MACROSOMIA, OBESITY, MACROCEPHALY AND OCULAR ABNORMALITIES (MOMO SYNDROME) IN 2 UNRELATED PATIENTS - DELINEATION OF A NEWLY RECOGNIZED OVERGROWTH SYNDROME

We describe 2 unrelated patients, a boy and a girl, with an overgrowth syndrome and the following common characteristics: macrocrania, obesity, ocular abnormalities (retinal coloboma and nystagmus), downward slant of palpebral fissures, mental retardation, and delayed bone maturation. Both cases are of sporadic occurrence with no consanguinity between the parents. We suggest that this syndrome is due to a new autosomal dominant mutation and propose to designate it with the acronym of ''MOMO syndrome'' (Macrosomia, Obesity, Macrocrania, Ocular anomalities).

Morphological abnormalities in a blue shark Prionace glauca (Chondrichthyes : Carcharhinidae) foetus from southern Brazil

This study records the occurrence of a blue shark Prionace glauca foetus, with morphological abnormalities in the trunk and cephalic region, from a female caught by a pelagic longline in October 2003, in southern Brazil. (c) 2006 the Authors.

Subclinical abnormalities of glucose metabolism in Brazilian women with a history of gestational diabetes mellitus

Background. To evaluate insulin release and insulin sensitivity in women with prior gestational diabetes mellitus (GDM) to gain a better understanding of type 2 diabetes pathogenesis.Methods. GDM women were individually matched for age, body mass index, and waist/hip ratio with those who were normal glucose tolerant in a previous pregnancy (NGT). All women presented with normal glucose tolerance. Twenty pairs were submitted to the oral glucose tolerance test (OGTT) with plasma glucose, insulin, and C-peptide determinations. of the 20 pairs, 18 participated in hyperglycemic (10.0 mmol/l) clamp experiments with frequent plasma glucose and insulin determinations, allowing us to calculate first- and second-phase insulin release and the insulin sensitivity index. GDM and NGT women were compared using Student's t-test, the Mann-Whitney U-test, Friedman's non-parametric test, and the two proportion test for independent groups.Results. GDM women showed higher glycosylated hemoglobin values; at OGTT, they showed late insulin peak with increased plasma insulin levels only during the second hour, and a similar plasma C-peptide response despite a higher plasma glucose curve; during hyperglycemic clamp procedures, they showed similar biphasic insulin release and insulin sensitivity index. Considering that a woman with previous GDM had a defect in insulin release and/or insulin sensitivity, if its magnitude was at least 25% lower than that of the matched NGT woman, 43.8% showed impairment of first-phase insulin release and 55.6% insulin resistance.Conclusions. GDM women showed some degree of glucose intolerance. It is therefore necessary to follow them for a longer time.

Tooth separation: A risk-free procedure?

This article reports the case of a 12-year-old patient with tooth extrusion, pain, gingival bleeding, and localized periodontitis near the maxillary second premolar. Despite probing and radiographic examination, it was not possible to establish the etiology. Tooth extraction was indicated because of the severe tooth mobility and extrusion. Curettage of the tooth socket revealed a rubber separator. Preventive approaches are suggested to avoid iatrogenesis and legal problems. (Am J Orthod Dentofacial Orthop 2012; 142:402-5)

DIAGNOSIS of REGIONAL CEREBRAL BLOOD FLOW ABNORMALITIES USING SPECT: AGREEMENT BETWEEN INDIVIDUALIZED STATISTICAL PARAMETRIC MAPS and VISUAL INSPECTION BY NUCLEAR MEDICINE PHYSICIANS WITH DIFFERENT LEVELS of EXPERTISE IN NUCLEAR NEUROLOGY

INTRODUCTION: Visual analysis is widely used to interpret regional cerebral blood flow (rCBF) SPECT images in clinical practice despite its limitations. Automated methods are employed to investigate between-group rCBF differences in research Studies but have rarely been explored in individual analyses.OBJECTIVES: To compare visual inspection by nuclear physicians with the automated statistical parametric mapping program using a SPECT dataset of patients with neurological disorders and normal control images.METHODS: Using statistical parametric mapping, 14 SPECT images from patients with various neurological disorders were compared individually with a databank of 32 normal images using a statistical threshold of p<0.05 (corrected for multiple comparisons at the level of individual voxels or clusters). Statistical parametric mapping results were compared with Visual analyses by a nuclear physician highly experienced in neurology (A) as well as a nuclear physician with a general background of experience (B) who independently classified images as normal or altered, and determined the location of changes and the severity.RESULTS: of the 32 images of the normal databank, 4 generated maps showing rCBF abnormalities (p<0.05, corrected). Among the 14 images from patients with neurological disorders, 13 showed rCBF alterations. Statistical parametric mapping and physician A completely agreed on 84.37% and 64.28% of cases from the normal databank and neurological disorders, respectively. The agreement between statistical parametric mapping and ratings of physician B were lower (71.18% and 35.71%, respectively).CONCLUSION: Statistical parametric mapping replicated the findings described by the more experienced nuclear physician. This finding suggests that automated methods for individually analyzing rCBF SPECT images may be a valuable resource to complement visual inspection in clinical practice.

Carbamide Peroxide Determination in Tooth Whitening Using a Reagentless HRP-Biosensor

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Morphology of the dental carinae in Mariliasuchus amarali (Crocodylomorpha, Notosuchia) and the pattern of tooth serration among basal Mesoeucrocodylia.

Carinated teeth are common in Mesoeucrocodylia, and the occurrence of denticles over the carinae is related to high predacious species, often referred as ziphodont. This characteristic is broadly recognized as homoplastic. Carinae morphology is cryptic, difficult to be studied under common techniques, and Scanning Electronic Microscopy (SEM) allows the access to detailed information, offering a higher degree of confidence. Previous SEM study allowed the recognition of true/false ziphodont patterns, according to the morphology of the denticles, but such studies on gondwanan mesoeucrocodyles are uncommon. Mariliasuchus amarali is an Upper Cretaceous notosuchian mesoeucrocodyle from South America (Bauru Group, Brazil), with carinated teeth and specialized dentition. Its geological and biochronological distribution are reappraised. SEM study of two teeth shows carinae composed of isolated tuberous anisomorphic true denticles, supporting previous study. Enamel ornamentation does not develop over the carinae, and fabric becomes anastomosed in middle and posterior teeth. Carinae only occur in posterior molariform teeth, related to food processing. Morphological variability of Mariliasuchus is commented, focusing on dentition. Overall characteristics, molariform morphology and wear planes support a non-predacious habit for Matiliasuchus. Matiliasuchus pattern could not be related to true/false ziphodont patterns, either by morphology or function, and is defined as ziphomorph. Ziphomorph pattern is evaluated within the range of mesoeucrocodyles. The detailed study of homoplastic characteristics, such as dental carinae, may provide useful apomorphic information for cladistic analysis.