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1.
Clin Dermatol ; 39(5): 879-886, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34785016

RESUMO

The Asian population currently constitutes a simple majority of the global population, comprising nearly 60%. The percentage of the US population that identifies as Asian is expected to grow to 41 million by the year 2050, making up an eventual 9% of the US population. As the world and US populations of Asian individuals increase, the demand for dermatologic care from this population will increase, requiring dermatologists to become more familiar with the diagnosis and treatment of Asian-specific skin characteristics and diseases. In this contribution, we review skin conditions specific to or relatively more common in Asian patients to help recognition and management of diseases in an increasing Asian patient population. We discuss prurigo pigmentosa, primary cutaneous plasmacytosis, lipodystrophia centrifugalis abdominalis infantilis, Epstein-Barr viru-positive T- and natural killer-cell lymphoproliferative disorders, acquired bilateral nevus of Ota-like macules, and BehÒ«et disease.


Assuntos
Lipodistrofia , Nevo , Prurigo , Neoplasias Cutâneas , Humanos , Pele
3.
Curr Med Res Opin ; 37(11): 1881-1889, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34490811

RESUMO

OBJECTIVE: To evaluate the effects of metreleptin in distinct subgroups of patients with generalized lipodystrophy (GL) and partial lipodystrophy (PL), using multivariate linear regression modeling to account for the role of patients' baseline usage of concomitant glucose and lipid-lowering medications and other covariates on their outcomes. MATERIALS AND METHODS: A post-hoc statistical analysis of two published single-arm, interventional, phase 2 clinical trials at NIH was conducted. Concomitant medication use was assessed for the clinical trial population using prescription fill data, measured at baseline and the post-one year following metreleptin initiation. Pre-specified co-primary efficacy endpoints measured were change from baseline in HbA1c at month 12, and the percent change from baseline in fasting serum triglycerides (TG) at month 12. Descriptive and statistical analyses were conducted for the overall population, the separate populations with GL and PL, and additional PL subgroups defined by baseline metabolic markers of elevated HbA1c and elevated fasting TG. RESULTS: As previously reported, improvement in HbA1c and fasting TG from baseline to 12 months on metreleptin were observed in the overall population (mean change -1.57 percentage points and median change -37.9%, respectively) and subgroups. For both HbA1c and TG, baseline levels were significant predictors of changes after metreleptin. After considering baseline characteristics such as disease type, age, sex, and baseline HbA1c, baseline insulin use was not found to be a significant predictor of HbA1c improvement following metreleptin initiation. Similar results were seen for TG levels, with the use of any lipid-lowering medications at baseline not found to be a significant predictor of reductions in fasting TG levels. CONCLUSIONS: Patients treated with metreleptin experienced statistically significant improvement in metabolic markers of glycemic and hypertriglyceridemic control-e.g. HbA1c and triglyceride levels-across various subgroups after controlling for baseline characteristics and concomitant medication usage.


Assuntos
Leptina , Lipodistrofia , Glicemia , Jejum , Humanos , Leptina/análogos & derivados
5.
Int J Mol Sci ; 22(18)2021 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-34576123

RESUMO

Nasu-Hakola Disease (NHD) is a recessively inherited systemic leukodystrophy disorder characterized by a combination of frontotemporal presenile dementia and lytic bone lesions. NHD is known to be genetically related to a structural defect of TREM2 and DAP12, two genes that encode for different subunits of the membrane receptor signaling complex expressed by microglia and osteoclast cells. Because of its rarity, molecular or proteomic studies on this disorder are absent or scarce, only case reports based on neuropsychological and genetic tests being reported. In light of this, the aim of this paper is to provide evidence on the potential of a label-free proteomic platform based on the Multidimensional Protein Identification Technology (MudPIT), combined with in-house software and on-line bioinformatics tools, to characterize the protein expression trends and the most involved pathways in NHD. The application of this approach on the Lymphoblastoid cells from a family composed of individuals affected by NHD, healthy carriers and control subjects allowed for the identification of about 3000 distinct proteins within the three analyzed groups, among which proteins anomalous to each category were identified. Of note, several differentially expressed proteins were associated with neurodegenerative processes. Moreover, the protein networks highlighted some molecular pathways that may be involved in the onset or progression of this rare frontotemporal disorder. Therefore, this fully automated MudPIT platform which allowed, for the first time, the generation of the whole protein profile of Lymphoblastoid cells from Nasu-Hakola subjects, could be a valid approach for the investigation of similar neurodegenerative diseases.


Assuntos
Lipodistrofia/metabolismo , Lipodistrofia/patologia , Linfócitos/metabolismo , Linfócitos/patologia , Osteocondrodisplasias/metabolismo , Osteocondrodisplasias/patologia , Proteômica , Panencefalite Esclerosante Subaguda/metabolismo , Panencefalite Esclerosante Subaguda/patologia , Análise por Conglomerados , Análise Discriminante , Humanos , Glicoproteínas de Membrana/metabolismo , Mapas de Interação de Proteínas , Receptores Imunológicos/metabolismo , Biologia de Sistemas
7.
Medicine (Baltimore) ; 100(29): e26743, 2021 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-34398053

RESUMO

ABSTRACT: The aim of this study was to explore the values of ultrasound for diagnosis and management of insulin-induced lipohypertrophy and further analyzing the impact of body mass index and subcutaneous fat thickness on ultrasound manifestations of lipohypertrophy.In this 3-month, prospective cohort study, a total of 162 patients with diabetes who used insulin therapy more than 1 year with unknown lipohypertrophy status were enrolled into this study. Demographic information, assessment of glycemic control and insulin injection technique were evaluated. Physical and ultrasound examination were separately performed to detect lipohypertrophy by a team of diabetes educator nurses or ultrasonographer in a blinded fashion. Patients with lipohypertrophy received insulin injection technique education based on ultrasound examination and Chinese guideline.Ultrasound examination detected 41.1% more patients (74.1% vs 52.5%; P < .001) with lipohypertrophy and 61.2% more lesions (216 vs 134; P < .001) than physical examination. Glycosylated hemoglobin A1c and fasting blood glucose were significantly decreased in patients with lipohypertrophy or subclinical lipohypertrophy (lipohypertrophy without visual and palpation changes) after receiving insulin injection technique education based on ultrasound examination and Chinese guideline than baseline at 3 months (P < .001). The proportion of lesions with ultrasound manifestation 2 (distortion of surrounding connective tissue) in obese and STF (>15 mm) groups were no more than 50% and showed a decreased trend with increased subcutaneous fat thickness and body mass index (P < .001).Lipohypertrophy has characteristic ultrasound manifestations which can detect more accurate results than palpation alone and provide detailed information to promote effective education on lipohypertrophy management, thereby improving glycemic control.


Assuntos
Lipodistrofia/diagnóstico , Glicemia , China , Estudos de Coortes , Diabetes Mellitus Tipo 1/tratamento farmacológico , Diabetes Mellitus Tipo 2/tratamento farmacológico , Feminino , Hemoglobina A Glicada , Humanos , Insulina/efeitos adversos , Lipodistrofia/induzido quimicamente , Lipodistrofia/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Ultrassonografia
8.
Pediatr Dermatol ; 38(5): 1292-1297, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34418138

RESUMO

Annular lipoatrophy of the ankle is a rare and unique acquired lipoatrophic panniculitis that mainly affects children. There is no consensus on treatment, and the long-term course is not well known. We present four new pediatric cases that contribute to the understanding of this rare disease.


Assuntos
Lipodistrofia , Paniculite , Tornozelo , Atrofia/patologia , Criança , Humanos , Lipodistrofia/diagnóstico , Paniculite/patologia , Gordura Subcutânea/patologia
9.
Nutrients ; 13(8)2021 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-34444659

RESUMO

Lipodystrophy syndromes (LD) are a heterogeneous group of very rare congenital or acquired disorders characterized by a generalized or partial lack of adipose tissue. They are strongly associated with severe metabolic dysfunction due to ectopic fat accumulation in the liver and other organs and the dysregulation of several key adipokines, including leptin. Treatment with leptin or its analogues is therefore sufficient to reverse some of the metabolic symptoms of LD in patients and in mouse models through distinct mechanisms. Brown adipose tissue (BAT) thermogenesis has emerged as an important regulator of systemic metabolism in rodents and in humans, but it is poorly understood how leptin impacts BAT in LD. Here, we show in transgenic C57Bl/6 mice overexpressing sterol regulatory element-binding protein 1c in adipose tissue (Tg (aP2-nSREBP1c)), an established model of congenital LD, that daily subcutaneous administration of 3 mg/kg leptin for 6 to 8 weeks increases body temperature without affecting food intake or body weight. This is associated with increased protein expression of the thermogenic molecule uncoupling protein 1 (UCP1) and the sympathetic nerve marker tyrosine hydroxylase (TH) in BAT. These findings suggest that leptin treatment in LD stimulates BAT thermogenesis through sympathetic nerves, which might contribute to some of its metabolic benefits by providing a healthy reservoir for excess circulating nutrients.


Assuntos
Tecido Adiposo Marrom/efeitos dos fármacos , Leptina/farmacologia , Lipodistrofia/tratamento farmacológico , Termogênese , Tecido Adiposo Marrom/inervação , Tecido Adiposo Marrom/metabolismo , Animais , Modelos Animais de Doenças , Lipodistrofia/genética , Lipodistrofia/metabolismo , Lipodistrofia/fisiopatologia , Masculino , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Receptores de LDL/genética , Receptores de LDL/metabolismo , Proteína de Ligação a Elemento Regulador de Esterol 1/genética , Proteína de Ligação a Elemento Regulador de Esterol 1/metabolismo , Sistema Nervoso Simpático/efeitos dos fármacos , Sistema Nervoso Simpático/metabolismo , Sistema Nervoso Simpático/fisiopatologia , Tirosina 3-Mono-Oxigenase/metabolismo , Proteína Desacopladora 1/metabolismo
10.
Sci Rep ; 11(1): 14560, 2021 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-34267289

RESUMO

Lipodystrophic mice are protected from cartilage damage following joint injury. This protection can be reversed by the implantation of a small adipose tissue graft. The purpose of this study was to evaluate the relationship between the gut microbiota and knee cartilage damage while controlling for adiposity, high fat diet, and joint injury using lipodystrophic (LD) mice. LD and littermate control (WT) mice were fed a high fat diet, chow diet, or were rescued with fat implantation, then challenged with destabilization of the medial meniscus surgery to induce osteoarthritis (OA). 16S rRNA sequencing was conducted on feces. MaAslin2 was used to determine associations between taxonomic relative abundance and OA severity. While serum LPS levels between groups were similar, synovial fluid LPS levels were increased in both limbs of HFD WT mice compared to all groups, except for fat transplanted animals. The Bacteroidetes:Firmicutes ratio of the gut microbiota was significantly reduced in HFD and OA-rescued animals when compared to chow. Nine novel significant associations were found between gut microbiota taxa and OA severity. These findings suggest the presence of causal relationships the gut microbiome and cartilage health, independent of diet or adiposity, providing potential therapeutic targets through manipulation of the microbiome.


Assuntos
Cartilagem/fisiopatologia , Dieta Hiperlipídica/efeitos adversos , Microbioma Gastrointestinal/fisiologia , Osteoartrite/microbiologia , Adiposidade , Animais , Bacteroidetes/genética , Feminino , Firmicutes/genética , Microbioma Gastrointestinal/genética , Lipodistrofia/microbiologia , Lipopolissacarídeos/sangue , Masculino , Menisco/cirurgia , Camundongos Transgênicos , Obesidade/microbiologia , Osteoartrite/etiologia , RNA Ribossômico 16S/genética , Líquido Sinovial/metabolismo
11.
BMJ Case Rep ; 14(7)2021 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-34290016

RESUMO

Acquired localised lipoatrophy is a focal loss of subcutaneous fat, which is commonly secondary to trauma, injections of medications such as antibiotics or corticosteroids, pressure, previous surgery or panniculitis. We present a case of a patient who experienced focal fat loss in the left gluteal region from a previous left transgluteal drainage of a suspected abscess. There was no medical history of corticosteroid, antibiotic injection or use of highly active antiretroviral therapy. Lipoatrophy occurring as a consequence of a deep pelvic abscess drainage has not been reported in the literature; however, based on the lack of other aetiologies, the diagnosis of acquired localised lipoatrophy secondary to a transgluteal drainage was made in this patient. The aim of this report was to present this rare cause of lipoatrophy that has not previously been described and to acknowledge lipoatrophy as a potential side effect of a deep abscess drainage.


Assuntos
Lipodistrofia , Paniculite , Abscesso/etiologia , Nádegas , Drenagem , Humanos , Lipodistrofia/induzido quimicamente
12.
Int J Mol Sci ; 22(12)2021 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-34205507

RESUMO

Complement overactivation has been reported in most patients with Barraquer-Simons syndrome (BSS), a rare form of acquired partial lipodystrophy. Complement Factor D (FD) is a serine protease with a crucial role in the activation of the alternative pathway of the complement system, which is mainly synthesized by adipose tissue. However, its role in the pathogenesis of BSS has not been addressed. In this study, plasma FD concentration was measured in 13 patients with BSS, 20 patients with acquired generalized lipodystrophy, 22 patients with C3 glomerulopathy (C3G), and 50 healthy controls. Gene expression and immunohistochemistry studies were assayed using atrophied adipose tissue from a patient with BSS. We found significantly elevated FD levels in BSS cases compared with the remaining cohorts (p < 0.001). There were no significant differences in FD levels between sexes but FD was strongly and directly associated with age in BSS (r = 0.7593, p = 0.0036). A positive correlation between FD and C3 was seen in patients with C3G, characterized by decreased FD levels due to chronic C3 consumption, but no correlation was detected for BSS. Following mRNA quantification in the patient's adipose tissue, we observed decreased CFD and C3 but elevated C5 transcript levels. In contrast, the increased FD staining detected in the atrophied areas reflects the effects of persistent tissue damage on the adipose tissue, thus providing information on the ongoing pathogenic process. Our results suggest that FD could be a reliable diagnostic biomarker involved in the pathophysiology of BSS by promoting unrestrained local complement system activation in the adipose tissue environment.


Assuntos
Fator D do Complemento/metabolismo , Lipodistrofia/sangue , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Estudos de Casos e Controles , Criança , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
13.
Med Clin North Am ; 105(4): 681-697, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34059245

RESUMO

Diabetes mellitus is a significant worldwide health concern and cutaneous manifestations are common. This review describes characteristic skin findings of diabetes, general skin findings related to diabetes, and findings related to diabetes treatment with a focus on clinical presentation, diagnosis, pathophysiology, epidemiology, and treatment. As the prevalence of diabetes continues to rise, cutaneous manifestations of diabetes mellitus likely will be encountered more frequently by physicians in all disciplines including dermatologists and primary care physicians. Accordingly, knowledge regarding the prevention, diagnosis, and management of cutaneous manifestations is an important aspect in the care of patients with diabetes.


Assuntos
Complicações do Diabetes/patologia , Diabetes Mellitus/epidemiologia , Dermatopatias/diagnóstico , Dermatopatias/prevenção & controle , Dermatopatias/fisiopatologia , Acantose Nigricans/etiologia , Acantose Nigricans/patologia , Acantose Nigricans/terapia , Dermatologistas/estatística & dados numéricos , Pé Diabético/etiologia , Pé Diabético/patologia , Pé Diabético/terapia , Saúde Global/estatística & dados numéricos , Humanos , Conhecimento , Lipodistrofia/etiologia , Lipodistrofia/patologia , Lipodistrofia/terapia , Pessoa de Meia-Idade , Necrobiose Lipoídica/etiologia , Necrobiose Lipoídica/patologia , Necrobiose Lipoídica/terapia , Médicos de Atenção Primária/estatística & dados numéricos , Prevalência , Escleredema do Adulto/etiologia , Escleredema do Adulto/patologia , Escleredema do Adulto/terapia , Dermatopatias/epidemiologia
14.
BMJ Case Rep ; 14(6)2021 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-34155011

RESUMO

A 35-year-old woman with acquired partial lipodystrophy (PLD) and features of type 2 membranoproliferative glomerulonephritis (MPGN-II), presented with difficulty in her fine detailed vision over the past year. She had right amblyopia from a hypermetropic anisometropia with astigmatism, displaying a best-corrected visual acuity of 0.50 and 0.00 LogMAR, in the right and left eye, respectively. Funduscopy showed bilateral symmetrical drusenoid deposits most prominent in the temporal macula with clusters in the superior and inferior retina, outside the temporal vascular arcades. Multimodal retinal imaging was performed, which confirmed hyperautofluorescent drusen located between the retinal pigment epithelium and Bruch's membrane. Electroretinography showed bilateral mild peripheral macular dysfunction, but normal central macular function on the pattern electroretinogram. Both PLD and macular drusen, are rare as distinct disease entities, but an association does exist and may be linked to MPGN-II.


Assuntos
Glomerulonefrite Membranoproliferativa , Lipodistrofia , Drusas Retinianas , Adulto , Lâmina Basilar da Corioide , Feminino , Glomerulonefrite Membranoproliferativa/complicações , Glomerulonefrite Membranoproliferativa/diagnóstico , Humanos , Drusas Retinianas/complicações , Drusas Retinianas/diagnóstico , Epitélio Pigmentado da Retina
15.
Diabetes Res Clin Pract ; 178: 108924, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34153354

RESUMO

In the article recently published in Diab Res Clin Pract (1), we described one of the most exciting paths in the history of medicine from the perspective of diabetologists and people with type 1 diabetes. Such a history lasted 100 years, from the discovery of insulin to the most technologically advanced technologies aimed at making treatment as close to physiology and user-friendly as possible. Indeed, we are luckier than others because, by living in Italy and the USA, respectively, we have access to miniaturized and computerized insulin delivery systems, but this is not the case worldwide. Due to that, while receiving many favorable comments from colleagues and friends, we were encouraged to further expand on the issue and go deeper into insulin injection technique.


Assuntos
Diabetes Mellitus Tipo 1 , Lipodistrofia , Diabetes Mellitus Tipo 1/tratamento farmacológico , Humanos , Insulina , Insulina Regular Humana , Itália/epidemiologia , Lipodistrofia/induzido quimicamente , Lipodistrofia/epidemiologia
17.
Arch Endocrinol Metab ; 64(5): 559-566, 2021 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-34033296

RESUMO

Objective: Our aim is to establish genetic diagnosis of congenital generalized lipodystrophy (CGL) using targeted massively parallel sequencing (MPS), also known as next-generation sequencing (NGS). Methods: Nine unrelated individuals with a clinical diagnosis of CGL were recruited. We used a customized panel to capture genes related to genetic lipodystrophies. DNA libraries were generated, sequenced using the Illumina MiSeq, and bioinformatics analysis was performed. Results: An accurate genetic diagnosis was stated for all nine patients. Four had pathogenic variants in AGPAT2 and three in BSCL2. Three large homozygous deletions in AGPAT2 were identified by copy-number variant analysis. Conclusion: Although we have found allelic variants in only 2 genes related to CGL, the panel was able to identify different variants including deletions that would have been missed by Sanger sequencing. We believe that MPS is a valuable tool for the genetic diagnosis of multi-genes related diseases, including CGL.


Assuntos
Subunidades gama da Proteína de Ligação ao GTP , Lipodistrofia Generalizada Congênita , Lipodistrofia , Alelos , Subunidades gama da Proteína de Ligação ao GTP/genética , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lipodistrofia/diagnóstico , Lipodistrofia/genética , Lipodistrofia Generalizada Congênita/diagnóstico , Lipodistrofia Generalizada Congênita/genética , Mutação/genética
18.
Eur J Neurol ; 28(8): 2603-2613, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33969597

RESUMO

BACKGROUND AND PURPOSE: Nasu-Hakola disease (NHD) is a rare, autosomal recessive disorder characterized by skeletal and neurological symptoms. Behavioral symptoms with cognitive impairment may mimic the behavioral variant of frontotemporal dementia (bvFTD) and other early-onset dementias. Our patients were analyzed and the literature was reviewed to delineate neurological and neuroimaging findings suggestive of NHD. METHOD: Fourteen patients carrying a pathogenic mutation in the TREM2 gene were found in our database. Demographic, clinical, laboratory and radiological data were retrieved and analyzed. RESULTS: The presenting clinical picture was behavioral changes with cognitive decline resembling bvFTD in all patients. The mean age was 37.1 ± 4.97 years and the mean duration of the disease was 8.9 ± 3.51 years. Only two patients had typical bone cysts. Seven patients had bilateral calcification of the basal ganglia in computed tomography of the brain. Magnetic resonance imaging of the brain revealed severe atrophy of the corpus callosum, enlargement of the ventricles, atrophy of the caudate nuclei and periventricular white matter changes in all patients. Symmetrical global atrophy of the brain mainly affecting frontoparietal and lateral temporal regions were observed in all cases, and 13 patients had atrophy of the hippocampus. Cerebrospinal fluid examination of 10 patients showed elevated protein levels in six and the presence of oligoclonal bands in four patients. CONCLUSION: A combination of white matter changes, enlarged ventricles, atrophy of the caudate nuclei and thinning of the corpus callosum in magnetic resonance imaging strongly suggests NHD in patients with FTD syndrome. Molecular genetic analysis should be performed in suspected cases, and families should receive genetic counseling.


Assuntos
Demência Frontotemporal , Lipodistrofia , Glicoproteínas de Membrana/genética , Osteocondrodisplasias , Receptores Imunológicos/genética , Panencefalite Esclerosante Subaguda , Adulto , Encéfalo/diagnóstico por imagem , Demência Frontotemporal/diagnóstico por imagem , Demência Frontotemporal/genética , Humanos , Imageamento por Ressonância Magnética , Neuroimagem
19.
Therapie ; 76(6): 617-628, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33958198

RESUMO

AIMS OF THE STUDY: This study aimed to assess the prevalence of lipohypertrophy (LH) in diabetes mellitus (DM) patients on insulin therapy and identify factors influencing the prevalence rates through a meta-analysis and meta-regression. METHODS: Databases of PubMed, Embase, Scopus, and Google Scholar were screened from inception to 20th Aug 2020. Data on prevalence was transformed using the logit transformation for pooling the proportions using the DerSimonian-Laird meta-analysis model. Random-effects meta-regression analysis was performed to assess the influence of the following moderators on the pooled prevalence: male gender, mean age, type of DM, DM duration, duration of insulin therapy, needle re-users, rotation of injection site, HbA1c, insulin dose, ≥8mm needle users and mean number of injections/day. RESULTS: Forty-five studies were included. LH was diagnosed by observation and palpation by all studies. On meta-analysis of data from 26,865 participants, the overall prevalence of LH was found to be 41.8% (95% CI: 35.9% to 47.6%). On region-wise analysis, pooled prevalence of LH in Europe was 44.6% (95% CI: 37.5% to 51.8%) in Africa was 34.8% (95% CI: 16.9% to 52.8%) and in Asia was 41.3% (95% CI: 27.2% to 55.3%). The pooled prevalence of LH in studies only on type 1 and type 2 DM patients was 39.9% (95% CI: 28.3% to 51.6%) and 45.9% (95% CI: 29.5% to 62.4%) respectively. Only insulin duration was found to significantly influence the prevalence of LH on meta-regression. CONCLUSION: Our study indicates that the prevalence of LH in DM patients on insulin therapy is estimated to be 41.8%. The longer the duration of insulin therapy, the higher is the prevalence of LH.


Assuntos
Diabetes Mellitus Tipo 2 , Lipodistrofia , Diabetes Mellitus Tipo 2/tratamento farmacológico , Diabetes Mellitus Tipo 2/epidemiologia , Humanos , Insulina/efeitos adversos , Masculino , Prevalência , Fatores de Risco
20.
AJNR Am J Neuroradiol ; 42(8): 1370-1374, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33958332

RESUMO

Pilomyxoid astrocytoma is a rare form of pediatric CNS malignancy first classified in 2007 by the World Health Organization. The tumors are similar to pilocytic astrocytomas, sharing both some imaging and histologic traits. However, pilomyxoid astrocytomas portend a more ominous prognosis, with more aggressive local tendencies and a greater proclivity for leptomeningeal spread. Although tissue sampling is ultimately required to differentiate pilocytic astrocytomas and pilomyxoid astrocytomas, some imaging features can be used to suggest a pilomyxoid astrocytoma, including homogeneous enhancement, leptomeningeal seeding, and lack of intratumoral cysts. In this article, a case of a hypothalamic pilomyxoid astrocytoma is described, in which the presenting disorder was profound generalized lipodystrophy. The aforementioned imaging characteristics of pilomyxoid astrocytomas are reviewed, as are the pathologic features of such tumors, including their angiocentric cellular arrangement and myxoid background.


Assuntos
Astrocitoma , Neoplasias Encefálicas , Lipodistrofia , Astrocitoma/diagnóstico por imagem , Neoplasias Encefálicas/diagnóstico por imagem , Criança , Humanos , Imageamento por Ressonância Magnética , Prognóstico
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