Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunotherapy with interleukin (IL)-2 possesses great potential in the treatment of immune-mediated diseases and cancers. However, only a few reports on a small number of children have appeared in the literature. From March 1988 to March 1989, 11 children and adolescents were treated with IL-2. They included 1 patient with hepatocellular carcinoma, 1 with hepatoblastoma, 6 with childhood atopic dermatitis, and 3 with juvenile rheumatoid arthritis. The dosages ranged from 10,000 to 50,000 U/kg every 8 hours by intravenous drip. The following side effects were observed: anorexia, fever, and chillness (100%), general malaise (82%), irritability (64%), diarrhea (100%), nausea and vomiting (73%), weight gain (82%), edema (82%), abdominal distension (73%), oliguria (82%), cough (91%), dyspnea (27%), pleural effusion (40%), hypotension (82%), skin eruption (82%), oral ulcer (18%), enlarged liver (73%) liver function abnormalities (82%), renal function impairment (36%), electrolyte imbalance (73%), anemia (91%), thrombocytopenia (54%), leukopenia (18%), and eosinophilia (73%). Immunologically, numbers of natural killer cells were increased and natural killer and lymphokine-activated killer cell activities were augmented after IL-2 treatment. There was a tendency for serum levels of IL-2 and receptor IL-2 to decrease, especially in patients with atopic eczema. Ten patients (91%) completed one course (9 to 12 days) of therapy, and the remaining patient interrupted the treatment because of intolerable adverse effects. Clinically, complete remission for 3 months was obtained in 1 juvenile rheumatoid arthritis patient, transient improvement (2 to 6 weeks) in all atopic dermatitis patients, minor response in the hepatoblastoma patient, and no response in the patient with hepatocellular carcinoma.
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PMID:Interleukin-2 immunotherapy in children. 217 36

Although there are various published studies on erythroderma from western and Asian countries, most of them have only included patients in the adult age groups. As we have an exclusively pediatric dermatology unit, we thought it would be intriguing to study the clinical, etiological and laboratory parameters of erythroderma in children. Seventeen erythroderma patients of both sexes were inducted into the study between 1993 to 1998. The mean age of onset was 3.3 years and the male:female ratio was 0.89:1. Eight (47%) of the patients were infants; 9 (53%) others belonged to the preschool and school going age group (age range between 1 to 12 years). An acute onset of the disease was seen in 47% of the patients while 53% of the patients had a chronic onset. The main presenting complaints were itching in 41% and burning in 18% of patients. Scalp involvement (71%), nail involvement (18%), and alopecia (6%) were the main cutaneous features observed while fever (53%), tachycardia (53%), pedal edema (12%), lymphadenopathy (18%), and hepatomegaly (12%) were the main systemic features observed in this study. Etiologically, drugs (29%), showed the highest incidence, followed equally (18%) by genodermatoses, psoriasis, and staphylococcal scalded skin syndrome (SSSS). Two (12%) patients had erythroderma due to atopic dermatitis, while one was (5%) due to infantile seborrheic dermatitis coexisting with dermatophytosis. Laboratory parameters contributed little towards diagnosis of the underlying dermatological condition. Thus, though erythroderma is a striking entity, it is yet uncommon in the pediatric age group. Because the drug induced group was the largest in this study, we recommend that drugs should be suspected as important causative factors of erythroderma in children.
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PMID:Erythroderma in children: a clinico-etiological study. 1048 5

An 18-month-old male infant was referred to us with pruritus from 6 months of age. He was being managed as atopic eczema with emollients and antihistamines without any response to treatment. On examination, he was found to have extensive scratch marks, mild hepatomegaly and no icterus. Blood investigations were suggestive of anicteric cholestatic liver disease. Liver biopsy was suggestive of progressive familial intrahepatic cholestasis type 3. He showed symptomatic improvement after starting ursodeoxycholic acid and fat-soluble vitamins and is under follow up.
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PMID:Chronic Pruritus in an 18-Month-Old Male Infant Due to Anicteric Cholestasis. 2808 65

L-histidine (HIS) is an essential amino acid with unique roles in proton buffering, metal ion chelation, scavenging of reactive oxygen and nitrogen species, erythropoiesis, and the histaminergic system. Several HIS-rich proteins (e.g., haemoproteins, HIS-rich glycoproteins, histatins, HIS-rich calcium-binding protein, and filaggrin), HIS-containing dipeptides (particularly carnosine), and methyl- and sulphur-containing derivatives of HIS (3-methylhistidine, 1-methylhistidine, and ergothioneine) have specific functions. The unique chemical properties and physiological functions are the basis of the theoretical rationale to suggest HIS supplementation in a wide range of conditions. Several decades of experience have confirmed the effectiveness of HIS as a component of solutions used for organ preservation and myocardial protection in cardiac surgery. Further studies are needed to elucidate the effects of HIS supplementation on neurological disorders, atopic dermatitis, metabolic syndrome, diabetes, uraemic anaemia, ulcers, inflammatory bowel diseases, malignancies, and muscle performance during strenuous exercise. Signs of toxicity, mutagenic activity, and allergic reactions or peptic ulcers have not been reported, although HIS is a histamine precursor. Of concern should be findings of hepatic enlargement and increases in ammonia and glutamine and of decrease in branched-chain amino acids (valine, leucine, and isoleucine) in blood plasma indicating that HIS supplementation is inappropriate in patients with liver disease.
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PMID:Histidine in Health and Disease: Metabolism, Physiological Importance, and Use as a Supplement. 3223 43