Biomedical subjects

I L Marques

Publications and source records attributed to I L Marques.

3 recordsLinked to original sources

PubMed2001

Clinical experience with infants with Robin sequence: a prospective study.

OBJECTIVE: To study the clinical course of patients with Robin sequence (RS) during the first 6 months of life. DESIGN: A longitudinal prospective study of children with RS. SETTING: Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru-SP, Brazil, 1997 and 1998. PATIENTS: Sixty-two children were studied from hospital admission to 6 months of age. Thirty-three (53.2%) presented with probable isolated RS (PIRS), 25 (40.3%) presented with syndromes or other malformations associated with RS, and 4 (6.5%) presented with RS with neurological involvement. INTERVENTIONS: The type of respiratory tract obstruction was defined by nasopharyngoscopy. The patients with type 1 and type 2 obstruction underwent nasopharyngeal intubation (NPI), and glossopexy was indicated in patients with type 1 obstruction who did not show clinical improvement with this procedure. Tracheostomy was indicated in patients with type 2 obstruction who did not show a good course after NPI, in patients with type 1 obstruction who did not show good course after glossopexy, and in patients with type 3 and type 4 obstruction. RESULTS: Prone position treatment (PPT) or NPI was the definitive treatment in 25 cases (75.8%) of PIRS and in 13 cases (52%) of syndromes or other malformations. Among the children with type 1 obstruction, 24 (51.1%) were submitted exclusively to PPT and 12 (25.5%) to NPI. With the type 2 groups, only one (12.5%) received PPT, and three (37.5%) were treated exclusively with NPI. All 15 infants treated exclusively with NPI (24.4%) presented with good weight, length, and neuromotor development. CONCLUSIONS: Most patients with PIRS and type 1 obstruction improved without surgical intervention. NPI should be the initial treatment in all patients with RS with type 1 and type 2 obstruction who present with important respiratory and feeding difficulties.

Abnormalities, Multiple↗

PubMed1998

A methodology report of a randomized prospective clinical trial to assess velopharyngeal function for speech following palatal surgery.

Cleft lip and palate occurs in approximately 1 in every 750 live human births, making it one of the most common congenital malformations. Surgical closure of the palatal cleft does not always result in a velopharyngeal port capable of supporting normal speech. The University of Florida (UF), in collaboration with the University of São Paulo (USP), is engaging in a 5-year prospective, randomized controlled study to compare velopharyngeal function for speech outcomes between patients undergoing palatoplasty for complete unilateral cleft lip and palate performed using the von Langenbeck procedure with intravelar velarplasty and those receiving the Furlow double-reversing Z-plasty palatoplasty. The von Langenbeck procedure was selected as the time-tested standard against which the Furlow procedure could be judged. The Furlow procedure, a relatively new operation, has been reported to yield substantially higher rates of velopharyngeal competency for speech than have most other reported series and theoretically should result in less disturbance to midfacial growth. A total of 608 patients will be entered into one of two age categories. Inclusion of two age groups will allow a comparison of results between patients having surgery before 1 year of age (9-12 months) and patients undergoing surgery at approximately 1.5 years of age (15-18 months). Speech data will be collected and will be available for definitive analysis throughout the last 3 years of the study. Collection of preliminary growth data will require more than 5 years; growth analysis is anticipated to continue until all patients have reached maturity. The Hospital for Research and Rehabilitation of Patients with Cleft Lip and Palate at the University of São Paulo (USP-HPRLLP) in Bauru, Brazil, is uniquely situated for conducting this study. The well-equipped and modern facilities are staffed by well-trained specialists representing all disciplines in cleft-palate management. In addition, an already existing social services network throughout Brazil will ensure excellent follow-up of study cases. The clinical caseload at this institution currently exceeds 22,000, and more than 1200 new cases are added annually. This project represents a unique opportunity to obtain prospective data from a large number of subjects while controlling the variables that have traditionally plagued cleft-palate studies. This study is designed to determine which of the two proposed surgical procedures is superior in constructing a velum capable of affecting velopharyngeal competency for the development of normal speech.

Cleft Palate↗

PubMed1998

Etiopathogenesis of isolated Robin sequence.

OBJECTIVE: To investigate the etiopathogenesis of isolated Robin sequence. DESIGN: A longitudinal and prospective study of children with isolated Robin sequence and no other associated syndromes or malformations. SETTING: The study was carried out at the Hospital de Reabilitação de Anomalias Craniofaciais (formerly the Hospital for Research and Rehabilitation of Cleft Lip/Palate), University of São Paulo, Bauru, SP, Brazil, which provides care for patients with lip/palate lesions throughout Brazil. PATIENTS: Forty-three children were initially included in the study, seven of whom were later found to be cases of Stickler syndrome and excluded. The remaining 36 children presenting only the anomaly triad of microretrognathia, glossoptosis, and cleft palate were followed up from the first month of life to 4 years of age with repetitive clinical and ophthalmological examination. MAIN OUTCOME MEASURE: A family history of cleft lip/palate was determined on the basis of information provided by the parents and, when possible, the affected relative was submitted to physical examination. RESULTS: A family history of cleft lip/palate was observed in 27.7% of cases, one case having a younger brother with only cleft palate but no microretrognathia or glossoptosis. Six cases of isolated cleft palate and three cases of cleft lip with or without cleft palate were present in distant relatives. Complete U-shaped cleft palate (wide cleft) was the most frequent type of cleft, which was present in 75% of cases. Only one case of incomplete cleft palate was observed, but U-shaped; 25% of the patients presented complete V-shaped cleft palate. CONCLUSIONS: We conclude that heredity could be a factor in the etiopathogenesis of isolated Robin sequence and suggest that cleft palate (usually complete and U-shaped) is the primary event in the determination of the triad of anomalies.

Brazil↗