OBJECTIVE: To investigate the experiences of young women with spontaneous premature ovarian failure with regard to the initial presenting symptom, promptness of diagnosis, and patient education. METHODS: We asked 50 patients previously diagnosed with spontaneous premature ovarian failure to participate in a structured interview survey consisting of 38 true-or-false, multiple-choice, and open-ended questions. RESULTS: Disturbance in menstrual pattern was the most common initial symptom in the 48 women who completed the interview (44 of 48, 92%). Over half of the 44 women who presented with this complaint reported visiting a clinician's office three or more times before laboratory testing was performed to determine the diagnosis. Over half of them reported seeing three or more different clinicians before diagnosis. In 25% of women it took longer than 5 years for the diagnosis of premature ovarian failure to be established. Patients who spent more than 5 minutes with the clinician discussing the diagnosis were significantly more likely to be satisfied with the manner in which they were informed (P <.001). Ninety percent of participants were college graduates, and 40% had graduate degrees. CONCLUSION: Women with spontaneous premature ovarian failure perceived a need for more aggressive evaluation of secondary amenorrhea and oligomenorrhea. Loss of menstrual regularity can be a sign of ovarian insufficiency, and the associated estrogen deficiency is a well-established risk factor for osteoporosis.

Adult↗

PubMed2002

Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome.

Hermansky-Pudlak syndrome (HPS) consists of oculocutaneous albinism, a platelet storage pool deficiency and, in patients with HPS1 gene mutations, a progressive, fatal pulmonary fibrosis. We investigated the safety and efficacy of an antifibrotic agent, pirfenidone (800 mg, t.i.d.), in treating 21 adult Puerto Rican HPS patients, including 20 homozygous for the same HPS1 mutation. Patients were examined every 4 months for up to 44 months in a randomized, placebo-controlled trial, with rate of change in pulmonary function values as outcome parameters. Using the complete data set of 130 patient admissions, a repeated measures model showed that 11 pirfenidone-treated patients lost FVC at a rate 5% of predicted ( approximately 400 mL) per year slower than 10 placebo-treated patients (p=0.001). A random coefficients model showed no significant difference. However, using data restricted to patients with an initial FVC >50% of predicted, both models showed the pirfenidone group losing FVC (p<0.022), FEV(1) (p<0.0007), TLC (p<0.001), and DL(CO) (p<0.122) at a rate approximately 8%/year slower than the placebo group. Clinical and laboratory side effects were similar in the two groups. Pirfenidone appears to slow the progression of pulmonary fibrosis in HPS patients who have significant residual lung function.

Adult↗

PubMed2002

Additive gastrointestinal effects with concomitant use of olestra and orlistat.

OBJECTIVE: To report a case of significant additive gastrointestinal effects with concomitant use of orlistat and an olestra-containing snack food. CASE SUMMARY: A 16-year-old African American girl with type 2 diabetes, hypercholesterolemia, and hypertension was participating in a pilot study that tested the safety and efficacy of orlistat. After 2 weeks of orlistat treatment, the patient presented to the clinic with complaints of soft, fatty/oily stools, flatus with discharge, abdominal pain, increased flatus, and fecal incontinence. On further questioning, it was determined that she was also consuming approximately 5 ounces of olestra-containing potato chips on a daily basis. The patient eliminated olestra from her diet and returned to the clinic with substantially diminished gastrointestinal adverse effects, despite continuing to take orlistat. DISCUSSION: This is the first published case describing additive gastrointestinal effects after concurrent use of orlistat and olestra. Education about the potential for serious additive gastrointestinal adverse effects is important to prevent premature and unnecessary discontinuation of orlistat therapy. Awareness of this potential interaction could be especially important for patients with underlying disease states in which severe gastrointestinal symptoms could result in significant complications. CONCLUSIONS: This case illustrates that significant gastrointestinal distress may result after olestra consumption during orlistat therapy. All patients receiving orlistat for the management of obesity should be properly educated about this potential drug-food interaction.

Adolescent↗