Diagnostic Criteria for Autoimmune Lymphoproliferative Syndrome (ALPS)

Autoimmune lymphoproliferative syndrome (ALPS) is characterized by dysregulation of the immune system due to an inability to regulate lymphocyte homeostasis through the process of lymphocyte apoptosis (a form of programmed cell death). The consequences of this include lymphoproliferative disease, manifested by lymphadenopathy, hepatomegaly, splenomegaly, and an increased risk of lymphoma, as well as autoimmune disease, typically involving blood cells.
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Diagnostic Criteria for Zollinger-Ellison Syndrome (ZES)

Diagnostic criteria for ZES include the following:

  1. Elevated levels of Basal Acid Output (BAO), greater than 15 mEq in unoperated patients and greater than 5 mEq if previous acid-reducing surgery has been performed;
  2. Elevated level of fasting serum gastrin (>100 pg/mL until 1994, >200 pg/mL since 1994);
  3. Abnormal results from stimulation testing with secretin (an increase of >200 pg/mL postinjection) or with calcium (an increase >395 pg/mL);
  4. Positive histologic confirmation of gastrinoma; or
  5. A combination of these criteria. Continue reading

Revised International Classification Criteria for Sjögren's Syndrome (SS)

I. Ocular symptoms: a positive response to at least one of the following questions:

  1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?

  2. Do you have a recurrent sensation of sand or gravel in the eyes?

  3. Do you use tear substitutes more than 3 times a day?

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Case Definition of Streptococcal Toxic-Shock Syndrome (Streptococcal TSS) and Necrotizing Fasciitis*

I. Streptococcal TSS

A. Isolation of group A Streptococcus

1. From a sterile site

2. From a nonsterile body site

B. Clinical signs of severity

1. Hypotension

2. Clinical and laboratory abnormalities (requires two or more of the following):

a) Renal impairment

b) Coagulopathy

c) Liver abnormalities

d) Acute respiratory distress syndrome

e) Extensive tissue necrosis, i.e., necrotizing fasciitis

f) Erythematous rash

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Case Definition of Staphylococcal Toxic Shock Syndrome (TSS)

An illness with the following clinical manifestations:

  1. Fever: temperature > 38.9º C (102º F)

  2. Rash: diffuse macular erythroderma

  3. Desquamation: 1-2 weeks after onset of illness, particularly palms and soles

  4. Hypotension: systolic blood pressure < 90 mm Hg for adults or less than fifth percentile by age for children <16 years of age; orthostatic drop in diastolic blood pressure greater than or equal to 15 mm Hg from lying to sitting, orthostatic syncope, or orthostatic dizziness

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Diagnostic Criteria of Paraneoplastic Neurological Disorder (PND) of the CNS

Definite PND

  1. Classic syndrome with cancer diagnosed within 5 years of neurological symptom development
  2. Nonclassic syndrome that resolves or significantly improves after cancer treatment
  3. Nonclassic syndrome with cancer diagnosed within 5 years of neurological symptom development and positive antineuronal antibodies
  4. Neurological syndrome (classic or not) without cancer and with well-characterized antineuronal antibodies
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Syndromes or Disease Entities That Have Been Associated with Polycystic Ovaries

A- Hyperandrogenism

o Steroidogenic enzyme deficiencies

§ Congenital adrenal hyperplasia

§ Aromatase deficiency

o Androgen-secreting tumors

§ Ovarian

§ Adrenal

o Exogenous androgens

§ Anabolic steroids

§ Transsexual hormone replacement

o Other

§ Acne

§ Idiopathic hirsutism

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Clinical Features of Nephrotic and Nephritic Syndrome

Clinical Features of Nephrotic Syndrome

The nephrotic syndrome is a clinical complex characterized by:

  • proteinuria of >3.5 g per 1.73 m2 per 24 h (in practice, >3.0 to 3.5 g per 24 h),
  • hypoalbuminemia,
  • edema,
  • hyperlipidemia,
  • lipiduria, and
  • hypercoagulability.

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