Course Of Steroid Therapy Research Articles

Idiopathic thrombocytopenic purpura of childhood

Idiopathic thrombocytopenic purpura (ITP) is a benign hemorrhagic disorder characterised by peripheral thrombocytopenia and increased megakaryocytes in the bone marrow. The exact pathogenesis of ITP is not well understood. The adherence of viral induced immune complexes to the platelet membrane is thought to trigger the phagocytosis of damaged platelets by macrophages in the reticuloendothelial system. The role of platelet associated IgG in the pathogenesis of ITP is under investigation. Although spontaneous recovery is observed in 80–90% of patients, a short course of steroid therapy is recommended to reduce the duration of thrombocytopenia. The steroids however, have no influence on the course or outcome of the disease, and their possible role in reducing the incidence of intracranial hemorrhage (ICH) is unknown. Emergency management of patients presenting with signs and symptoms suggestive of ICH is essential to prevent the fatal outcome. Approximately 10–20% of patients develop chronic ITP. Splenectomy, considered the treatment of choice in these patients, is not always curative. The post-splenectomy sepsis also imposes a great risk for these individuals. Recent experience with intravenous immunoglobulin (IV IgG) treatment indicates that the splenectomy could safely be deferred, or even avoided in chronic ITP. The use of IV IgG in acute ITP is being investigated.

Prednisone therapy of membranoproliferative glomerulonephritis in children

Six children with biopsy-proved type 1 membranoproliferative glomerulonephritis (MPGN) improved clinically during a 2-year course of prednisone therapy. All children had nephrotic syndrome. Creatinine clearance was less than or equal to 80 ml/min/1.73 m2 in four patients. Initial prednisone dosage ranged from 20 mg every other day to 2 mg/kg/day (maximum 60 mg), with subsequent modifications based on improvement. After completion of a 2-year course of steroid therapy, a repeat kidney biopsy was performed in each child; a decrease in glomerular disease activity was noted in five of them. After a mean follow-up of almost 5 years, all children have creatinine clearance greater than 120 ml/min/1.73 m2, and only one remains nephrotic. Although the use of prednisone in children with MPGN remains controversial, we have observed a diminution in proteinuria and normalization of creatinine clearance with therapy initiated early in the disease course.

Acute interstitial nephritis and iritis: Renal-ocular syndrome

Acute renal failure developed in a previously healthy woman in association with acute iritis. Renal biopsy revealed acute mononuclear interstitial nephritis. Known systemic illnesses that can present as acute renal failure and have ocular manifestations of iritis/uveitis were excluded by detailed testing and observation during a 24-month period. No cause for this renal-ocular syndrome was discovered. There was a dramatic improvement to normal renal function and complete resolution of ocular findings following a two-month course of steroid therapy.

Steroid-induced psychiatric syndromes: A report of 14 cases and a review of the literature

Although it is well-established that psychiatric symptoms can develop in association with the administration of corticosteroids, the nature of this relationship is poorly understood. We reviewed 14 previously unreported cases of steroid-induced psychiatric syndromes, 79 cases from the literature and 29 studies of the clinical efficacy of steroids in various medical illnesses. Our findings indicate that severe psychiatric reactions occur in approximately 5% of steroid-treated patients, and that a large proportion of these patients have affective and/or psychotic symptoms. Psychiatric disturbances usually occur early in the course of steroid therapy. Female sex, systemic lupus erythematosus and high doses of prednisone may be risk factors for the development of a steroid-induced psychiatric syndrome. Treatment with steroid-taper, neuroleptics or electroconvulsive therapy is generally effective, although tricyclic antidepressants do not appear to be useful therapeutic agents. Most patients recover within several weeks of the onset of symptoms.

Pseudotumor cerebri.

Pseudotumor cerebri, a disorder of intracranial pressure regulation, is characterized by elevated cerebrospinal fluid pressures. The symptoms and signs are those of elevated intracranial pressure alone. Extensive endocrine studies in patients with the primary disease have generally been normal. The major threat to the patient with pseudotumor cerebri is loss of vision; severe deficits occur in 4% to 12% of patients. The causative mechanism is not known. There are no controlled studies of treatment and interpretation of uncontrolled studies is limited by the high rate of spontaneous remission. A short course of steroid therapy is recommended for the patient with significant symptoms that have not remitted after 7 to 10 days of observation and serial lumbar punctures. Shunting is considered in refractory patients or when loss of vision is threatened.

Haber's Syndrome

Three Japanese brothers had the rosacea-like facies and multiple intraepidermal epitheliomas that characterize Haber's syndrome. They also had xerosis of the lower extremities. The facial lesions were dry, warm, erythematous, and indurated, with enlarged follicular orifices and telangiectasia. One patient had disturbing "burning" of the face, which was relieved by short courses of steroid therapy that were given for systemic effects.

A CASE OF MALIGNANT LYMPHOMA OF THE NODULAR, POORLY DIFFERENTIATED LYMPHOCYTIC TYPE

ABSTRACTA case of malignant lymphoma of the nodular, poorly differentiated lymphocytic type was presented. The patient, a 72‐year‐old woman, exhibited cutaneous nodules, generalized lymphadenopathy and splenomegaly. Treatment with two courses of steroid therapy and radiotherapy was carried out. After this therapy, skin tumors responded excellently. Histopathological findings of lymph nodes included proliferating forms of lymphoid tumor cells consisting of nodular and diffuse patterns. Peripheral lymphoid tumor cells, which had cleaved nuclei, clear nucleoli and mitoses were 30% increased in peripheral lymphocytes. Furthermore, the percentage of B cells in the subpopulation of peripheral lymphocytes was high, and that of T cells was low.In the present report, the histopathological and autopsy findings were studied, and some aspects of the classification of malignant lymphoma were discussed.

Testicular sarcoidosis

A 20 year old black man presented with shortness of breath, hilar adenopathy, monoarticular arthritis, polydipsia and polyuria, and bilateral testicular masses. Transbronchial biopsy revealed noncaseating granulomas consistent with sarcoidosis. Subsequent work-up demonstrated hypothalamic dysfunction, and joint fluid analysis was consistent with sarcoid arthropathy. Concern over the possibility of a testicular neoplasm led to right testicular biopsy which revealed noncaseating granulomas consistent with testicular sarcoidosis. A short course of steroid therapy resulted in clinical improvement and complete resolution of the remaining left testicular mass.

A CASE OF MULTIPLE NEONATAL HAEMANGIOMATOSIS WITH FAVOURABLE OUTCOME FOLLOWING STEROID THERAPY

A case of multiple neonatal haemangiomatosis is described. A short course of steroid therapy produced rapid and marked improvement of cutaneous lesions, without any effect on testicular involvement. The authors emphasize the value of an early treatment with steroids.

Sarcoid Granulomatous Nephritis Occurring as Isolated Renal Failure

A woman had clinically isolated renal failure caused by granulomatous sarcoid nephritis. At the onset of her illness, there were no historical, physical, or clinical features to suggest a definite causative diagnosis. After a renal biopsy specimen disclosed noncaseating epithelioid granulomas, a course of steroid therapy resulted in prompt disappearance of uremic symptoms and amelioration of her renal failure. Although leukopenia, delayed cellular immunity, and the response to steroids increased the suspicion that the patient had sarcoidosis, it was not until identical granulomas were obtained via mediastinal node biopsy that the diagnosis was confirmed. Sarcoidosis may occur as isolated renal failure. Renal biopsy is indicated to ensure early diagnosis and prompt treatment of this steroid-responsive lesions.

Immunologic Lung Disease Due to Aspergillus: Medical Unit Staff Round from the Cardiothoracic Institute of the Brompton Hospital, London

This 36-year-old man had asthma since the age of six months and missed school often as a teenager. He has had eczema since infancy. The precipitating allergic factors for his asthma were cats, nuts, dust, exercise, and drinking milk at night. For the past four to five years, he has had an almost continuous cough productive of a cupful of sputum per day without plugs, but with an occasional streaky hemoptysis. In 1962 he received a short course of steroid therapy for a week and in July 1973 a course of adrenocorticotropic hormone injections which increased his peak flow to about 300 L/min. At age seven he had a nasal polypectomy. His grandfather had asthma, and one brother and his son aged ten have eczema and asthma. On examination he was a pleasant man, with no shortness of breath at rest, cyanosis, or clubbing. There was some chest overexpansion with widespread rhonchi and crepitations throughout all lung fields. Blood pressure was 140/80 mm Hg.

Anaemia and temporary erythroblastopaenia in children. A syndrome.

SummaryOver a period of 7 years, 12 patients were seen at the Royal Alexandra Hospital for Children with a symptomatic normocytic, normochromic anaemia and reticulocytopaenia. The initial bone marrow aspirates revealed erythroblastopaenia as the only abnormality.The cause of this condition remains uncertain. However, all children fully recovered within a few weeks after diagnosis. A single course of steroid therapy was given to 3 children, whilst the remainder received no therapy. Recurrence was observed in one child only. Thus, on clinical and haematological grounds, differentiation from Gasser's series could be made.Failure to recognize this syndrome will lead to a number of unnecessary investigations. Furthermore, a life‐long iatrogenic diagnostic label may be given in error for a benign, self‐limiting condition in childhood.

Steroid-induced diabetic ketoacidosis in a 14-year-old boy with steroid-sensitive nephrotic syndrome: Case report and literature review.

In this report, we described the case of a 14-year-old boy with steroid-sensitive nephrotic syndrome who developed hyperglycaemia and ultimately, diabetic ketoacidosis, following high-dose steroid therapy for a primary renal disease. The nephrotic syndrome was diagnosed based on generalized oedema, massive proteinuria, hypoalbuminaemia and hypercholesterolaemia. Serum creatinine and random blood glucose levels were normal and there was no glycosuria. He was commenced on high dose prednisolone 40 mg 12 hourly and by the 8th day on prednisolone, he achieved remission and was discharged. However, four weeks later, he developed features of diabetic ketoacidosis (DKA) which was confirmed by the presence of hyperglycaemia (random blood glucose19.4 mmol/L), acidosis (serum bicarbonate 10 mmol/L) and ketonuria (2+). The DKA was managed with intravenous fluid (0.9% sodium chloride), continuous insulin infusion and antibiotics. After resolution of the DKA, he was switched to subcutaneous soluble insulin and thereafter, premixed insulin twice daily with a reduction in the dose of prednisolone and was discharged home after 30 days on admission. Blood glucose level has remained within normal range one year after discontinuing insulin and he is still in remission with regard to the nephrotic syndrome at follow up.Conclusion: The risk of diabetic ketoacidosis should be considered in the course of steroid therapy for nephrotic syndrome. To avoid missing of cases of steroid-induced diabetes mellitus, and ultimately DKA, both fasting and postprandial blood glucose values should be monitored.Key words: Adolescence, diabetes, ketoacidosis, nephrotic syndrome, steroid therapy.

A case of hypersensitivity to corticosteroids.

SUMMARY.— A patient is described who developed skin eruptions after both intra‐articular and oral administration of corticosteroids. Skin tests with highly purified steroid compounds were consistently and strongly positive, but an extensive series of in vitro tests for circulating and cell‐bound steroid‐specific antibody was negative. The possible reasons for this are discussed and brief descriptions given of 2 other patients who developed generalized reactions after intra‐articular injections of corticosteroids. It is suggested that steroid hypersensitivity is an entity which ought to be considered in patients who develop unusual skin reactions during the course of steroid therapy.

Long-term results of steroid therapy in adults with idiopathic nephrotic syndrome

This study analyzes the results in sixty-eight patients with the idiopathic nephrotic syndrome treated with adrenal corticoids and followed for a period averaging five and a half years. Approximately one fourth of the patients responded to steroid therapy with a complete remission (i.e., protein excretion less than 100 mg. per day) which, in most cases, was sustained after discontinuation of treatment; four patients, however, remained dependent upon steroid therapy. Beneficial steroid responses occurred only among patients treated within six months of onset of the disease. An additional 14 per cent of patients, whose proteinuria did not disappear during treatment, had a spontaneous remission of their disease at intervals ranging from one to five years after treatment had been discontinued. Thus slightly more than one third of the adults with the idiopathic nephrotic syndrome treated with steroids ultimately became free of proteinuria. Of the group who did not have complete remissions, roughly one-third have either died of uremia or now have significantly impaired renal function after an over-all follow-up of approximately five years. Deterioration of renal function developed only among those who continued to excrete more than 2 gm. of protein per day; the incidence of renal insufficiency in this group was more than 50 per cent. No patient whose protein excretion has fallen to less than 2 gm. per day has so fat shown evidence of significant renal deterioration. The incidence of complications due to treatment was 25 per cent in the group as a whole; however, these complications occurred mainly in patients who received a prolonged daily course of steroid therapy. Inasmuch as intermittent therapy is associated with a low incidence of significant side effects and is seemingly just as effective as continuous therapy, we recommend that patients with idiopathic nephrotic syndrome be treated on an intermittent schedule. Definitive evaluation of the effect of steroids on the natural history of the idiopathic nephrotic syndrome awaits a suitably controlled prospective study. We believe, however, that when the present findings are examined in the light of what is known about the untreated course of the disease, they strongly suggest that steroids have significant beneficial effects. Best results are to be expected when steroid administration is started within six months of the onset of the disease and is reserved for those patients with minimal renal lesions. Our data also suggest that life expectancy may be improved, even in patients who do not have a complete remission, if protein excretion can be reduced to less than 2 gm. per day.

Blood hyperosmolality and neurologic deficit. An experimental study.

THE ROLE that systemic blood hyperosmolality may play in causing neurologic disease has been called in question by the clinical description of hyperosmolal, hyperglycemic nonketotic coma. This condition, which occurs mostly in elderly persons with early diabetes mellitus, consists of coma, dehydration, elevation in blood sugar, hyperosomolality, and various neurologic deficits. There is a good clinical response to insulin and hypotonic fluids. Since the first report by Sament and Schwartz in 1957 1 the syndrome has been described in other disturbances such as severe burns, during peritoneal dialysis, and during the course of steroid therapy. 2-4 Recently, Maccario et al 5 have recorded seven cases presenting focal neurologic deficits in association with the above syndrome. In order to explain the occurrence of the clinical manifestations such factors as hyperosmolality, intracellular dehydration, and a direct toxic effect of glucose upon the nervous system, acting singly or in combination, have been

Granulomatous sarcoid nephritis

A patient with impaired renal function due to granulomatous sarcoid replacement of the renal parenchyma is described through two separate courses of therapy with corticosteroids. Serial measurements of renal function, periodic renal scintiscans and intravenous pyelograms, and serial histologic studies of the kidney by percutaneous renal biopsy are presented. The variety of modes by which sarcoidosis may affect the kidneys is reviewed. Hypercalcemia or hypercalciuria is responsible for most instances of renal disease from sarcoidosis. In the present case, kidney damage resulted from extensive replacement of renal parenchyma by sarcoid lesions in the absence of hypercalcemia or hypercalciuria. The value of renal biopsy is seriously limited by reason of the focal nature of the disease and the chance of sampling error. Renal scintiscan provided not only presumptive evidence for diagnosis but also a method to assess the extent of the disease and the effect of therapy. Serial determination of renal function demonstrated improvement following each course of therapy. However, a gradual decrease in creatinine clearance was observed during the prolonged second course of steroid therapy without increase in sarcoid “lesions” by scintiscan. It is considered likely that fibrosis associated with regression or healing of active granulomas may have been responsible. It is concluded that alternate periods of exacerbation followed by regression with steroid therapy should be avoided because of the potential of progressive fibrosis and destruction of renal parenchyma.

Jaundice

The clinical efficacy of a short course of steroid therapy in differentiating the etiology of jaundice was determined. Also, observations were made on the mechanism of action of steroids on bilirubin metabolism. A standard five-day course of prednisone, 60 mg daily, was given to 33 patients. The final diagnosis was established by biopsy, laparotomy, or autopsy. Clinically, the response to this course of steroids clearly separated extrahepatic jaundice due to malignancy from intrahepatic jaundice of viral inflammatory origin. In viral hepatitis, the suggested mechanism of action of steroids appears to be enhanced clearing of conjugated bilirubin from the serum.

Jaundice. Value of five-day steroid test in differential diagnosis.

The clinical efficacy of a short course of steroid therapy in differentiating the etiology of jaundice was determined. Also, observations were made on the mechanism of action of steroids on bilirubin metabolism. A standard five-day course of prednisone, 60 mg daily, was given to 33 patients. The final diagnosis was established by biopsy, laparotomy, or autopsy. Clinically, the response to this course of steroids clearly separated extrahepatic jaundice due to malignancy from intrahepatic jaundice of viral inflammatory origin. In viral hepatitis, the suggested mechanism of action of steroids appears to be enhanced clearing of conjugated bilirubin from the serum.

Experimental reactivation of subsiding rheumatic fever.

When anti-inflammatory therapy is reduced or discontinued in patients with acute rheumatic fever, a flare-up of disease activity frequently occurs, manifested by clinical abnormalities or by changes only in laboratory tests. The nature of this rebound phenomenon was recently analyzed in a retrospective survey of 265 patients with acute rheumatic fever (1, 2). The results indicated that most clinical rebounds were not due to any of the three possible causes to which they have been attributed in the past: 1) polycyclic rheumatic activity, 2) an insufficient duration of therapy or 3) relative hypoadrenalism. Instead, the data strongly suggested that these rebounds represented the appearance of the inflammation that had been suppressed during the antirheumatic therapy. This hypothesis was based on the findings that the incidence of clinical rebounds increased with greater severity of cardiac involvement, that rebounds were more frequent in patients treated with steroids alone than in those treated with salicylates, that rebounds were more likely to occur after long courses of steroid therapy than after short courses, that they were rare after long courses of salicylates, and finally, that the incidence of post-steroid rebounds could be reduced by adding salicylates when the steroid reduction began, and by continuing salicylates for several weeks after steroids were stopped. This new hypothesis has important implications regarding the natural history of rheumatic fever and has practical significance for its treatment. One of the implications has been used in the present study to provide a method of testing the validity of the theory. If the hypothesis is correct, it should be possible to create a rebound by giving anti-inflammatory therapy at a time when the convalescent rheumatic patient has no clinical abnormalities but is still in a subclinical inflammatory