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1.
JPGN Rep ; 5(2): 158-161, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38756132

RESUMO

We report the case of a 14-year-old patient with a known history of Crohn's disease who was incidentally diagnosed with an asymptomatic cecal lipoma. A routine surveillance colonoscopy as part of the management of the patient's Crohn's Disease revealed a well-defined, submucosal, yellowish mass in the patient's cecum. Histopathological examination of a biopsy specimen revealed submucosal adipose tissue, consistent with the endoscopic images showing the characteristic appearance of the lipoma. A computed tomography examination further confirmed the diagnosis. While colonic lipomas are infrequent and typically manifest later in life, few cases report the coexistence of a cecal lipoma with Crohn's disease, particularly in the pediatric population. In this case, managing this dual condition posed a notable challenge. Here, we present the conservative approach to managing a pediatric patient with cecal lipoma and Crohn's disease. The decision to leave the lipoma in situ was based on the absence of symptoms and potential risks associated with surgical removal.

2.
Stem Cells Int ; 2012: 452102, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22936950

RESUMO

Background. Human cardiac-derived progenitor cells (hCPCs) have shown promise in treating heart failure (HF) in adults. The purpose of this study was to describe derivation of hCPCs from pediatric patients with end-stage HF. Methods. At surgery, discarded right atrial tissues (hAA) were obtained from HF patients (n = 25; hAA-CHF). Minced tissues were suspended in complete (serum-containing) DMEM. Cells were selected for their tissue migration and expression of stem cell factor receptor (hc-kit). Characterization of hc-kit(positive) cells included immunohistochemical screening with a panel of monoclonal antibodies. Results. Cells, including phase-bright cells identified as hc-kit(positive), spontaneously emigrated from hAA-CHF in suspended explant cultures (SEC) after Day 7. When cocultured with tissue, emigrated hc-kit(positive) cells proliferated, first as loosely attached clones and later as multicellular clusters. At Day 21~5% of cells were hc-kit(positive). Between Days 14 and 28 hc-kit(positive) cells exhibited mesodermal commitment (GATA-4(positive) and NKX2.5(positive)); then after Day 28 cardiac lineages (flk-1(positive), smooth muscle actin(positive), troponin-I(positive), and myosin light chain(positive)). Conclusions. C-kit(positive) hCPCs can be derived from atrial tissue of pediatric patients with end-stage HF. SEC is a novel culture method for derivation of migratory hc-kit(positive) cells that favors clinical translation by reducing the need for exogenously added factors to expand hCPCs in vitro.

3.
Comp Med ; 59(2): 139-46, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19389305

RESUMO

Mucopolysaccharidosis type IIIB (MPS IIIB; Sanfilippo syndrome type B) is a metabolic disorder with devastating clinical characteristics starting in early childhood and leading to premature death. A knockout mouse strain was developed that models this disease. Mice of the strain B6.129S6- Naglu(tm1Efn)/J are invaluable for investigating pathogenesis and possible treatment modalities. However, the mouse strain also exhibits some objectionable phenotypic features. One such feature, urinary retention, not only is atypical of human MPS IIIB but often leads to early termination of experiments due to animal welfare concerns. The aim of this study was to investigate abnormalities associated with the urinary retention. Necropsies were performed on 9-mo-old mice; urinalysis, hematology and blood chemistry parameters were evaluated, and urogenital specimens were microscopically examined. Histopathologic examinations of urinary tract specimens proved illuminating regarding pathology in the urinary tract. A large mononuclear cell infiltrate was discovered in mutant mice of both sexes, more pronounced in females compared with male mice. The infiltrate comprises of large rounded or polygonal cells with generous variably vacuolated, granular eosinophilic cytoplasm and small round vesicular nuclei. These cells were present throughout and expand the interstitium of the lower urinary tract. Either this results in extrinsic compression of the lumen of the urethra, eventually leading to obstructive uropathy, bladder hyperdistension, and urinary retention or possibly interferes with the neurogenic component of micturition needs to be further investigated. The novel finding of an unexpected mononuclear cell infiltrate in the urinary tract in the knockout mice B6.129S6- Naglu(tm1Efn)/J is reported.


Assuntos
Mucopolissacaridose III/complicações , Mucopolissacaridose III/patologia , Retenção Urinária , Acetilglucosaminidase/genética , Acetilglucosaminidase/metabolismo , Animais , Pré-Escolar , Modelos Animais de Doenças , Feminino , Humanos , Rim/citologia , Rim/patologia , Masculino , Camundongos , Camundongos Knockout , Mucopolissacaridose III/fisiopatologia , Fenótipo , Urinálise , Retenção Urinária/etiologia , Retenção Urinária/patologia , Sistema Urinário/patologia , Vagina/citologia
4.
FASEB J ; 20(3): 485-7, 2006 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-16401642

RESUMO

Numerous data support passage of maternal cells into the fetus during pregnancy in both human and animal models. However, functional benefits of maternal microchimerism in utero are unknown. The current study attempted to take advantage of this route for prenatal delivery of alpha-N-acetylglucosaminidase (Naglu) enzyme into the enzyme-deficient mouse model of Sanfilippo syndrome type B (MPS III B). Enzymatically sufficient mononuclear cells from human umbilical cord blood (MNC hUCB) were intravenously administered into heterozygote females modeling MPS III B on the 5th day of pregnancy during blastocyst implantation. The major findings were 1) administered MNC hUCB cells transmigrated and diffused into the embryos (E12.5); 2) some transmigrated cells expressed CD34 and CD117 antigens; 3) transmigrated cells were found in both the maternal and embryonic parts of placentas; 4) transmigrated cells corrected Naglu enzyme activity in all embryos; 5) administered MNC hUCB cells were extensively distributed in the organs and the blood of heterozygote mothers at one week after transplantation. Results indicate that prenatal delivery of Naglu enzyme by MNC hUCB cell administration into mothers of enzyme-deficient embryos is possible and may present a significant opportunity for new biotechnologies to treat many inherited disorders.


Assuntos
Acetilglucosaminidase/genética , Transplante de Células-Tronco de Sangue do Cordão Umbilical , Terapias Fetais , Leucócitos Mononucleares/transplante , Troca Materno-Fetal , Mucopolissacaridose III/terapia , Acetilglucosaminidase/deficiência , Animais , Antígenos CD34/análise , Linhagem da Célula , Movimento Celular , Feminino , Terapias Fetais/métodos , Humanos , Leucócitos Mononucleares/enzimologia , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Modelos Animais , Mucopolissacaridose III/embriologia , Mucopolissacaridose III/enzimologia , Mucopolissacaridose III/genética , Placenta/ultraestrutura , Gravidez , Proteínas Proto-Oncogênicas c-kit/análise , Transplante Heterólogo
5.
Stem Cells Dev ; 14(2): 173-80, 2005 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-15910243

RESUMO

Leukocytes of the macrophage lineage are abundant in the ovarian tissues and have an important function in both follicular development and regression of postovulatory follicles. In this study, we tested the hypothesis that continuous production of macrophages in the ovarian stroma is maintained by a resident population of progenitors. We established a long-term culture of ovarian follicular stromal cells from BALB/c and green fluorescent protein-transgenic (GFP-TG) C57BL/6 mice. Nonadherent cells were collected and tested for hematopoietic function in vitro and in vivo. Histological and ultrastructural analyses revealed a homogenous population of monocyte-like rounded cells. Nonadherent cells continued to proliferate in culture for several months without senescence. When plated at very low density in methylcellulose, these cells formed colonies consisting of monocyte-like cells. Ovarian monocyte-like cells reacted with CD45, CD11b, CD11c, and Ly6-Gr-1 cell surface markers. A distinct CD45low population within these cells reacted with CD117 (C-kit) surface marker, suggestive of a primitive hematopoietic progenitor. Fifty thousand nonadherent cells failed to provide radioprotection to lethally irradiated mice and thus were not considered to be equivalent to pluripotent hematopoietic stem cells. Ovarian nonadherent stromal cells were positive for alkaline phosphatase but lacked embryonic cell antigens stage-specific embryonic antigen (SSEA-1) and Oct-4. We conclude that in the ovaries, a higher requirement for macrophages is provided by a resident stromal population of progenitors whose progeny is restricted to the production of cells of the monocyte-macrophage lineage.


Assuntos
Monócitos/citologia , Ovário/citologia , Células-Tronco/citologia , Fosfatase Alcalina/metabolismo , Animais , Antígeno CD11b/biossíntese , Antígeno CD11c/biossíntese , Adesão Celular , Linhagem da Célula , Proliferação de Células , Transplante de Células , Células Cultivadas , Proteínas de Ligação a DNA/biossíntese , Feminino , Citometria de Fluxo , Proteínas de Fluorescência Verde/metabolismo , Imunofenotipagem , Antígenos Comuns de Leucócito/biossíntese , Leucócitos/citologia , Antígenos CD15/metabolismo , Macrófagos/citologia , Macrófagos/metabolismo , Metilcelulose/metabolismo , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Microscopia Eletrônica , Monócitos/metabolismo , Fator 3 de Transcrição de Octâmero , Ovário/patologia , Fenótipo , Proteínas Proto-Oncogênicas c-kit/biossíntese , Fatores de Transcrição/biossíntese
6.
Comp Med ; 53(6): 622-32, 2003 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-14727810

RESUMO

Sanfilippo syndrome type B or mucopolysaccharidosis type III B (MPS IIIB) is a lysosomal storage disorder that is inherited in autosomal recessive manner. It is characterized by systemic heparan sulfate accumulation in lysosomes due to deficiency of the enzyme alpha-N-acetylglucosaminidase (Naglu). Devastating clinical abnormalities with severe central nervous system involvement and somatic disease lead to premature death. A mouse model of Sanfilippo syndrome type B was created by targeted disruption of the gene encoding Naglu, providing a powerful tool for understanding pathogenesis and developing novel therapeutic strategies. However, the JAX GEMM Strain B6.129S6-Naglutm1Efn mouse, although showing biochemical similarities to humans with Sanfilippo syndrome, exhibits aging and behavioral differences. We observed idiosyncrasies, such as skeletal dysmorphism, hydrocephalus, ocular abnormalities, organomegaly, growth retardation, and anomalies of the integument, in our breeding colony of Naglu mutant mice and determined that several of them were at least partially related to the background strain C57BL/6. These background strain abnormalities, therefore, potentially mimic or overlap signs of the induced syndrome in our mice. Our observations may prove useful in studies of Naglu mutant mice. The necessity for distinguishing background anomalies from signs of the modeled disease is apparent.


Assuntos
Acetilglucosaminidase/genética , Modelos Animais de Doenças , Camundongos Endogâmicos C57BL/genética , Mucopolissacaridose III/genética , Mutação , Acetilglucosaminidase/deficiência , Acetilglucosaminidase/metabolismo , Envelhecimento/genética , Envelhecimento/metabolismo , Envelhecimento/patologia , Animais , Anormalidades Congênitas/genética , Anormalidades Congênitas/patologia , Feminino , Genótipo , Masculino , Camundongos , Camundongos Endogâmicos C57BL/anormalidades , Camundongos Endogâmicos C57BL/metabolismo , Camundongos Knockout , Mucopolissacaridose III/enzimologia , Mucopolissacaridose III/patologia , Fenótipo , Reprodução/genética
8.
J Pediatr Hematol Oncol ; 24(2): 154-7, 2002 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-11990705

RESUMO

The authors report a child younger than age 15 years with a rare hepatosplenic gamma/delta T-cell lymphoma, which is highly aggressive and primarily seen in young men. A 9-year-old girl presented with thrombocytopenia and hepatosplenomegaly. Bone marrow analysis revealed a metastatic pleomorphic lymphoma of peripheral T-cell phenotype, with rearrangement of the T-cell receptor gamma/delta and expression of CD3 and CD16/56. Instead of the previously reported primary, nonrandom, chromosomal abnormalities, isochromosome 7q and trisomy 8, this patient had four copies each of chromosome 7q, including isochromosome 7[i(7)(q10)] and der(21)t(7;21), as well as chromosome 8. This entity needs to be considered in women and children with lymphoma. Conventional therapy appears to be inadequate for cure.


Assuntos
Cromossomos Humanos Par 21/ultraestrutura , Cromossomos Humanos Par 7/ultraestrutura , Cromossomos Humanos Par 8 , Isocromossomos , Neoplasias Hepáticas/genética , Linfoma de Células T/genética , Receptores de Antígenos de Linfócitos T gama-delta/genética , Neoplasias Esplênicas/genética , Translocação Genética , Trissomia , Aneuploidia , Antígenos de Neoplasias/análise , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medula Óssea/patologia , Transplante de Medula Óssea , Complexo CD3/análise , Antígeno CD56/análise , Criança , Feminino , Hepatomegalia/etiologia , Hepatomegalia/patologia , Humanos , Imunofenotipagem , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/terapia , Linfoma de Células T/tratamento farmacológico , Linfoma de Células T/terapia , Monossomia , Células-Tronco Neoplásicas/química , Células-Tronco Neoplásicas/patologia , Receptores de Antígenos de Linfócitos T gama-delta/análise , Receptores de IgG/análise , Indução de Remissão , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/terapia , Esplenomegalia/etiologia , Esplenomegalia/patologia , Transplante Homólogo , Cromossomo X
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