ABSTRACT
In humans, intrauterine growth retardation is correlated to high levels of serum IGF binding protein-1 (IGFBP-1). This present study analyzes in vivo the impact of circulating IGFBP-1 on body growth associated to bone mineralization and carbohydrate resources. Transgenic mice used in this work overexpressed human IGFBP-1 in liver from embryonic day (E)14.5, concomitantly to the appearance of ossification centers, through to adulthood. Growth retardation was observed as early as E17.5 in homozygous (HM) mice being 20% smaller at birth (postnatal d 1). Anatomical analysis of the skeletons by alizarin red and alcian blue staining showed that the mice exhibited pleiotropic defects of several skeletal units. Some bones were small and dysmorphic. Our results showed reduced mineralization in the posterior area of the skull (delayed suture closure), as well as in the appendicular and axial skeleton. Heterozygous crossings showed a loss of HM animals. Moreover, IGFBP-1 overexpression contributed to decreased fetal hepatic glycogen and neonate blood glucose levels which constitute the main reservoir of carbohydrate resources for neonates. Thus, this reduced carbohydrate pool contributed to perinatal mortality. Maternal IGFBP-1 expression was also clearly associated with neonate growth retardation (newborn weights from HM mothers were 20% smaller than newborns from NT mothers) and reduced fetal carbohydrate resources. In conclusion, antenatal growth retardation and delayed mineralization in transgenic mice are related to overexpressed fetal and maternal circulating human IGFBP-1. Similar perturbations could be observed in human intrauterine growth retardation suggesting the IGF/IGFBP system is involved in fetal growth, biomineralization, and energetic status in humans.
Subject(s)
Fetal Growth Retardation/physiopathology , Insulin-Like Growth Factor Binding Protein 1/genetics , Insulin-Like Growth Factor Binding Protein 1/physiology , Animals , Animals, Newborn , Blood Glucose/metabolism , Calcification, Physiologic/physiology , Carbohydrate Metabolism/physiology , Cartilage/growth & development , Female , Genotype , Growth/physiology , Humans , Insulin-Like Growth Factor Binding Protein 1/blood , Liver Glycogen/metabolism , Mice , Mice, Inbred CBA , Mice, Transgenic , Osteogenesis/physiology , Pregnancy , Reverse Transcriptase Polymerase Chain Reaction , Somatomedins/biosynthesis , SurvivalABSTRACT
Pemphigus vulgaris (PV) is an autoimmune disease accounting for 80% of all cases of pemphigus. Before the advent of corticosteroid therapy, pemphigus was fatal, with a mortality rate of up to 75% in the first year. It is still a serious disorder, but the 5% to 10% mortality rate is now primarily due to the side effects of therapy. In 75% to 80% of cases, PV lesions first appear in the oral cavity. Dentists are therefore in a unique position to recognize the oral manifestations of the disease, allowing early diagnosis and initiation of treatment. The diagnosis is based on pathological examination and immunofluorescence testing. Systemic corticosteriods and steroid-sparing agents are the mainstays of treatment; topical corticosteroids may also be used to accelerate healing of persistent oral lesions. This article describes a 71-year-old woman with multiple chronic ulcers in the oral cavity, in whom PV was diagnosed 4 months after the symptoms first appeared. The article also reviews the current literature on diagnosis and treatment of the condition.
Subject(s)
Mouth Diseases/pathology , Mouth Mucosa/pathology , Pemphigus/pathology , Aged , Anti-Inflammatory Agents/therapeutic use , Beclomethasone/therapeutic use , Diagnosis, Differential , Female , Fluorescent Antibody Technique, Direct , Humans , Immunosuppressive Agents/therapeutic use , Methotrexate/therapeutic use , Mouth Diseases/drug therapy , Pemphigus/drug therapy , Prednisone/therapeutic useABSTRACT
Sialolithiasis is the main pathology of major salivary glands. Sialolithiasis of minor salivary glands is, however, generally considered to be extremely rare. Lithiasis of accessory salivary glands apparently occurs more frequently than alleged and therefore should be included in differential diagnosis of tumors of the oral mucous membrane. The elementary lesion consists of a firm nodule located under the mucosal membrane on the upper lip or cheek. Nodules should be excised and examined by histopathology.
Subject(s)
Salivary Gland Calculi/pathology , Salivary Glands, Minor/pathology , HumansABSTRACT
Infantile pancreatic vascular tumors are very unusual and are described with an old and imprecise terminology. We report two new cases in 2 and 4 months old children, the first one associated with Kasabach-Merritt syndrome, the second revealed by cholestasis. The histology diagnosis was kaposiform hemangioendothelioma and juvenile capillary hemangioma respectively. Outcome was favourable after selective embolization for one and biliary derivation for the other. These case reports show that infantile vascular pancreatic tumors form a heterogeneous group. Most of them would be in fact kaposiform hemangioendothelioma with a benign behavior, in contrast with aggressive growth for retroperitoneal localizations.
