Successful treatment of pure red cell aplasia associated with systemic lupus erythematosus with oral danazol and steroid

We describe a middle-aged Chinese systemic lupus erythematosus (SLE) patient developing steroid refractory and transfusion dependent red cell aplasia. Oral danazol 200 mg twice per day was started together with low-dose prednisolone therapy. There was no further recurrence of anemia 1 month after this combined therapy.     

Hypermobility syndrome in 105 women with pure urinary stress incontinence and in 105 controls

We compared the frequency of Hypermobility Syndrome (HS) in 105 patients with urinary stress incontinence (USI) with the frequency of HS in 105 healthy controls that matched for age and parity. A Beighton score (BS) of more than 3 was used to make the clinical diagnosis of HS. Thirty-six patients (34.28%) from the USI group and 28 patients (26.66%) from the control group were diagnosed as HS. The mean BS values were 6.44±0.35 and 5.21±0.29 respectively. The difference between the two groups was statistically significant (P<0.05).     

Update and review of moderate and deep sedation

The administration of sedation/analgesia to allow patients to tolerate diagnostic or therapeutic procedures and tests is a fact of life. Approximately 200,000,000 such sedations are administered every year in the United States. Any morbidity or mortality associated with sedation is likely to be deemed avoidable. There will never be adequate resources or personnel so that trained anesthesia providers administer all sedations, and there probably is no need for such an approach. Clinicians from many specialties now safely and effectively administer sedation. With attention to updated guidelines and concepts, as outlined in this article, the safety and efficacy of sedation/analgesia can and should be excellent.     

Treatment of protozoan infections

Protozoan infections can have a variety of different cutaneous manifestations in addition to systemic signs and symptoms of disease. Recognition and diagnosis can be difficult, as additional laboratory tests, in addition to biopsies, may be required. Treatment options for different protozoa vary and resolution of disease may be refractory despite lengthy treatment courses. An overview of cutaneous manifestations, diagnosis, and treatment regimen of amebiasis, leishmaniasis, trypanosomiasis, and toxoplasmosis is outlined in this article.     

Azathioprine metabolite measurements in the treatment of autoimmune hepatitis in pediatric patients: a preliminary report

Potential adverse effects of azathioprine (AZA), such as neutropenia and hepatotoxicity, make its use in autoimmune hepatitis (AIH) problematic. OBJECTIVE: To determine longitudinal AZA metabolite levels in a cohort of children with AIH, correlate them with therapeutic effects, medication-induced toxicity and adherence. METHODS: From January 2000 to January 2002, 122 blood samples from 30 pediatric patients with AIH were prospectively analyzed. Ten patients had previously been treated with AZA (mean dose of 1.3mg/kg/day) for an average of 30 months. At the outset, 24 patients were taking steroids and 10 had cirrhosis/hypersplenism. Routine biochemical studies, 6-thioguanine (6-TG) and 6-methylmercaptopurine (6-MMP) levels were assessed every 8 weeks. Red blood cell thiopurine methyltransferase (TPMT) enzyme activity was determined in each patient. AZA dose was adjusted to achieve a target 6-TG level 235-450 pmoles per 8 x 10 RBC. RESULTS: 8/10 patients who had previously been treated with standard doses of AZA had 6-TG below target levels. Increasing AZA mean dose by 50% in those patients resulted in 6/10 patients in target range; ALT levels and steroid requirements were reduced. AZA dosing was safely increased in patients with cirrhosis/hypersplenism. In spite of normal TPMT levels, 64% of patients did not make measurable concentrations of 6-MMP. Inappropriately low 6-TG levels revealed non-adherence in 5 patients. Two patients were identified with AZA hepatotoxicity. CONCLUSION: AZA metabolite testing in children with AIH is useful in identifying medication toxicity and non-adherence. AZA dose escalation is safe and may be required in order to achieve 6-TG target levels described for inflammatory bowel disease.     

Idiopathic intracranial hypertension with primary aldosteronism: report of 2 cases

Although unconfirmed, the syndrome idiopathic intracranial hypertension (IIH), commonly seen in overweight 20- to 50-year-old women, has been proposed to have its origins in an endocrine-based disturbance of electrolytes. Herein we report on 2 women with IIH and primary aldosteronism (PAL). Aged 57 and 55 (patients 1 and 2), each had a longstanding history of mild-to-moderate arterial hypertension, recurrent hypokalemia, and headaches. They were found to have IIH at ages 51 and 45. PAL was diagnosed at ages 57 and 35, respectively, due to proven left adrenal adenoma in patient 1; and presumptive adrenal nodular hyperplasia in patient 2. This is the first report to appear in the English medical literature that describes an association between IIH and PAL. It raises the prospect that in some cases of IIH associated with arterial hypertension, an autonomous production of aldosterone should be considered.