No abstract

Despite being a classical growth disorder, pituitary gigantism has not been studied previously in a standardized way. We performed a retrospective, multicenter, international study to characterize a large series of pituitary gigantism patients. We included 208 patients (163 males; 78.4%) with growth hormone excess and a current/previous abnormal growth velocity for age or final height O2 S.D. above country normal means. The median onset of rapid growth was 13 years and occurred significantly earlier in females than in males; pituitary adenomas were diagnosed earlier in females than males (15.8 vs 21.5 years respectively). Adenomas were R10 mm (i.e., macroadenomas) in 84%, of which extrasellar extension occurred in 77% and invasion in 54%. GH/IGF1 control was achieved in 39% during long-term follow-up. Final height was greater in younger onset patients, with larger tumors and higher GH levels. Later disease control was associated with a greater difference from mid-parental height (rZ0.23, PZ0.02). AIP mutations occurred in 29%; microduplication at Xq26.3 -X-linked acrogigantism (X-LAG) -occurred in two familial isolated pituitary adenoma kindreds and in ten sporadic patients. Tumor size was not different in X-LAG, AIP mutated and genetically negative patient groups. AIP-mutated and X-LAG patients were significantly younger at onset and diagnosis, but disease control was worse in genetically negative cases. Pituitary gigantism patients are characterized by male predominance and large tumors that are difficult to control. Treatment delay increases final height and symptom burden. AIP mutations and X-LAG explain many cases, but no genetic etiology is seen in O50% of cases.

ObjectiveTo evaluate extended dosing intervals (EDIs) with lanreotide Autogel 120 mg in patients with acromegaly previously biochemically controlled with octreotide LAR 10 or 20 mg.Design and methodsPatients with acromegaly had received octreotide LAR 10 or 20 mg/4 weeks for ≥6 months and had normal IGF1 levels. Lanreotide Autogel 120 mg was administered every 6 weeks for 24 weeks (phase 1); depending on week-24 IGF1 levels, treatment was then administered every 4, 6 or 8 weeks for a further 24 weeks (phase 2). Hormone levels, patient-reported outcomes and adverse events were assessed. Primary endpoint: proportion of patients on 6- or 8-week EDIs with normal IGF1 levels at week 48 (study end).Results107/124 patients completed the study (15 withdrew from phase 1 and two from phase 2). Of 124 patients enrolled, 77.4% were allocated to 6- or 8-week EDIs in phase 2 and 75.8% (95% CI: 68.3–83.3) had normal IGF1 levels at week 48 with the EDI (primary analysis). A total of 88.7% (83.1–94.3) had normal IGF1 levels after 24 weeks with 6-weekly dosing. GH levels were ≤2.5 μg/l in >90% of patients after 24 and 48 weeks. Patient preferences for lanreotide Autogel 120 mg every 4, 6 or 8 weeks over octreotide LAR every 4 weeks were high.ConclusionsPatients with acromegaly achieving biochemical control with octreotide LAR 10 or 20 mg/4 weeks are possible candidates for lanreotide Autogel 120 mg EDIs. EDIs are effective and well received among such patients.

The view of the clinical picture of primary hyperparathyroidism (PHPT) has essentially changed during the recent years under the influence of new data about its prevalence and the structure of the disease. The analysis of the database created at the Endocrinological Research Centre and including 1053 patients was used to obtain an epidemiological characteristics of various forms of PHPT recorded in Russia. PHPT morbidity in Moscow was estimated, based on the incidence of outpatient visits, to reach 0.05 per 1,000 adult population. This figure is significantly lower than that reported in foreign publications. The manifest form appears to be the most widespread form of PHTP in this country. The relative prevalence of the mild form has increased in the last years (till 2012) but does not exceed 28% or significantly lower than abroad where it is reported to prevail in the structure of PHPT morbidity. The asymptotic form accounts for 7.5% of all PHPT cases in the framework of type 1 multiple endocrine neoplasia syndrome (MEN-1) where it most frequently occurs in combination with pituitary adenomas (81%), in the first place prolactinomas, somatotropinomas, and pancreatic tumours (45%).