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1.
Eur J Pediatr ; 180(2): 387-395, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32959076

RESUMO

Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. Early diagnosis and treatment is mandatory in incomplete KD to reduce the risk of coronary involvement. Between 2002 and 2018, 124 patients have been diagnosed suffering from KD at the University Children's Hospital Regensburg (KUNO). We assessed luminal diameters of both CAs normalized as Z-scores by 2D-echocardiography. A total of 94 patients were analyzed. Of them, 31 (33%) were affected by an incomplete form of KD. In 24 children (26%), serial echocardiography was necessary in order to confirm diagnosis. Mean Z-scores for the left main coronary artery (LMCA), right main coronary artery (RMCA), and left anterior descending artery increased significantly between the initial (LMCA 0.79z, RMCA 0.15z, LAD 0.49z) and second (LMCA 1.69z, RMCA 0.99z, LAD 1.69z) examination (p < 0.05).Conclusion:To confirm diagnosis of KD, it might not be necessary to detect dilation or aneurysms. Our observation suggests that patients suspected having KD should be monitored with serial echocardiography in order to detect a possible enlargement of the CA diameters, even if Z-scores are within the normal range. What is Known: • Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. • Due to less classic clinical criteria in patients with incomplete KD, the risk for CA pathology is even higher. What is New: • A significant progression of patients' CA Z-scores in serial echocardiographic measurements may be helpful to ensure diagnosis of KD early even if Z-scores are within the normal range. • Twenty-seven patients (90%) with incomplete KD could be diagnosed within 10 days of fever, early enough to prevent significantly higher rates of CA aneurysm.


Assuntos
Aneurisma Coronário , Síndrome de Linfonodos Mucocutâneos , Criança , Pré-Escolar , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/etiologia , Vasos Coronários/diagnóstico por imagem , Dilatação Patológica , Ecocardiografia , Humanos , Lactente , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem
2.
Mol Genet Metab ; 126(1): 30-38, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30497809

RESUMO

Fatty acid transport protein4 (FATP4) is upregulated in acquired and central obesity and its polymorphisms are associated with blood lipids and insulin resistance. Patients with FATP4 mutations and mice with global FATP4 deletion exhibit skin abnormalities characterized as ischthyosis prematurity syndrome (IPS). Cumulating data have shown that an absence of FATP4 increases the levels of cellular triglycerides (TG). However, FATP4 role and consequent lipid and TG metabolism in the hepatocyte is still elusive. Here, hepatocyte-specific FATP4 deficient (Fatp4L-/-) mice were generated. When fed with chow, these mutant mice displayed no phenotypes regarding blood lipids. However when fed low-fat/high-sugar (HS) or high-fat/high-sugar (HFS) for 12 weeks, Fatp4L-/- mice showed a significant increase of plasma TG, free fatty acids and glycerol when compared with diet-fed control mice. Interestingly, Fatp4L-/- mice under HS diet had lower body and liver weights and they were not protected from HFS-induced body weight gain and hepatic steatosis. Male mutant mice were more sensitive to HFS diet than female mutant mice. Glucose intolerance was observed only in female Fatp4L-/- mice fed with HS diet. Lipidomics analyses revealed that hepatic phospholipids were not disturbed in mutant mice under both diets. Thus, hepatic FATP4 deletion rendered an increase of blood lipids including glycerol indicating a preferential fatty-acid channeling to TG pools that are specifically available for lipolysis. Our results imply a possible risk of hyperlipidemia as a result of abnormal metabolism in liver in IPS patients with FATP4 mutations who consume high-sugar diets.


Assuntos
Proteínas de Transporte de Ácido Graxo/genética , Hepatócitos/metabolismo , Metabolismo dos Lipídeos , Triglicerídeos/sangue , Animais , Dieta , Proteínas de Transporte de Ácido Graxo/deficiência , Ácidos Graxos/metabolismo , Fígado Gorduroso , Feminino , Glucose/administração & dosagem , Intolerância à Glucose , Resistência à Insulina , Lipólise , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Obesidade
3.
Psychopathology ; 43(6): 369-72, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20798576

RESUMO

BACKGROUND: Despite the notion that randomized controlled trials are regarded as the gold standard in psychotherapy research, questions about their generalizability have been raised. This paper focuses on the differences between participants and eligible nonparticipants of a randomized controlled trial for patients with borderline personality disorder (BPD). SAMPLING AND METHODS: One hundred forty-two patients were screened, and 122 were found eligible for study participation. Out of these, 64 patients (52.5%) gave informed consent and were included in the study. RESULTS: The 58 eligible nonparticipants showed a lower level of functioning (global assessment of functioning score), had a history of more outpatient treatment attempts and were living alone more often. Regarding acute symptoms and severity of BPD as indexed by suicide attempts, inpatient treatments, substance abuse and history of trauma, no differences between the groups could be detected. Moreover, participants showed significantly more eating disorders, whereas nonparticipants presented more affective and anxiety disorders. CONCLUSIONS: The results indicate that lower psychosocial functioning and comorbid affective and anxiety disorders decrease BPD patients' willingness to participate in an RCT.


Assuntos
Transtorno da Personalidade Borderline/psicologia , Seleção de Pacientes , Adolescente , Adulto , Transtorno da Personalidade Borderline/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Ensaios Clínicos Controlados Aleatórios como Assunto , Índice de Gravidade de Doença , Suicídio/psicologia
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