Criteria for the Diagnosis of Asthma

Asthma is a chronic inflammatory disease of the airways that is characterized by variable narrowing of the airways and symptoms of intermittent dyspnea, wheezing, and nighttime or early-morning coughing.

Criteria for the Diagnosis of Asthma

Presence of episodic symptoms of airflow obstruction or airway hyperresponsiveness
Objective assessment consisting of one of the following

Airflow obstruction that is at least partially reversible with the use of an inhaled short-acting beta 2-agonist, as shown by one of three variables

An increase in FEV1 of >/=12% from baseline
An increase in predicted FEV1 of >/=10 percentage points from baseline
An increase in PEF of >/=20% (or 60 liters/min) from baseline

Diurnal variation in PEF (measured twice daily) of more than 10%

* FEV1 denotes forced expiratory volume in 1 second, and PEF peak expiratory flow.

References:

1. Bel EH. Clinical Practice. Mild asthma. N Engl J Med. 2013 Aug 8;369(6):549-57.[Medline]
2. Mintz M. Asthma update: part I. Diagnosis, monitoring, and prevention of disease progression. Am Fam Physician. 2004;70:893-898. [Medline]

Intravenous Immune Globulin in Autoimmune and Inflammatory Diseases

Initially used for the treatment of immunodeficiencies, intravenous immunoglobulins (IVIg) have increasingly been used as immunomodulatory agent in autoimmune and inflammatory disorders. The mode of action of IVIg is enigmatic, probably involving Fc-dependent and/or F(ab')2-dependent non-exclusive mechanisms of action. IVIg broadly interacts with the different components of the immune system: cytokines, complement, Fc receptors and several cell surface immunocompetent molecules. IVIg also has an impact on effector functions of immune cells. These mechanisms of action of IVIg reflect the importance of natural antibodies in the maintenance of immune homeostasis.

Diseases for Which Intravenous Immune Globulin Has Been Shown to Be Beneficial

FDA-approved indications

• Primary immunodeficiency disease
• Chronic lymphocytic leukemia
• Pediatric HIV infection
• Kawasaki’s disease
• Allogeneic bone marrow transplantation
• Chronic inflammatory demyelinating polyneuropathy
• Kidney transplantation involving a recipient with a high antibody titer or an ABO-incompatible donor
• Multifocal motor neuropathy

Additional approved indications with criteria

Neuromuscular disorders

• Guillain–Barré syndrome
• Relapsing–remitting multiple sclerosis
• Myasthenia gravis
• Refractory polymyositis
• Polyradiculoneuropathy
• Lambert–Eaton myasthenic syndrome
• Opsoclonus–myoclonus
• Birdshot retinopathy
• Refractory dermatomyositis

Hematologic disorders

• Autoimmune hemolytic anemia
• Severe anemia associated with parvovirus B19
• Autoimmune neutropenia
• Neonatal alloimmune thrombocytopenia
• HIV-associated thrombocytopenia
• Graft-versus-host disease
• Cytomegalovirus infection or interstitial pneumonia in patients undergoing bone marrow transplantation

Dermatologic disorders

• Pemphigus vulgaris
• Pemphigus foliaceus
• Bullous pemphigoid
• Mucous-membrane (cicatricial) pemphigoid
• Epidermolysis bullosa acquisita
• Toxic epidermal necrolysis or Stevens–Johnson syndrome
• Necrotizing fasciitis

* This is an abbreviated list of conditions approved under Medicare Part D or Aetna Clinical Policy Bulletin (2012). Criteria include medical certainty of diagnosis, medical necessity owing to the failure of usual treatments, contraindications to usual treatments, rapid progression or relapse, documentation of progress, and attempts to adjust drug dosages without improvement. FDA denotes Food and Drug Administration, and HIV human immunodeficiency virus.

References:

1. Gelfand EW. Intravenous immune globulin in autoimmune and inflammatory diseases. N Engl J Med. 2012 Nov 22;367(21):2015-25. [Medline]
2. Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol. 2005 Oct;142(1):1-11. [Medline]

Diagnostic Criteria for Chronic HBV Infection

Chronic HBV infection is a necroinflammatory disease of the liver caused by persistent infection with HBV, and can be categorized as hepatitis B e antigen (HBeAg) positive or negative. Inactive hepatitis B surface antigen (HBsAg) carriers have HBV infection of the liver without significant, ongoing necroinflammatory disease. HBV infection is resolved when there is no further virologic, biochemical, or histologic evidence of active viral infection or disease.

Diagnostic criteria related to HBV include the following:

• Chronic hepatitis B: HBsAg positive for more than six months, serum HBV DNA greater than 20,000 IU per mL (lower values of 2,000 to 20,000 IU per mL often occur with HBeAg-negative chronic hepatitis B), persistent or intermittent elevation in alanine transaminase (ALT) or aspartate transaminase (AST) levels, and liver biopsy showing chronic hepatitis with moderate or severe necroinflammation.
• Inactive HBsAg carrier state: HBsAg positive for more than six months, HBeAg negative and anti-HBeAg positive, serum HBV DNA less than 2,000 IU per mL, persistently normal ALT and AST levels, liver biopsy confirming absence of significant hepatitis.
• Resolved hepatitis B: known history of acute or chronic hepatitis B or the presence of anti-hepatitis B core antigen (anti-HBcAg) with or without anti-HBsAg, HBsAg negative, undetectable serum HBV DNA (very low levels may be detectable with sensitive prostate-specific antigen assays), and normal ALT levels.

References:

1. Kuo A, Gish R. Chronic hepatitis B infection. Clin Liver Dis. 2012 May;16(2):347-69.[Medline]
2. Liaw YF, Chu CM. Hepatitis B virus infection. Lancet. 2009;373:582–92. [Medline]
3. Bonino F, Piratvisuth T, Brunetto MR, Liaw YF. Diagnostic markers of chronic hepatitis B infection and disease. Antivir Ther. 2010;15 Suppl 3:35-44. [Medline]

Revised Diagnostic Criteria for Rett Syndrome (RTT)

Rett syndrome (RTT) is a severe neurodevelopmental disease that affects approximately 1 in 10,000 live female births and is often caused by mutations in Methyl-CpG-binding protein 2 (MECP2).

Revised diagnostic criteria for RTT.

RTT Diagnostic Criteria 2010

• Consider diagnosis when postnatal deceleration of head growth observed.

Required for typical or classic RTT

1. A period of regression followed by recovery or stabilization*
2. All main criteria and all exclusion criteria
3. Supportive criteria are not required, although often present in typical RTT

Required for atypical or variant RTT

1. A period of regression followed by recovery or stabilization*
2. At least 2 out of the 4 main criteria
3. 5 out of 11 supportive criteria

Main Criteria

1. Partial or complete loss of acquired purposeful hand skills.
2. Partial or complete loss of acquired spoken language**
3. Gait abnormalities: Impaired (dyspraxic) or absence of ability.
4. Stereotypic hand movements such as hand wringing/squeezing, clapping/tapping, mouthing and washing/rubbing automatisms

Exclusion Criteria for typical RTT

1. Brain injury secondary to trauma (peri- or postnatally), neurometabolic disease, or severe infection that causes neurological problems***
2. Grossly abnormal psychomotor development in first 6 months of life#

Supportive Criteria for atypical RTT##

1. Breathing disturbances when awake
2. Bruxism when awake
3. Impaired sleep pattern
4. Abnormal muscle tone
5. Peripheral vasomotor disturbances
6. Scoliosis/kyphosis
7. Growth retardation
8. Small cold hands and feet
9. Inappropriate laughing/screaming spells
10. Diminished response to pain
11. Intense eye communication - “eye pointing”

*Because MECP2 mutations are now identified in some individuals prior to any clear evidence of regression, the diagnosis of “possible” RTT should be given to those individuals under 3 years old who have not lost any skills but otherwise have clinical features suggestive of RTT. These individuals should be reassessed every 6–12 months for evidence of regression. If regression manifests, the diagnosis should then be changed to definite RTT. However, if the child does not show any evidence of regression by 5 years, the diagnosis of RTT should be questioned.

**Loss of acquired language is based on best acquired spoken language skill, not strictly on the acquisition of distinct words or higher language skills. Thus, an individual who had learned to babble but then loses this ability is considered to have a loss of acquired language.

***There should be clear evidence (neurological or ophthalmological examination and MRI/CT) that the presumed insult directly resulted in neurological dysfunction.

#Grossly abnormal to the point that normal milestones (acquiring head control, swallowing, developing social smile) are not met. Mild generalized hypotonia or other previously reported subtle developmental alterations during the first six months of life is common in RTT and do not constitute an exclusionary criterion.

##If an individual has or ever had a clinical feature listed it is counted as a supportive criterion. Many of these features have an age dependency, manifesting and becoming more predominant at certain ages. Therefore, the diagnosis of atypical RTT may be easier for older individuals than for younger. In the case of a younger individual (under 5 years old) who has a period of regression and >/=2 main criteria but does not fulfill the requirement of 5/11 supportive criteria, the diagnosis of “probably atypical RTT” may be given. Individuals who fall into this category should be reassessed as they age and the diagnosis revised accordingly.

References:

1. Jeffrey L. Neul, Walter E. Kaufmann, Daniel G. Glaze, John Christodoulou, Angus J. Clarke, Nadia Bahi-Buisson, Helen Leonard, Mark E. S. Bailey, N. Carolyn Schanen, Michele Zappella, Alessandra Renieri, Peter Huppke, Alan K. Percy. Rett Syndrome: Revised Diagnostic Criteria and Nomenclature. Ann Neurol. 2010 December; 68(6): 944–950. [Medline]
2. Percy AK, Neul JL, Glaze DG, Motil KJ, Skinner SA, Khwaja O, Lee HS, Lane JB, Barrish JO, Annese F, McNair L, Graham J, Barnes K. Rett syndrome diagnostic criteria: lessons from the Natural History Study. Ann Neurol. 2010 Dec;68(6):951-5. [Medline]
3. Huppke P, Gärtner J. Molecular diagnosis of Rett syndrome. J Child Neurol. 2005 Sep;20(9):732-6. [Medline]

Diagnostic Criteria for Marfan Syndrome (MFS)

Marfan syndrome is currently diagnosed using criteria based on an evaluation of the family history, molecular data, and 6 organ systems.

Diagnostic Criteria for Marfan Syndrome (MFS)


The Ghent criteria consist of major and minor criteria. The major criteria are features or symptoms that are common in people with Marfan syndrome and rare in people who do not have the syndrome.

Minor criteria are features or symptoms that are present in people with Marfan syndrome, but are also present in people who do not have it.

To be diagnosed with Marfan syndrome using the Ghent criteria, you must have a number of different criteria as described below.

If you have a family history of Marfan syndrome, you will need to have one of the major criteria and one of the minor criteria that affect different systems in your body, such as your skeleton and your blood vessels.
If you do not have a family history of Marfan syndrome, you will need to have two major criteria and one of the minor criteria that affect different systems in your body.

ILAR Classification Criteria for Juvenile Idiopathic Arthritis (JIA)

Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years.

International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA)

JIA can be diagnosed if age at onset is under 16 years, disease duration is 6 weeks or greater, and other known conditions are excluded.

• Systemic-onset JIA: Systemic arthritis is diagnosed if there is arthritis in 1 or more joints with, or preceded by, fever of at least 2 weeks' duration. Signs or symptoms must have been documented daily for at least 3 days and accompanied by 1 or more of the following: evanescent rash, generalised lymphadenopathy, hepato/splenomegaly, serositis. (Exclusions are A, B, C, and D from the exclusion list below.)
• Persistent or extended oligoarthritis: Oligoarthritis is diagnosed if there is arthritis affecting 1 to 4 joints during the first 6 months. Persistent oligoarthritis affects up to 4 joints throughout the course of the disease, and extended oligoarthritis affects more than 4 joints after the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)
• RF-negative polyarthritis: Polyarthritis (RF-negative) is diagnosed if there is rheumatoid factor (RF)-negative arthritis affecting 5 or more joints during the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)
• RF–positive polyarthritis: Polyarthritis (RF-positive) is diagnosed if there is RF-positive arthritis affecting 5 or more joints during the first 6 months of disease. Two or more RF tests (taken at least 3 months apart) are positive during the first 6 months of disease. (Exclusions are A, B, C, and E from the exclusion list below.)
• Psoriatic JIA: Psoriatic arthritis is diagnosed if there is arthritis and psoriasis, or arthritis and at least 2 of the following: dactylitis, nail pitting, onycholysis, and/or family history of psoriasis (in a first-degree relative). (Exclusions are B, C, D, and E from the exclusion list below.)
• Enthesitis-related arthritis: Enthesitis-related arthritis is diagnosed if there is arthritis and/or enthesitis with at least 2 of the following: presence or history of sacroiliac joint tenderness with or without inflammatory lumbosacral pain; presence of HLA B27 antigen; onset of arthritis in a male over 6 years of age; acute (symptomatic) anterior uveitis; history of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, or acute anterior uveitis in a first-degree relative. (Exclusions are A, D, and E from the exclusion list below.)
• Undifferentiated: Undifferentiated arthritis is diagnosed if there is arthritis that does not fulfil criteria in any of the above categories or that fulfils criteria for 2 or more of the above categories.

Screening for breast cancer

Recommendations

Major Recommendations

Note from the National Guideline Clearinghouse (NGC) and the U.S. Preventive Services Task Force (USPSTF): On December 4, 2009, the USPSTF unanimously voted to update the language of their recommendation regarding women under 50 years of age to clarify their original and continued intent. The following recommendations reflect that change.
The U.S. Preventive Services Task Force (USPSTF) grades its recommendations (A, B, C, D, or I) and identifies the Levels of Certainty regarding Net Benefit (High, Moderate, and Low). The definitions of these grades can be found at the end of the "Major Recommendations" field.
Summary of Recommendations and Evidence

• The USPSTF recommends biennial screening mammography for women aged 50 to 74 years. This is a B recommendation.
• The decision to start regular, biennial screening mammography before the age of 50 years should be an individual one and take patient context into account, including the patient’s values regarding specific benefits and harms. This is a C recommendation.
• The USPSTF concludes that the current evidence is insufficient to assess the additional benefits and harms of screening mammography in women 75 years or older. This is an I statement.
• The USPSTF recommends against teaching breast self-examination (BSE). This is a D recommendation.
• The USPSTF concludes that the current evidence is insufficient to assess the additional benefits and harms of clinical breast examination (CBE) beyond screening mammography in women 40 years or older. This is an I statement.
• The USPSTF concludes that the current evidence is insufficient to assess the additional benefits and harms of either digital mammography or magnetic resonance imaging (MRI) instead of film mammography as screening modalities for breast cancer. This is an I statement.

Management of women with cervical intraepithelial neoplasia or adenocarcinoma in situ

Recommendations

Major Recommendations

The ratings of the strength of recommendation (A-E), the quality of the evidence (I-III), and terminology used by the consensus conference (recommended, preferred, acceptable, unacceptable) are defined at the end of the Major Recommendations.
Note from the American Society for Colposcopy and Cervical Pathology (ASCCP): The management of low-grade cervical intraepithelial neoplasia (CIN) grade 1 has been modified significantly since 2001. Previously, management depended on whether colposcopy was satisfactory and treatment using ablative or excisional was acceptable for all women with CIN 1. In the new guidelines, cytological follow-up is the only recommended management option for women with CIN 1 who have low-grade referral cervical cytology, regardless of whether the colposcopic examination is satisfactory. Treatment is particularly discouraged in adolescents. The basic management of women in the general population with CIN 2,3 underwent only minor modifications, but options for the conservative management of adolescents with CIN 2,3 have been expanded. Moreover, management recommendations for women with biopsy-confirmed adenocarcinoma in situ are now included.

The management of erectile dysfunction

Recommendations

Major Recommendations

Definitions of the strength of the recommendations (standard, recommendation, option) are defined at the end of the "Major Recommendations" field.
Initial Management and Discussion of Treatment Options with Patients
Recommended Therapies and Patient Information
Standard: The management of erectile dysfunction begins with the identification of organic comorbidities and psychosexual dysfunctions; both should be appropriately treated or their care triaged. The currently available therapies that should be considered for the treatment of erectile dysfunction include the following: oral phosphodiesterase type 5 (PDE5) inhibitors, intra-urethral alprostadil, intracavernous vasoactive drug injection, vacuum constriction devices, and penile prosthesis implantation. These appropriate treatment options should be applied in a stepwise fashion with increasing invasiveness and risk balanced against the likelihood of efficacy (based on review of data and Panel consensus).
Standard: The patient and, when possible, his partner should be informed of the relevant treatment options and their associated risks and benefits. The choice of treatment should be made jointly by the physician, patient, and partner, when possible, taking into consideration patient preferences and expectations and the experience and judgment of the physician (based on Panel consensus).

Incontinence in women

Major Recommendations

Note from the European Association of Urology (EAU) and the National Guideline Clearinghouse (NGC): The following recommendations were current as of the publication date. However, because EAU updates their guidelines frequently, users may wish to consult the EAU Web site for the most current version available.
Note from the National Guideline Clearinghouse (NGC) and the European Association of Urology (EAU): Following the complete updating for print in 2009 of the EAU Guidelines on Urinary Incontinence, the Incontinence Guidelines Writing Panel considered it helpful to provide an addendum to the Guidelines on the role of weight loss in urinary incontinence. Recent high-quality scientific publications underpin the statements made here. The new recommendation is labeled 2010 Addendum.
Levels of evidence (1a-4) and the grades of recommendations (A-C) are provided at the end of the "Major Recommendations" field.

Management of acute coronary syndromes

Recommendations

Major Recommendations

Notes from the National Guideline Clearinghouse (NGC)

• In March 2008, the National Heart Foundation of Australia/Cardiac Society of Australia and New Zealand released an addendum to their 2006 guidelines for the management of acute coronary syndrome, highlighting evidence "that strengthens the recommendations in the guidelines or provides alternatives to current recommended practice that should be considered based on the circumstances of the individual patient and setting." This new information is presented under the heading "2008 Addendum: Implications of the Findings."
• The National Heart Foundation of Australia/Cardiac Society of Australia and New Zealand released an additional addendum in August 2011. This addendum "summarises clinical trial evidence published since 2007 that is relevant to the recommendations contained in the Heart Foundation's Guidelines for the management of acute coronary syndromes 2006 and 2007 addendum to the National Heart Foundation of Australia/Cardiac Society of Australia and New Zealand Guidelines for the management of acute coronary syndromes 2006. These recommendations are directed at the management of patients with spontaneous acute coronary syndromes, rather than those occurring as a result of other conditions (e.g., anaemia or thyrotoxicosis) where management may be directed at the underlying cause. This new information is presented under the heading "2011 Addendum."
• Definitions for the strength of recommendations and levels of evidence for the 2011 addendum are provided at the end of the "Major Recommendations" field.

Gonococcal infections

Description of Methods Used to Formulate the Recommendations

2010 Guideline
Centers for Disease Control and Prevention (CDC) staff members and invited consultants (including public- and private-sector professionals knowledgeable in the treatment of patients with sexually transmitted diseases [STDs]) assembled in Atlanta, Georgia, in April 2009, for a meeting where all evidence from the literature reviews pertaining to STD management was discussed.
Specifically, participants identified key questions regarding STD treatment that emerged from the literature reviews and discussed the information available to answer those questions. Discussion focused on four principal outcomes of STD therapy for each individual disease: 1) treatment of infection based on microbiologic eradication, 2) alleviation of signs and symptoms 3) prevention of sequelae, and 4) prevention of transmission. Cost-effectiveness and other advantages (e.g., single-dose formulations and directly observed therapy [DOT]) of specific regimens also were discussed. The consultants then assessed whether the questions identified were relevant, ranked them in order of priority, and answered the questions using the available evidence. In addition, the consultants evaluated the quality of evidence supporting the answers on the basis of the number, type, and quality of the studies.
2012 Addendum
During September–December 2011, CDC and five external Gonococcal Isolate Surveillance Project (GISP) principal investigators, each with Neisseria gonorrhoeae–specific expertise in surveillance, antimicrobial resistance, treatment, and antimicrobial susceptibility testing, reviewed antimicrobial susceptibility trends in GISP through August 2011 to determine whether to update CDC's current recommendations for treatment of uncomplicated gonorrhea.

Caregiving strategies for older adults with delirium, dementia and depression

Description of Methods Used to Formulate the Recommendations

June 2004 Guideline
In January of 2003, a panel of nurses and researchers with expertise in practice, education, and research related to gerontology and geriatric mental health care was convened under the auspices of the Registered Nurses' Association of Ontario (RNAO). At the onset, the panel discussed and came to a consensus on the scope of the best practice guideline.
Following the extraction of identified recommendations and content from eight guidelines, the panel underwent a process of review, discussion, and consensus on the key evidence-based assessment criteria.
The panel members divided into subgroups to undergo specific activities using the short-listed guidelines, other literature, and additional resources for the purpose of drafting recommendations for nursing interventions. This process yielded a draft set of recommendations. The panel members as a whole reviewed the recommendations, discussed gaps and available evidence, and came to consensus on a draft guideline.

Best practice guideline for the subcutaneous administration of insulin in adults with type 2 diabetes

Description of Methods Used to Formulate the Recommendations

June 2004 Guideline
In January of 2003, a panel of nurses with expertise in practice and education related to diabetes, from institutional and community settings, was established by the Registered Nurses Association of Ontario (RNAO). At the onset, the panel discussed and came to consensus on the scope of the best practice guideline.
The panel members divided into subgroups to undergo specific activities using the short-listed guidelines, other literature, and additional resources for the purpose of drafting recommendations for nursing interventions. This process yielded a draft set of recommendations. The panel members as a whole reviewed the recommendations, discussed gaps and available evidence, and came to consensus on a draft guideline.

Prevention of post-lumbar puncture headaches

Major Recommendations

2000 Guideline
The quality of evidence ratings, I-III, and the strength of recommendations (Type A-Type E) are defined at the end of the "Major Recommendations" field.

1. Class I and Class II data in the anesthesiology literature and either Class I or Class II data in the neurology series show that smaller needle size is associated with reduced frequency of post-lumbar puncture headache (PLPHA) (Type A). The actual choice of needle size will be influenced by balancing other considerations, such as ease of use, the need to measure pressures, and the flow rate, with the desire to prevent PLPHA.
2. Class I data in the anesthesiology literature show that, when using a cutting needle, ensuring that the bevel direction is parallel to the dural fibers reduces the frequency of PLPHA. (Type A).
3. Class I data using a noncutting needle show that replacement of the stylet before the needle is withdrawn is associated with lower frequency of PLPHA. (Type A).
4. For spinal anesthesia, Class I data show that non-cutting needles reduce the frequency of PLPHA (Type A). However, for diagnostic lumbar punctures (LPs), the data are inconclusive.
5. Class I and Class II data have not demonstrated that the duration of recumbency following a diagnostic lumbar puncture influences the occurrence of PLPHA.
6. There is no evidence that the use of increased fluids prevents PLPHA.

Management of patients with ST-elevation myocardial infarction

Consensus guidelines for the management of women with abnormal cervical cancer screening tests

Recommended Management of Women with Atypical Squamous Cells of Undetermined Significance (ASC-US)
General Management Approaches
A program of DNA testing for high-risk (oncogenic) types of HPV, repeat cervical cytologic testing, or colposcopy are all acceptable methods for managing women over the age of 20 years with ASC-US. (AI) When liquid-based cytology is used or when cocollection for HPV DNA testing can be done, "reflex" HPV DNA testing is the preferred approach. (AI)
Women with ASC-US who are HPV DNA negative can be followed up with repeat cytologic testing at 12 months. (BII) Women who are HPV DNA positive should be managed in the same fashion as women with LSIL and be referred for colposcopic evaluation. (AII) Endocervical sampling is preferred for women in whom no lesions are identified (BII) and those with an unsatisfactory colposcopy (AII) but is acceptable for women with a satisfactory colposcopy and a lesion identified in the transformation zone. (CII) Acceptable postcolposcopy management options of women with ASC-US who are HPV positive, but in whom cervical intraepithelial neoplasia (CIN) is not identified, are HPV DNA testing at 12 months or repeat cytological testing at 6 and 12 months. (BII) It is recommended that HPV DNA testing not be performed at intervals less than 12 months. (EIII)