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Serological and biochemical genetic markers in congenital cleft lip and cleft palate anomalies.

Serological and biochemical genetic markers in 76 patients with cleft lip with or without cleft palate [CL(P)] and 24 patients with isolated cleft palate (CP) were studied and compared with healthy control subjects. Blood group A phenotype and homozygous plasma haptoglobin (2-2) were found associated with CL(P). The findings suggest that the individuals possessing these markers are more likely to develop congenital CL(P).

ABO Blood-Group System↗

Factors determining occurrence of cleft lip and cleft palate.

The modern care of newborn infants with oral clefts renders their survival possible. Since they will eventually reproduce, a slight increase in the incidence may be expected and genetic counseling will be requested on occasion. The cleft lip with or without cleft palate has a different genetic inclination from isolated cleft palate and the risk of recurrence is different. Drugs, such as antiepileptics, salicylates, benzodiazepines and cortisone, have a role in causing oral clefts. When an oral cleft is a part of a syndrome, the genetics of the particular syndrome must be outlined and genetic counseling is given accordingly. Oral clefts alone are usually multifactorial.

Animals↗

Fetal mortality associated with cleft lip and cleft palate.

Analysis of 1,516 pregnancies in sibships of probands with cleft lip with or without cleft palate [CL(P)], and 774 pregnancies in those of probands with isolated cleft palate (CP) indicated that fetal mortality significantly increases with liability to clefting. These data are compatible with the multifactorial two-threshold model, according to which a lower threshold level of liability results in a cleft formation whereas a higher level of liability causes a fetal death.

Cleft Lip↗

Genetics of cleft lip and cleft palate in China.

During the past 10 years, 60 cases of cleft lip with or without cleft palate [CL(P)] were recorded among 45,072 newborns at Shanghai International Peace Maternity and Infant Hospital, China. The incidence was 1.33 per 1,000 births. The family histories of 163 CL(P) patients were analyzed. The incidences of CL(P) in the first-, second-, and third-degree relatives of CL(P) patients were 11/246 (4.47%), 10/1,032 (0.97%), and 6/1,727 (0.35%), respectively. Of the 163 probands, three had a history of consanguinity of the parents (1.8%), in contrast to 0.77% in the general population. These data are suggestive of multifactorial inheritance. The heritability of CL(P) in our study calculated by Falconer's formula was 77.6%.

Adult↗

[Simultaneous repair of cleft lip and closure of cleft hard palate with vomer flaps in patients with unilateral complete cleft lip and palate].

OBJECTIVE: The purpose of this study was to retrospect the prognosis of simultaneous repair of cleft lip and closure of cleft hard palate with vomer flaps in patients with unilateral complete cleft lip and palate. METHODS: A retrospective study was carried out in 47 patients with unilateral complete cleft lip and palate and, simultaneously received repair of cleft lip and closure of cleft hard palate with vomer flaps. The duration of operation, as well as the blood loss during the operation was recorded, and compared with those patients who only received cleft lip repair. RESULTS: All the operations were successful, and the wound healed well. The procedure of simultaneous repair of cleft lip and closure of cleft hard palate with vomer flaps did not prolong the operating time, compared with simple cleft lip repair. No blood transfusion was needed due to closure of cleft hard palates with vomer flaps. CONCLUSION: Simultaneous repairs of cleft lip and closure of cleft hard palate with vomer flaps are safe for patients with unilateral complete cleft lip and palate.

Abnormalities, Multiple↗

Craniofacial variability in parents of children with cleft lip and cleft palate.

The purpose of this study was to determine whether there are variations in the craniofacial morphology between parents of children with non-syndromic cleft lip and palate and normal controls with no history of cleft in the families in a Saudi sample. Frontal skull radiographs were obtained from a study group consisting of 40 couples, who have a child with cleft deformity and control group of 32 males and 35 females, who have no history of clefts in the family. A total of 18 landmarks were digitized for each individual by a custom made computer program. T-test, logistic regression analysis were applied to the data. Significant findings were obtained for both males and females of the affected children. The males displayed wider nasal cavity and a narrower maxillary width with asymmetry of the face. Females showed smaller facial dimension as a whole, and asymmetry of the face.

Adult↗

Mental, motor, and social behavior of infants with cleft lip and/or cleft palate.

This is a report of analyses of cross-sectional and longitudinal samples of the mental, motor, and social behavior of children with cleft lip and/or palate as measured by the Bayley Scales for Infant Development. Seventy-five patients of the Lancaster Cleft Palate Clinic were the subjects for the sample. The longitudinal sample consisted of 28 of these Ss for whom there were complete data at 6, 12, 18, and 24 months. The results indicated that there were no significant differences in mental and motor functioning. In both analyses, the social behavior of patients with oral-facial clefts was more passive than the Bayley normative sample. The implications of these findings for future research are discussed.

Child Behavior↗

Syndromes with cleft lip and cleft palate.

A series of tables is presented as a diagnostic aid for the clinician when he confronts a patient who has a cleft lip and/or palate, together with associated anomalies. The tables provide a rapid way of sorting through the recognized syndromes with orofacial clefting in search of a possible overall diagnosis. Today, 154 such syndromes are recognized. This is more than twice as many as were known in 1971. Undoubtedly, many new syndromes with orofacial clefting will be delineated in the future.

Abnormalities, Multiple↗

[Measurements of the soft tissues in patients with cleft lip and cleft palate].

A comparative study was made on the pressure of the oral soft tissues during sequences of physiological functions and at rest in eugnathic subjects and patients with cleft lips and palates. The data obtained by means of electronic measuring instruments revealed marked differences between the two groups.

Child, Preschool↗

[Synthesis of the standard of treatment of cleft lip and cleft palate by 11 francophone teams en 2001].

Influenced by Eurocleft project, the authors proposed a synthesis of the protocol of treatment for cleft palate and/or cleft lip patient by 11 french and belgian teams. If the goal are similar (restored anatomy with good appearance, good speech, normal hearing, facial growth and dental arch correct, good social integration) the methods to obtain this outcome are different in each cleft unit. Consensual points and dissensions were described in the discussion.

Belgium↗

[Growth of the jaw after preoperative orthodontic treatment of cleft lip and cleft palate].

In 48 patients a systematized therapeutic model plan for the preoperative and postoperative orthodontic treatment of patients with unilateral or bilateral cleft lips and palates is pursued. In addition to stimulation and growth control of the maxillary alveolar segments in a transverse direction and at the segment poles, increasing sagittal development is observed in a later developmental phase.

Child, Preschool↗

Neonatal cleft lip and cleft palate repair.

Facial clefting is one of the most common birth defects and having a child born with this defect can be a devastating experience for parents and family members. Until recently, repair of cleft lip and palate deformities was deferred until the infant had reached 10 weeks of age, 10 pounds in weight, and a hemoglobin level of 10 gm per 100 mL. This criteria eliminated neonates 28 days of age or younger from the possibility of early repair. Waiting until these criteria were achieved often caused problems with parent-infant bonding and infant growth and development. Advances in neonatology and pediatric anesthesia now have made it possible to perform cleft surgery during the neonatal period. This article discusses the anatomy and embryology of cleft deformities and how cleft repair surgery now can be performed on the neonate.

Aftercare↗

Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate.

Otitis media with effusion (OME) is common among children with cleft palate, and may lead to such long-term consequences as hearing loss, tympanic membrane retraction, and chronic otitis media (COM). In total, 104 children with cleft lip and/or palate treated for OME at our institution were reviewed. Mean duration of follow-up was 6.9 years, and mean age at latest follow-up was 9.6 years. The incidence of COM was 19%, and the incidence of cholesteatoma was 1.9%. Ears showing such long-term sequelae of OME as hearing loss, tympanic membrane retraction, and chronic otitis media, were noted to have undergone a significantly greater number of ventilation tube insertions than ears not showing these sequalae. Our findings would suggest that a conservative approach to the management of OME in children with cleft palate is more likely to be beneficial in the long term.

Child↗

B group vitamins and cleft lip and cleft palate.

B group vitamins including folic acid supplementation during pregnancy have been shown to be effective in preventing cleft lip and palate (CLP) in humans. The clinical trials for the prevention of malformation have been mostly empirically based. The aim of the present study was directed toward the elucidation of the mechanisms underlying the preventive measures. The teratogenic potency of vitamin deficiency over the whole period of gestation (days 1-18) and of food restriction during the critical period of palatogenesis (days 12 and 13) were investigated in the genetically different strains of NMRI and A/WySn mice. Furthermore the potential benefit of vitamin B supplementation/treatment in the genetically susceptible CLP strain was demonstrated for comparison with former work on a teratogenetically induced cleft palate model. The results illustrate the higher susceptibility of the NMRI strain to the teratogenic action of deficiency (increase of the CP rate from 3.8% to 25%) in contrast to A/WySn mice, which actually have a high spontaneous but relative teratogenic-resistant clefting rate (28-44%). A deficiency of each of the individual B vitamins is teratogenic, however total B group deficiency has the strongest effect in the case of deficiency of all B vitamins. This produces up to 25% cleft palates in the NMRI strain. Alternatively, vitamin B group treatment in pregnant A/WySn mice did not substantially influence the clefting rate in contrast to our former experience in Halle:AB mice. The results may help to elucidate the interplay of genetic conditions and exogenous (nutritional) factors in both the aetiology and prevention of CLP. This may further clarify the role of the B vitamins in empiric preventive clinical trials.

Animals↗

[Interactions between oral surgery and orthodontics in the treatment of children with cleft lip and cleft palate].

The oral-surgical and the orthodontic treatment of patients with cleft lip and palate are interdependent. Consequently, they cannot be performed separately. In most cases, a continuous coordinated treatment planning by both specialties, from the first day of life, will permit to obtain optimal treatment results. The ultimate aim of co-ordinated treatment is not only the aesthetic and functional rehabilitation, but also the securing of the age-related growth of the midface.

Child, Preschool↗

[Cleft lip and cleft palate birth rate in Bavaria before and after the Chernobyl nuclear power plant accident].

BACKGROUND: Cleft lip and palates (CLP) occur with a frequency of between 1 and 2 cases in 1000 live births and thus belong to the most frequent congenital anomalies. In the former German Democratic Republic (GDR), records covering 1967-1989 for CLP newborns show a 9.4% increase of the prevalence of CLP from 1987 to 1989, possibly due to Chernobyl. DATA AND STATISTICAL METHOD: In Bavaria, all congenital malformations in children's hospitals have been recorded from 1984 to 1991. Among these data, 1324 cases with CLP were found. A spatial-temporal analysis aimed at uncovering a possible association of the CLP occurrence with the Chernobyl fallout on a district level, as well as a synoptic analysis of the GDR and Bavarian data, were carried out. RESULTS: In Bavaria, from October 1986 to December 1990, the CLP frequency increased by 9.5% (p=0.10) relative to the trend as computed from the remaining years. The association of CLP rates with fallout on a district level is reflected by a significant relative risk (RR) per kBq/m(2) of RR=1.008 (p=0.03). A synoptic analysis of the Bavarian data and the GDR data restricted to the overlapping time window from 1984 to 1989 discloses a simultaneous significant jump of the CLP prevalence by 8.6% (p=0.02) after 1986. CONCLUSION: The presumption of a long-term increase of CLP after exposure to Chernobyl fallout is corroborated by the analysis of the Bavarian congenital malformation data.

Abnormalities, Radiation-Induced↗