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Neonatal Teeth

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181. DiGeorge Syndrome (Diagnosis)

include the following (see the images below) [ ] : Retrognathia or micrognathia Long face High and broad nasal bridge Narrow palpebral fissures Small teeth Asymmetrical crying face Downturned mouth Short philtrum Low-set, malformed ears Hypertelorism Dimple on the tip of the nose Congenital heart defects, a cleft palate or incompetence of the soft palate, and immune deficiencies are common. Patients may have short stature and occasional instances of growth hormone deficiency. Renal, pulmonary (...) and cardiovascular abnormalities in 22q11.2DS include the following: Radiography Magnetic resonance imaging (MRI) Computed tomography (CT) scanning Echocardiography Angiography and magnetic resonance angiography (MRA) See for more detail. Management Congenital heart defect If a heart murmur and or other signs of a heart defect are present, consult a cardiologist right away, especially in the neonatal period. Hypocalcemia Begin calcium supplementation after proper tests (simultaneous serum calcium and serum PTH

2014 eMedicine.com

182. Granulocytopenia (Diagnosis)

in cases involving drug exposure: If the identity of the causative agent is not known, stop administration of all drugs until the etiology is established Use careful oral hygiene to prevent infections of the mucosa and teeth Avoid rectal temperature measurements and rectal examinations Administer stool softeners for constipation Use good skin care for wounds and abrasions: Skin infections should be managed by someone with experience in the treatment of infection in neutropenic patients Antibiotics (...) granular lymphocyte proliferation or leukemia In isoimmune neonatal neutropenia, the mother produces IgG antineutrophil antibodies to fetal neutrophil antigens that are recognized as nonself. This occurs in 3% of live births. The disorder manifests as neonatal fever, urinary tract infection, cellulitis, pneumonia, and sepsis. The duration of the neutropenia is typically 7 weeks. Chronic autoimmune neutropenia is observed in adults and has no age predilection. As many as 36% of patients will exhibit

2014 eMedicine.com

183. Benign Lesions of the Ovaries (Diagnosis)

from all 3 germ layers. Benign teratomas (also known as mature teratomas or dermoid cysts) are likely to contain more of the recognizable organic structures, such as thyroid, bronchial, and central nervous system tissue. [ ] In dermoid cysts, ectodermal structures such as hair, teeth, and skin predominate. Treatment In most instances, simple excision of the solid tumors is adequate therapy, particularly for women of reproductive age. Laparoscopic treatment of benign cystic teratomas of the ovaries (...) . The updated French guidelines indicate that transvaginal pelvic ultrasonography is the first-line modality for evaluating presumed benign ovarian tumors in adult women. [ ] Transvaginal ultrasonography is limited with regard to its role in assessing masses in neonates, children, and virginal adolescents. Color-coded Doppler ultrasonography improves the diagnostic accuracy of B-mode ultrasonography. Ultrasonography is easy, rapid, and able to provide critical information for the evaluation of an adnexal

2014 eMedicine.com

184. Bacterial Infections and Pregnancy (Diagnosis)

, and stillbirth; occasionally, GBS bacteremia leads to endocarditis or meningitis In postpartum women, GBS can cause urinary tract infections (UTIs) and pelvic abscesses In newborns, early-onset GBS infection occurs before age 7 days (mean age at presentation is age 12 hours) and primarily manifests as nonfocal sepsis, pneumonia, or meningitis Late-onset disease in neonates occurs at age 7-89 days (mean age, 36 days), and nonfocal bacteremia and meningitis are the most common presentations Babies who survive (...) or erythromycin has not been established, IV can be used The neonate must be carefully observed for signs and symptoms of disease Urinary tract infections Asymptomatic bacteriuria develops in 10-15% of pregnant women and can lead to complications such as pyelonephritis and , so all pregnant women should undergo screening with urine culture at least once during early pregnancy and should be treated if the results are positive. [ , ] Significant bacteriuria is defined as >100,000 colony-forming units (CFU

2014 eMedicine.com

185. Viral Infections of the Mouth (Diagnosis)

who are immunosuppressed or in those older than 50 years. Unusual complications can include devitalization of teeth, root resorption, osteonecrosis, and root resorption. [ ] arises when the virus emerges from latency in the geniculate ganglion. It involves cranial nerve VII (facial nerve), which has both motor and sensory functions. Manifestations may include paralysis that involves the levator palati muscle and the face; hoarseness; loss of secretory function (eg, dry mouth, loss of taste (...) as cytomegalovirus. Primary HHV-5 infection is usually asymptomatic in patients who are immunocompetent. The virus is shed by glandular secretions, including saliva. It occasionally is shed in urine. Primary HHV-5 infection can be asymptomatic, but it can also mimic mononucleosis. Clinical disease is more common in neonates and in patients who are immunosuppressed than in other individuals. HHV-5 can persist indefinitely in the host. Reactivation of latent infection can occur in patients who are immunosuppressed

2014 eMedicine.com

186. Lipodystrophy, Generalized (Diagnosis)

, or both may also play a role. The differential diagnosis of congenital generalized lipoatrophy includes acquired generalized lipoatrophy, leprechaunism, Werner syndrome, pubertal-onset generalized lipodystrophy due to LMNA mutations, and neonatal progeroid syndrome. [ ] (See images below.) Werner syndrome in a 37-year-old woman (left). Clinical features evident include premature aging, alopecia, sclerodermalike changes in the skin, and peripheral lipoatrophy (shown). Insulin-resistant diabetes (...) are not characterized as primary lipodystrophy syndromes but are associated with abnormal body fat distribution, among other complex abnormalities. Progeria syndromes, such as Werner syndrome (adult progeria syndrome, see first image above), [ ] Cockayne syndrome (a juvenile progeria syndrome), [ , ] carbohydrate-deficient glycoprotein syndrome type 1, [ ] SHORT (short stature, hyperextensibility, hernia, ocular depression, Rieger anomaly, and teething delay) syndrome, [ , ] and mandibuloacral dysplasia [ , ] [see

2014 eMedicine.com

187. Anetoderma (Diagnosis)

, Bradley RR, Winkelmann RK. Familial anetoderma. J Am Acad Dermatol . 1987 Feb. 16(2 Pt 1):341-5. . Thomas JE, Mehregan DR, Holland J, Mehregan DA. Familial anetoderma. Int J Dermatol . 2003 Jan. 42(1):75-7. . Zellman GL, Levy ML. Congenital anetoderma in twins. J Am Acad Dermatol . 1997 Mar. 36(3 Pt 1):483-5. . Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol . 2010 May. 146(5):565-7. . Karrer S (...) syndrome. J Am Acad Dermatol . 2007 May. 56(5):881-2. . Hodak E, David M. Primary anetoderma and antiphospholipid antibodies--review of the literature. Clin Rev Allergy Immunol . 2007 Apr. 32(2):162-6. . Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol . 2010 May. 146(5):565-7. . Herrero-Gonzalez JE, Herrero-Mateu C. Primary anetoderma associated with primary Sjogren's syndrome. Lupus . 2002. 11(2):124

2014 eMedicine.com

188. Atrial Septal Defect (Diagnosis)

mutations. Ellis van Creveld syndrome is an autosomal recessive disorder associated with skeletal dysplasia characterized by short limbs, short ribs, postaxial polydactyly, dysplastic nails and teeth, and a common atrium, occurring in 60% of affected individuals. [ ] Mutations in the cardiac transcription factor NKX2.5 have been attributed to the syndrome familial ASD associated with progressive atrioventricular block. [ , , ] This syndrome is an autosomal dominant trait with a high degree of penetrance (...) . . Thuny F, Di Salvo G, Belliard O, et al. Risk of embolism and death in infective endocarditis: prognostic value of echocardiography: a prospective multicenter study. Circulation . 2005 Jul 5. 112(1):69-75. . Tonni G, Ferrari B, Defelice C, Centini G. Neonatal porencephaly in very low birth weight infants: ultrasound timing of asphyxial injury and neurodevelopmental outcome at two years of age. J Matern Fetal Neonatal Med . 2005 Dec. 18(6):361-5. . Vick GW 3rd, Murphy DJ Jr, Ludomirsky A, et al

2014 eMedicine.com

189. Epidermal Inclusion Cyst (Diagnosis)

predilection has been identified. Pigmentation of epidermoid cysts is common in individuals with dark skin. In a study of Indian patients with epidermoid cysts, 63% of the cysts contained melanin pigment. [ ] Sex Epidermoid cysts are approximately twice as common in men as in women. Age Epidermoid cysts may occur at any age; however, they most commonly arise in the third and fourth decades of life. Small epidermoid cysts known as milia are common in the neonatal period. Previous Next: Prognosis Epidermoid (...) Acad Dermatol Venereol . 2001 Mar. 15(2):184-5. . Bechara FG, Sand M, Rotterdam S, Altmeyer P, Hoffmann K. Multiple epidermal inclusion cysts after axillary liposuction-curettage: a rare complication of a frequent procedure. Int J Dermatol . 2008 Nov. 47(11):1197-8. . Leppard B, Bussey HJ. Epidermoid cysts, polyposis coli and Gardner's syndrome. Br J Surg . 1975 May. 62(5):387-93. . Besser FS. Pachyonychia congenita with epidermal cysts and teeth at birth: 4th generation. Br J Dermatol . 1971 Jan

2014 eMedicine.com

190. Factor XIII (Diagnosis)

polymorphisms to specific populations should be expected. Since it is an autosomal disorder, homozygous FXIII deficiency occurs in either sex. Acquired inhibitors to FXIII can present in either males or females. Physiologically, reduced levels of FXIII are found in healthy newborns, with a gradual rise in levels into the reference range. Premature infants have lower values than full-term neonates. FXIII levels drop in the latter half of a normal pregnancy. Neonates with severe FXIII deficiency may present (...) with bleeding from the umbilical cord. Easy bruising and delayed and recurrent bleeding after trauma begin in childhood. Oral bleeding can begin with teething and cuts or abrasions to the lips, tongue, and frenulum. Bleeding remains a problem throughout life and requires replacement therapy. FXIII deficiency acquired as a result of autoantibodies has been reported in the older population, as has acquired hemophilia A. Both drug-induced autoantibodies and alloantibodies have been reported in severely

2014 eMedicine.com

191. Drug-Induced Gingival Hyperplasia (Diagnosis)

is associated with gingival enlargement. Morbidity can be severe in some cases because of gross overgrowth of gingival tissue, which can lead to gingival bleeding, pain, teeth displacement, and periodontal disease. The prognosis is better if patients maintain regular oral hygiene and plaque control. Previous Next: Patient Education Inform patients of the risk of developing gingival enlargement secondary to therapy and the role of oral health in minimizing complications from therapy. Advise patients to see (...) a pedodontist, a periodontist, and an oral medicine dentist for a baseline evaluation; full mouth x-ray films; tooth extractions, if needed; and dental hygiene before transplant or the use of any drug known to induce gingival overgrowth. For patient education resources, see the as well as and . Previous References Rodriguez-Vazquez M, Carrascosa-Romero MC, Pardal-Fernandez JM, Iniesta I. Congenital gingival hyperplasia in a neonate with foetal valproate syndrome. Neuropediatrics . 2007 Oct. 38(5):251-2

2014 eMedicine.com

192. Eclampsia (Diagnosis)

second trimester (ie, 14-20 weeks' gestation), the diagnosis of hydatiform mole or choriocarcinoma should be considered. Ruling out eclampsia in an obstetric patient who has been involved in an unexplained trauma is important. Immediately consult an obstetrician/gynecologist when the diagnosis of eclampsia is being considered. No single laboratory test or set of laboratory determinations is useful in predicting maternal or neonatal outcome in women with eclampsia. Imaging studies may be indicated (...) to a labor and delivery unit capable of providing intensive care until delivery of the neonate. In the event of premature delivery or fetal compromise, a pediatrician or neonatologist should be consulted. When initially evaluating a patient with eclampsia, become familiar with the level of care that the medical center can offer the patient, as eclampsia clearly poses a risk of considerable maternal and neonatal morbidity and mortality. Patients with eclampsia may benefit from management at a tertiary

2014 eMedicine.com

193. Congenital Clouding of the Cornea (Diagnosis)

temporal quadrant of the corneal limbus. However, they are occasionally present entirely within the cornea or confined to the conjunctiva. They may contain a variety of histologically aberrant tissues, including epidermal appendages, connective tissue, skin, fat, sweat gland, lacrimal gland, muscle, teeth, cartilage, bone, vascular structures, and neurologic tissue (including brain tissue). Malignant degeneration is extremely rare. The most common system for classifying dermoids is based (...) neonatally with a diffuse clouding of the central anterior corneal stroma with other normal corneal physical and nervous structures. The cornea is not edematous. It is nonprogressive. Its inheritance is autosomal dominant, and mutations in the decorin ( DCN ) gene have been implicated. Visual acuity is decreased. Strabismus and nystagmus may occur. Posterior polymorphous dystrophy (PPMD) is a slowly progressive, uncommon, dominantly inherited condition. It is usually bilateral but sometimes asymmetric

2014 eMedicine.com

194. Psychosocial and Environmental Pregnancy Risks (Treatment)

exposed to phenytoin in utero. Trimethadione: Fetal trimethadione syndrome is associated with single transverse palmar crease in the hands, cardiac anomalies, irregular teeth, and mental

2014 eMedicine.com

195. Syphilis (Treatment)

Prevention Conference, August 13-17, 2011 . HARRISON LW. The Oslo study of untreated syphilis, review and commentary. Br J Vener Dis . 1956 Jun. 32(2):70-8. . . ROCKWELL DH, YOBS AR, MOORE MB Jr. THE TUSKEGEE STUDY OF UNTREATED SYPHILIS; THE 30TH YEAR OF OBSERVATION. Arch Intern Med . 1964 Dec. 114:792-8. . McClure EM, Goldenberg RL. Infection and stillbirth. Semin Fetal Neonatal Med . 2009 Aug. 14(4):182-9. . Kupka R, Kassaye T, Saathoff E, Hertzmark E, Msamanga GI, Fawzi WW. Predictors of stillbirth (...) from Wisdom A. Color Atlas of Sexually Transmitted Diseases. Year Book Medical Publishers Inc; 1989. Syphilis. These photographs illustrate typical facies of congenital syphilis. Used with permission from Wisdom A. Color Atlas of Sexually Transmitted Diseases. Year Book Medical Publishers Inc; 1989. Syphilis. This photograph shows an example of Hutchinson teeth in congenital syphilis. Note notching. Used with permission from Wisdom A. Color Atlas of Sexually Transmitted Diseases. Year Book Medical

2014 eMedicine.com

196. Teratology and Drug Use During Pregnancy (Treatment)

, maternal health, and prenatal prescription records were linked to neonatal records, representing all live births in British Columbia during a 39-month period (1998-2001). Even after controlling for maternal illness profiles, infants exposed to prenatal serotonin reuptake inhibitors in combination with benzodiazepines had an increased incidence of congenital heart disease versus controls who had not been exposed. Serotonin reuptake inhibitor monotherapy was not associated with an increased risk (...) outcomes bases on all relevant human data, animal data, and the drugs pharmacology. Adverse developmental outcomes include the four groups of developmental toxicities: - "Structural abnormalities" describes dysmorphology, which includes malformations, variations, deformations, and disruptions. - "Embryo-fetal and/or infant mortality" describes development mortality, which includes miscarriage, stillbirth, and infant death (including neonatal death). - "Functional impairment" describes functional

2014 eMedicine.com

197. Syphilis (Treatment)

Prevention Conference, August 13-17, 2011 . HARRISON LW. The Oslo study of untreated syphilis, review and commentary. Br J Vener Dis . 1956 Jun. 32(2):70-8. . . ROCKWELL DH, YOBS AR, MOORE MB Jr. THE TUSKEGEE STUDY OF UNTREATED SYPHILIS; THE 30TH YEAR OF OBSERVATION. Arch Intern Med . 1964 Dec. 114:792-8. . McClure EM, Goldenberg RL. Infection and stillbirth. Semin Fetal Neonatal Med . 2009 Aug. 14(4):182-9. . Kupka R, Kassaye T, Saathoff E, Hertzmark E, Msamanga GI, Fawzi WW. Predictors of stillbirth (...) from Wisdom A. Color Atlas of Sexually Transmitted Diseases. Year Book Medical Publishers Inc; 1989. Syphilis. These photographs illustrate typical facies of congenital syphilis. Used with permission from Wisdom A. Color Atlas of Sexually Transmitted Diseases. Year Book Medical Publishers Inc; 1989. Syphilis. This photograph shows an example of Hutchinson teeth in congenital syphilis. Note notching. Used with permission from Wisdom A. Color Atlas of Sexually Transmitted Diseases. Year Book Medical

2014 eMedicine.com

198. Oral Manifestations of Autoimmune Blistering Diseases (Treatment)

, complications from poor oral care could lead to periodontal disease and early teeth loss. During the active disease stage, patient follow-up care every 4-6 weeks is recommended. Patients should be monitored for oral yeast infection. During the clinical remission stage, patient follow-up care every 6 months is recommended. Previous References Olivry T, Chan LS. Autoimmune blistering dermatoses in domestic animals. Clin Dermatol . 2001 Nov-Dec. 19(6):750-60. . Hill PB, Brain P, Collins D, Fearnside S, Olivry (...) . A common major histocompatibility complex class II allele HLA-DQB1* 0301 is present in clinical variants of pemphigoid. Proc Natl Acad Sci U S A . 1996 Aug 6. 93(16):8569-71. . . Amagai M, Nishikawa T, Nousari HC, Anhalt GJ, Hashimoto T. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest . 1998 Aug 15. 102(4):775-82. . . Mustafa MB, Porter SR, Smoller BR, Sitaru C

2014 eMedicine.com

199. Sleep Stage Scoring (Treatment)

silence during REM sleep may not be of help in distinguishing it from the preceding or subsequent sleep stages. Compounding the problem of interpreting EMG channels is intrusion of artifact into the signal. Some examples include cyclic jaw movements, teeth grinding (bruxism), or steady high-amplitude noise generated by increased pressure on an electrode (eg, as caused by lying on the chin). Additionally, muscle artifact spilling over into cortical leads is not an unusual finding. ECG signal (...) are useful in the diagnosis and management of sleep disorders. For example, increased sleep latency may be increased in primary insomnia and decreased in obstructive sleep apnea, whereas Stage R latency may be decreased in narcolepsy. Sleep architecture changes with advancing age. The percentage of stage R is greatest in neonates, where it may encompass 50% of total sleep time. This proportion decreases gradually to 20-25% at adolescence, with further slight decrease after age 65. Greater sleep

2014 eMedicine.com

200. Seizure Disorders in Pregnancy (Treatment)

acid. [ ] In addition, carbamazepine is also associated with a risk of cardiac anomalies; thus, for patients taking this drug, a fetal echocardiogram is recommended at 20-22 weeks' gestation. Trimethadione In 1975, a cluster of findings, including epicanthal folds, low-set ears, microcephaly, short stature, and irregular teeth, was ascribed to trimethadione. [ ] A review of 57 pregnancies published in 1977 revealed either malformations or fetal loss 87% of the time. [ ] Currently, trimethadione (...) in newborns suggest that the inhibition of vitamin K–dependent clotting factors (ie, II, VII, IX, X) secondary to increased vitamin K metabolism and the inhibition of placental transport of vitamin K results from AED use. Historically, most patients on AEDs received oral vitamin K supplementation at the end of pregnancy. However, a study of 204 neonates born to mothers taking AEDs who did not received vitamin K supplementation showed no evidence of coagulopathy. [ ] Upon delivery, clotting studies can

2014 eMedicine.com

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