Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Progressive familial intrahepatic cholestasis (PFIC) presents in early childhood with pruritus, jaundice, hepatomegaly, and growth failure. Medical therapy is unsuccessful, with progression from cholestasis to hepatic fibrosis, cirrhosis, and ultimately death before the age of 10 years. Because of evidence that biliary diversion can arrest or reverse progression to hepatic fibrosis, we have used partial biliary diversion (PBD) as primary therapy in PFIC, reserving orthotopic liver transplantation (OLT) for children who have progressive disease or established cirrhosis. Seventeen children with PFIC (aged 2 months to 19 years) have been treated. PBD was performed in eight cases. In these procedures, a 10-cm properistaltic jejunal segment was anastomosed to the side of the gallbladder, terminating as an end stoma for the collection and discard of bile. Eleven patients with hepatic insufficiency (or end-stage cirrhosis) received OLT using standard techniques, at the average age of 4 years. Six of the eight children treated with PBD had complete resolution of clinical symptoms and remain well 1 to 13 years postoperatively. These six patients have conjugated bilirubin values of less than 0.3 mg/dL, normal transaminases, and a serum bile salt concentration of less than 10 nmol/mL. All have had either reversal or no progression of the hepatic fibrosis. Postoperative bleeding complications occurred in two (25%), which required reoperation. One patient had an adhesive intestinal obstruction that was managed surgically 9 months postoperatively. Two patients had no benefit from PBD, and all of them had severe bridging fibrosis (1) or cirrhosis (3). These and nine others with cirrhosis at the time of presentation received orthotopic liver transplantation; of these, eight are alive (1 to 5 years postoperatively). These results show the importance of establishing a correct diagnosis in children with cholestasis. Clinical symptoms often are severe in children with PFIC before the development of irreversible hepatic fibrosis. Because several patients who appear to have been cured with PBD initially were scheduled for OLT, it is important that transplant surgeons recognize the feasibility of this approach.
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PMID:Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease). 874 12

During filaria surveys Mansonella streptocerca was found for the first time in Uganda, in 12 widely scattered villages in the Bundibugyo district between the Ruwenzori mountains and the Ituri forest, an area not endemic for Onchocerca volvulus. The crude prevalence in 806 examined adult persons ranged from 5 to 89% with an average of 61%. In 148 children the prevalence was 36%. The geometric mean of microfilaria (mf) densities in adult mf carriers was 1.7 mf/mg skin and the community microfilarial loads ranged from 1.0 to 13.7 mf/skin snip. In 476 persons skin snips from the shoulder contained mf in 58% and those from the buttocks in 48%. Skin snips from 68 persons digested with collagenase showed that only 30-37% of the mf emerged during 24 h incubation at 30 degrees C in isotonic salt solution. An itching acute or more often chronic papular dermatitis, predominantly on the upper parts of the body, was the typical skin lesion observed in 24% of 177 M. streptocerca mf carriers.
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PMID:Occurrence and diagnosis of Mansonella streptocerca in Uganda. 908 84

A 39-year-old woman had a three-year history of recurrent bullous eruption localized to her left cheek. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and direct immunoelectron microscopic studies performed on the perilesional salt-split skin. Topical corticosteroid treatment reduced pruritus and bullae formation. This case of localized epidermolysis bullosa acquisita on the face is reminiscent of Brunsting-Perry cicatricial pemphigoid. We also review the previously reported cases of localized epidermolysis bullosa acquisita.
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PMID:Epidermolysis bullosa acquisita localized to the face. 951 4

Local anesthetics do not penetrate readily through human skin if applied in their salt form; however, if applied in their base form various effects may be observed, such as a decrease in pricking pain and a change in burning, itch, and thermal sensations. These effects occur after skin penetration and may be attributed to the action of the anesthetics on nociceptors and thermoreceptors, i.e., on C and Adelta nerve fiber respectively. As there is little known about the time course of the pharmacodynamic response of cutaneously applied local anesthetic bases, this study was conducted to characterize various local anesthetics pharmacodynamically by measuring thermal thresholds over time with a thermal sensory analyzer. The results show that the investigated local anesthetics affect thermal thresholds to a different extent, with tetracaine and lidocaine being most efficient. From the response versus time profiles of all eight study subjects various response parameters were obtained: only the cold sensation parameters proved suitable for characterization of the local anesthetics, possibly because cold receptors are located in the epidermis and can easily be reached. Lag times of onset are short and the maximum anesthetic effect is reached within 2-3 h. Cold sensation parameters correlate linearly with the solubility of the local anesthetic bases in medium chain triglycerides and with the drug flux of 50% saturation, indicating that medium chain triglycerides may have similar properties with regard to the local anesthetics solubility as the stratum corneum lipids.
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PMID:Percutaneous penetration of local anesthetic bases: pharmacodynamic measurements. 1046 25

The tolerance of Onchocerca volvulus-infected individuals to diethylcarbamazine (DEC)-medicated salt (0.33% w/w) was assessed in 1996 in Tanzania in a double-blind placebo-controlled hospital-based trial involving 4 groups, each of 10 adult males. Groups I and II had O. volvulus microfilariae (mf) only, group III had both O. volvulus and Wuchereria bancrofti mf, and group IV had W. bancrofti mf only. Groups I, III and IV received DEC-medicated salt, whereas group II was a control to group I and received normal cooking salt. Medication was given for 10 days. The most pronounced adverse reactions in groups I and III were mild-to-moderate itching and rash, beginning after 3-4 days and lasting for the remaining medication period. The reactions did not interfere with normal daily activities. By 20 days after the end of medication, adverse reactions had disappeared in all individuals. The low daily dose of DEC had no significant effect on the O. volvulus pre-medication mf geometric mean intensities (GMIs). In contrast, the medication significantly reduced the pre-medication W. bancrofti mf GMIs. The prospects for using DEC-medicated salt for control of bancroftian filariasis in areas where incidental infections with O. volvulus occur are discussed.
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PMID:Tolerance to diethylcarbamazine-medicated salt in individuals infected with Onchocerca volvulus. 1112 54

Benzyl Alcohol is an aromatic alcohol used in a wide variety of cosmetic formulations as a fragrance component, preservative, solvent, and viscosity-decreasing agent. Benzoic Acid is an aromatic acid used in a wide variety of cosmetics as a pH adjuster and preservative. Sodium Benzoate is the sodium salt of Benzoic Acid used as a preservative, also in a wide range of cosmetic product types. Benzyl Alcohol is metabolized to Benzoic Acid, which reacts with glycine and excreted as hippuric acid in the human body. Acceptable daily intakes were established by the World Health Organization at 5 mg/kg for Benzyl Alcohol, Benzoic Acid, and Sodium Benzoate. Benzoic Acid and Sodium Benzoate are generally recognized as safe in foods according to the U.S. Food and Drug Administration. No adverse effects of Benzyl Alcohol were seen in chronic exposure animal studies using rats and mice. Effects of Benzoic Acid and Sodium Benzoate in chronic exposure animal studies were limited to reduced feed intake and reduced growth. Some differences between control and Benzyl Alcohol-treated populations were noted in one reproductive toxicity study using mice, but these were limited to lower maternal body weights and decreased mean litter weights. Another study also noted that fetal weight was decreased compared to controls, but a third study showed no differences between control and Benzyl Alcohol-treated groups. Benzoic Acid was associated with an increased number of resorptions and malformations in hamsters, but there were no reproductive or developmental toxicty findings in studies using mice and rats exposed to Sodium Benzoate, and, likewise, Benzoic Acid was negative in two rat studies. Genotoxicity tests for these ingredients were mostly negative, but there were some assays that were positive. Carcinogenicity studies, however, were negative. Clinical data indicated that these ingredients can produce nonimmunologic contact urticaria and nonimmunologic immediate contact reactions, characterized by the appearance of wheals, erythema, and pruritus. In one study, 5% Benzyl Alcohol elicited a reaction, and in another study, 2% Benzoic Acid did likewise. Benzyl Alcohol, however, was not a sensitizer at 10%, nor was Benzoic Acid a sensitizer at 2%. Recognizing that the nonimmunologic reactions are strictly cutaneous, likely involving a cholinergic mechanism, it was concluded that these ingredients could be used safely at concentrations up to 5%, but that manufacturers should consider the nonimmunologic phenomena when using these ingredients in cosmetic formulations designed for infants and children. Additionally, Benzyl Alcohol was considered safe up to 10% for use in hair dyes. The limited body exposure, the duration of use, and the frequency of use were considered in concluding that the nonimmunologic reactions would not be a concern. Because of the wide variety of product types in which these ingredients may be used, it is likely that inhalation may be a route of exposure. The available safety tests are not considered sufficient to support the safety of these ingredients in formulations where inhalation is a route of exposure. Inhalation toxicity data are needed to complete the safety assessment of these ingredients where inhalation can occur.
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PMID:Final report on the safety assessment of Benzyl Alcohol, Benzoic Acid, and Sodium Benzoate. 1176 31

Alagille syndrome (AGS) causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol. This study was performed to determine whether partial external biliary diversion (PEBD) is effective for relief of pruritus and xanthomas in AGS patients who fail conventional medical therapy. Between the years 1985 and 2001, 9 AGS patients underwent PEBD. Complete follow-up data were available for all patients. The average age at PEBD was 4.8 (range 1.4-10) years. The average duration of follow-up was 7.5 (range 0.5-16.0) years. All 9 patients had severe, mutilating pruritus (grade 4) prior to diversion. At 1 year post-PEBD, the average pruritus score was 1.1; 8 patients had only mild scratching when undistracted. Three patients with extensive xanthomas prior to PEBD had complete resolution within 1 year. Mean serum bile salt levels (n = 5) decreased from 136.5 to 37.1 micromol/L and mean cholesterol (n = 7) from 724 to 367 mg/dL 1 year after PEBD. A single 21-year-old patient with PEBD for 14 years experienced an increase in pruritus from grade 1 to grade 4 within 2 months of elective reversal of PEBD. In conclusion, PEBD is effective for treating severe pruritus and hypercholesterolemia in AGS patients without cirrhosis who did not respond to medical therapy. PEBD should be considered as a therapeutic option for these patients before referral for liver transplantation because of morbid complications.
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PMID:Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome. 1553 79

We describe a 76-year-old white woman with a 6-month history of intensive pruritus and excoriated papules resembling subacute prurigo. Histopathology showed signs of chronic dermatitis, whereas findings by direct and indirect immunofluorescence microscopy were compatible with bullous pemphigoid (BP). The patient's serum contained IgG autoantibodies that recognized epitopes on both BP180 and BP230 by Western blot analysis of epidermal extracts. In addition, we found strong reactivity with recombinant NC16A, an immunodominant region of BP180 targeted in the majority of BP sera, whereas no antibodies against the keratinocyte-derived soluble BP180 ectodomain (LAD-1) or the recombinant intracellular domain of BP180 were detected. The patient's disease responded well to oral methylprednisolone and mycophenolate mofetil. Disease activity correlated with enzyme-linked immunosorbent assay reactivity of antibodies to BP180 but not with titers of antibodies to the dermoepidermal junction as determined by indirect immunofluorescence on salt-split skin. Our findings suggest that the subacute prurigo form of BP is a true variant of BP.
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PMID:Subacute prurigo variant of bullous pemphigoid: autoantibodies show the same specificity compared with classic bullous pemphigoid. 1207 94

The familial cholestatic diseases Benign Recurrent Intrahepatic Cholestasis (BRIC) and Progessive Familial Intrahepatic Cholestasis type 1 (PFIC1) are characterized by intermittent or permanently elevated plasma bile salt levels, therapy-resistant extreme pruritus and peculiar biochemical abnormalities including low apolipoprotein apo A-I. Previously, symptomatic improvement has been demonstrated in BRIC patients after extracorporal albumin dialysis (MARS). We hypothesized that MARS improves cholestasis, induces changes in the bile salt profile and normalizes apo A-I serum levels in BRIC. A 17-year-old-female patient with BRIC experienced an episode of cholestasis lasting for more than 6 months with extreme pruritus and diarrhoea not responding to standard therapy. During a period of five days the patient was treated 3 x 8 h with MARS. The procedures were well tolerated and resulted in reduction of plasma bile salts by 58%. The plasma bile salt profile changed into a more hydrophilic composition after MARS. Diarrhoea discontinued and the pruritus improved significantly from 9 to 4 on a subjective scale. These effects lasted 4 months until a relapse occurred. Low plasma apo A-I levels (0.52 g/l) normalized after MARS (0.98 g/l). The procedures were well tolerated. Fatigue was noted as the only transient side-effect. In conclusion, MARS may induce a long-term symptomatic improvement and decrease of cholestatic markers in BRIC. Further studies evaluating efficacy and mechanism of MARS in patients with BRIC are needed.
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PMID:Extracorporal albumin dialysis (MARS) improves cholestasis and normalizes low apo A-I levels in a patient with benign recurrent intrahepatic cholestasis (BRIC). 1222 Mar 10

A survey of occupational skin problems, based on a questionnaire, was carried out among 883 workers in different types of seafood-processing industries in northern Norway. The prevalence of dry skin, itching, rash/eczema, chapped skin and chronic sores was significantly higher among production workers (55.6%) in the white fish-, shrimp- and salmon-processing industries, compared to administrative workers in the same industries (27.5%). Among production workers, there was a significantly higher prevalence of skin symptoms among females (60.2%) compared to males (50.1%). A strong sex division of work tasks rather than sex itself may explain this. There was no sex difference among administrative workers. Several risk factors for skin symptoms to occur are indicated. The workers are exposed to raw materials and a mixture of water and juice from the fish or shrimp, salt, detergents and disinfectants. Gloves may also cause skin problems. Major risk factors believed to cause skin symptoms were contact with raw materials, fish juice, water and gloves. The results also indicate that skin symptoms are of moderate severity and seldom interfere with working capacity.
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PMID:Skin symptoms in the seafood-processing industry in north Norway. 1572 89


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