Síndrome ocular isquêmica simulando retinopatia diabética unilateral: relato de caso e revisão da literatura

A síndrome ocular isquêmica (SOI) ocorre devido à hipoperfusão ocular crônica secundária à obstrução da artéria carótida. O quadro clínico inclui, entre outros, retinopatia proliferativa similar a retinopatia diabética. A SOI deve ser considerada principalmente nas retinopatias proliferativas unilaterais ou muito assimétricas e nos casos refratários ao tratamento por fotocoagulação. A indicação da endarterectomia nos pacientes com SOI isolada não é bem definida. Este trabalho relata uma paciente com SOI simulando retinopatia diabética proliferativa unilateral e tratada por endarterectomia.

Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis

Angiomyolipomas (AMLs) are mesenchymal neoplasms, named so because of the complex tissue composition represented by variable proportions of mature adipose tissue, smooth muscle cells, and dysmorphic blood vessels. Although AMLs may rise in different sites of the body, they are mostly observed in the kidney and liver. In the case of renal AMLs, they are described in two types: isolated AMLs and AMLs associated with tuberous sclerosis (TS). While most cases of AMLs are found incidentally during imaging examinations and are asymptomatic, others may reach huge proportions causing symptoms. Pulmonary lymphangioleiomyomatosis (LAM) is a rare benign disease characterized by cystic changes in the pulmonary parenchyma and smooth muscle proliferation, leading to a mixed picture of interstitial and obstructive disease. AML and LAM constitute major features of tuberous sclerosis complex (TSC), a multisystem autosomal dominant tumor-suppressor gene complex diagnosis. The authors report the case of a young female patient who presented a huge abdominal tumor, which at computed tomography (CT) show a fat predominance. The tumor displaced the right kidney and remaining abdominal viscera to the left. Chest CT also disclosed pulmonary lesions compatible with lymphangioleiomyomatosis. Because of sudden abdominal pain accompanied by a fall in the hemoglobin level, the patient underwent an urgent laparotomy. The excised tumor was shown to be a giant renal AML with signs of bleeding in its interior. The authors call attention to the diagnosis of AML and the huge proportions that the tumor can reach, as well as for ruling out the TSC diagnosis, once it may impose genetic counseling implications.

The coronal heating problem

The heating of the solar corona has been investigated during four of decades and several mechanisms able to produce heating have been proposed. It has until now not been possible to produce quantitative estimates that would establish any of these heating mechanism as the most important in the solar corona. In order to investigate which heating mechanism is the most important, a more detailed approach is needed. In this thesis, the heating problem is approached ”ab initio”, using well observed facts and including realistic physics in a 3D magneto-hydrodynamic simulation of a small part of the solar atmosphere. The ”engine” of the heating mechanism is the solar photospheric velocity field, that braids the magnetic field into a configuration where energy has to be dissipated. The initial magnetic field is taken from an observation of a typical magnetic active region scaled down to fit inside the computational domain. The driving velocity field is generated by an algorithm that reproduces the statistical and geometrical fingerprints of solar granulation. Using a standard model atmosphere as the thermal initial condition, the simulation goes through a short startup phase, where the initial thermal stratification is quickly forgotten, after which the simulation stabilizes in statistical equilibrium. In this state, the magnetic field is able to dissipate the same amount of energy as is estimated to be lost through radiation, which is the main energy loss mechanism in the solar corona. The simulation produces heating that is intermittent on the smallest resolved scales and hot loops similar to those observed through narrow band filters in the ultra violet. Other observed characteristics of the heating are reproduced, as well as a coronal temperature of roughly one million K. Because of the ab initio approach, the amount of heating produced in these simulations represents a lower limit to coronal heating and the conclusion is that such heating of the corona is unavoidable.

Tratamiento quirúrgico plástico de las mediastinitis post esternotomía media ... : utilización unilateral del colgajo de pectoral mayor frente a otros métodos empleados

Programa de Doctorado en Patología Quirúrgica, Reproducción Humana y Factores Psicológicos y el Proceso de Enfermar

Craniofacial morphology in complete unilateral cleft lip and palate patients consecutively treated with 1-stage repair of the cleft

OBJECTIVE: To retrospectively evaluate the craniofacial morphology of children with a complete unilateral cleft lip and palate treated with a 1-stage simultaneous cleft repair performed in the first year of life. METHODS: Cephalograms and extraoral profile photographs of 61 consecutively treated patients (42 boys, 19 girls) who had been operated on at 9.2 (SD, 2.0) months by a single experienced surgeon were analyzed at 11.4 (SD, 1.5) years. The noncleft control group comprised 81 children (43 boys and 38 girls) of the same ethnicity at the age of 10.4 (SD, 0.5) years. RESULTS: In children with cleft, the maxilla and mandible were retrusive; the palatal and mandibular planes were more open, and sagittal maxillomandibular relationship was less favorable in comparison to noncleft control subjects. Soft tissues in patients with cleft reflected retrusive morphology of hard tissues--subnasal and supramental regions were less convex, profile was flatter, and nasolabial angle was more acute relative to those of the control subjects. CONCLUSIONS: Craniofacial morphology after 1-stage repair was deviated in comparison with noncleft control subjects. However, the degree of deviation was comparable with that found after treatment with alternative surgical protocols.

Dental arch relationship in children with complete unilateral cleft lip and palate following one-stage and three-stage surgical protocols

The objective of this study is to compare dental arch relationship following one-stage and three-stage surgical protocols of unilateral cleft lip and palate. Dental casts of 61 children (mean age, 11.2 years; SD, 1.7), consecutively treated in one center with one-stage closure of the complete cleft at 9.2 months (SD, 2.0), were compared with a sample of 97 patients (mean age, 8.7 years; SD, 0.9), consecutively treated with a three-stage protocol including delayed hard palate closure in another center. The dental casts were assigned random numbers to blind their origin. Four raters graded dental arch relationship and palatal morphology using the EUROCRAN index. The strength of agreement of rating was assessed with kappa statistics. Independent t tests were run to compare the EUROCRAN scores between one-stage and three-stage samples, and Fisher's exact tests were performed to evaluate differences of distribution of the EUROCRAN grades. The intra- and inter-rater agreement was moderate to very good. Dental arch relationship in the one-stage sample was less favorable than in three-stage group (mean scores, 2.58 and 1.97 for one-stage and three-stage samples, respectively; p?

Coronal plane segmental flexibility in thoracic adolescent idiopathic scoliosis assessed by fulcrum-bending radiographs

Knowledge about segmental flexibility in adolescent idiopathic scoliosis is crucial for a better biomechanical understanding, particularly for the development of fusionless, growth-guiding techniques. Currently, there is lack of data in this field. The objective of this study was, therefore, to compute segmental flexibility indices (standing angle minus corrected angle/standing angle). We compared segmental disc angles in 76 preoperative sets of standing and fulcrum-bending radiographs of thoracic curves (paired, two-tailed t tests, p < 0.05). The mean standing Cobb angle was 59.7 degrees (range 41.3 degrees -95 degrees ) and the flexibility index of the curve was 48.6\% (range 16.6-78.8\%). The disc angles showed symmetric periapical distribution with significant decrease (all p values <0.0001) for every cephalad (+) and caudad (-) level change. The periapical levels +1 and -1 wedged at 8.3 degrees and 8.7 degrees (range 3.5 degrees -14.8 degrees ), respectively. All angles were significantly smaller on the-bending views (p values <0.0001). We noted mean periapical flexibility indices of 46\% (+1), 49\% (-1), 57\% (+2) and 81\% (-2), which were significantly less (p < 0.001) than for the group of remote levels 105\% (+3), 149\% (-3), 231\% (+4) and 300\% (-4). The discal and bony wedging was 60 and 40\%, respectively, and mean values 35 degrees and 24 degrees (p < 0.0001). Their relationship with the Cobb angle showed a moderate correlation (r = 0.56 and 0.45). Functional, radiographic analysis of idiopathic thoracic scoliosis revealed significant, homogenous segmental tethering confined to four periapical levels. Future research will aim at in vivo segmental measurements in three planes under defined load to provide in-depth data for novel therapeutic strategies.

Factors influencing the decision for Baha in unilateral deafness: the Bern benefit in single-sided deafness questionnaire

The data of 46 adults with single-sided sensorineural deafness who were candidates for bone-anchored hearing aids (Baha) CROS (contralateral routing of signals) were analyzed. All candidates tested a Baha with a headband in their normal environment. Subsequently, 29 of the candidates chose a permanent Baha CROS fitting, and 17 declined, thus forming the two study groups. No significant difference regarding age, sex or duration of deafness was found between the two groups. Similarly, the transcranial attenuation was not significantly different between those who accepted and declined a Baha. Subjects with some residual hearing in their poorer ear tended to decline a Baha, but the effect was not statistically significant. For a subset of 28 subjects, the Bern Benefit in Single-Sided Deafness questionnaire was administered. The questionnaire consists of 10 visual analogue scales rating the subjectively perceived benefit of the Baha or any other CROS device in different situations. Scores were found to be significantly higher for speech understanding at some distance (p = 0.026), for speech understanding in noise (p = 0.037), for group conversations (p < 0.01), and for the overall benefit (p < 0.01) for those candidates who chose to use a Baha as a CROS device permanently.

Evidence for a new fumarate hydratase gene mutation in a unilateral type 2 segmental leiomyomatosis

Multiple cutaneous and uterine leiomyomata syndrome (MCUL; MIM 150800) is a rare condition that sometimes predisposes to renal cancer. It is caused by deleterious mutations in the fumarate hydratase (FH) gene. In many patients, skin leiomyomas have been reported to develop according to a segmental type 1 or type 2 distribution. We report a patient showing multiple leiomyomas distributed according to a segmental type 2 distribution and covering several areas exclusively on the left side of his body.

Early alveolar bone grafting has a negative effect on maxillary dental arch dimensions of pre-school children with complete unilateral cleft lip and palate

To evaluate maxillary dental arch dimensions in pre-school children with a complete unilateral cleft lip and palate (CUCLP) after early alveolar bone grafting.

Effect of unilateral temporal-lobe excision on perception and imagery of songs

Auditory imagery for songs was studied in two groups of patients with left or right temporal-lobe excision for control of epilepsy, and a group of matched normal control subjects. Two tasks were used. In the perceptual task, subjects saw the text of a familiar song and simultaneously heard it sung. On each trial they judged if the second of two capitalized lyrics was higher or lower in pitch than the first. The imagery task was identical in all respects except that no song was presented, so that subjects had to generate an auditory image of the song. The results indicated that all subjects found the imagery task more difficult than the perceptual task, but patients with right temporal-lobe damage performed significantly worse on both tasks than either patients with left temporal-lobe lesions or normal control subjects. These results support the idea that imagery arises from activation of a neural substrate shared with perceptual mechanisms, and provides evidence for a right temporal- lobe specialization for this type of auditory imaginal processing.

Bilateral total extraperitoneal inguinal hernia repair (TEP) has outcomes similar to those for unilateral TEP: population-based analysis of prospective data of 6,505 patients

Whether bilateral total extraperitoneal (TEP) inguinal hernia repair is associated with worse outcomes than unilateral TEP continues to be a matter of debate. This study aimed to compare different outcomes of large cohorts of patients undergoing bilateral versus unilateral TEP.

Metopic and sagittal synostosis in Greig cephalopolysyndactyly syndrome: five cases with intragenic mutations or complete deletions of GLI3

Greig cephalopolysyndactyly syndrome (GCPS) is a multiple congenital malformation characterised by limb and craniofacial anomalies, caused by heterozygous mutation or deletion of GLI3. We report four boys and a girl who were presented with trigonocephaly due to metopic synostosis, in association with pre- and post-axial polydactyly and cutaneous syndactyly of hands and feet. Two cases had additional sagittal synostosis. None had a family history of similar features. In all five children, the diagnosis of GCPS was confirmed by molecular analysis of GLI3 (two had intragenic mutations and three had complete gene deletions detected on array comparative genomic hybridisation), thus highlighting the importance of trigonocephaly or overt metopic or sagittal synostosis as a distinct presenting feature of GCPS. These observations confirm and extend a recently proposed association of intragenic GLI3 mutations with metopic synostosis; moreover, the three individuals with complete deletion of GLI3 were previously considered to have Carpenter syndrome, highlighting an important source of diagnostic confusion.