[Behavior of various serum enzymes and unusual associations in the field of glycogenosis].
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Biomedical subjects
Publications and source records attributed to L Fasce.
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We review 30 cases of pediatric systemic lupus erythematosus followed over an 8-year period at our institution. The female to male ratio was 3.3:1; the age at diagnosis ranged between 3.5 and 16 years. On first admission, renal involvement was detected in the majority of the patients, as assessed by laboratory findings and/or clinical manifestations. Other frequently observed symptoms were fever, skin rashes, arthralgias and/or arthritis and serositis. All of the patients were treated with corticosteroids and most of them also received immunosuppressive drugs in order to control disease activity. Two patients were lost to the follow-up, five died and only one of the 23 evaluable patients is off therapy after a median follow-up of 5 years. This study confirms that pediatric systemic lupus erythematosus is a very aggressive disease.
The authors shortly report on the frequency and the peculiar clinical picture in the paediatric age. The usefulness of the pharmacological control (Ranitidine i.v. 50 mg thrice for 3 days) of acute haemorrhagic compliances is discussed in reference to the patient. The same drug was continued per os for two month at a dosage of 150 mg twice daily followed by 150 once daily for 8 months; during this period no relapses were observed and an endoscopic control did show the anatomical recovery of the ulcer. The authors underline also the good compliance and the absence of side effects of the drug employed.
Two new indications for the application of endo-urological techniques are reported. One case involved recanalization of stenosis at the ureterovesical junction, while in the other case a double J ureteral endoprosthesis was extracted by the transnephropyelostomy percutaneous route.
The authors refer their clinical experience of the use of embolization of the hypogastric arteries in cases of serious haemorrhage of the bladder and prostate. The immediate results in the 14 cases observed are decidedly positive, especially considering the serious conditions of certain neoplastic patients, which ruled out alternative surgical treatment. Nevertheless, even when the embolization technique has been correctly used, the possibility exists that bleeding may start up again after some time, and this is always caused by the revascularization of the neoplasia.
In this study we have investigated the efficacy and safety of cyclosporin A (CyA) in a group of pediatric patients with juvenile chronic arthritis (JCA, 9 cases) and polymyositis-dermatomyositis (PM-DM, 3 cases). Of the 9 JCA patients, 7 had the systemic and 2 the polyarticular form of the disease. All of the patients received CyA after the failure of corticosteroids and/or cytotoxic drugs. CyA was administered for 9 to 48 months at a mean dose of 5 mg/kg/day. Ten patients received corticosteroids with CyA. The results of CyA treatment were satisfactory overall, both in the PM-DM patients and in the JCA patients, as assessed by clinical and laboratory evaluation. CyA-related side effects included alopecia, hypertension, hypertrichosis, tremors, and hirsutism. One JCA patient developed polyserositis with hypoproteinemia of unknown origin while receiving CyA. CyA blood levels did not correlate with clinical efficacy and/or side effects. This study suggests that CyA represents a promising agent for the treatment of JCA and childhood PM-DM.