Polycystic Ovary Syndrome: An Under-recognized Cause of Abnormal Uterine Bleeding in Adolescents Admitted to a Children's Hospital.

STUDY OBJECTIVE: To evaluate whether ovulatory dysfunction due to polycystic ovary syndrome (PCOS) is a common underlying etiology of abnormal uterine bleeding (AUB) in adolescents who require hospitalization and to explore etiology, treatment, and complications of AUB with severe anemia in adolescents. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: We identified female patients aged 8-20 years admitted to a children's hospital for treatment of AUB from January 2000 to December 2014. Our hospital protocol advises hormonal testing for PCOS and other disorders before treatment for AUB. We reviewed medical records and recorded laboratory evaluations, treatments, and final underlying diagnoses as well as recurrences of AUB and readmissions in the subsequent year. RESULTS: Of the 125 subjects, the mean age was 16.5 ± 2.9 years; mean hemoglobin level was 7.0 ± 1.8 g/dL; 54% were overweight/obese; and 41% sexually active. PCOS accounted for 33% of admissions; hypothalamic pituitary ovarian axis immaturity 31%; endometritis 13%; bleeding disorders 10%. Girls with PCOS were more likely to be overweight/obese (74% vs 46%; P < .01) and girls with hypothalamic pituitary ovarian axis immaturity had lower hemoglobin levels (6.4 g/dL vs 7.4 g/dL; P < .05), than girls with all other etiologies of AUB. Treating physicians failed to diagnose endometritis as the etiology for AUB in 4 of 8 girls with positive tests for sexually transmitted infection and no other etiology. CONCLUSION: PCOS was the most common underlying etiology in adolescents hospitalized with AUB. Screening for hyperandrogenemia is important for early diagnosis of PCOS to allow ongoing management and prevention of comorbidities. Endometritis was frequently underestimated as an etiology for AUB.

Urban Adolescents' and Young Adults' Decision-Making Process around Selection of Intrauterine Contraception.

STUDY OBJECTIVE: To examine adolescent and young adults' priorities, values, and preferences affecting the choice to use an intrauterine contraceptive device (IUD). DESIGN: Qualitative exploratory study with analysis done using a modified grounded theory approach. SETTING: Outpatient adolescent medicine clinic located within an academic children's hospital in the Bronx, New York. PARTICIPANTS: Twenty-seven women aged 16 to 25 years of age on the day of their IUD insertion. INTERVENTIONS AND MAIN OUTCOME MEASURES: We conducted semistructured interviews exploring participant's decision making process around selecting an IUD. We were specifically interested in elucidating factors that could potentially improve IUD counseling. RESULTS: We identified 4 broad factors affecting choice: (1) personal; (2) IUD device-specific; (3) health care provider; and (4) social network. Most of the participants perceived an ease with a user-independent method and were attracted by the high efficacy of IUDs, potential longevity of use, and the option to remove the device before its expiration. Participants described their health care provider as being the most influential individual during the IUD decision-making process via provision of reliable, accurate contraceptive information and demonstration of an actual device. Of all people in their social network, mothers played the biggest role. CONCLUSION: Adolescents and young women who choose an IUD appear to value the IUDs' efficacy and convenience, their relationship with and elements of clinicians' contraceptive counseling, and their mother's support. Our results suggest that during IUD counseling, clinicians should discuss these device-specific benefits, elicit patient questions and concerns, and use visual aids including the device itself. Incorporating the factors we found most salient into routine IUD counseling might increase the number of adolescents and young women who choose an IUD as a good fit for them.

Advance supply of emergency contraception: a randomized trial in adolescent mothers.

OBJECTIVE: To examine whether the advanced provision of emergency contraception (AEC) to parenting youth would increase emergency contraception (EC) utilization, and whether AEC would impact the rates of unprotected sex and contraception use. DESIGN: Subjects were randomized to receive either information about EC or information and an actual supply of AEC. Subjects were interviewed at baseline, 6 and 12-month follow-up. SETTING: Urban non-medical case management office. PARTICIPANTS: 160 adolescent mothers (ages 13 to 20) who were receiving case management services. INTERVENTION: Advance supply of emergency contraception. MAIN OUTCOME MEASURES: Emergency contraception use, sexual activity, unprotected intercourse, contraceptive methods and use. RESULTS: Parenting teens who received AEC were much more likely to have used it than the control group at the 6-month interview (83% vs. 11%) and the 12-month interview (64% vs. 17%). Teens in the AEC treatment group were more likely to have unprotected sex at the 12-month follow-up interview (69% vs. 45%). There was no difference in condom use between the groups at either the 6-month, or the 12-month follow-up interviews. CONCLUSION: Advance provision of emergency contraception in parenting teens increases the likelihood of its use, and does not affect the use of condoms, or hormonal methods of birth control. Parenting teens who receive AEC may be more likely to have unprotected sex.

A recurring FBN1 gene mutation in neonatal Marfan syndrome.

BACKGROUND: Marfan syndrome is an autosomal dominant disorder of connective tissue caused by mutations in the fibrillin 1 gene (FBN1). FBN1 mutations have been associated with a broad spectrum of phenotypes. Neonatal Marfan syndrome has unique clinical manifestations and mutations. OBJECTIVE: To determine if there is a discernible genotypic-phenotypic correlation associated with the unique mutation in neonatal Marfan syndrome. STUDY DESIGN: A newborn exhibited many typical characteristics of neonatal Marfan syndrome, including arachnodactyly; contractures of both elbows, knees, and ankles; small-joint laxity; dilated cardiomyopathy; valvular dysplasia and insufficiency; congestive heart failure; and pulmonary emphysema. Three atypical features were also discovered: a right diaphragmatic hernia, a myocardial mass, and left main-stem bronchomalacia. She died at 3(1/2) months of age. Total RNA was extracted from skin fibroblasts and amplified by means of reverse transcriptase polymerase chain reaction amplification with FBN1-specific primers. The complementary DNA fragments were sequenced. RESULTS: A single T-to-C transition at nucleotide 3276 (T3276C) was identified and confirmed at the DNA level by sequencing of genomic DNA. This results in a substitution of threonine for isoleucine. CONCLUSIONS: Neonatal Marfan syndrome is a unique clinical entity with recurring mutation hot spots in exons 24 to 27 and 31 to 32 of the FBN1 gene. Some clinical features in this case report are unusual for neonatal Marfan syndrome. This is the third report of this T3276C mutation in the FBN1 gene with unusual clinical manifestations. We conclude that there is a genotypic-phenotypic correlation associated with this mutation.

If we prescribe it, will it come? Access to asthma equipment for Medicaid-insured children and adults in the Bronx, NY.

CONTEXT: Asthma is a major cause of morbidity in the United States. Self-management of asthma requires access to appropriate equipment. Clinical experience in an inner-city practice suggests that families encounter difficulties in filling prescriptions for spacers/holding chambers, peak flow (PF) meters, and nebulizer machines. OBJECTIVES: To determine whether Bronx, NY, pharmacies (1) carry spacers/holding chambers, PF meters, and nebulizer machines; (2) accept Medicaid insurance for them; and (3) perceive barriers to reimbursement by Medicaid for this equipment. DESIGN AND SETTING: Structured telephone survey of 100 Bronx pharmacies randomly selected from the 1999 telephone directory. PARTICIPANTS: Ninety-eight pharmacists and 2 pharmacy technicians in 100 different pharmacies. MAIN OUTCOME MEASURES: Pharmacists' reports of equipment availability, Medicaid acceptance, and reasons for not carrying equipment or accepting Medicaid. RESULTS: Overall equipment availability was as follows: spacers (68%), spacers with masks (57%), adult PF meters (40%), child-range PF meters (24%), and nebulizer machines (56%). For Medicaid recipients, equipment was less available: spacers (45%), spacers with masks (35%), adult PF meters (27%), child-range PF meters (17%), and nebulizer machines (33%). Surveyed pharmacists reported misconceptions about requirements for Medicaid reimbursement, which included the following: that Durable Medical Equipment permits are required (64% spacers and 33% PF meters), that special forms are needed (17% PF meters), or that this equipment is not covered by Medicaid (14% spacers and 8% PF meters). Of the 100 surveyed pharmacists, 32 reported difficulties with Medicaid reimbursement and 41 had never tried to receive reimbursement. CONCLUSIONS: These results suggest that (1) access to spacers/holding chambers, PF meters, and nebulizers for Medicaid-insured families is severely limited in Bronx pharmacies; (2) misunderstandings regarding Medicaid reimbursement policies are common; and (3) interventions to increase the proportion of pharmacies that dispense equipment are needed.