Delayed diagnosis of congenital hypopituitarism associated with low socio-economic status and/or migration.

The clinical presentation of combined pituitary hormone deficiency (CPHD) is variable. Some patients present with hypoglycemia during the neonatal period or during the first few years of life. Others present later in childhood with growth failure. We report on 7 patients with very late diagnosed severe hypopituitarism with pituitary stalk interruption syndrome. Five out of the 7 patients had recently migrated to Belgium and the 2 other patients were from low socio-economic status families. All of them presented to our clinic for short stature and some also complained of lack of pubertal development. Four out of the 7 patients reached final height which was within their target height, despite very delayed treatment.Conclusion: We illustrate the overall good outcome of these children with delayed diagnosed severe hypopituitarism. Adverse life conditions and social deprivation are thought to be the cause of their late diagnosis. In the current global socio-politic context, pediatricians in high-income countries should stay aware that migration and poor socio-economic status can be associated with specific clinical presentations.What is Known:• The clinical presentation of combined pituitary hormone deficiency (CPHD) is variable. Some patients present with hypoglycemia during the neonatal period or during the first few years of life. Others present later in childhood with growth failure.• A few case reports are published with very late diagnosis of congenital hypopituitarism.What is New:• We report on the largest series of delayed diagnosis of congenital hypopituitarism and illustrate the survival of these children with overall good prognosis.• Migration and social deprivation are thought to be the main cause of this late diagnosis.

Pediatric pituitary resection: characterizing surgical approaches and complications.

BACKGROUND: Although there has been extensive study evaluating adult pituitary surgery, there has been scant analysis among children. Our objective was to evaluate a population-based resource to characterize nationwide trends in surgical approach, hospital stay, and complications among children undergoing pituitary surgery. METHODS: The Kids' Inpatient-Database (KID) files (2009/2012) were evaluated for pituitary gland excisions. Procedure, patient demographics, length of inpatient stay, inpatient costs, hospital setting, and surgical complications were analyzed. RESULTS: A weighted incidence of 1071 cases were analyzed; the majority (77.6%) underwent transsphenoidal resections. These patients had significantly decreased hospital costs and lengths of stay. Patients undergoing transfrontal approaches had significantly greater rates of postoperative diabetes insipidus (DI) (66.5%), panhypopituitarism (38.8%), hydrocephalus, and visual deficits. Among transsphenoidal patients, males had greater rates of postoperative hydrocephalus (5.5%) and panhypopituitarism (17.5%) than females, and patients ≤10 years old had greater rates of these 2 complications (14.5%, 19.4%, respectively) as well as DI (61.3%). CONCLUSION: A greater proportion of children undergo transfrontal approaches for pituitary lesions than in their adult counterparts. This difference may harbor a potential to influence future sellar resection approaches in children toward a transsphenoidal operation when surgically feasible. Patients undergoing transfrontal procedures have greater risks for many intraoperative and postoperative complications relative to individuals undergoing transsphenoidal resections. Among patients undergoing transsphenoidal approaches, males had significantly greater rates of postoperative hydrocephalus and panhypopituitarism, and younger children had greater rates of postoperative DI, hydrocephalus, and panhypopituitarism. These data reinforce the need for greater vigilance in the postoperative care of younger children undergoing transsphenoidal surgery.

Assessment of quality of life in adult patients with GH deficiency: KIMS contribution to clinical practice and pharmacoeconomic evaluations.

Quality of life (QoL) has emerged as an important construct that has found numerous applications across healthcare-related fields, ranging from research and clinical evaluation of treatment effects to pharmacoeconomic evaluations and global healthcare policy. Impairment of QoL is one of the key clinical characteristics in adult GHD and has been extensively studied in the Pfizer International Metabolic Database (KIMS). We provide summarized evidence on GH treatment effects for both clinical and health economic applications based on the KIMS data. The primary focus is on those aspects of QoL research that cannot be investigated in the traditional clinical trial setting, such as specific patient subgroups, cross-country comparisons and long-term follow-up. First, the impact of age, gender, disease onset, primary aetiology, extent of hypopituitarism, previous radiotherapy and obesity on QoL before and during long-term GH replacement is discussed. Secondly, the studies on QoL in relation to country-specific normative values are reviewed. Finally, health economic data derived from KIMS including both burden of disease and utility assessment are evaluated. We conclude that the wide spectrum of analyses performed on the KIMS data allows for practical application of the results not only to research and clinical practice but also to health policy and global medical decision making.

[Once growth hormone, always growth hormone? Transition from growth hormone therapy in childhood to adulthood]. / Eenmaal groeihormoon, altijd groeihormoon? De transitie van groeihormoonbehandeling van kind naar volwassene.

The continuation of growth hormone treatment can be indicated in young adults who have been treated with growth hormone during childhood. However, in a large part of this population the diagnosis cannot be confirmed in adulthood. Therefore a retest procedure has to be performed once the final height has been attained. This procedure is only unnecessary in patients with deficiencies of two or more other pituitary hormone axes. The retest procedure can be performed one to three months after the growth hormone treatment has been discontinued, by means of an insulin tolerance test or, in the case of contraindications, by means of a combined growth hormone-releasing hormone(GHRH)-arginine test. If the growth hormone deficiency diagnosis is re-established, growth hormone treatment can be restarted. Patients are only eligible for a reimbursement of the growth hormone treatment costs from their health insurer, if the treatment indication is validated by the Dutch National Registry of Growth Hormone Treatment in Adults and the treatment results are included in a database. With this database insights into the long-term efficacy and safety of growth hormone treatment can be gained.

Cost of illness in adult patients with hypopituitarism.

OBJECTIVE: To compare the healthcare costs of patients with hypopituitarism with those of individuals from the general population. DESIGN: A retrospective study of costs over 1 year. Estimates of direct and indirect health-related costs were calculated for patients from the general population using existing databases, and for patients with hypopituitarism using records of all patients eligible to participate on 31 December 1989 who could be traced, were willing to participate and had not been treated for acromegaly or Cushing's disease. SETTING: The catchment area of the Endocrine Unit, Sahlgrenska Hospital, Gothenburg. The study was conducted from the societal perspective. Reference data were collected from official regional and national registries. PATIENTS: 199 patients with adult-onset hypopituitarism in whom replacement therapy was given to maintain the adrenal, thyroid and gonadal (but not the somatotropic) axes. MAIN OUTCOME MEASURES AND RESULTS: Direct and indirect costs incurred by patients with hypopituitarism were higher than those incurred by individuals from the general population. The total direct costs per patient were Swedish Crowns (SEK)22,920 vs SEK12,080 (p < 0.003) in the general population, and the highest costs were related to inpatient care. Of the patients aged 16 to 64 years, 22% had drawn a disability pension versus the expected 11.3% (p < 0.003) in the general population, and the patients had a mean sick leave of 38.4 days vs 23.5 (p < 0.001). Total excess costs for all patients with hypopituitarism were SEK 35,768 per patient (p < 0.007). CONCLUSIONS: Patients with hypopituitarism incur more health-related costs than individuals from the general population. They also take more sick leave days and are more likely to claim a disability pension than members of the general population. Further cost analyses are needed to determine whether improvements in diagnostic and surgical procedures, and hormone replacement therapy, can reduce the healthcare costs of patients with hypopituitarism.

Observational study in adult hypopituitary patients with untreated growth hormone deficiency (ODA study). Socio-economic impact and health status. Collaborative ODA (Observational GH Deficiency in Adults) Group.

OBJECTIVE: The aim of the present study was to assess the socio-economic impact at baseline and after one year of follow-up of clinical and health status characteristics and laboratory tests of adult-onset GH deficiency (AGHD), a well-known clinical entity, in a large group of Spanish hypopituitary patients with untreated AGHD. DESIGN AND METHODS: A total of 926 eligible patients with GHD (GH

The burden of illness of hypopituitary adults with growth hormone deficiency.

OBJECTIVE: The negative metabolic and psychosocial consequences of growth hormone deficiency (GHD) in adults are now well established. In the present study, an attempt was made to quantify the burden of illness, in terms of lost productivity and increased medical consumption, associated with hypopituitarism and untreated GHD. DESIGN AND SETTING: The study population consisted of 129 Belgian adults with untreated GHD associated with hypopituitarism after pituitary surgery. The Short-Form 36 Health Survey (SF-36) was used to assess health status, and the Health and Labour Questionnaire was used to measure production losses and labour performance. Data on medical consumption were also collected. MAIN OUTCOME MEASURES AND RESULTS: Hypopituitary patients reported a lower health status than that of the general population in all but two dimensions of the SF-36 (pain and physical functioning). Nearly 11% of the patients reported being incapacitated for paid employment due to health problems, compared with 4.8% of the general Belgian population. Patients in paid employment reported a mean of 19.8 days of sickness leave per year, which is twice that in the general population. The annual number of visits to general practitioners and specialists was also higher in the patients (9.6 and 6.5 visits, respectively, for the patients compared with corresponding figures of 2.1 and 1.5 for the general Belgian population). The average annual number of days spent in hospital was 3.5 for the patients compared with 2.3 in the general population. The annual healthcare costs and costs due to production losses calculated for hypopituitary patients who had received pituitary surgery amounted to 135,024 Belgian francs (BeF) or $US4340 (1995 values). This compares with the mean annual cost per person for the Belgian population as a whole of BeF68,569 or $US2204. CONCLUSIONS: Hypopituitary patients with untreated GHD therefore have a higher cost to society in terms of lost production and medical consumption than the average Belgian population.