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1.
Pediatr Pulmonol ; 54(6): 837-846, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30912317

RESUMO

INTRODUCTION: Pulmonary interstitial glycogenosis (PIG) is a rare infant interstitial lung disease characterized by an increase in the number of interstitial mesenchymal cells, presenting as enhanced cytoplasmic glycogen, and is considered to represent the expression of an underlying lung development disorder. METHODS: This study describes the clinical, radiological, and functional characteristics and long-term outcomes (median 12 years) of nine infants diagnosed with isolated PIG associated with alveolar simplification in the absence of other diseases. RESULTS: All patients presented with tachypnea. Additionally, seven patients had breathing difficulties and hypoxemia. Abnormalities in chest-computerized tomography (CT) with a pattern of ground-glass opacity, septal thickening, and air trapping were observed in all individuals, with images suggesting abnormal alveolar growth (parenchymal bands and architectural distortion). All lung biopsies showed alveolar simplification associated with an increased number of interstitial cells, which appeared as accumulated cytoplasmic glycogen. In the follow-up, all patients were asymptomatic. The respiratory function test was normal in only two patients. Five children showed an obstructive pattern, and two children showed a restrictive pattern. Chest-CT, performed after an average of 6.5 years since the initial investigation, revealed a partial improvement of the ground-glass opacity pattern; however, relevant alterations persisted. CONCLUSION: Although the patients with PIG in the absence of other associated pathologies had a good clinical outcome, significant radiographic alterations and sequelae in lung function were still observed after a median follow-up of 12 years, suggesting that PIG is a marker of some other persistent abnormalities in lung growth, which have effects beyond the symptomatic period.

2.
Ann Thorac Surg ; 108(5): e325-e327, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30926474

RESUMO

This case report describes a primary cardiac tumor, classified as venous malformation, diagnosed in an asymptomatic child. The tumor was located in the left atrium near the mitral valve without affecting the mitral valve's functioning. Complete resection of the lesion was performed because of the risk of systemic embolism. The lesion consisted of fibrous tissue with multiple venous vascular channels. The patient did not have similar lesions in other locations. Vascular primary cardiac tumors are extremely rare. Hemangiomas and lymphangiomas have been described previously, but to our knowledge, this is the first primary cardiac tumor identified as a venous malformation.

4.
Pediatr Infect Dis J ; 35(12): 1350-1351, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27626919

RESUMO

Acanthamoeba infections are rare and mostly occur in immunocompromised patients. Most of the reported cases after stem cell transplantation have been diagnosed postmortem. We present the case of a 3-year-old boy with chronic graft versus host disease post hematopoietic transplantation, who was successfully treated for Acanthamoeba.


Assuntos
Amebíase , Doença Enxerto-Hospedeiro/complicações , Transplante de Células-Tronco Hematopoéticas , Sinusite , Acanthamoeba , Amebíase/complicações , Amebíase/tratamento farmacológico , Amebíase/parasitologia , Amebicidas/uso terapêutico , Anfotericina B/uso terapêutico , Pré-Escolar , Humanos , Masculino , Mucosa Nasal/parasitologia , Mucosa Nasal/patologia , Sinusite/complicações , Sinusite/tratamento farmacológico , Sinusite/parasitologia
5.
Cytoskeleton (Hoboken) ; 73(2): 59-67, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26849407

RESUMO

Cells in vivo exist in a dynamic environment where they experience variable mechanical influences. The precise mechanical environment influences cell-cell interactions, cell-extracellular matrix interactions, and in-turn, cell morphology and cell function. Therefore, the ability of each cell to constantly and rapidly alter their behavior in response to variations in their mechanical environment is essential for cell viability, development, and function. Mechanotransduction, the process by which mechanical force is translated into a biochemical signal to activate downstream cellular responses, is thus crucial to cell function during development and homeostasis. Although much research has focused on how protein complexes at the cell cortex respond to mechanical stress to initiate mechanotransduction, the nucleus has emerged as crucial to the ability of the cell to perceive and respond to changes in its mechanical environment. This additional method for mechanosensing allows for direct transmission of force through the cytoskeleton to the nucleus, which can increase the speed at which a cell changes its transcriptional profile. This review discusses recent work demonstrating the importance of the nucleus in mediating the cellular response to internal and external force, establishing the nucleus as an important mechanosensing organelle.


Assuntos
Núcleo Celular/metabolismo , Mecanotransdução Celular , Animais , Humanos , Modelos Biológicos , Complexos Multiproteicos/metabolismo
6.
Gastroenterol. hepatol. (Ed. impr.) ; 34(10): 678-682, Dic. 2011.
Artigo em Espanhol | IBECS | ID: ibc-98665

RESUMO

La enfermedad celíaca es una enteropatía frecuente que se asocia a manifestaciones clínicas muy variadas en parte debidas a la malabsorción. En la mujer se ha asociado a alteraciones obstétricas y ginecológicas como abortos de repetición, retraso intrauterino de crecimiento, parto prematuro y bajo peso al nacer. Presentamos el caso de una mujer con enfermedad celíaca no diagnosticada y con un parto eutócico de feto muerto de 34 semanas de gestación con alteraciones morfológicas graves de hipomineralización compatibles con raquitismo. En la literatura médica el raquitismo congénito secundario a malabsorción por enfermedad celíaca de la madre es excepcional. Se comentan los conocimientos actuales sobre el metabolismo fosfocálcico materno-fetal. Relacionamos la celiaquía activa con la hipocalcemia severa durante la gestación y con el raquitismo fetal mortal. Se sugiere la necesidad de un cribado de dicha enfermedad en las gestantes con signos de malabsorción o anomalías en el desarrollo del feto (AU)


Celiac disease is a relatively frequent enteropathy associated with a wide range of clinical manifestations, due in part to malabsorption. In women, it has been associated with obstetric and gynecological alterations such as repeated miscarriages, intrauterine growth delay, premature delivery, and low birth weight. We present the case of a woman with undiagnosed celiac disease who gave birth to a stillborn foetus via normal delivery after 34 weeks of gestation. The foetus presented severe morphological alterations due to hypomineralization which were compatible with rickets.In the medical literature congenital rickets secondary to maternal celiac disease due to malabsorption is rare. We discuss the current knowledge on maternofoetal phospho-calcium metabolism and relate active celiac disease with severe hypocalcaemia during pregnancy and fatal rickets in the foetus. We recommend screening for celiac disease in pregnant women with signs of malabsorption or impaired fetal development (AU)


Assuntos
Humanos , Feminino , Gravidez , Adulto , Doença Celíaca/complicações , Transtornos da Nutrição Fetal/etiologia , Raquitismo/congênito , Complicações na Gravidez , Morte Fetal/etiologia , Anormalidades Múltiplas/etiologia , Hipocalcemia/etiologia
7.
Gastroenterol Hepatol ; 34(10): 678-82, 2011 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-21757262

RESUMO

Celiac disease is a relatively frequent enteropathy associated with a wide range of clinical manifestations, due in part to malabsorption. In women, it has been associated with obstetric and gynecological alterations such as repeated miscarriages, intrauterine growth delay, premature delivery, and low birth weight. We present the case of a woman with undiagnosed celiac disease who gave birth to a stillborn foetus via normal delivery after 34 weeks of gestation. The foetus presented severe morphological alterations due to hypomineralization which were compatible with rickets. In the medical literature congenital rickets secondary to maternal celiac disease due to malabsorption is rare. We discuss the current knowledge on maternofoetal phospho-calcium metabolism and relate active celiac disease with severe hypocalcaemia during pregnancy and fatal rickets in the foetus. We recommend screening for celiac disease in pregnant women with signs of malabsorption or impaired fetal development.


Assuntos
Morte Fetal/etiologia , Raquitismo/complicações , Doença Celíaca , Evolução Fatal , Feminino , Humanos , Gravidez , Complicações na Gravidez
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