Ichthyosis, Sex Linked
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  Ichthyosis, Sex Linked



Ichthyosis, Sex Linked

   Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.

RELATED TERMS
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Chronic
Ongoing or recurring. Chronic medical conditions include diabetes, epilepsy, and chronic fatigue syndrome.

Ichthyosis
An inherited skin disorder that produces dry, rough, scaly skin.

Steroid
One of a group of naturally occurring or synthetic hormones which are complex lipids based on the cholesterol molecule, and which affect chemical processes in the body, growth, and sexual and other physiological functions. They include adrenal cortical, testicular, and ovarian hormones and their derivatives.



SIMILAR TERMS
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Ichthyoses
Any of several generalized skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis. Most are genetic, but some are acquired, developing in association with other systemic disease or genetic syndrome.

Ichthyoses, Harlequin
A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate.

Ichthyoses, Lamellar
A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate.

Ichthyoses, Sex-Linked
Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.

Ichthyoses, X-Linked
Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.

Ichthyosiform Erythroderma, Bullous Congenital
A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by erythroderma and severe hyperkeratosis. It is manifested at birth by blisters followed by the appearance of thickened, horny, verruciform scales over the entire body, but accentuated in flexural areas.

Ichthyosiform Erythroderma, Congenital
Designation for several severe forms of ichthyosis, present at birth, that are characterized by hyperkeratotic scaling. Infants may be born encased in a collodion membrane which begins shedding within 24 hours. This is followed in about two weeks by persistent generalized scaling. The forms include bullous (HYPERKERATOSIS, EPIDERMOLYTIC), non-bullous (ICHTHYOSIS, LAMELLAR), wet type, and dry type.

Ichthyosiform Erythroderma, Nonbullous Congenital
A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate.

Ichthyosiform Erythrodermas, Congenital
Designation for several severe forms of ichthyosis, present at birth, that are characterized by hyperkeratotic scaling. Infants may be born encased in a collodion membrane which begins shedding within 24 hours. This is followed in about two weeks by persistent generalized scaling. The forms include bullous (HYPERKERATOSIS, EPIDERMOLYTIC), non-bullous (ICHTHYOSIS, LAMELLAR), wet type, and dry type.

Ichthyosis
An inherited skin disorder that produces dry, rough, scaly skin.

Ichthyosis Vulgaris
Most common form of ICHTHYOSIS characterized by prominent scaling especially on the exterior surfaces of the extremities. It is inherited as an autosomal dominant trait.

Ichthyosis Vulgaris, Dominant
Most common form of ICHTHYOSIS characterized by prominent scaling especially on the exterior surfaces of the extremities. It is inherited as an autosomal dominant trait.

Ichthyosis, Harlequin
A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate.

Ichthyosis, Lamellar
A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate.

Ichthyosis, Sex-Linked
Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.

Ichthyosis, X Linked
Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.

Ichthyosis, X-Linked
Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.



PREVIOUS AND NEXT TERMS
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IIIs, Mucopolysaccharidosis
Mucopolysaccharidosis characterized by heparitin sulfate in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.

IIIs, Mucolipidosis
A group of inherited metabolic diseases characterized by the accumulation of excessive amounts of acid mucopolysaccharides, sphingolipids, and/or glycolipids in visceral and mesenchymal cells. Abnormal amounts of sphingolipids or glycolipids are present in neural tissue. Mental retardation and skeletal changes, most notably dysostosis multiplex, occur frequently. (From Joynt, Clinical Neurology, 1992, Ch56, pp36-7)

ICYP
A highly selective and specific beta antagonist that is used to characterize beta-adrenoceptors.

IgA, Secretory
The principle immunoglobulin in exocrine secretions such as milk, respiratory and intestinal mucin, saliva and tears. The complete molecule (around 400 kD) is composed of two four-chain units of IMMUNOGLOBULIN A, one SECRETORY COMPONENT and one J chain (IMMUNOGLOBULINS, J-CHAIN).

ICRF187
An antimitotic agent with immunosuppressive properties. Dexrazoxane, the (+)-enantiomorph of razoxane, provides cardioprotection against anthracyline toxicity. It appears to inhibit formation of a toxic iron-anthracyline complex.

Ichthyosis, Sex Linked

IAP Element
Genes of IAP elements (a family of retrovirus-like genetic elements) which code for virus-like particles (IAPs) found regularly in rodent early embryos. (""Intracisternal"" refers to the cisternae of the endoplasmic reticulum.) Under certain circumstances, such as DNA hypomethylation they are transcribed. Their transcripts are found in a variety of neoplasms, including plasmacytomas, neuroblastoma, rhabdomyosarcomas, teratocarcinomas, and colon carcinomas.

IL 13
T-lymphocyte-derived cytokine that produces proliferation, immunoglobulin isotype switching, and immunoglobulin production by immature B-lymphocytes. It appears to play a role in regulating inflammatory and immune responses.

IL 2 Receptor
Receptors present on activated T- and B-cells as a complex consisting of a 55 kD peptide, which reacts with the anti-Tac monoclonal antibody, and a 75 kD non-Tac interleukin-2-binding peptide. The receptor is present in two forms, one with a very high affinity and the other with low affinity for IL-2. The high-affinity form appears to mediate exclusively the growth-promoting response to IL-2. The receptor is present in large numbers on resting HUMAN T-LYMPHOTROPIC VIRUS 1 leukemia cells, but not on normal resting cells.

IL 12
A heterodimeric cytokine that stimulates the production of interferon gamma from T-cells and natural killer cells, and also induces differentiation of Th1 helper cells. It is an initiator of cell-mediated immunity.

IL 1 Receptors
Specific molecular sites or structures on cells with which interleukin-1 reacts or to which it binds to modify the function of the cells. The IL-1 receptor on T-lymphocytes and fibroblasts is composed of a single polypeptide chain that binds both IL-1 alpha and IL-1 beta. The molecular weight of this high-affinity receptor is believed to be 80 kD.

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