Cleft Lip – A Comprehensive Review

Shkoukani, Mahdi A.; Chen, Michael; Vong, Angela

doi:10.3389/fped.2013.00053

Cited by 241 publications

(166 citation statements)

References 112 publications

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“…While cleft lip and palate may be visibly morphologically apparent immediately at birth [13], others, such as osteogenesis imperfecta (OI) or brittle bone disease, presents a variety of symptoms from loose joints to weak bones, which cause dysmorphism and premature death later in life [14]. Some rarer diseases, such as fetal skeletal dysplasia, exhibit stunted bone growth, poor mineralization, and limb agenesis [15].…”

Section: Faulty Osteogenesis As the Root Cause Of Skeletal Defectsmentioning

confidence: 99%

microRNA Regulation of Skeletal Development

Sera

Nieden

2017

Curr Osteoporos Rep

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Purpose of review Osteogenesis is a complex process involving the specification of multiple progenitor cells and their maturation and differentiation into matrix-secreting osteoblasts. Osteogenesis occurs not only during embryogenesis, but also during growth, after an injury, and in normal homeostatic maintenance. While much is known about osteogenesis associated regulatory genes, the role of microRNAs, which are epigenetic regulators of protein expression, is just beginning to be explored. While miRNAs do not abrogate all protein expression, their purpose is to finely tune it, allowing for a timely and temporary protein down-regulation. Recent findings The last decade has unveiled a multitude of microRNAs that regulate key proteins within the osteogenic lineage, thus qualifying them as ‘osteomiRs’. These miRNAs may endogenously target an activator or inhibitor of differentiation, and depending on the target, may either lead to the prolongation of a progenitor maintenance state or to early differentiation. Interestingly, cellular identity seems intimately coupled to the expression of miRNAs, which participate in the suppression of previous and subsequent differentiation steps. In such cases where key osteogenic proteins were identified as direct targets of miRNAs in non-bone cell types, or through bioinformatic prediction, future research illuminating the activity of these miRNAs during osteogenesis will be extremely valuable. Summary Many bone related diseases involve the dysregulation of transcription factors or other proteins found within osteoblasts and their progenitors, and the dysregulation of miRNAs, which target such factors, may play a pivotal role in disease etiology, or even as a possible therapy.

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Section: Faulty Osteogenesis As the Root Cause Of Skeletal Defectsmentioning

confidence: 99%

microRNA Regulation of Skeletal Development

Sera

Nieden

2017

Curr Osteoporos Rep

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“…For the extrusion GS doped filaments, a temperature of 175 • C was used. We established in a previous study the stability of GS during the extrusion and printing processes [14,17]. …”

Section: Filament Extrusionmentioning

confidence: 99%

“…Cleft lip and palate (CLP) is the second most common congenital birth defect in the United States (U.S.), trailing only the incidence of Down syndrome [17][18][19]. It is estimated that nearly 7000 infants are born with orofacial clefts in the U.S. annually [17].…”

Section: Introductionmentioning

confidence: 99%

“…It is estimated that nearly 7000 infants are born with orofacial clefts in the U.S. annually [17]. During early facial development, body tissues and special cells (neural crest cells) from each side of the head grow toward the center of the face and unite together to form our facial features, lips, nose, and mouth, etc.…”

Section: Introductionmentioning

confidence: 99%

“…During early facial development, body tissues and special cells (neural crest cells) from each side of the head grow toward the center of the face and unite together to form our facial features, lips, nose, and mouth, etc. [17][18][19][20]. Congenital defects result when the baby's lip or mouth do not form properly during pregnancy.…”

Section: Introductionmentioning

confidence: 99%

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The Use of 3D Printing in the Fabrication of Nasal Stents

et al. 2017

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Nasoalveolar molding of the cleft lip, nose, and alveolar palate has been a successful strategy for the restoration of oronasal function and appearance, but it has some drawbacks. The temporary implant that is inserted before surgical reconstruction is a large appliance requiring numerous adjustments, it can irritate delicate soft tissues, and interfere with the infant's ability to nurse or feed. In the early post-operative period and for months after cleft lip repair, patients wear standardized silicone stents that come in multiple sizes, but require significant sculpting to fit the unique cleft deformity. Three-dimensional (3D) printing offers the potential of highly personalized and patient-specific treatment. We developed a method that produces a customized 3D printed stent that matches the contours and unique features of each patient and permits modification and adjustments in size and shape as the patient ages. With 3D scanning technology, the device can be designed at the first visit to create an appliance that can be worn sequentially with minimal trauma, does not impede feeding, and a prosthesis that will improve compliance. The device will be worn intraorally to help shape the alveolus, lip, and nose before surgical repair. Furthermore, the stent can be doped with drugs as each patient's case warrants.

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How Should We Decide Whether and When Some Care Is Better Than No Care?

Koch

Meara

Wall

2019

AMA Journal of Ethics

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This case of cleft lip and palate repair by a surgical mission team is common. Low-risk, single-procedure surgical interventions requiring minimal follow-up with substantial quality of life improvement are well suited for this type of mission. However, cleft repair can also be quite complex and require multiple surgeries and other care over time, postoperative surveillance, and speech therapy. These benefits and burdens prompt us to investigate, from clinical and ethical perspectives, whether and when some surgical care is better than none. We argue that, when performing surgical interventions in low-and middle-income countries, mission teams should consider a systems-based approach that emphasizes collaboration, context, and sustainability. Case JJ was born with a cleft lip and palate. As a result, he has developed problems with oral competence, feeding, speech, and social acceptance-due to his appearance. He is 4 years old and lives in a rural community in Central Africa. JJ's family heard that a group of surgeons from a university in the United States were coming to a nearby town to offer free cleft lip and palate repairs. JJ and his family and siblings traveled an hour to seek care, and JJ's family waited all day to finally see the surgeon and resident physicians who repaired JJ's cleft.

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Cleft Lip – A Comprehensive Review

Cited by 241 publications

References 112 publications

microRNA Regulation of Skeletal Development

microRNA Regulation of Skeletal Development

The Use of 3D Printing in the Fabrication of Nasal Stents

How Should We Decide Whether and When Some Care Is Better Than No Care?

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