Use of Gonadotropin-Releasing Hormone for Intractable Seizures in a Girl with Precocious Puberty without Hypothalamic Hamartoma.

The use of gonadotropin-releasing hormone analogs has been reported in the treatment of gelastic seizures and precocious puberty associated with hypothalamic hamartomas, but not in other seizure types without hypothalamic hamartoma. We describe a 7.5 year-old girl whose seizures subsided after gonadotropin-releasing hormone analog implant, administered for precocious puberty.

Improving screening for diabetes in cystic fibrosis.

PURPOSE: Annual screening for cystic fibrosis-related-diabetes (CFRD) using oral glucose tolerance test (OGTT) is recommended, but national testing rates are low. The purpose of this paper is to implement the quality improvement (QI) initiative to improve cystic fibrosis (CF) annual screening rates among patients at one CF center. DESIGN/METHODOLOGY/APPROACH: To improve screening for CFRD at the CF Center, the authors used the Dartmouth Microsystem Improvement Ramp method and formed a collaborative working group. A process map was created to outline the steps and a fishbone analysis was performed to identify barriers and to utilize resources for implementing new interventions. FINDINGS: Prior to these interventions, 21 percent of eligible patients had completed annual screening and after the intervention, it rose to 72 percent. The initial completion rate with the first prescription was only 50 percent, but it improved steadily to 54/75 (72 percent) in response to reminder letters sent six weeks after the initial script was given. PRACTICAL IMPLICATIONS: Close tracking and reminder letters can improve adherence with annual OGTT screening for CFRD among CF patients, with special emphasis on high-risk patients. ORIGINALITY/VALUE: There should be a special emphasis on screening for CFRD in high-risk CF patients (those with low BMI or higher age). This QI initiative brought about several operational changes in the annual OGTT screening process that have now become the standard operating procedure at the center.

Pituitary abscess in an adolescent girl: a case report and review of the literature.

We report the case of a 15-year-old girl who presented with a history of recurrent bitemporal headaches for the last 2 months. In the prior few days, she complained of neck pain, emesis, phonophobia and photophobia, but no fever. Additional symptoms included polydipsia, polyuria and weight gain in the last year. Magnetic resonance imaging (MRI) of the brain demonstrated a cystic sellar and suprasellar mass with peripheral enhancement. Cerebrospinal fluid studies showed pleocytosis. Serum hormone levels were consistent with panhypopituitarism. Transnasal sphenoidotomy was performed, and 2 mL of purulent material was drained, confirming the diagnosis of pituitary abscess. The patient completed 6 weeks of parenteral antibiotics. She improved but continued to require home hormonal replacement therapy. A repeated MRI 3 months later showed abscess resolution. In addition to tumors, pituitary abscess should be considered in children who present with headache and panhypopituitarism, particularly in those who present with signs of meningeal inflammation. Prolonged parenteral antibiotics and surgical drainage are effective.

Neonatal thyrotoxicosis presenting as persistent pulmonary hypertension.

Neonatal hyperthyroidism is a rare condition caused either by transplacental passage of thyroid-stimulating immunoglobulins from a mother with Graves' disease or by activating mutations of the thyrotropin receptors and α-subunit of G-protein. The clinical features may vary. We report a case of neonatal thyrotoxicosis in an infant born to a mother with Graves' disease, who presented with cardiorespiratory failure and persistent pulmonary hypertension (PPHN). PPHN resolved with specific antithyroid treatment and extracorporeal membrane oxygenation was not required.

Education and multidisciplinary team care concepts for pediatric and adolescent diabetes mellitus.

The DCCT scientifically established the basis for optimizing blood glucose control in type 1 diabetes mellitus around the world using a multidisciplinary team approach and patient-centered adjustments of food and insulin based upon blood glucose data generated by the patient. Pediatric diabetologists no longer believe that it is prudent to allow higher blood glucose levels in prepubertal children but much educational emphasis must be placed upon minimizing serious episodes of hypoglycemia. Individualized treatment should be determined by a close working relationship between highly trained diabetes nurses, educators and dieticians with the patient as the focus of self-care decisions, and a pediatric diabetologist ideally setting the philosophical and medical goals. Rather than the diabetes health care team being the only ones to initiate treatment, patient and parents should be empowered to analyze their own data, identify patterns, solve problems with food and activity, and do so based upon actual blood glucose results. This empowerment paradigm helps decrease care frustrations and improve treatment outcomes. Survival education followed by in-depth problem solving education and organized follow-up education are all needed steps for successful diabetes management. Identification of psychosocial barriers and energy diverting behavioral and family issues just as knowledge about learning styles play key roles in this process. Dogma should be avoided. More physiological utilization of insulin analogs, greater insulin dosing flexibility with a multidose insulin regimen coupled with adaptation of insulin to food and activity, should allow maximum benefit. Four major types of learning styles are reviewed: concrete sequential learners, abstract sequential learners, abstract random learners and concrete random learners. Health Belief Models, Locus of Control constructs, and Self-Efficacy models all provide sophisticated ways to help identify and overcome learning and self-care barriers. Parental, child, adolescent and young adult responsibility for care also needs to be addressed and placed in the context of family functioning and glycemic goals. Age and developmental stages as well as parental and societal roles play important roles in the care needed to live well with a chronic illness. The role of the health care professionals who are part of the diabetes care team involves not only setting the stage and providing guidance but also supervising appropriate short- as well as long-term complications monitoring for early detection and treatment of microangiopathy. Applying not only telephone but also fax, e-mail and computers in modern diabetes care should facilitate applications of these psychological, educational and medical models to improve short-term and long-term diabetes treatment outcomes.