Wednesday, December 16, 2015

Osteoporosis: An increasing Concern in Pediatric Dentistry

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name:             Leslie M Slowikowski                                                         Date: 12/16/2015
Article Title: Osteoporosis: An increasing Concern in Pediatric Dentistry
Author(s): Marcio A. da Fonseca, DDS, MS 
Journal: Pediatric Dentistry
Date:  May/June 2011
Major Topic: Osteoporosis in pediatric population
Type of Article: Review of literature
Main Purpose: Overview of osteoporosis in children: testing, treatment and dental impacts
Key Points/Summary:  Over the past 3 decades there has been increase in bone fractures and are a leading cause for a 10-14y/o to be hospitalized. 

Osteoporosis is classically defined as a systemic skeletal disease characterized by low bone mass; alteration of ultrastructural quality of bone; deterioration in trabecular architecture increased cortical porosity reduced cortical thickness and decrease bone with putting the individual at risk for fractures. 

Important because a growing number of children are being diagnosed with low bone mineral density (BMD) and osteoporosis are being treated with drugs that may cause adverse effects in the oral cavity.  Pediatric dentists are in an important position to counsel patients about healthy lifestyles habits that can help prevent the condition from an early age.

By the end of adolescence 90% of peak bone mass has been acquired.  Poor bone mass in adolescences leads to an increased risk of osteoporosis and fractures in adulthood.

Diagnosis is difficult there is no great test due to lack of research in the pediatric population.  Usually defined by clinical history of long bone fractures and vertebral compression.

DEXA is used in adults to measure bone mineral density but is not as accurate in children. 

Treating osteoporosis in children and adolescents:

Anticipatory guidance regarding health lifestyles habits, such as regular physical activity, a balanced diet, and avoidance of tobacco, alcohol, and illicit drugs, are of great importance to prevent bone loss and should from an early age.  Dentist can make an impact on the patient’s health and, thus, should be addressed in least at every recall visit. 

Soft drink consumption negatively influences bond mineral accrual in adolescent girls more than in boys, while eating fruits and vegetables is positively associated with BMD in girls.

Beware of bisphosphonates treatment, IV is more concerning then oral and be aware of possible BRONJ. 

Evidence class: III

An Overview of Chronic Oral Graft vs Host Disease Following Pediatric Hematopoietic Stem Cell Transplantation

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: John Kiang                                                                                          Date: 12/16/15
Article Title: An Overview of Chronic Oral Graft vs Host Disease Following Pediatric Hematopoietic Stem Cell Transplantation
Author(s): Marcia da Fonseca and Hong
Journal: Pediatric Dentistry
Date: Mar/Apr 2008
Major Topic: GVHD
Type of Article:  Literature Review
Main Purpose:  Discuss chronic GVHD in children
Key Points/Summary:

·       Hematopoetic stem cell transplantation (HSCT) is the treatment of choice for many hematologic, genetic, oncologic, and immunological diseases.
·      It is also one of the most aggressive treatments among the different cancer therapies, and is associated with severe conditioning-related toxicity, which include profound neutropenia, a high level of prophylatic immunosuppression and graft vs host disease (GVHD).
·      GVHD remains a significant compmlication of HSCT and is a frequent reason for nonrelapse morbidity and mortality following allogenic HSCT
·      It primarily targets the skin, GI tract, and liver
·      Approximately 20% of patients who receive matched sibling transplants and 40% of matched unrelated donor recipients will develop chronic GVHD.
·      Risk factors include: 1. Hx of acute GVHD 2. Hematologic malignancy 3. Female donor to male recipient 4. Use of total body irradiation 5. Donor age of >5 years 6 recipients > 10 years of age
·      Dental Care: patient education, good OH, prevention of oral diseases
·      Risk factors: hyposalivation, loss of the protective properties of saliva, perioral fibrosis limiting mouth opening and oral pain that prevent optimal hygiene, high caloric diet due to weight loss, decreased mobility of the gonue making oral clearance difficult and frequent consumption of soft foods,
·      Fluoride supplements should be prescribed based on individual needs (products containing xylitol gums)
·      Should continue with 3 to 6 month routine care based on caries risk but do not resume routine polishing and scaling until adequate immunological reconstitution.
·      Always consult with physician if immediate dental care is needed (possible GA, abx, steroid supplementation, platelet transfusions)
·      Always know patients platelet count and absolutely neutrophil count for invasive dental procedures.

Dental care in the pediatric cancer patient

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Mark Dawoud, DMD                                                         Date: 12/16/15
Article Title: Dental care in the pediatric cancer patient.
Authors: da Fonseca, M.A.
Journal: Pediatr Dent
Date: 2004
Major Topic: Pediatric cancer patient
Type of Article: recommendations
Main Purpose: Recommendations for the dental care of the pediatric oncology patient.

·       Declines in mortality for childhood cancer are due to early diagnosis and improvements in therapy.
·       Incidence of childhood cancer is the greatest in the first year of life with a second peak at 2 to 3 years of age followed by a decline until age 9 and then steadily increasing through adolescence.
·       Boys are affected more than girls except in the first year of life. White children show a 30% higher frequency than blacks particularly during the first 5 years of life.
·       Acute lymphoid leukemia (ALL) is the most common malignancy, followed by CNS tumors then sarcomas.
·       The most common head, neck, and intraoral manifestations of ALL at the time of diagnosis are lymphadenopathy, sore throat, laryngeal pain, gingival bleeding, and oral ulceration. This is due to leukemic infiltrates.
·       Oral and dental infections may complicate the oncology treatment as well as delay it, leading to morbidity and an inferior quality of life for the child. Early and radical dental intervention reduces the frequency of problems, minimizing the risk for oral and associated systemic complications.
·       A good medical history is recommended. Most patients have a central line, which may dictate the use of antibiotics against endocarditis.
·       Myelosuppression may cause prolonged bleeding due to certain medications. Significant bleeding in unlikely to occur with a level >20,000/mm3.
·       ANC <1,000/mm3 – dental work should be deferred.
·       If spontaneous gingival bleeding is present, the physician must be notified because it may be a sign of internal hemorrhage.
·        Some patients may complain of paresthesias due to leukemic infiltration of the peripheral nerves or dental pain mimicking irreversible pulpitis. This is a side effect of vincristine and vinblastine, common chemotherapeutic agents.
·       The patient's blood counts normally start falling 5 to 7 days after the beginning of each treatment cycle, staying low for approximately 14 days before rising again.
·       Overall, routine dental care can be done when the ANC is >l,OOO/mm and platelet count is >50,000mm3.  Some recommend that endocarditis prophylaxis be prescribed when ANC is 1,000 and 2,000/mm3 and optional platelet transfusions be considered pre and 24 hours postoperatively when the level is between 40,000 an 75,000/mm3. During immunosuppression all elective dental treatment should be avoided.
·       For patients who need a platelet transfusion before dental treatment, it is important to note that the peak concentration of platelets is achieved 45 to 60 minutes following transfusion.
·        Orthodontic treatment may start of resume after completion of all therapy and after at least every 2 year disease-free survival. A panoramic radiograph should be obtained every 12 to 18 months to monitor the dental changes.

Internal Validity: III;

Congenital Genetic Disorders and Syndromes

Resident’s Name: Nicholas Paquin                                                                           Date: 12/16/2015

Article Title: Congenital genetic disorders and syndromes
Author(s): Rebecca L. Slayton
Journal: Casamassimo Text book
Major Topic: Genetics
Type of Article: Textbook
Main Purpose: Genetics
Key Points/Summary:
- Practicing dentists are confronted daily with conditions that are either primarily genetic or have a significant genetic contribution in their etiology. Common conditions such as congenitally missing teeth now are known in many cases to be caused by specific genetic mutations. Many syndromes involve craniofacial structures and have associated dental anomalies. Frequently, other major malformations are present in addition to the craniofacial anomalies.
-Traditionally, genetic diseases have been thought of in terms of Mendelian inheritance patterns. However, as we have learned more about genetics, we know there are other types of inheritance patterns that include imprinting, DNA triplet repeat expansion, mitochondrial DNA defects, and complex disorders in which multiple genes may be involved and in which sequence changes increase or decrease a person's susceptibility to disease.

-Autosomal Dominant - Dentinogenesis imperfecta is an example of an autosomal dominant disorder. The gene for type I dentinogenesis imperfecta has been identified (dentin sialophosphoprotein) and is located on chromosome 4. Other autosomal dominant disorders include achondroplasia (short-limbed dwarfism), some forms of amelogenesis imperfecta, and Marfan syndrome.

-Autosomal Recessive - Most frequently, each parent has one copy of the defective gene and is a carrier, and there is a 25% chance that both mutant genes will be passed on to their offspring, no gender predilection. Although autosomal recessive disorders are relatively uncommon, the carrier status in certain populations can be significant. For example, 1 in 25 people of northern European descent are carriers of cystic fibrosis.

- X-linked - Mutations in genes located on the X chromosome result in X-linked genetic disorders. Females have two X chromosomes and one is randomly inactivated in each cell, they are carriers and do not normally manifest the disorder. Males, on the other hand, only have one X chromosome, which is inherited from their mother. A son has a 50% chance of inheriting the defective gene from his mother and manifesting the disease. A daughter also has a 50% chance of inheriting the defective gene from her mother but will then be a carrier. X-linked disorders often appear to skip a generation because an affected male will only pass the affected X chromosome to a daughter and she will serve as a carrier to the next generation. Disorders with X-linked inheritance include factor VIII deficiency (hemophilia), X-linked hypohydrotic ectodermal dysplasia, fragile X syndrome, and X-linked amelogenesis imperfecta. Occasionally, as a result of nonrandom X inactivation, females may have mild symptoms of an X-linked disorder.

-Non-Traditional Inheritance - Other types of inheritance patterns that do not fit the traditional Mendelian patterns and that have been identified fairly recently are imprinting and triplet repeat expansion. Imprinted genes are turned off by methylation of the gene. This process controls the level of expression of a particular gene in the offspring. Depending on whether the imprinted gene is inherited from the mother or father determines if the child has a particular disease. In some cases, if the imprinted gene is inherited from the father, the child has one disease, but if the same imprinted gene is inherited from the mother, he or she has a different disease.
DNA triplet repeat expansion is a phenomenon where strings of repeated nucleotides increase in number. For example, within a particular gene, there may be 200 copies of the trinucleotide repeat “TAG.” Smaller numbers of repeats are often referred to as a premutation, but when the repeats are expanded in an offspring, they may cause the gene to be inactivated (often by methylation). Diseases caused by this type of defect include Huntington chorea and fragile X syndrome.
Epigenetic mechanisms affect the expression of genes and can be caused by environmental chemicals, developmental processes, drugs, or aging. These changes are not the result of DNA sequence alterations but rather are caused by factors such as DNA methylation and histone acetylation. Although the term epigenetics was coined in 1942, its relevance to inheritance of disease susceptibility has attracted substantial attention in recent years.

-Features such as the spacing between the eyes, the position and shape of the ears, and the relative proportions of the maxilla and mandible either are within the range of normal or vary enough to be considered dysmorphic. Many genetic syndromes result in dysmorphic facial features that frequently help to diagnose the syndrome. For example, children with Down syndrome have inner epicanthal folds, up-slanting palpebral fissures, and maxillary hypoplasia. This causes unrelated children with Down syndrome to have a similar appearance to each other.
In general, the children seen in a dental practice fit into one of three categories. They may be normally developed in every way; they may have been diagnosed with a developmental anomaly of some type (either physical or mental); or they may have a developmental anomaly that has not been diagnosed. As pediatric dentists, we see our patient frequently, not just when they are ill, this gives the dentist the opportunity to observe a child's growth and development and to note changes that are not within the range of normal. As health care professionals, it is incumbent on all dentists to recognize disease in their patients and to make the appropriate referral for definitive diagnosis and treatment. According to the text Smith's Recognizable Patterns of Human Malformation, 12 of the 26 categories of malformations used for diagnostic purposes involve features of the head or neck.8 Several are limited to oral structures, such as hypodontia, microdontia, micrognathia, and cleft lip/palate. In addition, having an understanding of the full spectrum of malformations associated with certain syndromes is essential for the safe and effective treatment of patients with these disorders.

Dental Anomalies - Anomalies of tooth development are relatively common and may occur as an isolated finding or in association with other minor and major anomalies. Hypodontia is the developmental absence of one or more primary or permanent teeth. Although there are a number of syndromes in which hypodontia is a feature, the occurrence of one or more missing teeth (other than third molars) is estimated to be 6%. Anomalies of teeth follow patterns that reflect the time in development when the malformation occurs. For example, disruptions in tooth initiation result in hypodontia or supernumerary teeth, whereas disruptions during morphodifferentiation lead to anomalies of size and shape such as macrodontia, microdontia, taurodontism, and dens invaginatus. Disruptions that occur during histodifferentiation, apposition, and mineralization result in dentinogenesis imperfecta, amelogenesis imperfecta, dentin dysplasia, and enamel hypoplasia.
When discussing potential developmental anomalies with parents, it is important to be very sensitive to their concerns and to avoid causing undue alarm. Depending on the circumstances, it may be best to observe the child at multiple appointments (assuming there are treatment needs) and to get to know the family better before broaching the subject. On the other hand, when there are clear or obvious concerns and a timely diagnosis is indicated, an immediate referral should be made. One disorder that a dentist is likely to be the first to diagnose is ectodermal dysplasia. Frequently, parents become concerned when their 2- or 3-year-old child does not have any visible teeth or has conically shaped teeth. This should be recognizable to a dentist who sees children as a developmental anomaly that should be further investigated. At this age, children frequently also have sparse hair, but the combination of hypodontia, sparse hair, and dry skin should cause a dentist to refer this child to determine if he or she has a form of ectodermal dysplasia. Similarly, if a child who is 10 or 12 years old has not lost any primary teeth, a dentist should investigate further to determine the reason the teeth have not exfoliated. In children with cleidocranial dysplasia, multiple supernumerary teeth are present that block the eruption of the permanent teeth; but there is also a genetic defect that keeps teeth from erupting even after the supernumerary teeth are removed.

Take home: We see our patients frequently, it is important to be able to identify certain clinical presentations of genetic diseases to identify, refer to the appropriate specialists and know how to treat the patients appropriately according to their conditions.

The Role of Pediatric Dentistry in Multidisciplinary Cleft Palate Teams.

Resident: Semantha Charles.        Date: 12/16/2015
Title: The Role of Pediatric Dentistry in Multidisciplinary Cleft Palate Teams.
Book: Dentistry For The Child And Adolescence
Author: David R. Avery
Main Purpose: the role of pediatric dentistry in multidisciplinary cleft palate teams.

Key Points:
Cleft lip and palate (CLP) is a congenital craniofacial anomaly produced by embryological defects during formation of the face. It is considered the most frequent developmental anomaly of the face (prevalence is 1 in 500 to 1:2,000, depending on the population affected).

-Cleft Palate Foundation recognizes that following disciplines should be represented on a cleft palate team: auidiology, surgery, pediatric dentistry, orthodontics, genetics, nursing, otolaryngology, pediatrics, psychology, speech-language pathology, and other specialists as needed.
-CLCP patients have high prevalence/severity of caries and malocclusion.

Aims of cleft treatment:

The ultimate goal is to attain normal form and function (especially speech and mastication) with the least possible damage to growth and development through surgical intervention.

Specific treatment objectives are:

Provide a long mobile palate capable of completely closing off the oropharynx from the nasopharynx.
Produce a full upper lip with a symmetrical cupid’s bow and reconstruction of the columella and the alar architecture of the nose.
Achieve an intact, well-aligned dental arch with a stable inter-arch occlusion.
Provide a pleasing facial appearance.

Multidisciplinary Team Approach to Cleft Lip and Palate Management

Department of Pediatric Dentistry

Lutheran Medical Center
Resident’s Name: John Diune                                                                        Date: 12/16/2015

Article Title: Multidisciplinary Team Approach to Cleft Lip and Palate Management
Author(s): LaQuia A. Walker Vinson, Donald V. Huebener, James E. Jones, Roberto L. Flores, Jeffery A Dean
Journal: Dentistry for the Child and Adolescent 10e
Date: 2016
Major Topic: Cleft Lip and Palate
Type of Article: Textbook chapter
Main Purpose: Discuss cleft lip and palate management
Key Points/Summary:
·         Presurgical Orthopedics
o   If lip surgery performed with premaxilla  in an abnormal position there is higher chance of lip dehiscence
o   Methods to reposition maxilla:
§  Lip taping across upper lip
§  DynaCleft   - premade topical approximation device
§  Latham appliance – dentomaxillary advancement appliance used to approximate segments actively
§  Nasoalveolar molding (NAM) appliance with a nasal extension to shape the nasal cartilages
·         Approximate alveolar segments, decrease nasal base width, elongate the columella, attempt to achieve symmetric nasal cartilages
·         Uses acrylic base adjusted weekly and using tapes with elastics and lip taping across upper lip
·         Cheiloplasty – cleft lip repair
o   Performed at approximately 3 months of age
o   Primary nasal reconstruction often also performed at this time
·         Maxillary orthopedics
o   After lip closure maxillary arch collapse may occur
o   Obturator can be used to provide cross-arch stability and support
·         Bone grafting of Alveolar Cleft Defects
o   Primary alveolar cleft bone grafting
§  Bone-grafting procedure involving alveolar clefts in children younger than 2 yo
§  Controversial
§  Does not preclude secondary alveolar cleft bone graft surgery
o   Gingivoperiosteoplasty (GPP)
§  Uses osteogenic properties of infants rather than grafts to bridge bony cleft
§  Mucoperiosteal flaps raised at time of lip repair and sealed over alveolar cleft without using primary bone graft
§  Requires presurgical orthopedics and closure of nasal floor
·         Palatoplasty
o   Closure of palate by 1 year of age primarily for normal speech development
o   Improves hearing and swallowing
o   Dedicated speech therapy critical
Stage II (primary dentition stage: 18 months to 5 years of age)
·         Establishing and maintaining oral health
·         Ectopic eruption of primary maxillary  anterior dentition common around cleft defect
·         3-4 month intervals for recall
Stage III (late primary or early mixed dentition stage: 6 – 10 or 11 years of age)
·         Correction of traumatic occlusion from ectopic erupting permanent and lateral incisors or crossbites
·         Secondary alveolar cleft bone graft
o   Provides bone support for teeth through eruption or orthodontic movement
o   Closure of oronasal fistula
o   Supports alar base of nose
o   Soft tissue associated with the cleft area are incised and elevated to allow labial and palatal closure
o   Bone harvested from iliac crest is used to fill into cleft defect
o   Success around 90%
o   Total rehabilitation that improves:
§  Speech
§  Dentition
§  Aesthetics
§  Psychosocial benefits
·         Facial aesthetics important  for individual and society interactions and acceptance
Stage IV (permanent dentition stage: 12-18 years of age)
·         Orthodontic-surgical approach may be needed – deferred until after maximum growth of maxilla and mandible attained
·         Cosmetic surgery
o   Nasal ridge and tip
o   Refinement of upper lip
Assessment of Article:  Level of Evidence/Comments: