ABSTRACT
Idiopathic intracranial hypertension (IIH) is a disorder of unknown origin, which is characterized by elevated intracranial pressure (ICP) without underlying etiological evidence of neurological disease. The purpose of the current study was to evaluate epidemiological features, clinical presentation, diagnostic findings and treatment of sixteen children (7 males and 9 females) with IIH. Medical records of the patients were obtained from the University Paediatric Hospital of Catania, Italy. Clinical features, investigations and treatment approaches were retrieved. The mean age of the sixteen children at onset of symptoms was 9 years (range: 4 to 16 years). Most of the patients were classified as pre-pubertal. Mean BMI was 28.9 kg/m2. In 93.75% of patients headache was the presenting clinical symptom; and in the same percentage papilledema was detected as the accompanied sign during diagnostic flow-chart. The mean lumbar puncture opening pressure (LPOP) was 350 mm H2O. Fifty percent of the cases had normal brain imaging, while 12.5% showed enlarged optic nerve diameter and one patient had an intraocular protrusion of the optic nerve on MRI. Two patients (12.5%) had venous sinus stenosis, and one case showed an abnormal spinal MRI. With regard to therapeutic approaches, 93.75% of the cases were successfully treated with Acetazolamide. None of the patients required surgical procedures, and all neuroimaging findings disappeared after receiving treatment. In the present study we investigated the association of IIH with venous sinus stenosis. We also found ocular ultrasound to be a useful non-invasive alternative method for determining papilledema in paediatric IIH, specifically in an emergency.
Subject(s)
Constriction, Pathologic/diagnostic imaging , Headache/diagnostic imaging , Intracranial Hypertension/diagnostic imaging , Papilledema/diagnostic imaging , Acetazolamide/therapeutic use , Adolescent , Anticonvulsants/therapeutic use , Child , Child, Preschool , Constriction, Pathologic/drug therapy , Constriction, Pathologic/epidemiology , Constriction, Pathologic/pathology , Cranial Sinuses/diagnostic imaging , Cranial Sinuses/drug effects , Cranial Sinuses/pathology , Female , Headache/drug therapy , Headache/epidemiology , Headache/pathology , Humans , Intracranial Hypertension/drug therapy , Intracranial Hypertension/epidemiology , Intracranial Hypertension/pathology , Magnetic Resonance Imaging , Male , Neuroimaging , Optic Nerve/diagnostic imaging , Optic Nerve/drug effects , Optic Nerve/pathology , Papilledema/drug therapy , Papilledema/epidemiology , Papilledema/pathology , Retrospective Studies , Spinal Puncture , Treatment OutcomeABSTRACT
NF-kappaB regulates liver cell death during development, regeneration, and neoplastic transformation. For example, we showed that oncogenic Ras- or Raf-mediated transformation of rat liver epithelial cells (RLEs) led to altered NF-kappaB regulation through IKK complex activation, which rendered these cells more resistant to TGF-beta1-induced apoptosis. Thus, based on these findings, we sought to determine whether NF-kappaB could also be involved in tumor growth of liver cells in vivo. Hepatocellular carcinomas (HCCs) derived from bitransgenic mice harboring TGF-alpha and c-myc transgenes targeted specifically to the liver were compared with HCCs from c-myc single transgenic mice. Tumors from bitransgenic mice are characterized by a higher frequency of appearance, lower apoptotic index, and a higher rate of cell proliferation. Here we show that NF-kappaB is activated in HCCs of double TGF-alpha/c-myc transgenic mice, but not of c-myc single transgenic mice, suggesting that TGF-alpha mediates induction of NF-kappaB. Activation of the IKK complex was observed in the HCCs of double TGF-alpha/c-myc transgenic mice, implicating this pathway in NF-kappaB induction. Lastly, activation of the Akt/protein kinase B (PKB), which has recently been implicated in NF-kappaB activation by PDGF, TNF-alpha, and Ras, was also observed. Importantly, human HCC cell lines similarly displayed NF-kappaB activation. Thus, these studies elucidate an anti-apoptotic mechanism by a TGF-alpha-Akt/PKB-IKK pathway, which likely contributes to survival and proliferation, thereby accelerating c-myc-induced liver neoplastic development in vivo.
Subject(s)
Carrier Proteins/physiology , Liver Neoplasms, Experimental/metabolism , NF-kappa B/biosynthesis , Protein Serine-Threonine Kinases , Proto-Oncogene Proteins c-myc/genetics , Proto-Oncogene Proteins/physiology , Transforming Growth Factor alpha/genetics , Animals , Apoptosis , Cell Division , Enzyme Activation , Gene Expression , Intracellular Signaling Peptides and Proteins , Liver/metabolism , Liver Neoplasms, Experimental/pathology , Male , Mice , Mice, Transgenic , NF-kappa B/metabolism , Proto-Oncogene Proteins c-akt , Proto-Oncogene Proteins c-myc/physiology , RNA-Binding Proteins , Transforming Growth Factor alpha/physiologyABSTRACT
UNLABELLED: The issue of whether silicone oil has a toxic effect on the retina is still being debated. In order to verify possible retinotoxic effects, the authors studied the variations of retinal function by means of computerized perimetry on patients who had undergone a vitreoretinal operation with injection of silicone oil in the vitreous cavity. Silicone oil remained in the eyes for a period of 120 days +/- 15. PATIENTS AND METHODS: 14 patients under treatment for vitreoretinal pathologies sparing the macula underwent five examinations (Macular Threshold Test of the Humphrey Field analyzer): two examination during the period silicone oil was in the vitreous cavity (30 days after injection and 10 days before removal) and three examinations after its removal (at 30, 90, and 180 days). RESULTS: When the paired t-test and ANOVA test were applied to our results, there were no significant differences in the light-difference sensitivity. CONCLUSIONS: These data do not indicate that silicone oil a retinotoxic effect (revealed by means of computerized perimetry) when it is removed within 4 months.
