCV negative (HCV-) cohorts. Depending on virologic clearance (VC), HCV+ were classified into HCV+ with VC and HCV+ without VC. Overall response rate (ORR), complete response, overall survival (OS), and progression-free survival (PFS) of HCV+ patients with and without VC were compared to HCV- patients. RESULTS: Of 397 patients with lymphoma, 40 had HCV. Black comprised 90% of HCV+ patients. Diffuse large B-cell lymphoma was most frequent (47%) in the HCV+ group. HCV+ patients without VC had significantly worse OS and PFS compared to HCV- patients. There were no differences in ORR, complete response, PFS, and OS of HCV+ patients with VC and HCV- patients. These results were consistent in subgroups of diffuse large B-cell lymphoma and aggressive lymphoma. CONCLUSION: HCV clearance is positively associated with lymphoma outcomes in black patients. Patients who clear HCV have noninferior outcomes to HCV- patients, while those who fail to clear HCV have significantly worse outcomes.

Ingeniería de diseño en Cirugía. ¿Cómo diseñar, probar y comercializar dispositivos quirúrgicos fabricados con impresión 3D? / Design Engineering in Surgery. How to Design, Test and Market Surgical Devices Made With 3D Printing?

La industria 4.0 ofrece nuevas oportunidades de desarrollo a los cirujanos. El diseño asistido por ordenador y la impresión 3D permiten materializar muchas ideas y conceptos, facilitando la accesibilidad al diseño y la creación de productos, bien como prototipos, bien como productos finales funcionales. Hasta ahora era difícil llegar a la fabricación de nuevos dispositivos. En estos momentos la principal limitación será nuestra creatividad, disponer de espacios que permitan poner a prueba nuestras creaciones y lograr financiación

Industry 4.0 offers new development opportunities for surgeons. Computer-aided design and 3D printing allow for the creation of prototypes and functional end products. Until now, it was difficult for new devices to get to the manufacturing phase. Nowadays, the main limitations are our creativity, available spaces to test our creations and obtaining financing

Design Engineering in Surgery. How to Design, Test and Market Surgical Devices Made With 3D Printing? / Ingeniería de diseño en Cirugía. ¿Cómo diseñar, probar y comercializar dispositivos quirúrgicos fabricados con impresión 3D?

Industry 4.0 offers new development opportunities for surgeons. Computer-aided design and 3D printing allow for the creation of prototypes and functional end products. Until now, it was difficult for new devices to get to the manufacturing phase. Nowadays, the main limitations are our creativity, available spaces to test our creations and obtaining financing.

Smaller platelet volumes associated with vascular malformations.

Various parameters in 91 adult participants with vascular malformations and 91 controls were studied. The mean of the participants' platelet volumes was 8.5 fL and that of their controls was 9.1 (P < .001). The mean of the participants' platelet mass was 2145 µL/L of blood and that of their controls was 2351 (P = .006). The other parameters studied were not significantly different than the controls. It is suggested that the lower platelet volume might be related to a compensatory mechanism to keep the total body platelet mass stable despite the increased vasculature.

Patient perceptions of 595 nm pulsed dye laser through glass compression in the treatment of thick port wine stains and superficial venous malformations.

In treating thickened port wine stains and superficial venous malformations with the 595 nm pulse dye laser, we have sometimes compressed the lesion with a glass slide to enable the laser beam to penetrate more deeply. In order to evaluate the patients' perception of this modality, 54 subjects were treated with glass compression using ice applications to cool the skin surface and without glass compression using a cryospray to cool the surface. The subjects were asked to rate the difference in 2 questionnaires after the first and second treatments as to effectiveness, pain of procedure, and overall preference. Of those subjects who perceived a difference between modalities, a significant number perceived treatments with glass compression to be more effective than without (p=0.0002 and p=0.0006), but more painful (p<0.0001). There was no significant difference in overall preference. Blistering occurred in 5 subjects when the glass slide was left on the skin more than 1 minute between ice applications.