Regulation of hepatokine gene expression in response to fasting and feeding: Influence of PPAR-α and insulin-dependent signalling in hepatocytes.

AIM: In hepatocytes, the peroxisome proliferator-activated receptor (PPAR)-α and insulin receptor (IR) are critical for transcriptional responses to fasting and feeding, respectively. The present report analyzes the effects of nutritional status (fasting vs feeding) on the expression of a large panel of hepatokines in hepatocyte-specific PPAR-α (Pparαhep-/-) and IR (IRhep-/-) null mice. METHODS: Pparαhep-/- and IRhep-/- mice, and their wild-type littermates, were subjected to fasting or feeding metabolic challenges, then analyzed for hepatokine gene expression. Experiments were conducted in mice of both genders. RESULTS: Our data confirmed that PPAR-α is essential for regulating fasting-induced Fgf21 and Angptl4 expression. In mice lacking PPAR-α, fasting led to increased Igfbp1 and Gdf15 gene expression. In the absence of hepatic IR, feeding induced overexpression of Igfbp1, follistatin (Fst) and adropin (Enho), and reduced activin E (Inhbe) expression. Gender had only a modest influence on hepatokine gene expression in the liver. CONCLUSION: The present results highlight the potential roles of hepatokines as a class of hormones that substantially influence nutritional regulation in both female and male mice.

Neuroblastoma neonatal estadio IV y IVs: Tratamiento quirúrgico con silo y reducción hepática / Neonatal Stage IV and IVs neuroblastoma: surgical treatment with silo and liver reduction

Las metástasis hepáticas del neuroblastoma neonatal pueden causar fallo multiorgánico debido al crecimiento rápido y hepatomegalia masiva. El objetivo de este trabajo es presentar dos neonatos con Neuroblastoma estadio IV y IVs en situación crítica que necesitaron un silo abdominal descompresivo y reducción hepática para lograr el cierre abdominal definitivo. Actualmente los dos niños tienen 6 años de edad y están libres de enfermedad. Conclusión. La colocación de un silo abdominal descompresivo continúa siendo una opción de rescate. La reducción hepática quirúrgica podría ser una alternativa para el cierre precoz del abdomen

Hepatic metastases of neonatal neuroblastoma can cause multiple organ failure due to rapid growth and severe hepatomegaly. The objective of this work is to present two infants with neuroblastoma stage IV and IVs in a critical situation that needed a decompressive abdominal silo and surgical liver reduction for definitive abdominal closure. Currently both children are 6 years old and they are free from disease. Conclusion. The placement of a silo for abdominal decompression is still an indication for salvage. Surgical hepatic reduction is an alternative for the early closure of the abdomen avoiding complications due to infection or dehiscence of the silo

[Neonatal stage IV and IVs neuroblastoma: surgical treatment with silo and liver reduction]. / Neuroblastoma neonatal estadio IV y IVs: Tratamiento quirúrgico con silo y reducción hepática.

UNLABELLED: Hepatic metastases of neonatal neuroblastoma can cause multiple organ failure due to rapid growth and severe hepatomegaly. The objective of this work is to present two infants with neuroblastoma stage IV and IVs in a critical situation that needed a decompressive abdominal silo and surgical liver reduction for definitive abdominal closure. Currently both children are 6 years old and they are free from disease. CONCLUSION: The placement of a silo for abdominal decompression is still an indication for salvage. Surgical hepatic reduction is an alternative for the early closure of the abdomen avoiding complications due to infection or dehiscence of the silo.

Management of child optic pathway gliomas: new therapeutical option.

OBJECTIVE: To present our experience in the treatment of child optic pathway gliomas in the last 25 years. MATERIAL AND METHODS: Seventeen children under 10 years of age have been analyzed and assessed from clinic, ophthalmologic, endocrinologic, neurological, neuropathologic, and imaginologic points of view. RESULTS: Predominance of female patients, 10 girls and 7 boys between 6 and 122 months old; mean age was 3 years and 8 months. The most frequent symptoms have been ophthalmologic and visual alterations in all 17 patients, endocrine alterations in 10, and neurological signs in 6. One of the patients presented neurofibromatosis type 1 (NF1), another patient had Down syndrome. Diagnosed using computed tomography or/and magnetic resonance imaging, histological studies showed pilocytic astrocytomas in 13 cases and a fibrillary astrocytoma grade II in 1 case. There were three patients without histological diagnosis; one of them had NF1. The treatment consisted of surgery, external beam radiotherapy, chemotherapy, and brachytherapy with iodine 125, separately or combined. Five patients died; the causes were secondary tumors in two children, tumor recurrence in one, sepsis secondary to respiratory and urinary tract infections in the child with Down syndrome, and finally, hydrocephaly due to hyperproteinorachia of tumor origin in one. Average survival was 89 months. CONCLUSION: Chemotherapy and brachytherapy are therapeutic methods to be considered, especially in children under 5. Marsupialization of the residual cyst into the ventricular system postradio or oncolytic treatment through endoscopic or stereotactic techniques is useful in the treatment of endocranial hypertension and/or hypothalamic compression in these patients.

[Gardner syndrome]. / Sindrome de Gardner.

Three patients, sisters of B, 12 and 14 years of age with a diagnosis of Gardner's Syndrome are reported. The family history indicated that their mother died of a carcinoma of the intestine at the age of 30. This Syndrome, which is of dominant autosomic inheritance, can be seen in aur city, with so heterogeneous hereditary features. Dentists must know the oral cavity characteristics of this disease (odontomas, cementomas, cysts, supernumerary teeth) that accompany gastric, intestinal, skeletal and tissular disturbances an of other organs (polyps, osteomas, cysts, adenomas), being intestinal poliposis of the colon the most important feature that becomes an "Adenocarcinoma", in the third ald fourth decade in a 100% of the cases. Its incidence in our city is real. The proper diagnosis is made when the clinical study is completed and the dentist may be the first one to watch this syndrome's feature in the oral cavity. A proper medical diagnosis would allow a preventive procedure and a patient's control until the "total surgery of the colon", which is the appropriate therapeutic procedure, is achieved; although there are controversies as regards the time to be carried out.

Sindrome de Gardner / [Gardner syndrome]

Three patients, sisters of B, 12 and 14 years of age with a diagnosis of Gardner’s Syndrome are reported. The family history indicated that their mother died of a carcinoma of the intestine at the age of 30. This Syndrome, which is of dominant autosomic inheritance, can be seen in aur city, with so heterogeneous hereditary features. Dentists must know the oral cavity characteristics of this disease (odontomas, cementomas, cysts, supernumerary teeth) that accompany gastric, intestinal, skeletal and tissular disturbances an of other organs (polyps, osteomas, cysts, adenomas), being intestinal poliposis of the colon the most important feature that becomes an [quot ]Adenocarcinoma[quot ], in the third ald fourth decade in a 100

of the cases. Its incidence in our city is real. The proper diagnosis is made when the clinical study is completed and the dentist may be the first one to watch this syndrome’s feature in the oral cavity. A proper medical diagnosis would allow a preventive procedure and a patient’s control until the [quot ]total surgery of the colon[quot ], which is the appropriate therapeutic procedure, is achieved; although there are controversies as regards the time to be carried out.

Sindrome de Gardner. / [Gardner syndrome]

Three patients, sisters of B, 12 and 14 years of age with a diagnosis of Gardners Syndrome are reported. The family history indicated that their mother died of a carcinoma of the intestine at the age of 30. This Syndrome, which is of dominant autosomic inheritance, can be seen in aur city, with so heterogeneous hereditary features. Dentists must know the oral cavity characteristics of this disease (odontomas, cementomas, cysts, supernumerary teeth) that accompany gastric, intestinal, skeletal and tissular disturbances an of other organs (polyps, osteomas, cysts, adenomas), being intestinal poliposis of the colon the most important feature that becomes an [quot ]Adenocarcinoma[quot ], in the third ald fourth decade in a 100

of the cases. Its incidence in our city is real. The proper diagnosis is made when the clinical study is completed and the dentist may be the first one to watch this syndromes feature in the oral cavity. A proper medical diagnosis would allow a preventive procedure and a patients control until the [quot ]total surgery of the colon[quot ], which is the appropriate therapeutic procedure, is achieved; although there are controversies as regards the time to be carried out.