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    Norman Spack

    Author Affiliation: Los Angeles Biomedical Research Institute, Harbor-University of California at Los Angeles Medical Center, Torrance (bspellberg@labiomed .org). Conflict of Interest Disclosures: The author has completed and submitted... more
    Author Affiliation: Los Angeles Biomedical Research Institute, Harbor-University of California at Los Angeles Medical Center, Torrance (bspellberg@labiomed .org). Conflict of Interest Disclosures: The author has completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and reported receiving grant and contract support from Cubist, Pfizer, Bristol-Myers Squibb, and Cempra; being a consultant for GlaxoSmithKline, Meiji, Cardeas, aRigen, and Synthetic Biologics; and receiving honoraria from Cubist.
    Gender identity disorder (transgenderism) is poorly understood from both mechanistic and clinical standpoints. Awareness of the condition appears to be increasing, probably because of greater societal acceptance and available hormonal... more
    Gender identity disorder (transgenderism) is poorly understood from both mechanistic and clinical standpoints. Awareness of the condition appears to be increasing, probably because of greater societal acceptance and available hormonal treatment. Therapeutic options include hormone and surgical treatments but may be limited by insurance coverage because costs are high. For patients seeking male-to-female (MTF) change, hormone treatment includes estrogens, finasteride, spironolactone, and gonadotropin-releasing hormone (GnRH) analogs. Surgical options include feminizing genital and facial surgery, breast augmentation, and various fat transplantations. For patients seeking a female-to-male (FTM) gender change, medical therapy includes testosterone and GnRH analogs and surgical therapy includes mammoplasty and phalloplasty. Medical therapy for both FTM and MTF can be started in early puberty, although long-term effects are not known. All patients considering treatment need counseling and medical monitoring.
    ABSTRACT The author, an endocrinologist and co-founder of Gender Management Services at Children's Hospital, discusses his experience treating disorders of sexual development in adolescents and young adult transgender individuals.... more
    ABSTRACT The author, an endocrinologist and co-founder of Gender Management Services at Children's Hospital, discusses his experience treating disorders of sexual development in adolescents and young adult transgender individuals. He began treating transgender young adults in 1985. He discusses his early career development as well as his use of a protocol developed in Holland for treating trans-youth.
    ABSTRACT The author, an endocrinologist and co-founder of Gender Management Services at Children's Hospital, discusses his experience treating disorders of sexual development in adolescents and young adult transgender individuals.... more
    ABSTRACT The author, an endocrinologist and co-founder of Gender Management Services at Children's Hospital, discusses his experience treating disorders of sexual development in adolescents and young adult transgender individuals. He began treating transgender young adults in 1985. He discusses his early career development as well as his use of a protocol developed in Holland for treating trans-youth.
    BACKGROUND Transgender children and adolescents, those who experience incongruence between assigned sex at birth and internal gender identity, are a poorly understood and understudied population in the United States. Since 2008, medical... more
    BACKGROUND Transgender children and adolescents, those who experience incongruence between assigned sex at birth and internal gender identity, are a poorly understood and understudied population in the United States. Since 2008, medical care for transgender youth has generally followed guidelines developed by professional consensus given the paucity of empirical research, particularly in a U.S. setting. OBJECTIVE The objective of this research is to provide evidence-based data to inform clinical care for transgender youth. The study aims to: 1) evaluate the impact of gonadotropin-releasing hormone (GnRH) agonists administered for puberty suppression on mental health, psychological well-being, and metabolic and physiologic parameters including bone health in a cohort of children and adolescents (Tanner stages 2-4) with gender dysphoria, comparing baseline and follow-up assessments; and 2) evaluate the impact of gender-affirming hormones (e.g. estradiol, testosterone, etc.) administer...
    This chapter focuses on new scientific developments in the physiology, management, and psychological impact of diabetes in adolescents.
    GENETICS AND ETIOLOGY: IMPLICATIONS FOR COUNSELING Until recently, diabetics were classified into juvenile- or maturity-onset categories on the basis of the age at onset of disease. We now consider diabetes to be a syndrome in which... more
    GENETICS AND ETIOLOGY: IMPLICATIONS FOR COUNSELING Until recently, diabetics were classified into juvenile- or maturity-onset categories on the basis of the age at onset of disease. We now consider diabetes to be a syndrome in which various factors, diminished or absent beta cell secretory capacity, increased levels of gluconeogenic hormones, or altered insulin receptor binding, can produce the common denominator of hyperglycemia. Although a rare form of mild diabetes has been characterized in children and appears to be inherited in autosomal dominant fashion (termed MODY-Maturity Onset Diabetes of the Young)1, the overwhelming majority of diabetic children and adolescents develop the insulin-dependent variety of the disease. Recent studies in Michigan define the prevalence of the latter form to be 1.89 cases per 1000 school-aged children (ages 5 to 18 years), with an annual incidence of 1.6 cases per 100,000 children (ages 5 to 18 years). Whereas increased risk has long been observed in relatives of insulin-dependent diabetics, several factors have confounded the geneticists: patients sharing the same genetic blueprint do not share equal likelihood for developing the disease. When one member of a monozygotic twinship develops diabetes under age 45, his twin has but a 50% chance of becoming diabetic. On the other hand, if a twin develops the disease over the age of 45, the risk to the other twin approaches 100%.
    Testosterone is commonly administered intramuscularly (IM) to treat hypogonadal males and female-to-male transgender (FTM) patients. However, these injections can involve significant discomfort and may require arrangements for... more
    Testosterone is commonly administered intramuscularly (IM) to treat hypogonadal males and female-to-male transgender (FTM) patients. However, these injections can involve significant discomfort and may require arrangements for administration by others. We assessed whether T could be administered effectively and safely by the subcutaneously (SC) as an alternative to IM injections. Retrospective cohort study. Outpatient Reproductive Endocrinology Clinic at an academic medical center. Sixty-three FTM transgender patients aged >18 years electing to receive SC T therapy for gender transition were included. Fifty-three patients were premenopausal. Patients were administered T cypionate or enanthate weekly at an initial dose of 50mg. Dose was adjusted if needed to achieve serum total T levels within the normal male range. Serum concentrations of free and total T and total estradiol (E2), masculinization and surveillance for reactions at injection sites. Serum T levels within the normal ...
    In 2007, an interdisciplinary clinic for children and adolescents with disorders of sex development (DSD) or gender identity disorder (GID) opened in a major pediatric center. Psychometric evaluation and endocrine treatment via pubertal... more
    In 2007, an interdisciplinary clinic for children and adolescents with disorders of sex development (DSD) or gender identity disorder (GID) opened in a major pediatric center. Psychometric evaluation and endocrine treatment via pubertal suppressive therapy and administration of cross-sex steroid hormones was offered to carefully selected patients according to effective protocols used in Holland. Hembree et al.'s (2009) Guidelines for Endocrine Treatment of Transsexual Persons published by the Endocrine Society endorsed these methods. A description of the clinic's protocol and general patient demographics are provided, along with treatment philosophy and goals.
    In 2007, an interdisciplinary clinic for children and adolescents with disorders of sex development (DSD) or gender identity disorder (GID) opened in a major pediatric center. Psychometric evaluation and endocrine treatment via pubertal... more
    In 2007, an interdisciplinary clinic for children and adolescents with disorders of sex development (DSD) or gender identity disorder (GID) opened in a major pediatric center. Psychometric evaluation and endocrine treatment via pubertal suppressive therapy and administration of cross-sex steroid hormones was offered to carefully selected patients according to effective protocols used in Holland. Hembree et al.'s (2009) Guidelines for Endocrine Treatment of Transsexual Persons published by the Endocrine Society endorsed these methods. A description of the clinic's protocol and general patient demographics are provided, along with treatment philosophy and goals.
    Evidence indicates an overrepresentation of youth with co-occurring autism spectrum disorders (ASD) and gender dysphoria (GD). The clinical assessment and treatment of adolescents with this co-occurrence is often complex, related to the... more
    Evidence indicates an overrepresentation of youth with co-occurring autism spectrum disorders (ASD) and gender dysphoria (GD). The clinical assessment and treatment of adolescents with this co-occurrence is often complex, related to the developmental aspects of ASD. There are no guidelines for clinical care when ASD and GD co-occur; however, there are clinicians and researchers experienced in this co-occurrence. This study develops initial clinical consensus guidelines for the assessment and care of adolescents with co-occurring ASD and GD, from the best clinical practices of current experts in the field. Expert participants were identified through a comprehensive international search process and invited to participate in a two-stage Delphi procedure to form clinical consensus statements. The Delphi Method is a well-studied research methodology for obtaining consensus among experts to define appropriate clinical care. Of 30 potential experts identified, 22 met criteria as expert in ...
    Purpose. To quantify retinal circulatory abnormalities in patients with type 1 diabetes; to compare blood speed and blood flow in major temporal retinal arteries as well as total retinal arterial cross-section measured in patients to that... more
    Purpose. To quantify retinal circulatory abnormalities in patients with type 1 diabetes; to compare blood speed and blood flow in major temporal retinal arteries as well as total retinal arterial cross-section measured in patients to that measured in controls without diabetes; to determine which factors are related to the measured abnormalities within the patient group. Methods. The laser Doppler technique
    To quantify retinal circulatory abnormalities in patients with type 1 diabetes; to 1 diabetes; to compare blood speed and blood flow in major temporal retinal arteries as well as total retinal arterial cross-section measured in patients... more
    To quantify retinal circulatory abnormalities in patients with type 1 diabetes; to 1 diabetes; to compare blood speed and blood flow in major temporal retinal arteries as well as total retinal arterial cross-section measured in patients to that measured in controls without diabetes; to determine which factors are related to the measured abnormalities within the patient group. The laser Doppler technique and monochromatic fundus photography were used to measure retinal circulatory parameters in 39 patients with type 1 diabetes with duration of diabetes between 7 and 20 years and 13 age-matched controls without diabetes. Blood pressure, intraocular pressure, and heart rate were measured in all subjects. Glycosylated hemoglobin was measured in the patients. Retinopathy was assessed using standardized color fundus photography and fluorescein angiography. Total retinal arterial cross-section was, on average, 17% higher (P = 0.007) in the patients than in the controls, and it increased wi...
    To review the clinical and MR imaging findings in adolescents with hemorrhagic pituitary adenomas and to compare those findings with pathologic results and outcome. We reviewed the clinical records, imaging examinations, surgical and... more
    To review the clinical and MR imaging findings in adolescents with hemorrhagic pituitary adenomas and to compare those findings with pathologic results and outcome. We reviewed the clinical records, imaging examinations, surgical and pathologic findings, and follow-up studies in 11 girls and six boys (12 to 20 years old; mean age, 16 years) with pituitary adenomas who were treated at our institution between August 1986 and June 1995. Of the 17 adenomas, eight were macroadenomas (> 1 cm) in patients 14 to 18 years old (three girls, five boys). Six of the macroadenomas were grossly hemorrhagic, and appeared as high-intensity intrasellar/suprasellar masses on all MR sequences obtained before definitive diagnosis and treatment. Clinical presentation in the patients with the hemorrhagic macroadenomas included headache (five), visual field deficits (three), and neuroendocrine symptoms (three). One patient was asymptomatic. The preliminary clinical and imaging diagnoses were craniophary...
    Studies with the aldose reductase inhibitor alrestatin in animal models have suggested that the sorbitol pathway may be of etiologic significance in the pathogenesis of peripheral neuropathy in diabetes. In normal subjects and in highly... more
    Studies with the aldose reductase inhibitor alrestatin in animal models have suggested that the sorbitol pathway may be of etiologic significance in the pathogenesis of peripheral neuropathy in diabetes. In normal subjects and in highly selected diabetic patients with severe peripheral neuropathy, alrestatin given either intravenously (50 mg/kg body weight) or orally (1 gm q.i.d.) produced no acute toxicity. The serum half-life of alrestatin was approximately 1 hr, and 99% was recovered in the urine within 24 hr. Two diabetic patients receiving alrestatin intravenously reported subjective improvements in clinical symptoms 2 days following the start of infusions. These improvements lasted approximately 3 wk after infusions were discontinued. However, there were no significant objective changes in peripheral nerve condition velocities, or on neurologic examination. In a 30-day oral trial with alrestatin in 4 diabetics, there were no subjective improvements in clinical symptoms nor were there objective improvements on neurologic examination or in peripheral nerve conduction velocities. In this study, peak serum levels of alrestatin were approximately 3 times lower than those obtained on intravenous administration, and it is possible that a high peak serum level is critical to the attainment of adequate tissue drug concentrations. Furthermore, the patients were suffering from severe clinical peripheral neuropathy, which could represent a stage of permanent irreversible nerve damage. Studies with alrestatin in newly diagnosed diabetics with peripheral nerve conduction velocity deficits but without clinical neuropathy might provide a better test of the sorbitol pathway hypothesis.
    The approach to gender identity disorder (GID) in childhood and adolescence has been rapidly evolving and is in a state of flux. In an effort to form management recommendations on the basis of the available literature, The Endocrine... more
    The approach to gender identity disorder (GID) in childhood and adolescence has been rapidly evolving and is in a state of flux. In an effort to form management recommendations on the basis of the available literature, The Endocrine Society published clinical practice guidelines in 2009. The guidelines recommend against sex role change in prepubertal children, but they recommend the use of gonadotropin-releasing hormone (GnRH) agonists to suppress puberty in adolescence, and the use of cross-sex hormones starting around age 16 for eligible patients. In actual practice, the approach to GID is quite variable due to continued lack of consensus and specific barriers to treatment that are unique to GID. Recent literature has focused on the mental health approach to prepubertal children with GID and short-term outcomes using pubertal suppression and cross-sex steroids in adolescents with GID. This review will describe the literature published since the release of The Endocrine Society guidelines regarding the management of GID in both children and adolescents.
    Few interdisciplinary treatment programs that tend to the needs of youth with gender nonconforming behaviors, expressions, and identities exist in academic medical centers with formal residency training programs. Despite this, the... more
    Few interdisciplinary treatment programs that tend to the needs of youth with gender nonconforming behaviors, expressions, and identities exist in academic medical centers with formal residency training programs. Despite this, the literature provides evidence that these youth have higher rates of poor psychosocial adjustment and suicide attempts. This article explores the logistical considerations involved in developing a specialized interdisciplinary service to these gender minority youth in accordance with the existing treatment guidelines.Demographic data will be presented and treatment issues will be explored. The impact that a specialized interdisciplinary treatment program has on clinical expansion, research development, education and training, and community outreach initiatives is discussed.
    At 18 months, Alexandra insisted on wearing boys' underwear before she would complete toilet training. At 3 years, she told her parents that she “should have been a boy,” and insisted on wearing boys' clothes.... more
    At 18 months, Alexandra insisted on wearing boys' underwear before she would complete toilet training. At 3 years, she told her parents that she “should have been a boy,” and insisted on wearing boys' clothes. Alexandra now refuses to wear dresses and wears a coat and tie to formal ...