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Etiology and pathogenesis of congenital cleft lip and cleft palate, an NIDR state of the art report.Teratology Dec 1972
Review
Topics: Abnormalities, Drug-Induced; Animals; Antineoplastic Agents; Cleft Lip; Cleft Palate; Cortisone; Disease Models, Animal; Female; Genotype; Humans; Male; Maternal-Fetal Exchange; National Institutes of Health (U.S.); Pregnancy; Research; Syndrome; Triamcinolone; United States
PubMed: 4566159
DOI: 10.1002/tera.1420060302 -
Progress in Orthodontics Mar 2019Dental caries is a polymicrobial disease and prevalent among cleft lip and palate (CLP) patients, although their oral hygiene is well maintained. Dysbiosis, the state of...
BACKGROUND
Dental caries is a polymicrobial disease and prevalent among cleft lip and palate (CLP) patients, although their oral hygiene is well maintained. Dysbiosis, the state of imbalance within the dental plaque microbiota, may cause caries prevalence among these patients. However, little is known about how dysbiosis occurs and affects cariogenicity. To find dysbiotic signs, here we conducted a metatranscriptomic analysis for the plaque microbiota in six CLP patients and four controls.
METHODS
Total bacterial RNA was extracted from each sample and sequenced. Bacterial composition and functional profiles were estimated from 16S rRNA and mRNA reads, respectively. The mRNA reads were further used for estimating bacterial composition. Species listed in both rRNA-based and mRNA-based bacterial composition were identified as viable taxa with in situ function (VTiF), and the VTiF with a high mRNA-to-rRNA ratio were considered to be transcriptionally active. A network was constructed for each group by connecting two VTiF if their mRNA abundances were positively correlated.
RESULTS
The bacterial composition and functional profiles themselves did not provide remarkable signs of dysbiosis in the CLP group. However, the group-specific active taxa were identified, including streptococcal and Prevotella species in the CLP group. Moreover, the network structure was different between groups; Actinomyces johnsonii and several species in the CLP group were the active taxa, which were connected based on positive correlations with statistical significance.
CONCLUSIONS
Functional dysbiosis within the plaque microbiota was observed such as difference of the network structure between groups, and may be associated with cariogenicity. The observed functional dysbiosis was an invisible change within the microbiota in the oral cavity of CLP patients. This may emphasize the importance of maintaining good oral hygiene of the patients with cleft anomalies.
Topics: Cleft Lip; Cleft Palate; Dental Caries; Dental Plaque; Dysbiosis; Humans; Microbiota; RNA, Ribosomal, 16S
PubMed: 30906959
DOI: 10.1186/s40510-019-0265-1 -
A pragmatic approach for implementation of value-based healthcare in Amsterdam UMC, the Netherlands.BMC Health Services Research Apr 2022The emphasis on implementation of value-based healthcare (VBHC) has increased in the Dutch healthcare system. Yet, the translation of the theoretical principles of VBHC...
BACKGROUND
The emphasis on implementation of value-based healthcare (VBHC) has increased in the Dutch healthcare system. Yet, the translation of the theoretical principles of VBHC towards actual implementation in daily practice has been rarely described. Our aim is to present a pragmatic step-by-step approach for VBHC implementation, developed and applied in Amsterdam UMC, to share our key elements. The approach may inspire others and can be used as a template for implementing VBHC principles in other hospitals.
METHODS
The local approach is developed in a major academic hospital in the Netherlands, based at two locations with 15,000 employees in total. Experience-based co-design is used, building on our learning experiences from implementing VBHC for 14 specific patient groups. The described steps and activities devolved from iterative and participative co-design sessions with various experienced stakeholders involved in the implementation of one or more VBHC pathways.
RESULTS
The approach includes five phases; preparation, design (team introduction, outcome selection, action agenda), building (outcome set integration in daily practice), implementation (training, outcome registration and implementation) and the continuous improvement cycle. We described two cases for illustration of the approach; the Cleft Lip and Palate and the Chronic Kidney Disease patient groups. For a good start, involvement of a clinical leader as driving force, ensuring participation of patient representatives and sufficient resources are needed.
CONCLUSION
We have experienced that several defining features of the development and implementation of this approach may have contributed to its completeness and applicability. Key elements for success have been organisational readiness and clinical leadership. In conclusion, the approach has provided a first step towards VBHC in our hospital. Further research is needed for evaluation of its effectiveness including impact on value for patients.
Topics: Cleft Lip; Cleft Palate; Delivery of Health Care; Health Facilities; Humans; Netherlands
PubMed: 35468765
DOI: 10.1186/s12913-022-07919-1 -
BMJ Case Reports Apr 2019Crohn's disease (CD) is a multifactorial, chronic immune-mediated disorder. The oral cavity is involved in 0.5% to 20% of the patients with CD. Oral manifestations of CD...
Crohn's disease (CD) is a multifactorial, chronic immune-mediated disorder. The oral cavity is involved in 0.5% to 20% of the patients with CD. Oral manifestations of CD are sometimes nonspecific and can be overlooked by the clinicians. These manifestations may precede intestinal symptoms and can serve as indicators for early diagnosis. To increase awareness and to contribute to the standard intervention, here we report a paediatric case with persistent idiopathic swelling of the lower lip and perianal fistula. Microscopic examinations revealed multiple non-necrotising granulomas with chronic inflammation, oedema and lymphangiectasia. The patient was treated with metronidazole 500 mg and ciprofloxacin 500 mg twice a day for one month. The perioral lesions were managed with topical 0.03% tacrolimus and oral prednisone 10 mgtwice a day for one month, followed by a tapered regimen of 10 mg/day for another month. The patient's symptoms improved without full remission at the 6-month follow-up.
Topics: Child; Crohn Disease; Humans; Lip Diseases; Male; Rectal Fistula
PubMed: 31005876
DOI: 10.1136/bcr-2019-229916 -
BMC Veterinary Research Oct 2018Cleft palate is a birth defect characterized by a lack of fusion between structures forming the palate. Causes include a multitude of factors, both genetic and...
BACKGROUND
Cleft palate is a birth defect characterized by a lack of fusion between structures forming the palate. Causes include a multitude of factors, both genetic and environmental. Computed tomography (CT) is widely used to evaluate morphological features and diagnose head disorders in adult dogs. However, there is less data about its use in neonatal dogs. The purpose of this study was to perform CT evaluation of palatal defects in one-day-old puppies and to present a novel approach of 3D modeling in terms of cleft palate assessment.
RESULTS
Macroscopic and CT examinations were performed in 23 stillborn or euthanized purebred newborn puppies. On the basis of CT data, a 3D model was prepared and the cleft surface area was then calculated. A multi-stage approach, which utilised software such as 3D Slicer and Blender, was applied. Palatal defects were found in ten dogs, of which five had cleft palate, three had bilateral cleft lip and palate, one had a unilateral cleft lip and palate and one had a unilateral cleft lip. The surface area of the clefts ranged from 31 to 213 mm, which made up respectfully 11 to 63% of the total surface area of the palate. No abnormalities were found in thirteen dogs and they made up the control group.
CONCLUSIONS
Computed tomography and 3D modeling were very effective in evaluation of palatal disorders in newborn dogs. 3D models adapted to the natural curvature of the palate were created and more precise data was obtained. Morphological characteristics, CT findings and advanced image analysis of cleft palate in neonates obtained from these models increase the knowledge about this malformation in dogs.
Topics: Animals; Animals, Newborn; Case-Control Studies; Cleft Lip; Cleft Palate; Dog Diseases; Dogs; Imaging, Three-Dimensional; Tomography, X-Ray Computed
PubMed: 30342508
DOI: 10.1186/s12917-018-1642-6 -
Reproductive Health Nov 2020Congenital anomalies are a significant cause of death and disability for infants, especially in low and middle-income countries (LMIC), where 95% of all deaths due to...
BACKGROUND
Congenital anomalies are a significant cause of death and disability for infants, especially in low and middle-income countries (LMIC), where 95% of all deaths due to anomalies occur. Limited data on the prevalence and survival of infants with congenital anomalies are available from Central America. Estimates have indicated that 53 of every 10,000 live births in Guatemala are associated with a congenital anomaly. We aim to report on the incidence and survival of infants with congenital anomalies from a population-based registry and classify the anomalies according to the International Classification of Disease, Tenth Revision (ICD-10).
METHODS
We conducted a planned secondary analysis of data from the Maternal Newborn Health Registry (MNHR), a prospective, population-based study carried out by the Global Network for Women's and Children's Health Research in seven research sites. We included all deliveries between 2014 and 2018 in urban and rural settings in Chimaltenango, in the Western Highlands of Guatemala. These cases of clinically evident anomalies were reported by field staff and reviewed by medically trained staff, who classified them according to ICD - 10 categories. The incidence of congenital anomalies and associated stillbirth, neonatal mortality, and survival rates were determined for up to 42 days.
RESULTS
Out of 60,142 births, 384 infants were found to have a clinically evident congenital anomaly (63.8 per 10,000 births). The most common were anomalies of the nervous system (28.8 per 10,000), malformations and deformations of the musculoskeletal system (10.8 per 10,000), and cleft lip and palate (10.0 per 10,000). Infants born with nervous system anomalies had the highest stillbirth and neonatal mortality rates (14.6 and 9.0 per 10,000, respectively).
CONCLUSIONS
This is the first population-based report on congenital anomalies in Guatemala. The rates we found of overall anomalies are higher than previously reported estimates. These data will be useful to increase the focus on congenital anomalies and hopefully increase the use of interventions of proven benefit.
TRIAL REGISTRATION
ClinalTrial.gov ID: NCT01073475 .
Topics: Child; Cleft Lip; Cleft Palate; Congenital Abnormalities; Female; Guatemala; Humans; Infant; Infant, Newborn; International Classification of Diseases; Pregnancy; Prevalence; Prospective Studies
PubMed: 33256772
DOI: 10.1186/s12978-020-01007-5 -
PloS One 2019Cleft lip (CL), cleft palate (CP) and cleft lip and palate (CLP) are the most common types of orofacial clefts in dogs. Orofacial clefts in dogs are clinically relevant...
Cleft lip (CL), cleft palate (CP) and cleft lip and palate (CLP) are the most common types of orofacial clefts in dogs. Orofacial clefts in dogs are clinically relevant because of the associated morbidity and high newborn mortality rate and are of interest as comparative models of disease. However, the incidence of CL, CP and CLP has not been investigated in purebred dogs, and the financial impact on breeders is unknown. The aims of this study were to document the incidence patterns of CL, CP and CLP in different breeds of dogs, determine whether defect phenotype is associated with skull type, genetic cluster and geographic location, and estimate the financial impact in breeding programs in the United States by means of an anonymous online survey. A total of 228 orofacial clefts were reported among 7,429 puppies whelped in the 12 preceding months. Breeds in the mastiff/terrier genetic cluster and brachycephalic breeds were predisposed to orofacial clefts. Certain breeds in the ancient genetic cluster were at increased odds of orofacial clefts. Male purebred dogs were at increased odds of CPs. Results confirm that brachycephalic breeds are overrepresented among cases of orofacial clefts. Furthermore, geographic region appeared to be a relevant risk factor and orofacial clefts represented a considerable financial loss to breeders. Improved understanding of the epidemiology of orofacial clefts (frequency, causes, predictors and risk factors) may help in identifying ways to minimize their occurrence. Information gained may potentially help veterinarians and researchers to diagnose, treat and prevent orofacial clefts.
Topics: Animals; Breeding; Cleft Lip; Cleft Palate; Dogs; Female; Incidence; Male; Surveys and Questionnaires; United States
PubMed: 31682628
DOI: 10.1371/journal.pone.0224574 -
Frontiers in Immunology 2022Orofacial clefts (OFC) are frequent congenital malformations characterized by insufficient separation of oral and nasal cavities and require presurgical infant...
Orofacial clefts (OFC) are frequent congenital malformations characterized by insufficient separation of oral and nasal cavities and require presurgical infant orthopedics and surgical interventions within the first year of life. Wound healing disorders and higher prevalence of gingivitis and plaque levels are well-known challenges in treatment of children with OFC. However, oral inflammatory mediators were not investigated after birth using non-invasive sampling methods so far. In order to investigate the impact of OFC on oral cytokine levels, we collected tongue smear samples from 15 neonates with OFC and 17 control neonates at two time points (T), T0 at first consultation after birth, and T1, 4 to 5 weeks later. The samples were analyzed using multiplex immunoassay. Overall, we found significantly increased cytokine levels (TNF, IL-1β/-2/-6/-8/-10) in tongue smear samples from neonates with OFC compared to controls, especially at T0. The increase was even more pronounced in neonates with a higher cleft severity. Further, we detected a significant positive correlation between cleft severity score and distinct pro-inflammatory mediators (GM-CSF, IL-1β, IL-6, IL-8) at T0. Further, we found that breast-milk (bottle) feeding was associated with lower levels of pro-inflammatory cytokines (IL-6/-8) in neonates with OFC compared to formula-fed neonates. Our study demonstrated that neonates with OFC, especially with high cleft severity, are characterized by markedly increased inflammatory mediators in tongue smear samples within the first weeks of life potentially presenting a risk for oral inflammatory diseases. Therefore, an inflammatory monitoring of neonates with (severe) OFC and the encouragement of mother to breast-milk (bottle) feed might be advisable after birth and/or prior to cleft surgery.
Topics: Female; Child; Infant; Infant, Newborn; Humans; Cytokines; Mouth Mucosa; Cleft Lip; Interleukin-6; Cleft Palate; Inflammation Mediators
PubMed: 36466891
DOI: 10.3389/fimmu.2022.1044249 -
Hua Xi Kou Qiang Yi Xue Za Zhi = Huaxi... Dec 2021Short-term outcome evaluation for the correction of unilateral cleft lip deformity with a new technique.
OBJECTIVES
Short-term outcome evaluation for the correction of unilateral cleft lip deformity with a new technique.
METHODS
Forty-four patients with unilateral cleft lip deformity were included in the study and in which the orbicularis oris muscle was reconstructed to achieve the optimal force balance of reconstructed orbicularis oris. The photometric two-dimensional indexes, including the philtrum oblique angle and asymmetry ratios (lip height, lip width, vertical distance from the white roll to the vermilion bottom at the Cupid's bow point, and vertical distance from the Cupid 's bow points to facial midline), were employed to measure and evaluate the outcome.
RESULTS
Several indexes showed statistically significant difference, and they included the philtrum oblique angle, asymmetry ratio of the lip height, and asymmetry ratio of the vertical distance from the white roll to the vermilion bottom at Cupid ' s bow points (<0.05).
CONCLUSIONS
The results suggested that the new muscle reconstruction technique can significantly improve the short-term outcome of the correction of unilateral cleft lip deformity.
Topics: Cleft Lip; Facial Muscles; Humans; Lip; Mouth Mucosa; Plastic Surgery Procedures
PubMed: 34859630
DOI: 10.7518/hxkq.2021.06.011 -
BMC Oral Health Dec 2022Patients with unilateral cleft lip and palate were associated with different nasomaxillary complex from the normal population. Although the biomechanical effects of...
The comparison of biomechanical effects of the conventional and bone-borne palatal expanders on late adolescence with unilateral cleft palate: a 3-dimensional finite element analysis.
BACKGROUND
Patients with unilateral cleft lip and palate were associated with different nasomaxillary complex from the normal population. Although the biomechanical effects of conventional rapid palatal expansion (Hyrax expansion) and bone-borne rapid palatal expansion (micro-implant-assisted expansion) in non-cleft patients have been identified by multiple studies, little is known in patients with unilateral cleft lip and palate. The purpose of this study was to investigate and compare the biomechanical effects of the conventional and bone-borne palatal expanders in a late adolescence with unilateral cleft lip and palate.
METHODS
A cone beam CT scan of a late adolescence with unilateral cleft lip and palate was selected to construct the three-dimensional finite element models of teeth and craniofacial structures. The models of conventional and born-borne palatal expanders were established to simulate the clinical maxillary expansion. The geometric nonlinear theory was applied to evaluate the Von Mises stress distribution and displacements in craniofacial structures and teeth.
RESULTS
Bone-borne palatal expander achieved more transverse movement than conventional palatal expander in the whole mount of craniofacial regions, and the maximum amount of expansion was occurred anteriorly along the alveolar ridge on cleft-side. The expanding force from born-borne palatal expander resulted in more advancement in nasomaxillary complex than it in conventional palatal expander, especially in the anterior area of the minor segment of maxilla. Stresses from the both expanders distributed in similar patterns, but larger magnitudes and ranges were generated using the bone-borne expander around the maxillary buttresses and pterygoid plates of sphenoid bone. The maximum expanding stresses from born-borne palatal expander were concentrated on palatal slope supporting minscrews, whereas those from conventional palatal expander were concentrated on the anchoring molars. In addition, the buccal tipping effect of teeth generated using the bone-borne expander was less than it using the conventional palatal expander.
CONCLUSION
Bone-borne expander generated enhanced skeletal expansion at the levels of alveolar and palate in transversal direction, where the miniscrews contributed increased expanding forces to maxillary buttresses and decreased forces to buccal alveolar. Bone-borne expanders presented a superiority in correcting the asymmetric maxilla without surgical assistant in late adolescence with unilateral cleft lip and palate.
Topics: Adolescent; Humans; Cleft Lip; Cleft Palate; Finite Element Analysis; Maxilla; Palatal Expansion Technique; Cone-Beam Computed Tomography
PubMed: 36514035
DOI: 10.1186/s12903-022-02640-1