| Marinesco-Sjogren syndrome | <syndrome> A rare neurologic disorder characterised by cerebellolental degeneration with mental retardation; autosomal recessive inheritance. Synonym: cataract-oligophrenia syndrome, Marinesco-Sjogren syndrome, Torsten Sjogren's syndrome. (05 Mar 2000) |
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| sjogren-larsson syndrome | <syndrome> Rare syndrome of autosomal recessive inheritance characterised by ichthyosis, mental retardation, and spastic pyramidal symptoms. It is associated with a defect in fatty alcohol metabolism. (12 Dec 1998) |
| Sjogren's syndrome | <syndrome> An immunologic disorder characterised by progressive destruction of the exocrine glands (sweat glands, lacrimal glands, salivary glands). Symptoms include dry eyes, dry mouth, persistent cough (dry airways) and lack of saliva. Approximately 30% also have rheumatoid arthritis. Kidney involvement (kidney dysfunction) is seen in 40% of patients. Sjogren's syndrome affects predominately females in their thirties to forties. The lungs, peripheral nerves, blood vessels (vasculitis) and thyroid may also be affected. (27 Sep 1997) |
| sjogren syndrome | <radiology> Chronic systematic inflammatory disorder of unknown aetiology characterised by dryness of mucous membranes, primary Sjogren syndrome: without underlying autoimmune disease, recurrent parotitis in children, sicca syndrome (xerophthalmia and xerostomia), secondary Sjogren syndrome: connective tissue diseases (RA, systemic lupus erythematosus, PSS, psoriasis, etc), lymphoprolifertive disorders (lymphocytic interstitial pneumonitis, pseudolymphoma, lymphoma, Waldenstrom macroglobulinaemia) Findings: CXR: reticulododular pattern, patchy consolidation/atelactisis, pneumonitis, with or without pleural effusion, ultrasound: multiple scattered parotid cysts bilaterally, enlarged lacrimal glands (12 Dec 1998) |
| Torsten Sjogren's syndrome | <syndrome> A rare neurologic disorder characterised by cerebellolental degeneration with mental retardation; autosomal recessive inheritance. Synonym: cataract-oligophrenia syndrome, Marinesco-Sjogren syndrome, Torsten Sjogren's syndrome. (05 Mar 2000) |
| Gougerot-Sjogren disease | <syndrome> An immunologic disorder characterised by progressive destruction of the exocrine glands (sweat glands, lacrimal glands, salivary glands). Symptoms include dry eyes, dry mouth, persistent cough (dry airways) and lack of saliva. Approximately 30% also have rheumatoid arthritis. Kidney involvement (kidney dysfunction) is seen in 40% of patients. Sjogren's syndrome affects predominately females in their thirties to forties. The lungs, peripheral nerves, blood vessels (vasculitis) and thyroid may also be affected. (27 Sep 1997) |
| Sjogren, Henrik | <person> Swedish ophthalmologist, *1899. See: Sjogren's disease, Sjogren's syndrome, Gougerot-Sjogren disease. (05 Mar 2000) |
| Sjogren's disease | <syndrome> An immunologic disorder characterised by progressive destruction of the exocrine glands (sweat glands, lacrimal glands, salivary glands). Symptoms include dry eyes, dry mouth, persistent cough (dry airways) and lack of saliva. Approximately 30% also have rheumatoid arthritis. Kidney involvement (kidney dysfunction) is seen in 40% of patients. Sjogren's syndrome affects predominately females in their thirties to forties. The lungs, peripheral nerves, blood vessels (vasculitis) and thyroid may also be affected. (27 Sep 1997) |
| Sjogren, Torsten | <person> Swedish physician, 1859-1939. See: Sjogren-Larsson syndrome, Torsten Sjogren's syndrome, Marinesco-Sjogren syndrome. (05 Mar 2000) |
| Spielmeyer-Sjogren disease | Cerebral sphingolipidosis, late juvenile type. (05 Mar 2000) |
| Aarskog-Scott syndrome | A syndrome of ocular hypertelorism, anteverted nostrils, broad upper lip, saddle-bag scrotum, and laxity of ligaments resulting in genu recurvatum, flat feet, and hyperextensible fingers; X-linked and autosomal dominant forms. Synonym: Aarskog-Scott syndrome. (05 Mar 2000) |
| Aarskog syndrome | <syndrome> Grier et al. (1983) reported father and 2 sons with typical Aarskog syndrome, including short stature, hypertelorism, and shawl scrotum. They tabulated the findings in 82 previous cases. X-linked recessive inheritance has been repeatedly suggested. The family reported by Welch (1974) had affected males in 3 consecutive generations. Thus, there is either genetic heterogeneity or this is an autosomal dominant with strong sex-influence and possibly ascertainment bias resulting from use of the shawl scrotum as a main criterion. Stretchable skin was present in the cases of Grier et al. (1983). Teebi et al. (1993) reported the case of an affected mother and 4 sons (including a pair of monozygotic twins) by 2 different husbands. They suggested that the manifestations were as severe in the mother as in the sons and that this suggested autosomal dominant inheritance. Actually, the mother seemed less severely affected, compatible with X-linked inheritance. Clinical signs: Mild to moderate short stature,normocephaly, Widow's peak hair, maxillary hypoplasia, broad nasal bridge, anteverted nostrils, long philtrum, broad upper lip, curved linear dimple below the lower lip, hypertelorism, ptosis, down-slanted palpebral fissures, ophthalmoplegia, strabismus, hyperopic astigmatism, large cornea, floppy ears, lop-ears,cleft lip/palate, shawl scrotum, saddle-bag scrotum, cryptorchidism, brachydactyly, digital contractures, clinodactyly, mild syndactyly, transverse palmar crease, lymphoedema of the feet, ligamentous laxity, osteochondritis dissecans, proximal finger joint hyperextensibility, flexed distal finger joints, genu recurvatum, flat feet, stretchable skin, cervical spine hypermobility, odontoid anomaly, macrocytic anaemia, hemochromatosis, hepatomegaly, portal cirrhosis, imperforate anus, rectoperineal fistula, interstitial pulmonary disease, sternal deformity. Inheritance: Sex-influenced autosomal dominant form, also X-linked form. (05 Aug 1998) |
| abdominal muscle deficiency syndrome | <syndrome> Congenital absence (partial or complete) of abdominal muscles, in which the outline of the intestines is visible through the protruding abdominal wall; in males, genitourinary anomalies (urinary tract dilation and cryptorchidism) are also found; genetics unclear. (05 Mar 2000) |
| abstinence syndrome | <syndrome> A constellation of physiologic changes undergone by persons or animals who have become physically dependent on a drug or chemical due to prolonged use at elevated doses, but who are abruptly deprived of that substance. The abstinence syndrome varies with the drug to which dependence has developed. Generally the effects observed are in an opposite direction from those produced by the drug; e.g., the withdrawal syndrome from central nervous system depressants such as barbiturates and benzodiazepines consists of insomnia, restlessness, tremulousness, hallucinations, and, in the extreme, tonic-clonic convulsions which may prove fatal. The onset time and severity of the abstinence syndrome depend upon how rapidly the drug disappears from the body. (05 Mar 2000) |
| Achard syndrome | <syndrome> Arachnodactyly with small receding mandible, broad skull, and joint laxity limited to the hands and feet; genetics unclear. (05 Mar 2000) |