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)

Two brothers with progressive familial intrahepatic cholestasis were followed during four years of phenobarbital therapy to assess its long-term safety and efficacy. Effects attributable to phenobarbital included sustained relief of pruritus, lowering of serum bilirubin levels, and enhanced hepatic excretion of 131I-rose bengal; serum bile acid levels were only transiently decreased. Two brief interruptions in therapy resulted in symptomatic deterioration. Phenobarbital did not retard growth nor impede the response to vitamin D therapy of concomitant rickets.
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PMID:Efficacy and safety of long-term phenobarbital therapy of familial cholestasis. 65 Mar 23

Osteodystrophy is almost universally present in chronic renal failure. Mild, but detectable, abnormalities--especially in parathyroid hormone (PTH) secretion--occur even when the glomerular filtration rate is greater than 30 cc/min. Osteomalacia is common in areas in which vitamin D intake and exposure to sunlight are minimal; when these factors are plentiful, osteitis fibrosa predominates. Osteoporosis is seen with increasing frequency in hemodialyzed patients. Nonosseous complications of secondary hyper-parathyroidism include hypercalcemia, metastatic calcification and pruritus. The most important factor in the medical therapy of osteodystrophy is control of serum phosphate levels. Next, a positive calcium balance must be provided either by giving vitamin D as dihyrdotachysterol, raising dialysate calcium or administering calcium orally. Parathyroidectomy is sometimes indicated, especially when the patients are transplant candidates and manifest hypercalcemia. Whether or not transplant is contemplated, patients with persistently high calcium-phosphate products, severe metastatic calcification or rapidly progressive osteodystrophy should be considered for parathyroidectomy. Newer, experimental vitamin D preparations, such as 1,25-dihydroxycholecalciferol or 1-alpha-hydroxycholecalciferol, should improve the managemet of patients with renal osteodystrophy and decrease the need for parathyroidectomies.
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PMID:Calcium metabolism in renal failure. 109 Jan 50

The immunologic mechanisms responsible for the development of primary biliary cirrhosis (PBC) remain poorly defined, although recent investigations have provided new clues as to the role of cellular membrane proteins such as the mitochondrial autoantigens and intercellular adhesion molecules. Additionally, new therapeutic agents have become available that markedly enhance the prospect for medical management of both hepatic and extrahepatic manifestations of PBC. Definitive therapy with ursodeoxycholic acid and/or methotrexate, and symptomatic relief of pruritus with rifampicin or metronidazole may become standard in the years ahead. The results of their use to date in the treatment of PBC are detailed in this review. Successful therapy of hepatic osteodystrophy associated with PBC has yet to be achieved, although early data suggest a role for ursodeoxycholic acid, estrogen, calcium, and vitamin D in the management of this debilitating problem. Orthotopic liver transplantation continues to be successful for the management of advanced disease (greater than 70% 5-year survival) and will remain an essential therapeutic tool until definitive medical therapy for PBC becomes available.
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PMID:Primary biliary cirrhosis: new therapeutic directions. 143 68

'Ageing is a multistep, multifaceted, time-dependent phenomenon characterized by the decreased ability of a system to respond to exogenous and endogenous stress from either physical, chemical or biologic agents'. Cutaneous ageing provides a visible model of the interaction between endogenous (intrinsic) factors and exogenous (extrinsic) factors. In skin, the principal extrinsic-factor is ultraviolet light (UV) which is responsible for the constellation of changes termed photoageing. In recent years, much interest has been directed towards defining the ageing processes in skin and excellent comprehensive reviews have been compiled. This review aims to highlight several areas of developing knowledge, and focuses on the potential importance of environmental changes as they influence skin ageing and carcinogenesis. Repeated reference to the effects of UV on the skin are inevitable in any review of skin ageing and this is scarcely surprising as the skin contains many cells as well as subcellular and extracellular chromophores which are capable of absorbing energy within the UV spectrum. Cellular chromophores include among others keratinocytes, melanocytes, Langerhans cells, dermal fibroblasts and mast cells. Subcellular chromophores include keratin, melanin, collagen, elastin and a number of proteins, lipids and steroids (such as vitamin D). Urocanic acid, a photoisomerization product of the amino-acid histidine, may provide some limited photoprotection and some believe it to be important in UV induced immunosuppression. Understanding events at the molecular and biochemical level has unfortunately not been paralleled by clinical advances and the common, troublesome skin-problems of old age such as cancer, xerosis and pruritus remain a major cause of morbidity and yet are poorly explained.
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PMID:Aspects of cutaneous ageing. 179 84

Idiopathic hypoparathyroidism was diagnosed in five young to middle-aged cats of mixed breeding. Three of the cats were male and two were female. Historic signs included lethargy (n = 5), anorexia (n = 5), muscle tremors (n = 4), weakness (n = 4), generalized seizures (n = 3), ataxia (n = 3), mental dullness or disorientation (n = 3), panting (n = 2), pruritus (n = 1), ptyalism (n = 1) and dysphagia (n = 1). Weakness (n = 4), dehydration (n = 2), cataracts (n = 2), hypothermia (n = 1), and bradycardia (n = 1) were found on physical examination. Results of electrocardiography revealed a prolonged Q-T interval in two cats. Results of initial laboratory tests revealed profound hypocalcemia and severe hyperphosphatemia with normal renal function. The diagnosis of hypoparathyroidism was made on the basis of the history, clinical signs, and results serum biochemical testing (i.e., severe hypocalcemia and hyperphosphatemia); in two cats, the diagnosis was also confirmed by histologic examination of parathyroid glands. Initial treatment included intravenous administration of 10% calcium gluconate and oral administration of large loading doses of calcium and vitamin D (dihydrotachysterol). Successful long-term management with dihydrotachysterol and calcium was achieved in all cats. The final dosage of dihydrotachysterol required to maintain normocalcemia in the five cats ranged from 0.004 to 0.04 mg/kg/day (mean = 0.015 mg/kg/day). Long-term calcium supplementation was given to three of the cats in dosages ranging from 29 to 53 mg/kg/day (mean = 42 mg/kg/day) of elemental calcium. One cat died after 28 months of therapy from widely metastatic hemangiosarcoma; the other three cats are still alive and well after 5 to 37 months of treatment.
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PMID:Idiopathic hypoparathyroidism in five cats. 202 14

Renal excretion is the major route of magnesium elimination from the body and a positive magnesium balance would be expected under conditions of renal insufficiency. However, a compensatory decrease in tubular reabsorption is operating to maintain an adequate urinary magnesium excretion even when glomerular filtration rates are very low. Nevertheless, in end-stage renal disease, the limited ability of the kidney to excrete an increased magnesium load may result in toxic concentrations of the ion in serum. While magnesium intoxication is a real hazard when magnesium-containing drugs are given, magnesium balance may be normal or even decreased in uraemic patients. This is usually due to decreased dietary intake combined with the impaired intestinal magnesium absorption which characterizes chronic renal failure. Impairment of magnesium absorption seems to be related to deficient synthesis of the active metabolite of vitamin D by the non-functioning kidney. Following the institution of chronic haemodialysis or continuous ambulatory peritoneal dialysis (CAPD) treatment, the major determinant of magnesium balance is the concentration of magnesium in the dialysate. Changes in the dialysate magnesium have been used to reduce the incidence of renal osteodystrophy, to alleviate uraemic pruritus, or to retard the development of arterial calcification in chronic renal disease. However, uncertainty about magnesium, calcium and parathyroid hormone relationships in renal failure makes a reasoned approach to such manipulations extremely difficult.
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PMID:Magnesium metabolism in chronic renal failure. 213 26

Autoimmune CAH is important to recognize, since it is highly responsive to treatment which undoubtedly prolongs life. Autoimmune CAH can rarely be cured; complete withdrawal of treatment leads to relapse in over 80% of patients. Prednisolone and azathioprine are the major drugs of choice, the former inducing remission while the latter maintains remission, either alone or in combination with prednisolone. Since both drugs are associated with substantial side-effects which tend to be dose-related, the object of treatment must be to induce and maintain remission with the minimum risk of relapse together with an acceptably low incidence of complications. Although PBC shares many features in common with autoimmune CAH, treatment of the underlying disease is generally unsuccessful. To date no drug has been shown to induce remission or to prolong survival. The main aim of treatment should be directed towards the complications of PBC, of which pruritus and osteoporosis are the two major complaints. Cholestyramine and antihistamines are the drugs of choice, but when these fail a variety of other therapies are also available, although many have only been shown to be effective on an anecdotal basis. No treatment has yet been shown to reverse the bone demineralization which occurs in PBC, but early calcium supplementation is recommended in this disorder. Osteomalacia is uncommon and can be prevented by prophylactic calcium and vitamin D supplementation in jaundiced patients. Liver transplantation is effective in treating PBC, and when successful leads to complete restoration of health with the prospects of increasingly long survival. Recurrence of PBC does not appear to be a significant problem.
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PMID:Autoimmune chronic active hepatitis and primary biliary cirrhosis. 265 50

A retrospective analysis was undertaken to better define the clinical presentation and therapy of patients with secondary hyperparathyroidism and the timing of surgical removal of the parathyroid glands in secondary hyperparathyroidism. Sixty-eight patients with end-stage renal disease (ESRD) underwent 74 parathyroid procedures over a 10-year period. There were 38 per cent women and 62 per cent men in this population--the same proportion of men and women with ESRD. These proportions are reversed when compared with patients with primary hyperparathyroidism. The mean time between initiation of dialytic therapy and parathyroidectomy was 5.4 years. Preoperative mean serum calcium and phosphorus levels were 10.1 +/- 0.2 and 6.1 +/- 0.2 mg/dl, respectively. All patients were symptomatic and 60 per cent of the patients had at least two symptoms before surgery. Renal osteodystrophy was the most common symptom (74%), whereas pruritus was noted in 65 per cent of this population. Patients underwent either subtotal (88%) or total (12%) parathyroidectomy with autotransplantation. Six patients required reoperation: five for recurrent disease (2 to 5 years after the initial surgery) and one for persistent disease. All patients were symptomatically improved after the surgery. Complications included the following: transient hoarseness (7%), hypocalcemia requiring calcium, and/or vitamin D therapy (32%) for 6 months after surgery. This analysis demonstrates that although the initial therapy of secondary hyperparathyroidism is medical, surgical therapy should be instituted before multiple symptoms develop in the ESRD population.
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PMID:Secondary hyperparathyroidism. The role of surgery. 272 73

In hyperparathyroidism associated with endstage renal failure, either subtotal, or total parathyroidectomy with autotransplantation have been advocated as potentially curative for those patients non-responsive to medical therapy. Seventeen such patients managed by the Wellington renal unit were reviewed as to indications for, and responses to, surgery. Itch, psychiatric symptoms, joint ache, muscular weakness, gritty eyes and thirst were the major symptoms for which surgery was recommended. Hypercalcaemia (universal in women) and deterioration in bone radiology were additional indications for operation. All showed remarkable postoperative improvement. Symptomatic hypocalcaemia was significant in 41%. This observation has led to intensified pre- and postoperative vitamin D and calcium therapy. Postoperative radiologic improvement at three months was apparent in 80% of patients. We advocate subtotal parathyroidectomy as the effective surgical treatment of choice for uraemic hyperparathyroidism.
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PMID:Parathyroid surgery in chronic renal failure. 386 72

A number of different chronic diseases affect the intrahepatic bile radicles or cholangioles. They include primary and secondary sclerosing cholangitis, primary biliary cirrhosis, chronic cholestatic drug jaundice, atresia, and carcinoma. Aetiological factors include infection, immunological changes, hormones, and congenital defects.Patients with chronic cholestasis have decreased bile salts in the intestinal contents and suffer from a bile salt deficiency syndrome. Failure to absorb dietary fat is managed by a low-fat diet and by medium-chain trigly-cerides which are absorbed in the absence of intestinal bile salts. Fat-soluble vitamin deficiencies are prevented by parenteral vitamins A, D, and K(1). Calcium absorption is defective, and improvement may follow intramuscular vitamin D, medium-chain triglycerides, a low-fat diet, and oral calcium supplements.In partial intestinal bile salt deficiency the anionic bile-salt-chelating resin cholestyramine controls pruritus though steatorrhoea increases. Pruritus associated with total lack of intestinal bile salts is managed by methyl-testosterone or norethandrolone, though the jaundice increases.
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PMID:Chronic cholangitides: aetiology, diagnosis, and treatment. 497 Oct 54


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