[Interface of speech-language pathology in the process of gender reassignment from male to female]. / Die Schnittstelle der Logopädie im Prozess der Geschlechtsangleichung von Mann zu Frau.

BACKGROUND: Voice is crucial for gender identification during the transition process, as voice feminization plays a significant role in the success of passing. In order to be able to classify the role of voice treatment during the interdisciplinary gender reassignment process of MzF-trans*, possible recommendations for action for the cooperation with the medical doctors are needed. MATERIAL AND METHODS: Four expert interviews from the disciplines of otolaryngology, endocrinology, and plastic surgery were conducted to establish a potential guideline consensus for the role of speech-language pathology as an interface to the male-to-female gender reassignment process. RESULTS: Based on the expert interviews, it is uniformly recommended that voice treatment should be considered early in the transition. Primarily, conservative therapy should be considered to avoid e.g. irreversible surgery of the larynx. The focus of voice treatment in voice feminization is centrally on the adaption of the speaking fundamental frequency. In postoperative cases, speech-language pathology is intended to adapt the speech pattern to the new anatomy and prevent complications, such as uneconomic voice and speech. CONCLUSION: The current interviews represent a first insight into the cooperation between speech-language pathology and the medical specialties for the treatment of MzF-Trans*. In order to implement the recommendations from the preliminary expert interviews for a potential guideline consensus, consultation with involved professional societies and more randomized trials of specific voice treatments in MzF-trans* are needed.

[Modern concepts in the treatment of intersexuality]. / Zum aktuellen Stand der Intersextherapie.

Treatment of intersexuality is demanding and requires experience and interdisciplinary cooperation. Preconditions for normal development and clear gender identification are correct (not emergency) diagnosis and gender assignment and adequate hormonal and surgical treatment. Surgery should be done early (6th to 15th month) as atraumatically as possible with cosmetically and functionally satisfying results. These preconditions are not met consistently, resulting in a 20-25% rate of mistakes in diagnosis and treatment. In experienced centers, feminizing genitoplasty, even of the severest forms, is carried out through a perineal one-stage approach. Masculinization corresponds to surgery for severe hypospadias. The high risk of malignant degeneration requires removal of all inadequate structures such as streak gonads, uterus, and tubes. In 5-alpha deficiency, early gonadectomy and feminization are not recommended since gyneophile behavior can be expected. Late or non-correction is rejected by the majority of psychiatrists. Many problems remain unclear and controversial due to lack of knowledge. In the future they can only be solved through cooperation, documentation, and observation of these individuals over their lifetime.

Familial hyperestrogenism in both sexes: clinical, hormonal, and molecular studies of two siblings.

Familial hyperestrogenism is a rare clinical condition of unknown etiology in which patients present excessive androgen to estrogen conversion. Excessive aromatization is primarily ascribed to abnormalities in the CYP19. Mice that lack steroid 5alpha-reductase type 1 also exhibit hyperestrogenism due to an increased availability of androgen precursors. Here we studied two adult siblings, born to unrelated parents, who presented clinical and hormonal evidence of estrogen excess. The man was treated with topical dihydrotestosterone, which promoted adequate virilization. The woman was treated with anastrazole, a potent aromatase inhibitor, with normalization of menstrual cycles. Genetic linkage to the steroid 5alpha-reductase type 1 gene (SRD5A1) was ruled out in this family. A similar analysis did not rule out linkage to CYP19, although no mutation was identified in the coding region of this gene. Aromatase mRNA was at least 10-fold more abundant in the female patient's skin fibroblasts vs. the control. Southern analysis of genomic DNA did not reveal rearrangements or amplification of the coding region of CYP19. We conclude that the phenotype of familial hyperestrogenism includes prepubertal gynecomastia, hypogonadism, and short stature in men, and precocious thelarche, macromastia, enlarged uterus, and menstrual irregularities in women. Topical dihydrotestosterone is an efficient alternative treatment in men with hyperestrogenism; in addition, second generation aromatase inhibitors are useful in both sexes.

Endocrine disorders of the pancreas and adrenal cortex in pediatric patients.

Hyperinsulinemia, a common cause of persistent hypoglycemia in infants and children, can result in permanent damage to the central nervous system. Thus, early diagnosis and treatment are important. The typical clinical manifestation of hyperinsulinemic hypoglycemia is symptomatic hypoglycemia that responds poorly to medical therapy. Affected infants may have tremors, jitteriness, apnea, cyanosis, or seizures. If initial medical therapy (frequent feedings, large amounts of intravenously administered glucose, diazoxide, and glucocorticoids) fails to stabilize plasma glucose levels, subtotal pancreatectomy is indicated. This surgical intervention does not completely correct the hypoglycemia in all patients, but it effectively reduces the severity of the condition. Surgical treatment of pediatric patients with Cushing's syndrome or aldosteronoma has also been effective. In our experience, survival of patients with adrenocortical adenomas has been 100%, whereas only five of nine children with adrenocortical carcinomas survived, and four of the five were younger than 10 years of age.

Feminization in hepatocellular carcinoma corrected by chemotherapy: a case report.

A 17-year-old boy with metastatic hepatocellular carcinoma and feminization became asymptomatic after administration of antineoplastic agents. Serum estradiol level, urine estrogens, and tumor estrogen receptors were changed after the treatment. Achievement of partial remission resulted in normalization of the serum sex hormone profile and disappearance of the feminization in spite of the presence of residual tumor. Thus, serum hormone levels alone cannot be used as tumor markers for control of therapy. This suggests that chemotherapy may have altered the biological behavior of the tumor, which resulted in loss of its ability to synthesize estrogen estrogen receptors.