Complicated osteoporosis in progeroid syndrome: treatment with teriparatide.

Human progeroid syndromes (PSs) include a group of genetic "premature aging" diseases that affect a variety of organ systems. Bone diseases are common sequelae of patients diagnosed with PSs. Teriparatide therapy is recommended for elderly men with low bone mineral density (BMD; T-score <-2.5) and at least 1 fragility fracture who are unable to tolerate bisphosphonates. We describe a 20-yr-old patient affected by PS and severe osteoporosis complicated with femoral fracture. The patient experienced a significant improvement in lumbar spine BMD after treatment with teriparatide.

[Primary hyperparathyroidism. An alternative to the surgery]. / Hiperparatiroidismo primario. Una alternativa a la cirugía.

Primary hyperparathyroidism is caused by an adenoma/hyperplasia in the parathyroid glands in which hypercalcemia is mainly due to an increased secretion of parathormone (PTH). The only definitive treatment is surgery. There are some patients at high surgical risk or who refuse surgery, and whose hypercalcemia cannot be controlled with conventional medical therapy such as hydration, diuretics and/or oral biphosphonates. We suggest the use of two drugs indicated for the treatment of hypercalcemia of other etiologies: zoledronic acid, a parenteral bisphosphonate, and cinacalcet, a calcimimetic agent that reduces PTH secretion. We present the case of a woman with hypercalcemia due to primary hyperparathyroidism caused by an adenoma, who was treated with both drugs.

Acute and long-term effects of zoledronate in adult patients with osteogenesis imperfecta. An observational Spanish study with five years of follow-up. / Efectos agudos y a largo plazo del tratamiento con zolendronato en pacientes adultos con osteogénesis imperfecta. Estudio español observacional con 5 años de seguimiento.

Osteogenesis imperfecta (OI) is an inherited disorder that causes low mineral density and bone fragility. Previous studies have shown the efficacy of bisphosphonates to increase bone mineral density (BMD). This study assessed changes over time in BMD and biochemical markers of bone metabolism in adult patients with osteogenesis imperfecta treated with intravenous zoledronic acid and the safety of this treatment. PATIENTS AND METHODS: A prospective, observational study in patients with OI, osteoporosis or osteopenia (T score <-2) who were administered zoledronic acid infusions (4mg IV) every 6 months for three years and annually thereafter. Densitometry was performed annually. Acute changes in complete blood count and calcium, phosphate, and creatinine levels, as well as side effects of the infusion, were recorded 24 and 48h after treatment. Calcium, phosphate, parathyroid hormone (iPTH), 25OH-vitamin D and bone turnover markers (bone alkaline phosphatase, ß-crosslaps and urinary deoxypyridinoline) were measured at baseline and every 12 months. Adverse events and new fractures were recorded. RESULTS: Twenty patients (6 men and 14 women) were treated. Median follow-up time was five years. Calcium levels and platelet counts significantly decreased 24 and 48hours after the first infusion, and the red blood cell count decreased at 24hours. These changes were not clinically relevant. Seven patients experienced a flu-like episode after the first dose. Treatment induced significant increases in BMD in the lumbar spine (6.7%) after 12 months of follow-up (0.791±0.178 vs. 0.791±0.140g/cm2, p=.003) and at three (5.7%) and five years (9%) of follow-up. Femoral neck BMD significantly increased after 3 years (11.1%): 0.648±0.148 vs. 0.720±0.138g/cm2; p=.01. In total hip, increase in BMD (10.1%) was significant after three years of treatment (0.706±0.118 vs. 0.720±0.138, p=.01). There were no significant differences in calcium and 25OH-vitamin D levels during follow-up, phosphorus significantly decreased after one year, and iPTH increased at three years. ß-crosslaps decreased after one year of treatment. Only one patient sustained new fractures. CONCLUSIONS: Zoledronic acid is a convenient, safe, and effective treatment that increases BMD in adult patients with OI.

Implication of gestational diabetes treatment on maternal weight gain and low neonatal weight: a large retrospective cohort study.

INTRODUCTION: Objective: the treatment for gestational diabetes is based on diet, and this may modify maternal weight gain. The limited maternal weight gain is related to newborns with small weight for their gestational age (SGA), and many studies have found an increase of SGA in women with gestational diabetes (GD), but the reason for this is not clear. The objective of this study is to evaluate the effects of gestational diabetes treatment on maternal weight gain and neonatal weight. Methods: a retrospective cohort study of 1,765 patients with GD, according to the National Diabetes Data Group (NDDG) criteria. We assessed: pre-pregnancy BMI, total maternal weight gain (MWG), weight gain during the third trimester, gestational week of starting the treatment, and treatment modality (diet or diet plus insulin). Birth weight was adjusted by gestational age and gender: SGA (≤ 10th) and large for gestational age (LGA) (> 90th). Results: the percentage of newborns with weight ≤ 10 was 14.8 %. The diet and the time of initiation of the treatment were related to maternal weight gain (MWG) in the third trimester. For every 1 kcal/kg of variation in the diet (increase or decrease), a MWG variation of 0.03 (0.001-0.06) kg occurred (p < 0.01). For each week before the beginning of treatment, the mother did not gain 0.13 ± [(-0.15) - (-0.11)] kg in the third trimester (p < 0.01). The SGA was related to the lowest MWG in total gestation: 7.0 (IQR 3.0-10.4) kg vs 8.4 (IQR 5.0-11.6) kg (p < 0.01), and in the third trimester: 0.3 (IQR -0.9-1.5) kg vs. 0.9 (IQR -0.3-2.2) kg (p < 0.01). Conclusion: the dietary treatment for gestational diabetes leads to a lower maternal weight gain and induces an impact on neonatal weight.

INTRODUCCIÓN: Objetivo: el tratamiento para la diabetes gestacional se basa en la dieta y esto puede modificar el aumento de peso materno. Un aumento de peso materno limitado está relacionado con recién nacidos con bajo peso para su edad gestacional (SGA). Muchos estudios han encontrado un aumento de niños con bajo peso en mujeres con diabetes gestacional, pero la razón no está clara. El objetivo es evaluar los efectos del tratamiento de la diabetes gestacional sobre el aumento de peso materno y el peso neonatal. Métodos: estudio de cohortes retrospectivo en 1765 pacientes con diabetes gestacional. Evaluamos: IMC antes del embarazo, aumento de peso materno total, aumento de peso durante tercer trimestre, semana gestacional de inicio y modalidad de tratamiento (dieta o dieta más insulina). El peso al nacer se ajustó por edad gestacional y género: SGA (≤ 10) y grande para la edad gestacional (> 90). Resultados: el porcentaje de recién nacidos con peso ≤ 10 fue 14,8%. La dieta y el momento de inicio del tratamiento se relacionaron con aumento de peso materno en el tercer trimestre. Por cada 1 kcal/kg de variación en dieta (aumento o disminución) se produjo una variación de aumento del peso materno de 0,03 (0,001-0,06) kg (p < 0,01). Por cada semana antes de inicio del tratamiento la madre dejó de ganar 0,13 ± [(- 0,15)-(- 0,11)] kg en el tercer trimestre (p < 0,01). El SGA se relacionó con un aumento de peso materno más bajo en el total de la gestación: 7,0 (IQR 3,0-10,4) kg versus 8,4 (IQR 5,0-11,6) kg (p < 0,01), y en el tercer trimestre: 0,3 (IQR -0,9-1,5) kg vs. 0,9 (IQR -0,3-2,2) kg (p < 0,01). Conclusión: el tratamiento dietético para la diabetes gestacional puede conducir a un menor aumento de peso materno y a su influir en el peso neonatal.

Radioiodine therapy for multinodular goiter.

Treatment options for large, compressive goiters are currently limited to surgery and radioiodine administration. Classically, the first-line option has been surgery, with radioiodine therapy being reserved as an alternative treatment in patients with high surgical risk. We describe the case of an 81-year-old woman with a large, compressive multinodular goiter and hyperthyroidism, substernal extension and associated co-morbidity, contraindicating surgery. We review the efficacy of different treatment options for compressive multinodular goiter, as well as the potential secondary complications.

[Casuistry review of patients undergoing surgery for pituitary adenomas in Getafe University Hospital (Spain)]. / Revisión casuística en el hospital universitario de Getafe de pacientes con patología hipofisaria adenomatosa intervenidos quirúrgicamente.

OBJECTIVE: To perform a casuistry review of patients diagnosed with pituitary adenomas (PA) who underwent surgery performed by the same neurosurgeon after 1995. MATERIAL AND METHODS: A descriptive and retrospective study was performed in 98 patients with PA undergoing surgery from 1995-2008. Tumor size and data on functionality, pathology and postprocedural complications were analyzed. The study was divided into two periods: 1995-2002 (first period) and 2003-2008 (second period). RESULTS: A total of 110 surgical interventions (59 in the first period and 51 in the second) were performed for 49 non-hormone-producing PA and 61 hormone-producing PA. There were 85 macroadenomas and 25 microadenomas. Cure was achieved in 31 patients (36%) with macroadenomas and in 21 patients (84%) with microadenomas (P=0.05).The number of complications was significantly higher in the first period [32 patients (54 %)] than in the second period [16 patients (31.3%)] (P<0.05). Cure was achieved in 28 patients (47.4%) in the first period compared with 31 (52.1%) in the second (P=0.1). The mortality rate was 0.9%. CONCLUSIONS: As described in previous studies, the cure rate was significantly higher for microadenomas than for macroadenomas. There was a significant reduction in the incidence of surgical complications and a trend toward an increase in the percentage of overall healing in the second period of the study. These results are probably related to the neurosurgeon's greater experience and surgical skill.

[Effects of zoledronic acid in adults with osteogenesis imperfecta]. / Efectos del tratamiento con ácido zoledrónico en pacientes adultos con osteogénesis imperfecta.

BACKGROUND AND OBJECTIVE: Osteogenesis imperfecta (OI) is a genetic disorder that results in bone fragility. Several studies have demonstrated the effectiveness of bisphosphonate therapy. The aim of this study was to evaluate the effects of intravenous zoledronic acid on bone mineral density (BMD) and biochemical markers of bone turnover in adults with OI. MATERIAL AND METHODS: We carried out a prospective non-randomized study in patients with osteoporosis or severe osteopenia (T score < -2) related to OI and intolerance or contraindication to oral bisphosphonates. The patients were treated with a zoledronic acid infusion every 6 months. Densitometry was carried out annually. Calcium (Ca), phosphate (P), intact parathormone (PTH), 25 hydroxyvitamin D and biochemical markers of bone turnover [bone alkaline phosphatase (BAP), beta-cross-laps (CTX) and urinary deoxypyridoxine (DOP)] were measured every year. Adverse events and new fractures were registered. RESULTS: Ten patients (2 men and 8 women) were treated. Treatment increased BMD measured in the lumbar spine after 24 (0.738+/-0.141 vs 0.788+/-0.144 g/cm(2); p=0.048) and 36 months (0.720+/-0.139 vs 0.820+/-0.128; p=0.01). Significant increases in BMD were also observed after 24 months in the femoral neck (0.677+/-0.121 vs 0.703+/-0.122 g/cm(2); p<0.016). Serum Ca, P, BAP and CTX concentrations remained unchanged. PTH concentrations increased and vitamin D concentrations decreased after 36 months of treatment. DOP excretion decreased significantly after 24 months. Seven patients had mild influenza-like symptoms occurring within the first 24 h after the first infusion. No severe adverse events were observed. None of the patients had new fractures. CONCLUSION: Zoledronic acid seems to be a safe and effective treatment option in adults with osteoporosis related to OI.