Distinct cellular origin and genetic requirement of Hedgehog-Gli in postnatal rhabdomyosarcoma genesis.

Dysregulation of the Hedgehog (Hh)-Gli signaling pathway is implicated in a variety of human cancers, including basal cell carcinoma (BCC), medulloblastoma (MB) and embryonal rhabdhomyosarcoma (eRMS), three principle tumors associated with human Gorlin syndrome. However, the cells of origin of these tumors, including eRMS, remain poorly understood. In this study, we explore the cell populations that give rise to Hh-related tumors by specifically activating Smoothened (Smo) in both Hh-producing and -responsive cell lineages in postnatal mice. Interestingly, we find that unlike BCC and MB, eRMS originates from the stem/progenitor populations that do not normally receive active Hh signaling. Furthermore, we find that the myogenic lineage in postnatal mice is largely Hh quiescent and that Pax7-expressing muscle satellite cells are not able to give rise to eRMS upon Smo or Gli1/2 overactivation in vivo, suggesting that Hh-induced skeletal muscle eRMS arises from Hh/Gli quiescent non-myogenic cells. In addition, using the Gli1 null allele and a Gli3 repressor allele, we reveal a specific genetic requirement for Gli proteins in Hh-induced eRMS formation and provide molecular evidence for the involvement of Sox4/11 in eRMS cell survival and differentiation.

Opitz (BBB/G) syndrome: oral manifestations.

We studied a new case of the G (Opitz BBB/G) syndrome in a 12-year-old boy. Several relatives had partial manifestations of the disorder. A comprehensive dental evaluation of the propositus was conducted; included is, to our knowledge, the first published cephalometric analysis of a G syndrome patient. We reviewed 139 cases of the G syndrome; 48 of them had at least one oral abnormality. These included clefting, micrognathia, ankyloglossia, and high-arched palate. Male G syndrome patients are more likely to have oral anomalies than affected females.

Gingival and cutaneous xanthomatosis associated with primary biliary cirrhosis. Report of a case.

The first comprehensive case report of intraoral xanthomatosis associated with primary biliary cirrhosis is detailed. The lesions were present along the free gingival margin and labial vestibule. Crevicular fluid was noted to be tinged yellow. Generalized cutaneous eruptions were also seen. The xanthomas were attributed to the underlying hyperlipoproteinemia. Advanced progression of the hepatic disease necessitated a liver transplant. Disappearance of both oral and skin lesions were subsequently observed.

The Rieger anomaly concomitant with multiple dental, craniofacial, and somatic midline anomalies and short stature.

An unusual, isolated case of the Rieger anomaly coincident with a multitude of dental, craniofacial, and systemic anomalies is described. Significant dental findings were severe enamel hypoplasia, conical and misshapen teeth, hypodontia, and impactions. Craniofacial disorders were underdevelopment of the maxilla, mandible, and anterior and posterior cranial bases, low-set ears, and a wide nasal bridge. Reported for the first time is the association of this genetic disorder with bilateral microcondyles and bilateral choanal atresia. Embryologic disturbance of the neural crest ectoderm is suspected. The patient also manifested anal atresia, scoliosis, kyphosis, and short stature. A discussion distinguishing this case report from the Rieger syndrome is presented. In addition, the possibility that the patient exhibited a previously unreported syndrome is also considered, and the term Short-F-R-A-M-E is proposed to name this new syndrome.

Detection of intracoronal resorption in an unerupted developing premolar: report of case.

Identification of a resorptive lesion in an unerupted premolar is detailed in this report of a 10-year-old female. The lesion was discovered during a periodic recall examination. Early detection and treatment of this resorptive process will minimize its potentially destructive capacity. Radiographic inspection of this lesion in the preeruptive dentition should be routinely performed.

The Marcus Gunn phenomenon. Discussion and report of a case.

A detailed clinical description of the Marcus Gunn phenomenon is featured. Related ophthalmologic aberrations associated with mandibular movement are briefly discussed. A report of a case of Marcus Gunn phenomenon, which was significant for a paternal history of bilateral cleft lip and palate, is presented. The general and the dental considerations in the management of this disorder are summarized. Protective eye shields are recommended for use during dental procedures.

Geographic stomatitis: review of the literature and report of five cases.

Five new cases of geographic stomatitis have been presented along with data from 24 previously reported patients. For the first time, basic parameters of this disorder have been tabulated from this population. Although the earliest documented case appeared in the literature in 1955, the majority of articles has been published during the last 15 years. This increased frequency of reporting indicates a growing awareness of this rather innocuous lesion. Thus, the general practitioner is advised to become familiar with the salient features of geographic stomatitis. Prompt recognition and diagnosis of this lesion, based on the clinical findings and history, will usually mitigate the need for biopsy.

The effects of hormonal oral contraceptives on the female human periodontium and experimental animal models, a review of the literature.

PIP: This paper discusses the influence of hormonal oral contraceptives (OCs) on the health of the periodontium. Characteristics oral manifestations of OC intake are similar to oral changes associated with pregnancy, such as the pronounced vascularity of the gingiva, hyperplestic gingivitis, and pyogenic granuloma. There are as yet no reports in the literature of the influence of OC on the health of the alveolar bone in humans, although there are conflicting data gathered on animal studies. How OCs influence vscular changes in the periodontium is unclear; prostaglandin E2 may be implicated as a possible mediator of the inflammatory process.^ieng

Enrichment of albumin and IgG in the airway secretions of dogs breathing ozone.

A study was undertaken of the acute effect of the inhalation of ozone (1.0-1.5 ppm) on the concentration of proteins in the airway secretions harvested from the trachea of intact anesthetized dogs. Secretions were first collected during a control day during which room air was breathed. The dogs breathed ozone (1.0-1.5 ppm) on a second day. This protocol permitted each dog to serve as its own control. During the inhalation of ozone the concentration of total aqueous-soluble proteins, as well as of albumin and IgG were not significantly effected. However, when expressed as a percentage of total protein, both albumin and IgG were significantly enriched in the secretions. The quantitative changes of certain proteins in airway secretions may be an important reflection of the pulmonary response to ozone.

Weak compactness and control measures in the space of unbounded measures.

We present a synthesis theorem for a family of locally equivalent measures defined on a ring of sets. This theorem is then used to exhibit a control measure for weakly compact sets of unbounded measures. In addition, the existence of a local control measure for locally strongly bounded vector measures is proved by means of the synthesis theorem.

On finitely additive vector measures.

In this paper we extend the well-known Vitali-Hahn-Saks and Nikodým theorems for measures to finitely additive vector-valued set functions.

On the vitali-hahn-saks and nikodym theorems.

We present simplified proofs of the well-known Vitali-Hahn-Saks and Nikodým theorems for measures.