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Biomedical subjects

M R Laufer

Publications and source records attributed to M R Laufer.

At least 19 recordsLinked to original sources

High risk of infertility and long term gonadal damage in males treated with high dose cyclophosphamide for sarcoma during childhood.

BACKGROUND: Therapy with alkylating agents, such as cyclophosphamide, can be associated with irreversible gonadal toxicity in male survivors of adult cancer. To the authors's knowledge the effect of high dose therapy with cyclophosphamide during childhood on adult testicular reproductive and endocrine function has not been established. METHODS: Gonadal function was studied in 17 adult male survivors of childhood sarcomas treated with high dose pulse cyclophosphamide therapy as part of a VAC (vincristine, actinomycin, and cyclophosphamide) or Adria-VAC (doxorubicin, vincristine, actinomycin, and cyclophosphamide) chemotherapy regimen. Patients answered a questionnaire concerning sexual functioning and underwent a comprehensive physical examination, semen analysis, and hormonal evaluation. RESULTS: Of the 17 males who underwent semen analysis, 10 (58.8%) had azoospermia, 5 (29.4%) had oligospermia, and only 2 (11.8%) were found to have a normal sperm count. All patients treated prior to the onset of puberty had an abnormal semen analysis. The 2 patients with normal sperm counts received the lowest doses of cyclophosphamide (< 7.5 g/m(2)). The baseline follicle-stimulating hormone level was elevated in only 10 of 14 patients with abnormal sperm counts (71.4%). Testosterone levels were normal in 15 of 16 patients (93.8%); however, the baseline luteinizing hormone (LH) level was elevated in 6 of 15 patients with normal testosterone levels (40%). Gonadotropin-releasing hormone-stimulated LH levels were > 3 times that of baseline in 13 of /14 patients (92.9%), suggesting some degree of Leydig cell insufficiency. CONCLUSIONS: The results of the current study show a high risk of gonadal dysfunction in men exposed to cyclophosphamide during childhood as part of a VAC/Adria-VAC chemotherapy regimen. Exposure prior to puberty was not found to be protective, and the risk of infertility appeared to increase with higher doses of therapy. To the authors' knowledge the clinical significance of impaired Leydig cell function beginning at a young age is unknown and merits further study.

Adolescent↗

Three years of a clinical practice guideline for uncomplicated pelvic inflammatory disease in adolescents.

PURPOSE: To study the effect of continued use of a clinical practice guideline (CPG) on the course of admissions for uncomplicated pelvic inflammatory disease (PID) over 3 consecutive fiscal years (FY). METHODS: Medical charts, computerized laboratory records, and hospital charge data were reviewed for 165 consecutive inpatient admissions of adolescents meeting clinical criteria for PID during FY 1994, 1995, and 1996. Data were analyzed to compare demographics, clinical variables, length of stay (LOS), and hospital charges (total, nursing, and pharmacy) across the three FYs. RESULTS: Of admissions for clinical PID, 65% had a discharge diagnosis of PID. Of those, 90% were uncomplicated PID. Among admissions with a discharge diagnosis of uncomplicated PID, reductions were seen in mean LOS (3.75 days in FY 1994 vs. 3.24 days in FY 1995 vs. 3.08 days in FY 1996; p =.047), proportion of admissions lasting longer than 3 days (48% vs. 24% vs. 20%; p < or =.022), and mean pharmacy charge ($946 vs. $806 vs. $731; p =.002). For all admissions to CPG, mean LOS, proportion of prolonged admissions, and mean total and pharmacy charges also decreased over the first 2 years but increased in FY 1996. More patients in FY 1996 met the three major clinical criteria plus at least one additional criterion (76% in FY 1996 vs. 26% in FY 1994 and 53% in FY 1995; p <.0005) and had pelvic ultrasounds (80% in FY 1996 vs. 56% in FY 1994 and 45% in FY 1995; p < or =.001) than in other FYs. CONCLUSIONS: Continued use of a CPG can reduce hospital LOS, charges, and prolonged admissions of adolescents with uncomplicated PID. Over 3 years, variations in clinical practice such as admitting sicker patients may attenuate the effect of the CPG.

Adolescent↗

Surgicel in the management of labial and clitoral hood adhesions in adolescents with lichen sclerosus.

STUDY OBJECTIVE: Lichen sclerosus (LS) is an inflammatory dermatosis of the vulva with potentially destructive consequences to the young woman's perineum. Long-term sequelae include atrophy of the labia minora, scarring of the clitoral hood, and labial and/or clitoral hood adhesions. This study aims to find techniques for preventing these devastating effects. PARTICIPANTS: Three young women, ages 14 and 15 years, with a long history of lichen sclerosus and labial adhesions, presented with recurrent labial and periclitoral adhesions. Increasing pain prior to presentation, exquisite enough to inhibit even walking, necessitated urgent operative intervention. Scarring of the clitoral hood with an area of firmness beneath the scarring was present. Sharp dissection of the clitoral hood was performed with the entrapped keratinaceous debris and hair expressed. The adherent labia were separated. Surgicel, oxidized regenerated cellulose gauze (Johnson & Johnson, Arlington, TX), was sutured to the exposed clitoral hood and labial surfaces with vicryl suture. Complete dissolution of the Surgicel occurred between postoperative day 4-6 without recurrence of adhesions. One-year follow-up did not reveal any evidence of recurrence in any of the three patients. CONCLUSION: The recurrence of labial and clitoral hood adhesions in young women with a history of LS was prevented for at least 1 year by surgical lysis and application of Surgicel to the affected area. This technique has prevented the recurrence during the interval when these surfaces are at highest risk of re-agglutination.

Adolescent↗

Conservative management of bilateral asynchronous adnexal torsion with necrosis in a prepubescent girl.

Conservative management of ovarian torsion consisting of detorsion and surgical stabilization of the involved ovary, and possibly including the uninvolved ovary, has been described in the adult population. A 6-year-old girl with a history of prior ovarian torsion resulting in oophorectomy presented with 72 hours of intermittent abdominal pain, anorexia, and 1 episode of vomiting. The patient had torsion of her remaining ovary diagnosed surgically and was managed with detorsion and oophoropexy, despite the severely necrotic appearance of the ovarian tissue. Postoperative serial ultrasound scans confirmed the viability and position of the ovary. In the prepubertal girl, ovaries may torse despite the absence of enlarging lesions such as tumors or cysts. To maximize the potential success of conservative therapy, torsion always must be included in the differential diagnosis of abdominal pain. Surgical management should attempt to salvage the torsed ovary, despite possible necrotic appearance, and also consider interventions to prevent recurrence, because bilateral torsion is a rare but potentially devastating complication.

Child↗

Microlaparoscopy with conscious sedation in adolescents with suspected pelvic inflammatory disease.

STUDY OBJECTIVE: To develop a protocol for emergency department microlaparoscopy with conscious sedation in adolescents with clinically suspected pelvic inflammatory disease (PID), and to evaluate the feasibility and tolerability of microlaparoscopy in this population. DESIGN: Prospective study involving adolescents and young adults age 13 to 24 meeting clinical criteria for uncomplicated PID. Laparoscopy subjects underwent microlaparoscopy in the Children's Hospital Emergency Department (ED) and comparison subjects were admitted for treatment of PID. Chi-square, Mann-Whitney U tests, Wilcoxon Rank Sum tests, and repeated measures of analysis of variance (MANOVA) were used for analysis. RESULTS: Twenty-four patients were enrolled: 6 laparoscopy subjects and 18 comparison subjects. Laparoscopy and comparison subjects did not differ with respect to age, mean white blood cell (WBC) count, mean temperature, or mean erythrocyte sedimentation rate. Mean surgical induction time was 13.5 minutes, operative time 19.0 minutes, and total procedure time 32.5 minutes. Mean requirement for midazolam was 2.8 mg and for fentanyl 225 microg. Pain assessment over the first 90 minutes did not differ significantly between laparoscopy and comparison subjects. Four of 6 laparoscopy subjects (67%) and 10 of 18 comparison subjects (56%) were diagnosed with PID (p = NS). CONCLUSIONS: ED microlaparoscopy appears to be feasible, safe, and well tolerated in this small sample of adolescents and young adults with suspected PID.

Adolescent↗

Developmental abnormalities of the female reproductive tract.

Developmental abnormalities of the female reproductive tract are a group of heterogeneous anomalies that may also affect other organ systems. Our review outlines the diverse abnormalities, etiologies, modes of diagnosis, and treatment options currently available.

Female↗

Obstructive anomalies of the female reproductive tract.

Obstructive anomalies of the female reproductive tract preclude the outflow of menstruation and may allow the collection of blood in the uterus and/or vagina and increase the likelihood of retrograde flow. These conditions occur in young women and may result in pelvic masses, endometriosis and/or pain. In addition, the long-term sequelae of endometriosis may influence fertility. Pediatricians, primary care physicians and gynecologists need to maintain a high index of suspicion in order to aggressively diagnose these congenital anomalies in young adolescents. In order to maintain this vigilance and assist in maintaining future fertility, it is important to review the embryologic sources of these anomalies, understand the modes of diagnosis and recognize the treatment options currently available.

Adolescent↗

Pelvic pain.

Pelvic pain is a common symptom in the adolescent female. Acute pain may represent a life-threatening situation and torsion, ectopic pregnancy, and PID must be considered. For the young patient who presents with chronic pelvic pain, a multidisciplinary approach is essential to facilitate diagnosis and management. Whenever possible, organic disease such as endometriosis, adhesions, and obstructive malformations should be identified and treated as indicated. Developing a treatment team, recognizing psychosocial and environmental factors, and encouraging long-term relationships are critical components in the care of these patients and in the prevention of recurrent symptom formation and future disability.

Acute Disease↗

Vulvar disorders in adolescents.

Bacterial and fungal vulvitis, dermatitis, inflammatory dermatoses, secondary drug reactions, viral infections, and a variety of vulvar tumors can all present as primary vulvar problems in adolescents. In addition, systemic disease can present with vulvar involvement. These disorders can be extremely anxiety-provoking in adolescent females who are dealing with issues surrounding self-image, physical maturation, and sexuality. A detailed history and physical exam can provide many clues to the underlying problem, but sometimes a biopsy is necessary to establish a diagnosis. Consultations from other services, such as dermatology or infectious diseases, can be very helpful. The best approach involves a combination of empathetic reassurance, careful diagnosis, and successful treatment. In order to facilitate optimal care of adolescents with vulvar disorders, a comprehensive review of the literature is presented.

Adolescent↗

Endometriosis in adolescents. Incidence, diagnosis and treatment.

OBJECTIVE: To review the incidence and diagnosis of endometriosis in adolescents and to evaluate the surgical and medical treatment options for this special population. STUDY DESIGN: Literature research. RESULTS: Endometriosis is the most common cause of chronic pelvic pain in adolescents, affecting up to 70% of girls with chronic pelvic pain unresponsive to medical management. There may be a natural progression of endometriosis from atypical lesions in adolescents to classic lesions in adults. CONCLUSION: Endometriosis should be strongly suspected in adolescent girls with chronic pelvic pain unresponsive to oral contraceptives and nonsteroidal antiinflammatory drugs. Initial management of endometriosis involves surgical resection or destruction at the time of diagnosis followed by medical management with continuous oral contraceptives. Gonadotropin-releasing hormone agonists should be considered for adolescents over 16 years of age who have completed pubertal maturation.

Adolescent↗

Limitations of the medical evaluation for child sexual abuse.

The purpose of this review is to highlight the inherent difficulties that a clinician faces during the evaluation of child sexual abuse cases. Each component of the medical evaluation has its own unique problems. Some of them are caused by the nature of the abusive relationship, while others are the result of the children's limited verbal skills. Readers should be aware of these limitations as they formulate an opinion at the conclusion of the examination.

Child↗

Reproductive disorders associated with pelvic pain.

Pelvic pain is common in adolescents and can result from a number of physiological and pathological etiologies, both gynecologic and nongynecologic in origin. The evaluation, diagnosis, and management of these conditions involve both medical and surgical approaches. In this review, the authors present a comprehensive approach to the care of adolescents with pelvic pain associated with dysmenorrhea, endometriosis, and obstruction of the genital tract.

Adolescent↗

Evaluation of a pregnancy-testing protocol in adolescents undergoing surgery.

STUDY OBJECTIVE: The purpose of this study was to determine the rate of unknown and nondisclosed pregnancies identified from a preoperative protocol for pregnancy testing of adolescents. DESIGN: A protocol was developed for pregnancy testing of all 12-21-year-old females presenting to the day surgery unit (DSU) at a large children's hospital. The charts of all patients identified with a positive urine ICON II human chorionic gonadotropin (uhCG) assay result were reviewed for demographic data, presurgical and postsurgical diagnosis, and proposed and actual procedure performed. Adolescents were asked the date of their last menstrual period (LMP), whether they were sexually active (SA) (engaged in vaginal intercourse), and whether they might be pregnant, and a uhCG was performed. SETTING: DSU at a large children's hospital in Boston, Massachusetts. PARTICIPANTS: All 12-21-year-old females presenting to DSU. MAIN OUTCOME MEASURES: Disclosure of sexual activity, date of LMP, and any possibility of pregnancy. The initial decision (continuation, abortion, or undecided) at the time of identification of the positive uhCG, compliance with follow-up referral appointments, outcome of the pregnancy, and subsequent choice of contraception were also documented. RESULTS: A total of 801 adolescent girls presented to the DSU for a surgical procedure between October 1994 and July 1996 and had a uhCG. Six of the 801 girls had a positive uhCG; 4 of 801 (0.49%) were pregnant. The remaining two had false-positive uhCG tests associated with ovarian failure and elevated gonadotropins. An LMP was recorded for the four pregnant patients, but only two patients were able to identify an exact date. SA was admitted by two of four and denied by the other two. One of the two who disclosed SA admitted to the possibility of being pregnant in response to the question, the other denied the possibility of pregnancy. The two who denied SA also failed to disclose the possibility of pregnancy. After disclosure of a positive uhCG, the surgical procedure was postponed in all four individuals. CONCLUSIONS: A routine preoperative uhCG testing program identified 4 of 801 (0.49%) patients with previously undiagnosed pregnancy. The current protocol has contributed to enhanced care and identifies pregnant adolescents who need counseling before surgical procedures are performed.

Abortion, Induced↗

Endosalpingiosis: clinical presentation and follow-up.

OBJECTIVE: To define the clinical presentation and follow-up of women found to have endosalpingiosis. METHODS: Subjects included for retrospective study were identified as having had a pathologic diagnosis of endosalpingiosis. Those subjects without coinciding pathologic diagnosis of endometriosis were then identified; their clinical charts were reviewed for information regarding the clinical presentation, surgical findings, therapeutic management, and clinical follow-up. RESULTS: Review of 1,648 pathologic reports from Children's Hospital, Boston and 380 reports from Brigham and Women's Hospital identified 18 subjects with endosalpingiosis without pathologic evidence of endometriosis. Two clinical scenarios were identified, and designated Groups I and II. Group I consisted of 15 patients presenting with pelvic pain, and Group II consisted of 3 patients presenting with infertility, pancreatic cancer, and an abdominal abscess. Follow-up was obtained for 8 of the 15 patients in Group I for an average of 17 months, none of whom were pain-free off medications (oral contraceptives, danazol, or GnRH agonist), and 3 (38%) have required additional surgery (6, 10 or 12 months after the initial surgery) at which time all were found to have endometriosis. Follow-up was obtainable for the patient with infertility in Group II for 5 months, with no complaint of pelvic pain. CONCLUSIONS: We report 18 patients with endosalpingiosis without confounding endometriosis. The clinical presentation is one of either pelvic pain or that of an incidental finding; the clinical course and response of those with pain is similar to that of endometriosis.

Abdominal Abscess↗

"Add-back" therapy for endometriosis in adolescents.

OBJECTIVE: To review the impact of gonadotropin-releasing hormone agonists (GnRH-as) on bone mass in adolescents and options for GnRH-a with "add-back" for long-term therapy for adolescents with endometriosis not responding to conventional therapy. STUDY DESIGN: Literature research. RESULTS: The use of GnRH-a plus add-back therapy has not been specifically studied in the adolescent population, and thus the ideal dosage of add-back sex steroids for adolescents has not been delineated. CONCLUSION: GnRH-a plus add-back can be considered for usage in adolescents with endometriosis who are refractory to conventional therapy, but steroids are needed to determine the ideal dosage and long-term effects.

Adolescent↗