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BACKGROUND: Functional impairment is the main consideration when it comes to choosing therapy for infantile hemangiomas (IH). However, since most hemangiomas are treated for cosmetic reasons, it is important to know the cosmetic outcome assessed by the parents. OBJECTIVE: To evaluate the aesthetic outcomes of IH, considering the characteristics of the lesions and the treatments used. PATIENTS AND METHODS: The Spanish Infantile Hemangioma Nationwide Prospective Cohort (2016-2022) recruited all consecutive patients diagnosed with IH in 12 Spanish hospitals. The children included had 2 photos of the IH lesion (at both baseline and at the end of the study). A panel of parents blindly assessed all available photos using a scale from 0 (worst cosmetic outcomes) to 10 (best cosmetic outcomes). The different scores -both before and after treatment-as well as the outcomes percent considered excellent (> 9) were described and compared. We analyzed the effect of receiving different therapies and performed causal model analyses estimating the mean treatment effect of parents' assessments. RESULTS: The median follow-up was 3.1 years. A total of 824 photos were evaluated. Baseline aesthetic impact was higher in the propranolol group vs the topical timolol and observation treatment groups (1.85 vs 3.14 vs 3.66 respectively; p < 0.001). After treatment, the aesthetic impact was similar between both treatment groups (7.59 vs 7.93 vs 7.90; p > 0.2). The causal model could only be applied to the comparison between topical timolol and observation, revealing no differences whatsoever. CONCLUSION: This is the first prospective cohort to analyze the aesthetic outcome of IH. The final aesthetic results of the 3 therapies were similar, with nearly 40% of patients achieving excellent aesthetic outcomes.
Assuntos
Hamartoma , Nevo , Esclerose Tuberosa , Humanos , Esclerose Tuberosa/complicações , Hamartoma/complicações , ColágenoAssuntos
Hamartoma , Nevo , Esclerose Tuberosa , Humanos , Esclerose Tuberosa/complicações , Hamartoma/complicações , ColágenoRESUMO
Hair shaft disorders, involving dysplastic abnormalities in the shaft, may be either congenital or acquired. Two large categories have been defined according to the presence or not of hair fragility. A diagnosis can usually be made after taking a thorough medical history and performing a physical examination. Trichoscopy has become a useful, cost-effective tool in recent years, particularly for examining the hair of children, because it facilitates inspection without removal of hairs. Structural abnormalities in the hair shaft are sometimes clues to the diagnosis of more complex diseases in which early treatment can improve prognosis. This review describes key features that enable the diagnosis of the most common hair shaft disorders and discusses the various treatments currently available.
RESUMO
Hair shaft disorders, involving dysplastic abnormalities in the shaft, may be caused by genetic mutations or acquired through environmental exposures. The second part of this review presents these disorders classified according to the degree of hair fragility. It is important to take a thorough medical history and examine the hair to detect changes in texture, density, quality, and whether fragility is observed or not. Trichoscopy is a useful, noninvasive tool that can suggest a diagnosis in most cases. Specific treatments for hair shaft disorders are not available at present. We recommend general care practices to prevent hair damage; examples are avoiding excessive brushing, chemical products, hairstyles that introduce tension, and exposure to excessive heat. Some hair shaft disorders improve with puberty. Others may respond to treatments such as topical applications of minoxidil.
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BACKGROUND: There are several therapeutic options for infantile haemangiomas (IH). Propranolol is used according to a pivotal trial. We aimed to describe the characteristics of IH in clinical practice, including the therapies used, and to compare the characteristics of patients treated with propranolol with those of the trial to assess its external validity. METHODS: Consecutive patients attending 12 Spanish hospitals from June 2016 to October 2019 were included (n=601). RESULTS: The mean age was 3.9 (SD:1.9) months, with a 2:1 female-to-male ratio. Most IHs were localized (82%, 495), superficial (64%, 383) and located in the face (25%, 157) and trunk (31%, 188). Median size was 17 (IR: 10-30) x 12 (IR: 7-20) mm. Complications were found in 16 (3%) patients. Treatment was initiated for 52% (311). Most patients received timolol (76%, 237); propranolol was reserved for complications or high-risk IHs. Aesthetic impairment was the main reason for starting therapy (64%, 199). Several characteristics of the patients and IHs treated with propranolol are similar to those of the pivotal clinical trial, but 1/3 of IHs did not reach the minimum diameter to meet the inclusion criteria, and important prognostic information was not reported. CONCLUSIONS: As most patients receive treatment for aesthetic impairment, there is a need to better understand the aesthetic results of therapies and to increase evidence on the use of timolol, which is currently the most common therapy. Propranolol is being used in a population generally similar to that of the trial; however, this statement cannot be definitely confirmed.
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Dermatoses da Mão/patologia , Ceratose/patologia , Idoso , Dermoscopia , Feminino , Humanos , Pessoa de Meia-Idade , FotografaçãoRESUMO
Ras-associated autoimmune leucoproliferative disorder (RALD) is a nonmalignant syndrome associated with somatic KRAS mutations. We report a patient with RALD and cutaneous lesions, the first such case reported, to our knowledge. An 8-year-old boy presented with erythematous plaques on his face and body, along with lymphadenopathies and spleen enlargement without systemic symptoms. An increased number of monocytes were found in skin biopsy, peripheral blood and bone marrow (BM). Juvenile myelomonocytic leukaemia (JMML) was suspected. Genetic study using peripheral blood showed no mutations in the KRAS, PTPN11, NRAS, CBL or BCR-ABL genes, but bone marrow analysis revealed a mutation (p-G12S/c.34 G>A) in the KRAS gene. The karyotype was normal. No KRAS mutations were found using molecular analysis of saliva. The diagnosis of RALD was proposed. The differential diagnosis between RALD and JMML is challenging because there are no established criteria to differentiate between them. The clinical course of RALD is uncertain, so long-term follow-up is recommended.
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Síndrome Linfoproliferativa Autoimune/diagnóstico , Proteínas Proto-Oncogênicas p21(ras) , Dermatopatias/etiologia , Pele/patologia , Síndrome Linfoproliferativa Autoimune/complicações , Síndrome Linfoproliferativa Autoimune/genética , Síndrome Linfoproliferativa Autoimune/patologia , Biópsia , Criança , Análise Mutacional de DNA , Diagnóstico Diferencial , Genes ras , Humanos , Leucemia Mielomonocítica Juvenil/diagnóstico , Masculino , Mutação , Proteínas Proto-Oncogênicas p21(ras)/genéticaRESUMO
OBJECTIVE: To explore if the changes in vasoactive substances observed during early pregnancy in the rat are modulated by maternal or fetoplacental factors. METHODS: Urinary excretion of cGMP, 6-keto-prostaglandin-F1 alpha (6-keto-PGF1 alpha), thromboxane B2 and kallikrein activity was measured in pregnant (P, n = 11), pseudopregnant (PSP, n = 12), and virgin (n = 13) rats and in ovariectomized virgin rats supplemented with slow-release pellets containing either progesterone (50 mg/pellet) or estradiol (0.5 mg/pellet) or a combination of both hormones, for 21 days. RESULTS: The cGMP excretion was higher in PSP rats than in virgin rats at day 5 (virgin = 82 +/- 7, P = 93 +/- 5, PSP = 110 +/- 8 nmol/24 h, p < 0.05); at day 10, values were significantly increased in P and PSP rats. 6-keto-PGF1 alpha excretion was similarly elevated in P and PSP rats at day 5 (virgin = 120 +/- 10, P = 160 +/- 10, and PSP = 174 +/- 14 ng/24 h, p < 0.01). This trend was still present at day 10. Thromboxane B2 excretion showed a nonsignificant increase in P and PSP rats in day 5; at day 10, values were significantly elevated in both experimental groups (virgin = 23 +/- 2, P = 32 +/- 4, and PSP = 32 +/- 2 ng/24 h, p < 0.05). Kallikrein excretion was significantly increased in PSP and P rats at days 5 and 10. Estradiol or progesterone administration caused a significant decrease in serum aldosterone and an increase in urinary kallikrein activity. CONCLUSIONS: These results indicate that during the first half of rat pregnancy, the increment in vasoactive substances is modulated by maternal and not by fetoplacental factors.
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Pseudogravidez/urina , 6-Cetoprostaglandina F1 alfa/urina , Aldosterona/sangue , Aldosterona/urina , Animais , Biomarcadores/sangue , Biomarcadores/urina , GMP Cíclico/urina , Feminino , Ovariectomia , Gravidez , Progesterona/farmacologia , Pseudogravidez/sangue , Ratos , Ratos Sprague-Dawley , UrinaRESUMO
La emesis es un gran problema en la quimioterapia con cis-platino. Probamos un nuevo agente (ondansetron) versus metoclopramida (terapia clásica). Un estudio cross-over prospectivo, doble ciego, con un período de wash-out de 3 semanas entre una droga y la otra. La droga inicial se asignó randomizadamente. Se enrolaron 20 pacientes y todos terminaron al estudio. 30 minutos antes de que el cis-platino fuera inyectado al paciente recibió LV ya sea 0.15 mg/kg de ondansetron o 1 mg/kg de metoclopramida. La dosis fue repetida c/8 horas por 2 veces. El principal fue el número de vómitos en cada paciente ese día Excelente: fue 0 vómitos, buena respuesta: < 2 vómitos y mala: > 3 vómitos. También como resultado secundarios, en una escala analógica se midió náusea e ingesta de alimentos. Considerando vómito hubo respuesta excelente en 60 por ciento de los pacientes con ondansetron versus 25 por ciento con metoclopramida. Mala respuesta en el 15 por ciento para ondansetron versus 70 por ciento para metoclopramida. El score promedio para náusea en la escala analógica fue 1.1 para pacientes con ondansetron y 4.5 para metoclopramida. Usando análisis estadístico de varianza bajo un diseño cross-over, para vómito los resultados fueron altamente significativos en favor de ondansetron (p < 0.0005). Lo mismo ocurrió para náusea (p < 0.0013) y para ingesta de alimentos (p < 0.0044). Ondansetron es claramente superior a Metoclopramida como antiemético en quimioterapia
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Humanos , Masculino , Cisplatino/efeitos adversos , Metoclopramida/administração & dosagem , Ondansetron/administração & dosagem , Neoplasias Testiculares/tratamento farmacológico , Antieméticos/administração & dosagem , Náusea/tratamento farmacológico , Vômito/tratamento farmacológicoRESUMO
Se presentan 3 casos de pacientes portadores de oncocitoma renal operados durante los últimos 10 años (1,8 por ciento). En todos se realizó nefrectomía radical con buena evolución durante 17 meses de seguimiento. Se revisa la literatura
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Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Adenoma Oxífilo/diagnóstico , Neoplasias Renais , Adenoma Oxífilo/cirurgia , Adenoma Oxífilo/ultraestrutura , Nefrectomia , UrografiaRESUMO
Levels of prostatic specific antigen may be modified with procedures over the prostatic gland. We measured this antigen in 36 hospitalized patients before and immediately after a digital rectal examination. Antigen levels were 8.3 +/- 21.3 ng/ml before and 13.8 +/- 27.5 ng/ml after the examination (NS). It is concluded that digital rectal examination did not modify antigen levels in our sample.