User Guide

The International Classification of Pediatric Endocrine Diagnoses (ICPED) is divided into 14 chapters based on the structure of the original ESPE classification from which it was derived. The first 13 chapters address broad areas of traditional relevance to clinical pediatric endocrine practice; the final chapter is focused on syndromic conditions that include major endocrine features. Each chapter is divided into 2 to 8 major subsections, denoted by capital letters after the chapter number. For example, in Chapter 1 (Short Stature) section 1A is primary growth failure, section 1B is secondary growth failure, and section 1C is idiopathic short stature.

The ICPED has a tree-like hierarchical structure using codes that combine numbers and letters for clarity (for example, 4A.2b.1c). Each successive level of the “tree” represents a more specific diagnosis than the level above it.

The same diagnostic entity may be coded in several chapters within ICPED, depending on the specific clinical focus. For example, Turner syndrome has entries in Chapter 1 (Short Stature), Chapter 3 (Puberty), Chapter 4 (Sex and Gender), Chapter 5 (Overweight and Obesity), Chapter 10 (Ovary), Chapter 11 (Glucose and Triglyceride Metabolism) and Chapter 14 (Syndromes with Endocrine Features). The entry in a specific chapter therefore also includes hyperlinked cross references to codes for entries in other ICPED chapters, denoted at the top of the column as “ICPED cross-ref”.

In addition to the internal cross references, where available the system also includes cross references to similar entries in Online Mendelian Inheritance in Man (OMIM^®), denoted at the top of the column as “OMIM^® ref” and to the International Classification of Diseases, revision 10 (ICD-10), denoted at the top of the column as “ICD-10 ref”. Following the hyperlink to the external resource opens the relevant page in a new screen. Clicking the back button on the browser returns the user to the original location in ICPED.

Exclusions

Where applicable, diagnoses excluded from a particular chapter are noted at the beginning of the chapter or section, designated by “Exclusions”, followed by the excluded diagnosis/es and links to their primary locations within ICPED.

Disorders Classified Elsewhere

Conditions classified elsewhere in ICPED are designated at the beginning of a section with a code ending in “0” or “a”, depending on the level of the tree at which this occurs. For example, hypergonadotropic hypogonadism due to conditions classified elsewhere in ICPED is coded as 9A.0, with Klinefelter syndrome (9A.0a), Lawrence-Moon-Bardet-Biedl syndrome (9A.0b), Noonan syndrome (9A.0c) and Steinert myotonic dystrophy (9A.0d) listed and links to the locations of their primary codes. As an example of a condition classified elsewhere at a lower level of the tree, peripheral isosexual precocious puberty (3A.2) has a lower level code 3A.2a, representing peripheral isosexual precocious puberty due to disorders primarily classified elsewhere.

Other Specified and Other Unspecified

Because there will always be situations in which no perfect diagnosis can be found in the current version of the ICPED, the system allows for coding a condition as “Other, specified”, when the diagnosis is known to the clinician but not listed in the system. These codes are denoted by letter ‘y’ or number ‘88’, depending on the level of the tree at which this occurs. For example, under congenital adrenal hyperplasia (8A.1) code 8A.1y is provided for “congenital adrenal hyperplasia, other specified”. As an example of this occurring at a lower level of the tree, under congenital isolated TSH deficiency (6A.2a) code 6A.2a.88 is provided for “congenital isolated TSH deficiency, other cause, specified”. A similar structure exists for “Other, unspecified”, which may be used when the diagnosis is unknown, denoted by codes ‘z’ or ‘99’; in this example the code would be 6A.2a.99 for “congenital isolated TSH deficiency, other cause, unspecified”.

For certain diagnoses coding hints (designated with the symbol) are provided to suggest additional codes that may be useful in specific clinical situations. For example, if a patient with growth hormone deficiency also has gonadotropin deficiency, a hint is provided for how to also code the gonadotropin deficiency.

For certain diagnoses, clicking on the symbol opens up a field where additional descriptive information is provided. provides general comments regarding the topic/diagnosis, examples relevant to the topic or hormonal data; provides brief clinical or phenotypic information; Synonyms lists the synonyms for the diagnosis (e.g. leprechaunism as a synonym for Donohue syndrome); provides key references.

Hormonal findings, provided in the when appropriate, are given in an abbreviated form. Hormone names are provided as standard abbreviations; the full name of the hormone can be seen by hovering over the abbreviation (e.g. hovering over “T” shows “testosterone”). Hormone concentrations are summarized using easily recognized symbols; for example, “increased” is indicated as “+”, “decreased” as “-”, and “normal” as “N”. As an example, T(+) means increased testosterone concentration.

The ICPED uses American English spelling because this is preferred by developers of information technology coding systems and is becoming the global standard for medical terminology. Reflecting the direction in which medical nomenclature has progressed, syndromes and conditions that bear the names of physicians and scientists are provided in the nominative rather than possessive form (e.g., Turner syndrome rather than Turner’s syndrome; Addison disease, rather than Addison’s disease).

To open a chapter click on the , the boxed chapter number, or the chapter heading. Similarly, to open a diagnosis click on the , the ICPED code or the diagnosis text. Multiple diagnoses can be open at the same time.

To search for a specific diagnosis, click on the Search tab to open a search box on the main screen, or type the search term into the smaller Search box on the top blue bar with icon. The search will provide a drop-down list of all instances where the word appears. For example typing “Noonan” into the Search box results in a list of 4 locations where the diagnosis is found. Click on one of the items in the drop-down list and then click the icon. The system will then return a list of all instances of this diagnosis, with their hierarchy and ICPED codes, listed in order of relevance. Clicking on the diagnosis code or condition name opens the diagnosis in a new page. Clicking the browser back button returns the user to the previous location.

To cite the ICPED as an entity in a reference list, use the following format: International Classification of Pediatric Endocrine Diagnoses (ICPED), 2015, ICPED Consortium, accessed [Day Month Year]; www.icped.org.

To cite a specific diagnosis in text, use the following format: International Classification of Pediatric Endocrine Diagnoses (ICPED), 2015, ICPED code 3A.1b.2e (example).