Beyond Traditional Sunscreens: A Review of Liposomal-Based Systems for Photoprotection.

Sunscreen products are essential for shielding the skin from ultraviolet (UV) radiation, a leading cause of skin cancer. While existing products serve this purpose, there is a growing need to enhance their efficacy while minimizing potential systemic absorption of UV filters and associated toxicological risks. Liposomal-based formulations have emerged as a promising approach to address these challenges and develop advanced photoprotective products. These vesicular systems offer versatility in carrying both hydrophilic and lipophilic UV filters, enabling the creation of broad-spectrum sunscreens. Moreover, their composition based on phospholipids, resembling that of the stratum corneum, facilitates adherence to the skin's surface layers, thereby improving photoprotective efficacy. The research discussed in this review underscores the significant advantages of liposomes in photoprotection, including their ability to limit the systemic absorption of UV filters, enhance formulation stability, and augment photoprotective effects. However, despite these benefits, there remains a notable gap between the potential of liposomal systems and their utilization in sunscreen development. Consequently, this review emphasizes the importance of leveraging liposomes and related vesicular systems as innovative tools for crafting novel and more efficient photoprotective formulations.

Pharmaceutical Care Practice and Consequences on Quality of Life and Satisfaction of Diabetic Patients: A Randomized Clinical Trial

Abstract The pursuit of quality of life, which has become marked in recent years, has translated into important population health benefits. This study assessed the quality of life of patients with type 2 diabetes (T2DM) who received pharmaceutical care, and their satisfaction with the service provided in community pharmacies. This single blind, randomized controlled clinical trial included 100 patients. The intervention group (N=47) received pharmaceutical care by a clinical pharmacist and the control group (N=42) received standard care. The Quality of Life Diabetes Questionnaire (DQOL) was used to measure all participants´ quality of life at study enrollment and six months later. Satisfaction of the participants in the intervention group was measured using a validated 14-items questionnaire. At the end of the study, participants in the intervention group had a statistically significant improvement in DQOL scores ( -0.62 x 1.57, p < 0.001) and were highly satisfied with the intervention (96% excellent satisfaction scores). Pharmaceutical care practice was associated with high satisfaction and increased quality of life scores in T2DM patients. These results underscore the important role of pharmacists to improve the health of diabetic patients.

Current trends on cannabidiol delivery systems: where are we and where are we going?

INTRODUCTION: Cannabidiol (CBD), a phytocannabinoid from Cannabis sativa, has several therapeutic properties. However, its high lipophilicity, metabolization, and instability impair its bioavailability and translational use in clinical settings. Several advanced drug delivery systems (ADDSs) have been evaluated as CBD carriers to overcome these drawbacks. These systems can improve the CBD dissolution profile, protect it against metabolization, and produce a site-specific release, increasing its bioavailability and making CBD administration clinically effective. AREAS COVERED: This review summarizes scientific reports on cannabidiol advanced delivery systems (CBD-ADSs) that have been (i) developed, and (ii) applied therapeutically; reports published in the main scientific databases until January 2020 were included. Studies without experimental data and/or published in languages other than English were excluded. Moreover, pharmaceutical technology tools in CBD therapeutic use have been discussed, emphasizing the clinical translation of CBD carrier use. EXPERT OPINION: Studies reporting CBD-ADS use for medicinal applications were reviewed and revealed multifaceted systems that can overcome the physicochemical drawbacks of CBD and improve its biological activities. Therefore, researchers concluded that the developed CBD-ADS can be used as an alternative to traditional formulations because they show comparable or superior effectiveness in treatment protocols. Although several criteria remain to be met, our findings emphasize the potential of CBD-ADSs for translational therapeutics, particularly for neurological-disorders.

Xylan microparticles for controlled release of mesalamine: Production and physicochemical characterization.

Xylan extracted from corn cobs was used to produce mesalamine-loaded xylan microparticles (XMP5-ASA) by cross-linking polymerization using a non-hazardous cross-linking agent. The microparticles were characterized by thermal analysis (DSC/TG), X-ray diffraction (XRD), Infrared spectroscopy (FTIR-ATR) and scanning electron microscopy (SEM). A comparative study of the in vitro drug release from XMP5-ASA and from gastro-resistant capsules filled with XMP5-ASA (XMPCAP5-ASA) or 5-ASA was also performed. NMR, FTIR-ATR, XRD and DSC/TG studies indicated molecularly dispersed drug in the microparticles with increment on drug stability. The release studies showed that XMPCAP5-ASA allowed more efficient drug retention in the simulated gastric fluid and a prolonged drug release lasting up to 24 h. XMPCAP5-ASA retained approximately 48 % of its drug content after 6 h on the drug release assay. Thus, the encapsulation of 5-ASA into xylan microparticles together with gastro-resistant capsules allowed a better release control of the drug during different simulated gastrointestinal medium.

Self-Assembled Cationic-Covered Nanoemulsion as A Novel Biocompatible Immunoadjuvant for Antiserum Production Against Tityus serrulatus Scorpion Venom.

This study assesses the efficacy of different nanoemulsion formulations as new and innovative adjuvants for improving the in vivo immunization against the Tityus serrulatus scorpion venom. Nanoemulsions were designed testing key-variables such as surfactants, co-solvents, and the influence of the temperature, which would be able to induce the phase transition from a liquid crystal to a stable nanoemulsion, assessed for four months. Additionally, cationic-covered nanoemulsion with hyper-branched poly(ethyleneimine) was prepared and its performance was compared to the non-cationic ones. The physicochemical properties of the selected nanoemulsions and the interactions among their involved formulation compounds were carefully monitored. The cytotoxicity studies in murine macrophages (RAW 264.7) and red blood cells were used to compare different formulations. Moreover, the performance of the nanoemulsion systems as biocompatible adjuvants was evaluated using mice immunization protocol. The FTIR shifts and the zeta potential changes (from -18.3 ± 1.0 to + 8.4 ± 1.4) corroborated with the expected supramolecular anchoring of venom proteins on the surface of the nanoemulsion droplets. Cell culture assays demonstrated the non-toxicity of the formulations at concentrations less than 1.0 mg/mL, which were able to inhibit the hemolytic effect of the scorpion venom. The cationic-covered nanoemulsion has shown superior adjuvant activity, revealing the highest IgG titer in the immunized animals compared to both the non-cationic counterpart and the traditional aluminum adjuvant. In this approach, we demonstrate the incredible potential application of nanoemulsions as adjuvants, using a nanotechnology platform for antigen delivery system on immune cells. Additionally, the functionalization with hyper-branched poly(ethyleneimine) enhances this recognition and improves its action in immunization.

Self-assembled cationic-covered nanoemulsion as a novel biocompatible immunoadjuvant for antiserum production against Tityus serrulatus scorpion venom

This study assesses the efficacy of different nanoemulsion formulations as new and innovative adjuvants for improving the in vivo immunization against the Tityus serrulatus scorpion venom. Nanoemulsions were designed testing key-variables such as surfactants, co-solvents, and the influence of the temperature, which would be able to induce the phase transition from a liquid crystal to a stable nanoemulsion, assessed for four months. Additionally, cationic-covered nanoemulsion with hyper-branched poly(ethyleneimine) was prepared and its performance was compared to the non-cationic ones. The physicochemical properties of the selected nanoemulsions and the interactions among their involved formulation compounds were carefully monitored. The cytotoxicity studies in murine macrophages (RAW 264.7) and red blood cells were used to compare different formulations. Moreover, the performance of the nanoemulsion systems as biocompatible adjuvants was evaluated using mice immunization protocol. The FTIR shifts and the zeta potential changes (from −18.3 ± 1.0 to + 8.4 ± 1.4) corroborated with the expected supramolecular anchoring of venom proteins on the surface of the nanoemulsion droplets. Cell culture assays demonstrated the non-toxicity of the formulations at concentrations less than 1.0 mg/mL, which were able to inhibit the hemolytic effect of the scorpion venom. The cationic-covered nanoemulsion has shown superior adjuvant activity, revealing the highest IgG titer in the immunized animals compared to both the non-cationic counterpart and the traditional aluminum adjuvant. In this approach, we demonstrate the incredible potential application of nanoemulsions as adjuvants, using a nanotechnology platform for antigen delivery system on immune cells. Additionally, the functionalization with hyper-branched poly(ethyleneimine) enhances this recognition and improves its action in immunization

Therapeutic bullfrog oil-based nanoemulsion for oral application: Development, characterization and stability.

The aim of this study was to develop, optimize, and characterize a stable therapeutic bullfrog oil based nanoemulsion for oral application using a rational experimental design approach. The optimized oral nanoemulsion contained 0.2 % sodium benzoate and 0.02 % propyl-paraben as preservatives; 0.1 % sucralose and 0.4 % acesulfam K as sweeteners and 0.1 % tutti-frutti as flavoring to mask the unpleasant organoleptic characteristics of bullfrog oil. The oral O/W-nanoemulsion showed the droplet size, PDI, zeta potential, and pH of 410 ± 8 nm, 0.20 ± 0.02, -38 ± 2.5 mV, and 6.43 ± 0.05, respectively. The optimized oral nanoemulsion showed a milky single-phase and optimal physical stability at 25 °C for 90 days. Indeed, higher oxidation induction time and lower formation of peroxides in the oral nanoemulsion were responsible for improving its stability. A therapeutic delivery system containing bullfrog oil for oral application was successfully developed and optimized with ideal thermo-oxidative stability.

A Functional Wound Dressing as a Potential Treatment for Cutaneous Leishmaniasis.

Cutaneous leishmaniasis (CL) is a parasitic disease characterized by progressive skin sores. Currently, treatments for CL are limited to parenteral administration of the drug, which presents severe adverse effects and low cure rates. Therefore, this study aimed to develop poly(vinyl-alcohol) (PVA) hydrogels containing Amphotericin B (AmB) intended for topical treatment of CL. Hydrogels were evaluated in vitro for their potential to eliminate promastigote forms of Leishmania spp., to prevent secondary infections, to maintain appropriate healing conditions, and to offer suitable biocompatibility. AmB was incorporated into the system in its non-crystalline state, allowing it to swell more and faster than the system without the drug. Furthermore, the AmB release profile showed a continuous and controlled behavior following Higuchi´s kinetic model. AmB-loaded-PVA-hydrogels (PVA-AmB) also showed efficient antifungal and leishmanicidal activity, no cytotoxic potential for VERO cells, microbial impermeability and water vapor permeability compatible with the healthy skin's physiological needs. Indeed, these results revealed the potential of PVA-AmB to prevent secondary infections and to maintain a favorable environment for the healing process. Hence, these results suggest that PVA-AmB could be a suitable and efficient new therapeutic approach for the topical treatment of CL.

Understanding Drug Release Data through Thermodynamic Analysis.

Understanding the factors that can modify the drug release profile of a drug from a Drug-Delivery-System (DDS) is a mandatory step to determine the effectiveness of new therapies. The aim of this study was to assess the Amphotericin-B (AmB) kinetic release profiles from polymeric systems with different compositions and geometries and to correlate these profiles with the thermodynamic parameters through mathematical modeling. Film casting and electrospinning techniques were used to compare behavior of films and fibers, respectively. Release profiles from the DDSs were performed, and the mathematical modeling of the data was carried out. Activation energy, enthalpy, entropy and Gibbs free energy of the drug release process were determined. AmB release profiles showed that the relationship to overcome the enthalpic barrier was PVA-fiber > PVA-film > PLA-fiber > PLA-film. Drug release kinetics from the fibers and the films were better fitted on the Peppas-Sahlin and Higuchi models, respectively. The thermodynamic parameters corroborate these findings, revealing that the AmB release from the evaluated systems was an endothermic and non-spontaneous process. Thermodynamic parameters can be used to explain the drug kinetic release profiles. Such an approach is of utmost importance for DDS containing insoluble compounds, such as AmB, which is associated with an erratic bioavailability.

Preparation and characterization of safe microparticles based on xylan.

This work describes the preparation and evaluation of safe xylan-based microparticles prepared by cross-linking polymerization using sodium trimetaphosphate. The resulting microparticles were evaluated for morphology, particle size, polymer-cross-link agent interaction, and in vitro toxicity. The microparticles showed narrow monodisperse size distributions with their mean sizes being between 3.5 and 12.5 µm in dried state. FT-IR analyzes confirmed the interaction between sodium trimetaphosphate and xylan during the cross-linking process with formation of phosphate ester bonds. Additionally, the X-ray diffraction patterns and FT-IR analyzes suggested that little or no cross-linking agent remained inside the microparticles. Furthermore, the in-vitro studies using Artemia salina and human erythrocytes revealed that the microparticles are not toxic. Therefore, the overall results suggest that these xylan microparticles can be used as a platform for new drug delivery system.

The renoprotective effect of oral Tadalafil pretreatment on ischemia/reperfusion injury in rats.

PURPOSE: To evaluate the effect of tadalafil in renal ischemia/reperfusion (I/R) injury in rats Methods: Group I/R saline rats (n=6) were subjected to 45 minutes of left renal ischemia and treated with saline; the I/R tadalafil rats (n=6) received oral 10mg/kg tadalafil microemulsion one hour before ischemia. In both groups, 8 hours after ischemia, laboratory analysis were performed Results: Better tissue perfusion was lower in ischemic left/kidney than in right/kidney in saline group, suggesting reduced kidney clearance. Fluorescence in left/kidneys of tadalafil treated rats was lower than in right/kidneys (difference not significant). The fluorescence signal intensity in kidneys of tadafil treated rats was higher than in saline rats. TNF-α levels were significantly lower in I/R tadalafil group rats compared to I/R saline group (154±10.3 vs 391.3±12.3), as well as IL-1ß (163.4±13.2 vs 279±11.5pg/dL), and IL-6 (122.9±8.1 vs 173.7±6.3 respectively; p=0.0001). Urea, creatinine and C-reactive protein were significantly lower in tadafil treated rats then in saline group Conclusion: Tadalafil therapy decreased the expression of circulating pro-inflammatory cytokines in a renal I/R rodent model, while improving kidney function proofs.

The renoprotective effect of oral Tadalafil pretreatment on ischemia/reperfusion injury in rats

Abstract Purpose: To evaluate the effect of tadalafil in renal ischemia/reperfusion (I/R) injury in rats Methods: Group I/R saline rats (n=6) were subjected to 45 minutes of left renal ischemia and treated with saline; the I/R tadalafil rats (n=6) received oral 10mg/kg tadalafil microemulsion one hour before ischemia. In both groups, 8 hours after ischemia, laboratory analysis were performed Results: Better tissue perfusion was lower in ischemic left/kidney than in right/kidney in saline group, suggesting reduced kidney clearance. Fluorescence in left/kidneys of tadalafil treated rats was lower than in right/kidneys (difference not significant). The fluorescence signal intensity in kidneys of tadafil treated rats was higher than in saline rats. TNF-α levels were significantly lower in I/R tadalafil group rats compared to I/R saline group (154±10.3 vs 391.3±12.3), as well as IL-1β (163.4±13.2 vs 279±11.5pg/dL), and IL-6 (122.9±8.1 vs 173.7±6.3 respectively; p=0.0001). Urea, creatinine and C-reactive protein were significantly lower in tadafil treated rats then in saline group Conclusion: Tadalafil therapy decreased the expression of circulating pro-inflammatory cytokines in a renal I/R rodent model, while improving kidney function proofs.

Antibacterial properties and healing effects of Melipona scutellaris honey in MRSA-infected wounds of rats.

PURPOSE: To investigate the antimicrobial, immunological and healing effects of Melipona scutellaris honey on infected wounds of rat skin. METHODS: Twenty four Wistar rats were distributed in four groups (6-each). The uninfected skin wounds of group I rats were treated daily with saline for 7 days. Uninfected wounds (group II) rats were treated with honey. In group III (treated with saline) and group IV (treated with honey) wounds were inoculated with MRSA ATTC43300. The first bacterial culture was performed 24 hours later. In the 7th day new culture was done, and wound biopsies were used for cytokines dosage and histopathology. RESULTS: In group I and III rats the CFU/g count of S. aureus in wounds was zero. In group II rats the CFU/g counts in the wound tissue were significantly higher than in wounds of group IV rats. The density histopathological parameters and the expression of TNF-α, IL1-ß, Il-6 were significantly higher on wounds of group IV then in the other groups. CONCLUSION: Honey of Melipona scutellaris was effective in the management of infected wounds, by significant bacterial growth inhibition, enhancement of cytokine expression, and positively influenced the wound repair.

Antibacterial properties and healing effects of Melipona scutellaris honey in MRSA-infected wounds of rats

ABSTRACT PURPOSE : To investigate the antimicrobial, immunological and healing effects of Melipona scutellaris honey on infected wounds of rat skin. METHODS: Twenty four Wistar rats were distributed in four groups (6-each). The uninfected skin wounds of group I rats were treated daily with saline for 7 days. Uninfected wounds (group II) rats were treated with honey. In group III (treated with saline) and group IV (treated with honey) wounds were inoculated with MRSA ATTC43300. The first bacterial culture was performed 24 hours later. In the 7th day new culture was done, and wound biopsies were used for cytokines dosage and histopathology. RESULTS: In group I and III rats the CFU/g count of S. aureus in wounds was zero. In group II rats the CFU/g counts in the wound tissue were significantly higher than in wounds of group IV rats. The density histopathological parameters and the expression of TNF-α, IL1-β, Il-6 were significantly higher on wounds of group IV then in the other groups. CONCLUSION: Honey of Melipona scutellaris was effective in the management of infected wounds, by significant bacterial growth inhibition, enhancement of cytokine expression, and positively influenced the wound repair.

How can micelle systems be rebuilt by a heating process?

The aim of this work was to evaluate how an aqueous micellar system containing Amphotericin B (AmB) and sodium deoxycholate (DOC) can be rebuilt after heating treatment. Also, a review of the literature on the physicochemical and biological properties of this new system was conducted. Heated (AmB-DOC-H) and unheated (AmB-DOC) micelles were then diluted at four different concentrations (50 mg · L(-1), 5 mg · L(-1), 0.5 mg · L(-1), and 0.05 mg · L(-1)) to perform physicochemical studies and a pharmacotoxicity assay, in which two cell models were used for the in vitro experiments: red blood cells (RBC) from human donors and Candida parapsilosis (Cp). While potassium (K(+)) and hemoglobin leakage from RBC were the parameters used to evaluate acute and chronic toxicity, respectively, the efficacy of AmB-DOC and AmB-DOC-H were assessed by K(+) leakage and cell survival rate from Cp. The spectral study revealed a slight change in the AmB-DOC aggregate peak from 327 nm to 323 nm, which is the peak for AmB-DOC-H. Although AmB-DOC and AmB-DOC-H exhibited different behavior for hemoglobin leakage, AmB-DOC produced higher leakage than AmB-DOC-H at high concentrations (from 5 mg · L(-1)). For K(+) leakage, both AmB-DOC and AmB-DOC-H showed a similar profile for both cell models, RBC and Cp (P < 0.05). AmB-DOC-H and AmB-DOC also revealed a similar profile of activity against Cp with an equivalent survival rate. In short, AmB-DOC-H showed much less toxicity than AmB-DOC, but remained as active as AmB-DOC against fungal cells. The results highlight the importance of this new procedure as a simple, inexpensive, and safe way to produce a new kind of micelle system for the treatment of systemic fungal infections.

Prática do abortamento entre adolescentes: um estudo em dez escolas de Maceió (AL, Brasil) / Practice of abortion among teenagers: a study in ten schools of Maceió (AL, Brazil)

Trata-se de um estudo de corte transversal, realizado com o objetivo de investigar as razões que levaram adolescentes a provocarem o aborto, relacionando com idade e tipo de escola que frequentavam. A amostra foi calculada considerando o número de internações para curetagem pós-abortamento. Usou-se como instrumento um questionário semiestruturado, anônimo, aplicado em dez escolas, sorteadas dentre todas da cidade de Maceió (Alagoas), pesquisando-se adolescentes dos 12 aos 19 anos, do sexo feminino. Os dados foram analisados pelo Programa Epi Info, usando-se odds ratio e risco relativo para verificar associação entre variáveis e intervalo de confiança a 95 por cento. Em uma amostra de 2.592 jovens, 559 (21,6 por cento) tinham vida sexual ativa, 182 (7,0 por cento) referiram ter engravidado e 149 (26,7 por cento) abortado. Medo da reação dos pais, idade, falta de apoio do companheiro e rejeição da gravidez foram razões para provocar o aborto, sendo medo a mais citada, em ambos os tipos de escola. O aborto foi mais citado nas escolas públicas, sendo significativo e protetor o risco para abortar antes dos 15 anos, e significativa a relação entre abortar e estudar em escolas públicas. O medo da reação dos pais como razão mais frequente sugere a necessidade de novos estudos sobre sexualidade e comunicação entre pais e filhos.

This is a cross-cut study that was carried out with the objective of investigating the causes through which adolescents have provoked abortion, relating it to age and the type of school attended. The sample was calculated by taking into account the number of hospital admissions for post-abortion curettage. A semi-structured and anonymous questionnaire was used as a research instrument which was applied in ten schools randomly chosen among all schools, of Maceió (Alagoas, Brazil), to reach 12-19 years old female teenagers. The data were assessed by the Epi Info Program making use of odds ratio and a relative risk to verify any association among variables and a confidence interval at 95 percent. At a sample of 2,592 adolescents, 559 (21.6 percent) had an active sexual life, 182 (7.0 percent) informed to have been pregnant and 149 (26.7 percent) to have aborted. The fear of the parents' reaction, age, lack of support of the partner and pregnancy rejection were the explanation to stimulate abortion. Fear was the most mentioned cause in both types of school. Abortion was more mentioned in public schools, being significant and protective the risk of aborting before the age of 15. The fear of the parents' reaction as a frequent cause suggests the necessity for additional studies on sexuality and communication between parents and children.

[Cardiometabolic abnormalities in patients with Berardinelli-Seip syndrome]. / Anormalidades cardiovasculares e metabólicas em pacientes com a síndrome de Berardinelli-Seip.

BACKGROUND: Berardinelli-Seip syndrome (BSS) or Generalized Congenital Lipodystrophy often affects the cardiovascular system and also promotes metabolic abnormalities involving glycidic and lipid metabolisms. OBJECTIVE: To assess the prevalence of cardiometabolic abnormalities in patients with BSS. METHODS: Twenty-two patients from the state of Rio Grande do Norte, Brazil, diagnosed with BSS, underwent clinical evaluation, resting electrocardiogram, echodopplercardiogram, chest X-ray, 24-hour ambulatory electrocardiogram monitoring, exercise testing and laboratory analysis. RESULTS: The patients were predominantly young adults, most of whom women. The whole sample showed insulin resistance, acanthosis nigricans and diminished HDL-cholesterol. The presence of splenomegaly, hepatomegaly, type II diabetes and elevated triglycerides was constant. Metabolic syndrome was characterized in most patients, which were predominantly women and with a high degree of paternal consanguinity. SAH and prehypertension blood pressure were found in more than half of the patients (77.3%). The echodopplercardiogram showed the presence of CLVH (50%), eccentric left ventricular hypertrophy (4.5%), and normal left ventricular geometry (45.5%). High arrhythmia rates were observed by Holter monitoring, such as ventricular ectopic beats, supraventricular ectopic beats and sustained supraventricular tachycardia. Chronotropic incompetence (54.5%) was observed during exercise testing. CONCLUSION: A high prevalence of cardiovascular and metabolic abnormalities was observed in young asymptomatic individuals with BSS. These findings point to the need for systematic cardiological follow-up and of preventive measures in this high-risk group.

Anormalidades cardiovasculares e metabólicas em pacientes com a síndrome de Berardinelli-Seip / Cardiometabolic abnormalities in patients with berardinelli-Seip syndrome / Anormalidades cardiovasculares y metabólicas en pacientes con el síndrome de Berardinelli-Seip

Fundamento: A síndrome de Berardinelli-Seip (SBS) ou lipodistrofia generalizada congênita acomete, frequentemente, o aparelho cardiovascular e também promove anormalidades metabólicas que envolvem os metabolismos glicídico e lipídico. Objetivo: Avaliar a prevalência das anormalidades cardiovasculares e metabólicas em portadores da SBS. Métodos: Vinte e dois pacientes do Estado do Rio Grande do Norte (Brasil), com diagnóstico da SBS, foram submetidos a avaliação clínica, eletrocardiograma de repouso, ecodopplercardiograma, radiografia de tórax, eletrocardiografia dinâmica de 24 horas, teste ergométrico e análise laboratorial. Resultados: Os pacientes eram, predominantemente, adultos jovens, sendo a maioria do sexo feminino. A totalidade da amostra apresentou resistência à insulina, acanthosis nigricans e HDL-colesterol diminuído. A presença de esplenomegalia, hepatomegalia, diabetes mellitus tipo II e triglicérides elevados era constante. A síndrome metabólica foi caracterizada na maioria dos pacientes, com predominância no sexo feminino e com um alto grau de consanguinidade paterna. A hipertensão arterial sistêmica e a pré-hipertensão foram encontradas em mais da metade dos pacientes (77,3 por cento). O ecodopplercardiograma mostrou a presença de hipertrofia concêntrica do ventrículo esquerdo (50 por cento), hipertrofia excêntrica do ventrículo esquerdo (4,5 por cento) e geometria normal do ventrículo esquerdo (45,5 por cento). Elevada taxa de arritmia foi evidenciada no holter, tais como extrassístoles ventriculares, extrassístoles supraventriculares e taquicardia supraventricular sustentada. A incompetência cronotrópica (54,5 por cento) foi observada no teste ergométrico. Conclusão: Anormalidades cardiovasculares e metabólicas foram observadas em elevada prevalência em indivíduos jovens e assintomáticos com SBS. Esses achados apontam para a necessidade de acompanhamento cardiológico sistemático e de medidas preventivas nesse grupo de risco.

Background: Berardinelli-Seip syndrome (BSS) or Generalized Congenital Lipodystrophy often affects the cardiovascular system and also promotes metabolic abnormalities involving glycidic and lipid metabolisms. Objective: To assess the prevalence of cardiometabolic abnormalities in patients with BSS. Methods: Twenty-two patients from the state of Rio Grande do Norte, Brazil, diagnosed with BSS, underwent clinical evaluation, resting electrocardiogram, echodopplercardiogram, chest X-ray, 24-hour ambulatory electrocardiogram monitoring, exercise testing and laboratory analysis. Results: The patients were predominantly young adults, most of whom women. The whole sample showed insulin resistance, acanthosis nigricans and diminished HDL-cholesterol. The presence of splenomegaly, hepatomegaly, type II diabetes and elevated triglycerides was constant. Metabolic syndrome was characterized in most patients, which were predominantly women and with a high degree of paternal consanguinity. SAH and prehypertension blood pressure were found in more than half of the patients (77.3 percent). The echodopplercardiogram showed the presence of CLVH (50 percent), eccentric left ventricular hypertrophy (4.5 percent), and normal left ventricular geometry (45.5 percent). High arrhythmia rates were observed by Holter monitoring, such as ventricular ectopic beats, supraventricular ectopic beats and sustained supraventricular tachycardia. Chronotropic incompetence (54.5 percent) was observed during exercise testing. Conclusion: A high prevalence of cardiovascular and metabolic abnormalities was observed in young asymptomatic individuals with BSS. These findings point to the need for systematic cardiological follow-up and of preventive measures in this high-risk group.

Fundamento: El síndrome de Berardinelli-Seip (SBS) o lipodistrofia generalizada congénita, afecta frecuentemente el aparato cardiovascular y también promueve anormalidades metabólicas que involucran los metabolismos glucídico y lipídico. Objetivo: Evaluar la prevalencia de las anormalidades cardiovasculares y metabólicas en portadores de SBS. Métodos: Veintidós pacientes del Estado de Rio Grande do Norte (Brasil), con diagnóstico de SBS, se sometieron a evaluación clínica, electrocardiograma de reposo, ecocardiograma doppler, radiografía de tórax, electrocardiografía dinámica de 24 horas, ergometría y análisis de laboratorio. Resultados: Los pacientes eran, predominantemente, adultos jóvenes, siendo en su mayoría del sexo femenino. La totalidad de la muestra presentó resistencia a la insulina, acanthosis nigricans y HDL-colesterol disminuido. La presencia de esplenomegalia, hepatomegalia, diabetes mellitus tipo II y triglicéridos elevados era constante. El síndrome metabólico se caracterizó en la mayoría de los pacientes, con predominancia en el sexo femenino y con un alto grado de consanguinidad paterna. En más de la mitad de los pacientes (77,3 por ciento) se encontraron hipertensión arterial sistémica y la prehipertensión. El ecocardiograma Doppler mostró presencia de hipertrofia concéntrica del ventrículo izquierdo (50 por ciento), hipertrofia excéntrica del ventrículo izquierdo (4,5 por ciento) y geometría normal del ventrículo izquierdo (45,5 por ciento). Se evidenció tasa elevada de arritmia en el holter, tales como extrasístoles ventriculares, extrasístoles supraventriculares y taquicardia supraventricular sostenida. La incompetencia cronotrópica (54,5 por ciento) se observó en la ergometría. Concludión: Anormalidades cardiovasculares y metabólicas se observaron en elevada prevalencia en individuos jóvenes y asintomáticos con SBS. Estos hallazgos señalan la necesidad de seguimiento cardiológico sistemático y de medidas preventivas...

Alteraçõs cardiovasculares e metabólicas da lipodistrofia generalizada congênita (Síndrome de Seip-Berardinelli / Cardiovascular and metabolic aspects of congenital generalized lipodystrophy (Seip-Berardinelli Syndrome)

A lipodistrofia é uma doença bem pouco estudada pela comunidade médica nacional e internacional. O surgimento de lesões do tipo de lipodistrofia adquirida associada ao uso de terapia retroviral nos portadores de HIV tem despertado maior interesse pela investigação nesta área. O objetivo deste artigo de atualização é descrever os aspectos cardiovasculares que acometem uma classe de pacientes portadores da forma de lipodistrofia generalizada congenita (LGC) também denominada de sindrome de Seip-Berardinelli. Inicialmente serão enfatizados os tipos conhecidos de lipodistrofias para, que em seguida, ser apresentado os relatos de casos encontrados na literatura, desde 1885 ate os dias atuais. As anormalidades cardiovasculares nos portadores desta sindrome são frequentes, sendo observada a presença de hipertensão arterial sistêmica, hipertrofia ventricular esquerda, cardiomiopatia hipertrófica e cardiomiopatia dilatada. Diabetes mellitus, hiperglicemia e hipertrigliceridemia também são ferquentes.