Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Those caring for children should recognize that cutaneous findings are common in children with host defense defects. Atopic dermatitis, recurrent or persistent pyodermas, candidiasis and lupus-like syndromes, should signal the possibility of host defense deficiencies. Particularly the findings of atopic dermatitis and recurrent skin abscesses should alert the clinician to determine serum IgE levels and neutrophil chemotaxis in such patients. The triad of generalized seborrheic dermatitis, failure to thrive, and diarrhea in an infant should bring to mind Leiner disease or severe combined immunodeficiency disease.
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PMID:Cutaneous manifestations of defective host defenses. 32 7

An infant with short stature and progressive skin lesions of cheeks and dorsum of the hands is described. Further problems such as recurrent diarrhoea and respiratory infections suggested zinc-deficiency, malabsorption-syndrome, Bloom syndrome and early Lupus Erythematosus respectively. Finally Rothmund-Thomson syndrome was diagnosed. This rare genetic disorder is characterized by variable expression of typical cutaneous changes, cataracts, skeletal anomalies, short stature, abnormal hair growth and defective nails and teeth, mental retardation, hypogonadism and a typical facial appearance.
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PMID:[An infant with short stature and red cheeks (Rothmund-Thomson syndrome)]. 177 48

Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patient's mother had an undiagnosed subacute cutaneous lupus erythematosus. The various forms of onset of neonatal lupus erythematosus syndrome are emphasized.
Lupus 1991 Nov
PMID:Neonatal lupus erythematosus with multisystem organ involvement preceding cutaneous lesions. 184 64

Fourteen patients with poor-prognosis cervical cancer were treated with concurrent chemotherapy (cisplatin and mitomycin-C), external radiation therapy (RT), and high-dose-rate (HDR) brachytherapy. Pelvic RT was delivered as (a) external-beam radiation (four-field box technique, 40.0 Gy), (b) brachytherapy using HDR 60Co or 192Ir (3.80 Gy/fraction, thrice weekly; total dose, 46.83 Gy) with intrauterine stent, and (c) parametrial boost using an AP field with custom-fabricated step wedges. Post-radical-hysterectomy patients received 50.40 Gy external RT and 3.23 Gy/day vaginal cylinder HDR at 1/2-cm depth (total dose, 16.15 Gy). Complete clinical and radiographic response was noted in all evaluable patients who are alive with no evidence of disease, 3 to 27 months after completion of therapy (median, 9 months). Toxicity consisted of grade 2 to 3 hematologic toxicity (4 patients) and nausea and vomiting in all, but grade 3 in only 2 patients. One patient had grade 2 diarrhea. The only major complication (small bowel obstruction) occurred in a patient with lupus vasculitis. This pilot study demonstrates the feasibility of this regimen in an outpatient setting with acceptable toxicity. More prolonged follow-up of our patients is required to determine its impact on long-term survival.
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PMID:High-dose-rate afterloading brachytherapy, external radiation therapy, and combination chemotherapy in poor-prognosis cancer of the cervix. 195 85

Fourteen cases of primary lupus-associated protein-losing enteropathy have now been reported in the English-language literature. These cases were reviewed to find any consistent pattern of presentation. Lupus-associated protein-losing enteropathy typically occurs in young women, and is characterized by the onset of profound edema and hypoalbuminemia. In many cases it is the first obvious manifestations of systemic lupus erythematosus. Diarrhea is present about 50% of the time, but steatorrhea is absent. Diagnosis of protein-losing enteropathy can be successfully made by radioisotopic studies or 24-hour stool alpha 1-antitrypsin clearance. A normal lymphocyte count, elevated serum cholesterol, and absence of lymphangiectasia on intestinal biopsy help distinguish lupus-associated protein-losing enteropathy from protein-losing enteropathies due to direct or indirect lymphatic obstruction. Normal endoscopy and mucosal biopsy can rule out protein loss due to mucosal disruption. Prognosis appears to be excellent with corticosteroids, although other immunosuppressive therapies have been successfully used. A typical and illustrative case is presented as a focal point for review and discussion.
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PMID:Lupus-associated protein-losing enteropathy. 206

Systemic lupus erythematosus was diagnosed in a dog with concurrent nematode infection. The clinical signs of disease were unusually severe and included multiple neurologic deficits, polyarthritis, and weight loss. The dog was thrombocytopenic, and serotest results included positive lupus erythematosus test, positive rheumatoid factor test, positive antinuclear antibody test, hypergammaglobulinemia, and high platelet-associated IgG concentration. After treatment of hookworm, whipworm, and heartworm infections concurrently with corticosteroid and empiric treatment, the dog's condition improved. However, 10 days later, cyclophosphamide administration was necessary for continued immunosuppression. The dog was euthanatized because of progressive deterioration and development of canine coronavirus diarrhea. Serotest data generated from the dog's serum obtained at the time of referral suggested that autoantibodies and circulating immune complexes may have included IgE isotypes.
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PMID:Systemic autoimmune disease and concurrent nematode infection in a dog. 255 67

Antibodies to Extractable Nuclear Antigens (ENAs) namely Sm, nRNP, SS-A and SS-B were studied in 397 patients with various connective tissue diseases (CTD), 146 patients with inflammatory polyarthropathies, 16 cases of systemic vasculitides, and 39 normal subjects using counterimmunoelectrophoresis and double immunodiffusion methods. Anti-ENA antibodies were positive in 40.8 percent cases of Systematic lupus erythematosus (SLE) (n = 191), 36.4 percent of overlap CTD (OCTD, n = 44), 27.8 percent of Sjogren's syndrome (n = 18), 10.6 percent of progressive systemic sclerosis (PSS, n = 66) and 2.7 percent of rheumatoid arthritis (n = 111) patients. The correlation of these antibodies with disease features was done. The significant finding was negative association of anti-nRNP antibodies (when present alone) with renal involvement. Anti-Sm antibodies did not correlate with any disease feature. The other associations included correlation of anti-nRNP with pulmonary parenchymal lesions, anti-SS-A with serositis and pulmonary hypertension, and anti-SS-B with myocarditis and recurrent diarrhoea. We conclude that Anti-ENAs may correlate with certain subsets of these diseases but the subject is controversial.
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PMID:Antibodies to extractable nuclear antigens in connective tissue disorders in India: prevalence and clinical correlations. 262 64

A case of lupus cystitis in a 23-year-old male is reported. The patient began to complain of diarrhea and vomiting in October, 1985. When the diagnosis of systemic lupus erythematosus (SLE) was established at the Department of Internal Medicine in our hospital, he was referred to our clinic for examination of pollakisuria on November 22. DIP revealed a loss of bladder distensibility, and bilateral hydronephrosis and hydroureter. Transurethral cold cup biopsies revealed subcutaneous edema. A diagnosis of lupus cystitis was made and he was treated with steroids, which resulted in symptomatic and radiographic improvement.
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PMID:[A case of lupus cystitis]. 273 71

Spontaneous bacterial peritonitis (SBP), a fascinating disease that had been reported perhaps 50 times in varying guises over the preceding century, suddenly burst forth in the 1960s and was recognized in clusters of cases almost simultaneously in Paris, London, and West Haven, Connecticut. The spectrum of the disease has broadened. Initially, it was associated almost exclusively with alcoholic cirrhosis, but it has now been found in association with posthepatitic cirrhosis, cryptogenic cirrhosis, chronic active liver disease, and, occasionally, in biliary cirrhosis and cardiac cirrhosis. Recently, it has been reported in alcoholic hepatitis and acute viral hepatitis. It occurs occasionally in malignant ascites and in pancreatitis in the absence of cirrhosis. It is surprisingly common in disseminated lupus, in which it occurs relatively more commonly than in alcoholic cirrhosis. A similar syndrome, primary peritonitis, occurs frequently in children with nephrotic ascites. The clinical pattern of SBP has broadened. Initially it consisted of abdominal pain, fever, rebound tenderness, hypoactive bowel sounds, hypotension, encephalopathy, and cloudy ascites with large numbers of polymorphonuclear leukocytes in ascitic fluid. Each and every symptom, sign, and laboratory abnormality may be absent; indeed, the syndrome can be completely silent. Initially, the causative bacteria appeared to be almost exclusively enteric, but now the list of bacteria isolated in cases of SBP looks like a bacteriology textbook. Anaerobes are rare. Multiple organisms usually suggest nonspontaneous origin such as perforation or vasopressin induction. The differentiation between spontaneous and nonspontaneous bacterial peritonitis is crucial in the differential diagnosis. The great majority of cases of SBP develop in the hospital, 80% more than one week after admission. It is therefore a nosocomial disease that may be precipitated by procedure-induced bacteremia, gastrointestinal bleeding, or diarrhea, and it tends to occur in patients with low ascitic fluid protein (complement) concentrations and severe portal-systemic shunting.
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PMID:Spontaneous bacterial peritonitis: variant syndromes. 368 33

The antimalarials, chloroquine, hydroxychloroquine, and quinacrine, are used primarily for malaria; but they can be beneficial for cutaneous lupus erythematosus (LE), polymorphous light eruption, solar urticaria, and porphyria cutanea tarda. Antimalarials bind to deoxyribonucleic acid (DNA) which prevents DNA and ribonucleic acid (RNA) polymerase reactions and DNA heat inactivation; and they inhibit the LE cell phenomenon, antinuclear antibody reactions, and suppress lymphocyte transformation. By competing with calcium ion, they stabilize membranes and have an anesthetic effect. Their anti-inflammatory potential is due to their inhibition of hydrolytic enzymes, stabilization of lysosomes, interference with prostaglandin synthesis, blocking of chemotaxis, and antagonism of histamine responses. The antimalarials have no sunscreening properties. The most common toxic effects are cutaneous pigmentation, nausea, vomiting, diarrhea, mild ileus, and cycloplegia. There has been a reluctance to use chloroquine and hydroxychloroquine because of the possibility of retinopathy. However, if the "safe" daily dose limit of chloroquine, 2 mg per pound of body weight, and of hydroxychloroquine, 3.5 mg per pound of body weight, is followed, the chance of retinopathy is slight. Quinacrine does not cause retinopathy, but it has more cutaneous side effects than the other two agents.
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PMID:Antimalarials. 616 44


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