A Case of Familial Male-Limited Precocious Puberty in a Child With Klinefelter Syndrome.

Familial male-limited precocious puberty (FMPP) is an autosomal dominant, male-limited disorder that causes peripheral precocious puberty in boys. Klinefelter syndrome (47, XXY) is the most common chromosomal aberration in males with associated infertility, hypogonadism, and learning disability. We report here a case of Klinefelter syndrome in a patient with FMPP. A 6-year-old boy was referred to our pediatric endocrinology department for accelerated linear growth and premature pubic hair development. He was diagnosed with FMPP based on clinical, laboratory, and genetic sequencing. Increased levels of gonadotropins prompted further investigation, leading to a subsequent diagnosis of Klinefelter syndrome through karyotype analysis. This case illustrates that patients with FMPP and elevated gonadotropins should encourage further investigation by physicians. We recommend the use of karyotype analysis in such patients who are not receiving aromatase inhibitor therapy. We hypothesize that his mutation or pretreatment with aromatase inhibitors may have a protective effect on testosterone production and sperm viability.

Development and Evaluation of a Pediatric Diabetes Program by a Single Provider.

PURPOSE: Although the use of a team approach is ideal for a pediatric population, such an approach is expensive, with programs running at a negative balance of $400,000 to $800,000 per year. To address the problem, a "state of the art" pediatric diabetes program was implemented that was cost neutral and did not compromise patient care. METHODS: Four years after starting a pediatric diabetes program run by a single provider, diabetes goals were evaluated by checking A1C levels while keeping costs in check. A1C levels were obtained every 2 to 3 months and analyzed over several months. Two hundred patients with type 1 diabetes were managed in the program. The cost of the program was analyzed on the basis of rental fees, staff salaries, and basic equipment and supplies required. A1C levels were performed using the Siemens HbA1c DCA Vantage Analyzer. Patients are able to call, fax, text, and e-mail between visits. This has resulted in excellent control and high satisfaction. RESULTS: The mean A1C level was 7.2% to 7.4%. The pediatric diabetes program is now cost neutral. Survey results indicated that patients were satisfied with the care they received. CONCLUSIONS: Children with type 1 diabetes can be managed by a single provider and achieve treatment goals that far exceed those obtained in most pediatric diabetes programs. This can be done at a fiscally responsible cost.

Técnicas de abordaje utilizadas en tratamientos de emergencias endodónticas en la práctica privada en la zona de la colonia médica ubicada en el área metropolitana de San Salvador / Approach techniques used in endodontic emergency treatments in private practice in the area of the medical colony located in the metropolitan area of San Salvador

El propósito del presente trabajo es conocer las técnicas y materiales odontológicos, utilizadas en el abordaje de las emergencias Endodónticas por odontólogos en su práctica privada. Por lo que visitamos consultorios odontológicos, ubicados en La Colonia Médica del área Metropolitana de la ciudad de San Salvador, en donde se abordaron a los odontólogos que laboran en dichas clínicas a través de una cédula de entrevista, con cinco preguntas cerradas y una abierta, todas ellas relacionadas con el tema de investigación. Encontramos que la mayoría de los profesionales entrevistados dejan cerrada la apertura cameral con algodón y cemento provisional; y el medicamento intracanal de mayor uso es el Hidróxido de Calcio. Por lo que se concluye que los profesionales atienden casos de emergencias y al ejecutar la apertura cameral el Hidróxido de Calcio es el material más utilizado en las emergencias Endodónticas.

The purpose of this work is to know the dental techniques and materials used in the approach to Endodontic emergencies by dentists in their private practice. Therefore, we visited dental offices, located in La Colonia Médica in the Metropolitan area of ​​the city of San Salvador, where the dentists who work in these clinics were approached through an interview card, with five closed questions and one open question. all of them related to the research topic. We find that most of the professionals interviewed leave the chamber opening closed with cotton and temporary cement; and the most widely used intracanal drug is Calcium Hydroxide. Therefore, it is concluded that professionals attend to emergencies and when executing the chamber opening, Calcium Hydroxide is the most used material in Endodontic emergencies.

Final adult height in children with Prader-Willi syndrome with and without human growth hormone treatment.

Short stature is characteristic of children with Prader-Willi syndrome (PWS). While previous studies have demonstrated acceleration of linear height velocity with growth hormone (GH) treatment, the long-term benefit on final adult height (AH) has not been reported. The objective of this study was to compare AH attained in PWS subjects with and without GH treatment. We reviewed the records of 21 children (aged 8.3 +/- 2.7 years) with PWS and confirmed GH deficiency that attained AH after receiving human GH treatment (0.25 +/- 0.06 mg/kg/week) for a period of 7.9 +/- 1.7 years. A group of 39 non-GH-treated adults with matched initial height standard deviation score (SDS) at age 6.8 +/- 1.3 years was used as control. In the GH-treated group the mean initial height and AH-SDS was -1.9 +/- 1.7 and -0.3 +/- 1.2 respectively (P < 0.0001), whereas the mean initial and AH-SDS in the control group was -1.9 +/- 1.3 and -3.1 +/- 1 respectively (P < 0.0001). Scoliosis was seen in 43% and 39% in the GH-treated and control group respectively. Premature adrenarche (PA) was noticed in 57% of GH-treated group. Six subjects in the control group but none of the GH-treated subjects developed type 2 diabetes mellitus. Our data show that administration of GH to children with PWS restores linear growth and final AH without significant adverse effects other than PA. Further studies will be necessary to determine related morbidity and mortality in individuals with PWS that reached final AH with or without GH treatment.