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How Common is Rubella in the United States?

Patient Presentation
A pediatrician received a written notice from the Department of Public Health that a newborn infant needed follow-up for failing their newborn hearing screening. The physician did not recognize the patient’s name and the infant had not been seen in the practice. When the family was contacted to arrange followup, the physician was told that the female infant had been diagnosed with congenital rubella syndrome and was seeing another physician. The parent also said that the infant had failed the followup hearing screening and was also being followed by an ophthalmologist for cataracts. Additionally the family was already receiving support services through local agencies. The physician was very surprised because he knew the rubella had been decreasing in the United States.

Discussion
Rubella (sometimes called German measles) is caused by a togavirus. The post-natally acquired infection causes erythematous macules that occur first on the face and then spreads to extremities and trunk (spreading distally). The rash fades in the same direction. Fever, posterior cervical lymphadenopathy or arthritis may also occur. The incubation period is 14-23 days and patients are contagious from 7 days before the rash until 14 days after the rash. The rash generally lasts 3 days, but the rash may be absent in 20-50% of cases.

Congenital rubella syndrome (CRS) is group of birth defects that includes:

  • Central nervous system
    • Behavior problems
    • Meningoencephalitis
    • Mental retardation
  • Congenital heart disease
    • Patent ductus arteriosus
    • Peripheral pulmonary artery stenosis
  • Ears
    • Sensorineural hearing impairment
  • Eyes
    • Blindness
    • Congenital cataract
    • Congenital glaucoma
    • Microopthalmia
    • Pigmentary retinopathy

Presentation of CRS in the neonatal period includes:

  • Bone disease, radiolucencies
  • Exantham – “blueberry muffin” caused by dermal erythropoiesis
  • Growth retardation
  • Hepatosplenomegaly
  • Pneumonitis, interstitial
  • Thrombocytosis
  • No or few clinical signs

CRS is more likely to be contracted the earlier in gestation the maternal infection occurs: 85% of fetuses develop CS if infected in the first 12 weeks of gestation, 54% in 13-16 weeks gestation, and 25% from 16-24 weeks gestation.

Rubella presents similarly to measles and therefore can be mistaken. Measles is caused by a paramyxovirus and clinically causes erythematous macules and papules that first appears on the lateral and posterior neck, and that progresses to involve the face, trunk and extremities (spreading distally). The rash fades in the same direction. Cough, coryza, Koplik spots and fever also occur. The incubation period is 8-12 days. Patients are contagious from 1-2 days before the rash until 4 days after the rash.

A review of common viral exanthams can be found here.

Learning Point
Rubella is considered non-endemic in the United States since 2010. “…[The Centers for Disease Control] defines absence of endemic transmission as the lack of existence of any continuous U.S.-acquired chain of transmission that persists for [greater than or equal to] 12 months in any defined geographic area.” This does not mean that there are no cases of rubella in the U.S.. The number of rubella cases in the U.S. has slowly but dramatically decreased after the initiation of Rubella vaccine in 1969 from 12.5 million cases in 1962-1965, to 7 and 9 cases respectively in 2003 and 2004. A similar decrease in CRS has been seen.

The demographics of rubella and CRS have changed from being endemic in the U.S. to being foreign-born. Rubella in the U.S. is most often found in persons born outside the U.S. who are unvaccinated or the vaccine status is unknown. Many of these people are from countries who have recently begun rubella vaccination programs or are improving their programs. Overall there has been dramatic progress towards controlling rubella worldwide, but rubella still remains endemic in other parts of the world and thus importable. Vaccination is highly efficacious with 95%+ rates of immunity after 1 dose of vaccine. Vaccination programs in some countries use single agent Rubella. In the U.S. rubella vaccine is routinely incorporated into measles vaccine which is given twice, giving an additional safeguard for rubella.

Questions for Further Discussion
1. How common is rubella in your own country or region of the world?
2. List common congenital infections and their presentations.
3. How common is measles in your own country or region of the world?
4. What services are available locally for children with congenital rubella syndrome?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Rubella

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Centers for Disease Control. Achievements in Public Health: Elimination of Rubella and Congenital Rubella Syndrome — United States, 1969-2004. MMWR. March 25, 2005.
Available from the Internet at http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5411a5.htm (cited 4/2/2012).

American Academy of Pediatrics. Rubella, In Pickering LD, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2009;579-584.
Centers for Disease Control. Progress Toward Control of Rubella and Prevention of Congenital Rubella Syndrome — Worldwide, 2009. MMWR. October 15, 2010.
Available from the Internet at http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5940a4.htm (cited 4/2/2012).

Muscat M, Zimmerman L, Bacci S, Bang H, Glismann S, Molbak K, Reef S; the EUVAC.NET group. Toward rubella elimination in Europe: An epidemiological assessment.Vaccine. 2012 Mar 2;30(11):1999-2007.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Systems Based Practice
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • Does He Have Acromegaly or Something Similar?

    Patient Presentation
    A 14-year-old male came to clinic for his health supervision visit. He and his parents had no concerns other than his growth. “He’s already 6 feet like me and I’m having problems finding shoes to fit him,” stated his mother. He had always been taller (> 95%) with a medium build. He was doing well in school, played basketball and threw the discus and shot put in track. The past medical history showed no concerns The family history revealed that mother was 6 feet tall and went through puberty at 13 years, his father was 6 feet 6 inches and went through puberty “late” and was slow to go through it per his mother. Both parents stated they were average size for their families. Mother joked, “Our families always play forward on the basketball teams.” His younger brother was also > 95% for height. A maternal grandmother had a goiter at an older age, and a paternal uncle had some heart problem in his late 40′s. There was another paternal uncle who died in a car accident in his 40′s. The parents were of Germanic and Scandinavian heritage. The review of systems was negative and he denied any drug or supplement use.

    The pertinent physical exam showed a height of 72 inches (> 95%), weight of 165 pounds (90-95%) with normal vital signs. HEENT demonstrated some mild facial and back closed comedomal acne. He had a normal thyroid gland and no frontal bossing, large chin, or facial edema. His heart examination was also normal with no abdominal bruits. Genitourinary examination showed a Tanner III male. Musculoskeletal examination found his upper segment proportional to his lower segment and his arm span was within 1/2 inch of his height. The diagnosis of a healthy male was made, but the physician was concerned that although his height concerns were most likely familial, perhaps he had acromegaly, Marfan syndrome or some other abnormality that should be identified. The patient was referred to endocrinology. The radiologic and laboratory evaluation produced a normal bone age, thyroid studies, IGF-1 and growth hormone levels in addition to a normal echocardiogram confirming the diagnosis of familial growth stature. The patient’s clinical course at age 18 showed his adult height to be 6 feet 7 inches. His brother was following a similar growth pattern also.

    Discussion
    Acromegaly is caused by abnormal growth hormone and IGF-1 (insulin-like growth factor 1) after the growth plates are closed. It is called gigantism if it occurs when the growth plates are open and causes increased linear growth. Acromegaly, since it acts on closed growth plates, shows other physical signs including frontal bossing, prognathism, coarse facial features, macrocephaly, moderate obesity, thickened skin, thickened fingers and toes, hyperhidrosis and other problems. If the IGF-1 exposure is prolonged other problems occur such as cardiac hypertrophy and osteoarthritis. Benign tumors are also relatively common with acromegaly, but increased risk of malignancies is controversial. Acromegaly usually occurs in the 3rd decade but gigantism can occur at any age prior to growth plate fusion and death is usually due to cardiac and respiratory problems.

    IGF-1 excess can be caused by pituitary excess secretion, pituitary dysregulation or excess IGF binding protein which prolongs IGF half-life. If caused by pituitary excess secretion it is often a part of other endocrinopathies especially abnormal growth hormone, and increased prolactin. It can be seen as a part of other abnormalities including McCune-Albright syndrome, Tuberous Sclerosis, and Neurofibromatosis.

    Acromegaly is usually diagnosed with an increase in IGF-1. Imaging of the pituitary is commonly done to look for adenomas or other pituitary abnormalities. Treatment may include surgery and hormone agonists (e.g. dopamine, somatostatin, etc.) and antagonists (e.g. growth hormone receptor).

    Learning Point
    The differential diagnosis of excessive height/length includes:

    • Familial or constitutional (most common)
    • Acromegaly or gigantism
    • Beckwith-Wiedemann Syndrome (as infant)
    • Congenital adrenal hyperplasia (untreated)
    • Eosinophilic adenoma
    • Homocystinuria
    • Lipodystrophy
    • Marfan’s syndrome
    • Neurofibromatosis
    • Soto syndrome
    • Weaver’s syndrome
    • Various genetic various of sex chromosomes
      • Fragile X
      • Kleinfelter’s syndrome
      • 47 XYY
      • 48 XXYY

    A review of overgrowth syndromes can be found here.

    Questions for Further Discussion
    1. How successful is surgery for treatment of gigantism or acromegaly?
    2. What is the differential diagnosis of short stature?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Growth Disorders

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Illingworth RS. Common Symptoms of Disease in Children. Blackwell Scientific Publications: Oxford. 1988:28-29.

    Renehan AG, Brennan BM. Acromegaly, growth hormone and cancer risk. Best Pract Res Clin Endocrinol Metab. 2008 Aug;22(4):639-57.

    Ferry RJ Jr, Kemp S. Gigantism and Acromegaly. Medscape.
    Available from the Internet at http://emedicine.medscape.com/article/925446-workup (rev. 7/10/2010, cited 3/26/3012).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • How Long Do Body Piercings Take to Heal?

    Patient Presentation
    An 18-year-old female came to clinic because of a problem with a navel ring. She had the piercing placed 2 months previously and had been doing appropriate care. About 1 week ago, the jewelry caught on clothing and caused a small tear and bleeding. The initial redness and swelling had not subsided, and she now noticed that the redness was spreading and there was increased tenderness. She noted that the initial clearish fluid was now thicker and yellow. She denied previous problems with her piercing, sharing jewelry, or other trauma. The review of systems showed no fever, chills, myalgia or other wounds.

    The pertinent physical exam showed a healthy appearing female with normal vital signs.
    Her navel was pierced at the 12 o’clock position with a ring and ball-type jewelry piece. She had erythema, ~1.5 cm extending in a fan-shaped pattern from 9 to 3 o’clock around the piercing, with a small amount of yellow fluid able to be expressed from around the jewelry. She had moderate tenderness over the jewelry and erythematous skin. The rest of her examination was normal. The diagnosis of localized cellulitis was made. The area was cleaned and some of the drainage was sent for gram stain and culture. She was instructed to keep the jewelry in place, use isotonic saline soaks (gauze pads saturated) several times a day, and clean warm compresses several times/day. She was also started on cephalexin and told to return if she had increasing wound symptoms, or new abdominal pain or fever over the night. By the next day, the erythema had decreased to around a 1 cm fan-shaped area and she had decreased tenderness. The gram-stain showed gram-positive cocci in clusters but was too early for identification. She was instructed to continue the current regimen and finish all of the antibiotics. She was also instructed to continue her after-care regimen until the piercing fully healed over the next several months.

    Discussion
    Body piercing is the intentional perforation of a body tissue and insertion of an ornament that is for decorative, cultural, spiritual, or other reasons. It is one form of body art which also includes tattooing. Body art has been used over centuries of time across the globe.

    It is estimated that body piercing prevalence is 30-50% in the 18-25 year old US population. It is popular among all ages, social classes and occupations. It does vary with certain ethnic classes and females are more common than males to have a body piercing. Basically almost any area can be pierced including the genitals and eye globe itself.

    There is a high rate of complications (20-35%) which most are relatively self-limited.
    Risks of body piercing include:

    • Local – bleeding, infection, skin irritation, mechanical tissue tearing, hypersensitivity to the metal, granuloma, scarring, embedded jewelry, edema and ischemia, and dental problems
    • Systemic – endocarditis, sepsis, brain abscess, upper-airway compromise, viral infections (e.g. Hepatitis B, Hepatitis C, HIV)

    Group A beta-hemolytic Streptococcus, Staphylococcus (methicillin sensitive and resistant) and Pseudomonas are the most common bacterial infections. Body piercing has been associated with increased risk-taking in adolescents including substance abuse, sexual risk taking, and violence and suicide.

    The United States and other countries regulate body piercing, but many people will self-pierce or use non-licensed practitioners. A listing of legal standards for minors in the US can be found here. Listings of local licensed practitioners may be available though local health departments who oversee the industry. At least one professional association is available in the United States, the Association of Professional Piercers. Like other professional organizations, membership is voluntary, but criteria standards are set for initial and continuing membership along with opportunities for continuing professional development and education of the lay public.

    Learning Point
    Healing time depends on several factors including the individual, piercing location, type of jewelry and aftercare of the piercing. Cartilage takes longer to heal than skin itself (ear lobe healing is around 6-8 weeks versus pinna piercing which may take 3-8 months). Areas that are covered or easily irritated such as navel, genitals and nipples also take longer. In general, ear lobes and tongues heal around 6-8 weeks, face around 3 months, and navel and cartilage heal in 3-8 months. Fastidious aftercare is necessary to decrease the potential side effects and needs to be continued much longer than most individuals believe. Even a healed piercing needs aftercare for up to a year so the skin epithelizes well. Jewelry with niobium, titanium, surgical steel, gold and plastic polymers are recommended for initial piercings. There are industry standards for materials used for different types of jewelry and its components.

    After care includes washing hands prior to touching the piercing, soaking with saline for 5-10 minutes 1-2x/day, washing with a small amount of mild soap daily and rinsing thoroughly. Drying the area with disposable paper products is recommended as even clean cloth items such as towels could harbor bacteria or cause mechanical irritation. Alcohol, Hibiclens®, and Betadine® are not recommended because they may cause more skin irritation. When there is a local infection it is usually best to try to leave the jewelry in place (removing can cause abscess formation) which allows for improved drainage and epidermal healing. After 5-7 days if it is not healing then the patient probably needs to have the jewelry removed along with possible surgical drainage and IV antibiotics.

    Questions for Further Discussion
    1. At what age is a minor too young for a body piercing?
    2. How would you counsel someone considering a body piercing to look for in a reputable piercer?
    3. What are signs of contact dermatitis or metal hypersensitivity with piercings?
    4. What are the risks associated with tattooing?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Piercing and Tattoos

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Carroll ST, Riffenburgh RH, Roberts TA, Myhre EB. Tattoos and body piercings as indicators of adolescent risk-taking behaviors. Pediatrics. 2002 Jun;109(6):1021-7.

    Armstrong ML, Koch JR, Saunders JC, Roberts AE, Owen DC. The hole picture: risks, decision making, purpose, regulations, and the future of body piercing. Clin Dermatol. 2007 Jul-Aug;25(4):398-406.

    Hogan L, Armstrong ML. Body piercing: more than skin deep. Skin Therapy Lett. 2009 Sep;14(7):4-7.

    Miller JM, Fitzpatrick JJ. Piercing: does health education make a difference? Nurse Pract. 2010 Jun;35(6):48-52.

    Fijalkowska M, Pisera P, Kasielska A, Antoszewski B. Should we say NO to body piercing in children? Complications after ear piercing in children. Int J Dermatol. 2011 Apr;50(4):467-9.

    Body Aftercare Instructions. Association of Professional Piercers. Available from the Internet at http://www.safepiercing.org/publications/brochures/body-aftercare/ (cited3/23/2012).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital