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).

Complex cytogenetic rearrangement in a case of unicameral bone cyst

Cytogenetic analysis of a unicameral bone cyst surgically resected in an 11-year-old boy revealed a highly complex clonal structural rearrangement involving chromosomes 4, 6, 8, 16, 21, and both 12. These findings reinforce the need for further studies on unicameral bone cysts to verify the frequency and to understand the significance of chromosome anomalies in this type of lesion.

Pediatric Chronic Sclerosing Sialadenitis: Kuttner Tumor

Chronic sclerosing sialadenitis is an uncommon cause of salivary gland enlargement mainly occurring in the fifth and seventh decade of life. In the Western population, chronic sclerosing sialadenitis has been characterized as an IgG4-related disease. Although rare, this lesion occurs in children. To increase awareness about this entity in the pediatric age group, we report the case of an 11-year-old boy with a hard, 4.0-cm circumscribed mass in the right submandibular gland. Histologically there was marked distortion of the gland architecture by a dense lymphocytic infiltrate and extensive fibrosis with septa that crossed and distorted the gland, leaving atrophic acini and dilated, irregular ducts. The lymphoid infiltrate formed multiple follicles with active germinal centers, numerous plasma cells, and areas with diffuse arrangement. Immunophenotyping showed abundant CD20- and CD3-positive lymphocytes; cytokeratin AE1/AE3 highlighted the distorted architectural pattern; IgG staining showed large numbers of positive cells infiltrating the interstitium and surrounding the atrophic acini and ducts. IgG4 staining revealed a large proportion of positive infiltrating elements. Kuttner tumor belongs to the group of IgG4-related sclerosing diseases. The differential diagnosis includes pleomorphic adenoma and other salivary gland neoplasms. Its recognition in children is important clinically because this entity is amenable to steroid treatment, and additional work up and follow up is warranted to stave off other IgG4-related diseases/complications.

Interstitial long-arm deletion of chromosome 7 and ectrodactyly

An interstitial deletion of 7q21 was found in a boy with mental retardation, microcephaly, convergent strabismus, micrognathia, genital anomalies, and other findings, including ectrodactyly.

Rhabdomyosarcoma in a congenital pigmented nevus

A 7-month-old boy had a giant pigmented lesion involving the trunk and thighs that exhibited many hyperpigmented hairy and verrucous nevi. One of the nevi ulcerated and on histological examination consisted of pleomorphic rhabdomyosarcoma cells that stained for muscle-specific actin (HHF-35), desmin, and myoglobin. Around the tumor, in the dermis, benign pigmented nevus cells were observed. The occurrence of malignant tumors, other than malignant melanoma, in pigmented nevi is rarely described.

Ectima gangrenoso: Relato de caso

Report of a case of ecthyma gangrenosum with septicemia by Pseudomonas aeruginosa with favorable evolution in a 3-month-old boy. This disease is relatively rare, potentially lethal and the early diagnosis and treatment has an important role in the clinical evolution.

Conservative management of severe intrusion in a primary tooth: A 4-year follow-up

The purpose of this report was to describe the case of an 18-month-old boy who was referred to the pediatric clinic of the School of Dentistry of Araçatuba, São Paulo State University, Araçatuba, São Paulo, Brazil, 3 days after sustaining severe trauma that led to the complete intrusion of the primary maxillary right lateral incisor, a crown fracture of the primary maxillary right central incisor without pulp involvement, and disruption of the superior labial frenum. Four months later, spontaneous re-eruption was observed in the intruded tooth and no endodontic intervention was necessary in either traumatized teeth. Four years after the trauma, a morphological change in the germ of the permanent successor was noted. Clinical follow-up and periodic radiographies are necessary after traumatic intrusion of primary teeth to monitor possible sequelae in the permanent successors.

Desmoplastic small round cell tumor of the kidney mimicking wilms tumor: A case report and review of the literature

Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm usually present with the widespread abdominal serosal involvement and affects mainly adolescents and young adults. When presenting within visceral organs, as kidney, the diagnosis of DSRCT imposes significant difficulties. We present a case of primary DSRCT of the kidney in a 10-year-old boy mimicking clinically and pathologically Wilms tumor. The tumor showed morphologic and immunohistochemical features of DSRCT and the presence of the Ewing sarcoma and Wilm tumor 1 fusion transcripts resulting from the t(11;22) (p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of Wilm tumor and other small blue-round cell tumors of the kidney. © 2009 by Lippincott Williams & Wilkins.

Developmental Disturbance of an Unerupted permanent incisor due to trauma to its predecessor

Developmental disturbances of permanent teeth can result from trauma to primary teeth because of the proximity of the root of the primary teeth to their permanent successors. We describe the case of a 14-month-old boy who was referred to the baby clinic of the School of Dentistry, Universidad Estadual Paulista, Araçatuba, Brazil, after sustaining a severe trauma that led to intrusion of the right primary central incisor. Radiographic examination 4 years after the trauma showed a developing morphological change in the germ of the permanent successor. On eruption of the permanent central incisor, a crown malformation along with enamel hypoplasia was observed. We concludethat radiographic follow-up is indicated after trauma to monitor possible sequelae in the permanent successors even before their eruption. © 2011 Canadian Dental Association.

Tracheostomy-dependent child with temporomandibular ankylosis and severe micrognathia treated by piezosurgery and distraction osteogenesis: Case report

Ankylosis of the temporomandibular joint in children is one the most difficult and complex conditions managed by oral and maxillofacial surgeons, and often leads to some facial deformity. Distraction osteogenesis of the mandible provides an excellent treatment for mandibular airway obstruction in children who do not respond to conservative measures, and allows for early removal of the tracheostomy. We report the case of a 1-year-old boy with severe micrognathia and temporomandibular ankylosis who was dependent on a tracheostomy; he was treated with piezosurgery and mandibular advancement by distraction osteogenesis. © 2010 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Ensaio sobre como Macunai{dotless acute}ma me ajudou a falar sobre Educação Matemática

As if aesthetics were not content. But as we are faced with some other language (frequently called incorrect), our inclination is to correct it or to want to correct it: not luv, boy! But that's what we hear, not what should be. This essay is, based on aesthetics, a testimony about not being ashamed of its contribution to the field (Mathematics Education) - as a contribution and not 'only' style. The usual parametres for research papers are kept on check.

Interação de um aluno com paralisia cerebral com colegas de classe durante atividades ludicas

The study aimed to examine the quality of interpersonal relationships developed between a child with cerebral palsy (CP) and his classmates during group play activities carried out in the classroom and during recess. The participants of the study were a boy with clinical diagnosis of quadriplegic CP, his teacher and classmates. Data collection was carried out by means of a structured interview with the teacher and of video recording the child with CP interacting with his classmates in play activities. The interview data analysis was carried out using qualitative procedures and the video recording was analyzed using a category system. The results showed that the teacher perceived the child with CP to be interested; he participated in play activities and interacted successfully both with familiar partners and with all classmates in general. On the other hand, the analysis of the video recorded activities showed that even though interaction did in fact occur positively, the child with CP remained mostly an observer of the activities rather than an active participant, possibly due to the gap between the child's possibilities and the requirements imposed by the activities underway in the school setting.

Low-level laser therapy in pediatric bell's palsy: Case report in a three-year-old child

Objectives: The objective of this study was to apply low-level laser therapy (LLLT) to accelerate the recovery process of a child patient with Bell's palsy (BP). Design: This was a prospective study. Subject: The subject was a three-year-old boy with a sudden onset of facial asymmetry due to an unknown cause. Materials and methods: The low-level laser source used was a gallium aluminum arsenide semiconductor diode laser device (660 nm and 780 nm). No steroids or other medications were given to the child. The laser beam with a 0.04-cm2 spot area, and an aperture with approximately 1-mm diameter, was applied in a continuous emission mode in direct contact with the facial area. The duration of a laser session was between 15 and 30 minutes, depending on the chosen points and the area being treated. Light was applied 10 seconds per point on a maximum number of 80 points, when the entire affected (right) side of the face was irradiated, based on the small laser beam spot size. According to the acupuncture literature, this treatment could also be carried out using 10-20 Chinese acupuncture points, located unilaterally on the face. In this case study, more points were used because the entire affected side of the face (a large area) was irradiated instead of using acupuncture points. Outcome measures: The House-Brackmann grading system was used to monitor the evolution of facial nerve motor function. Photographs were taken after every session, always using the same camera and the same magnitude. The three-year-old boy recovered completely from BP after 11 sessions of LLLT. There were 4 sessions a week for the first 2 weeks, and the total treatment time was 3 weeks. Results: The result of this study was the improvement of facial movement and facial symmetry, with complete reestablishment to normality. Conclusions: LLLT may be an alternative to speed up facial normality in pediatric BP. © Copyright 2013, Mary Ann Liebert, Inc. 2013.

Effect of a hyperbolide mastication apparatus for the treatment of severe sleep bruxism in a child with cerebral palsy: Long-term follow-up

Purpose: Sleep bruxism is common among the various oromotor alterations found in individuals with cerebral palsy (CP). Few studies have investigated the use of the mastication device denominated hyperbola (HB) and none was found describing the use of such a device for the treatment of bruxism in children with CP. The aim of the present study was to evaluate the effect of the HB on electromyographic (EMG) activity in the jaw-closing muscles and the reduction in sleep bruxism in a child with CP using surface EMG analysis before and after nine months of treatment. Methods: A seven-year-old boy with severe spastic CP and sleep bruxism was enrolled in this study. The HB was chosen as the treatment option for sleep bruxism in this case because the child did not accept an occlusal splint. The HB has a hyperbolic shape and is made of soft, non-toxic, odorless, tasteless silicone. There are five different sizes of HB manufactured based on the diversity of tooth sizes. This device produces proprioceptive excitation in the dentoalveolar nerve, spindles and Golgi tendon organs. HB has been employed for the treatment of temporomandibular disorder, abnormal oro-dental development, abnormal occlusion, xerostomy, halitosis and bruxism. HB therapy was performed for 5 min six times a day over a nine-week period. Surface EMG of the mandible at rest and during maximum contraction was performed on the masseter and temporalis muscles bilaterally to evaluate electromyographic activity before and after nine months of HB usage. Results: HB usage led to a visible tendency toward the reorganization of mastication dynamics, achieving a marked balance in electromyographic activity of the jaw-closing muscles and improving the child's quality of life. Conclusion: Based on the findings of the present study, this noninvasive therapy may be useful for individuals with cerebral palsy due to its positive effects and low cost, which allows its use in the public health realm. Further clinical studies with a larger sample size are needed to validate these results and allow the development of a new treatment protocol for patients with spastic cerebral palsy. © 2013 Elsevier Ltd. All rights reserved.