Infantile hypopituitarism: etiological variability evidenced by MRI

In this study, magnetic resonance imaging (MRI) was performed to document potentially present morphostructural abnormalities of the hypothalamopituitary region in seven infants (age 0–21 months) who presented very early in life with clinical and biochemical evidence of hypopituitarism. Four infants had associated congenital cerebro-facial malformations.The following anatomical abnormalities were identified in variable combinations: ectopic neurohypophysis absence of the pituitary stalk, extreme elongation of the pituitary stalk, aplasia of the anterior pituitary lobe and no identification of the hypothalamopituitary complex.MRI proved to be very sensitive in the identification of structural malformations of the hypothalamopituitary region in infants with or without cerebro-facial malformations. AllhwuLll the magnetic resonance image does not appear to be a good predictor of endocrine dysfunction, it provides us more insight into the precise aetiology of this disorder and may be therefore of diagnostic, prognostic and therapeutic importance.     

让垂体瘤的治疗更趋完善

垂体瘤患者术后进行垂体功能的评估可以及时地对垂体功能减退者进行合适的替代治疗.但我们当前的临床现实是能够接受恰当的术后评估的患者还不到50％.只有加强内分泌科与神经外科医师的技术互补协作以及三级医院医师和基层医院医师的密切联系,提供以患者为中心的治疗才能弥补当前垂体瘤治疗存在的缺陷.     

The natural history of post-traumatic hypopituitarism: implications for assessment and treatment

Purpose

Hypopituitarism has been reported in up to half of long-term survivors of traumatic brain injury. We attempted to define the natural history of post-traumatic hypopituitarism to devise guidelines for the optimal timing of patients’ assessment and hormone replacement.

Subjects and methods

Fifty consecutive patients with severe or moderate head trauma were enrolled in a prospective study of pituitary function during the acute phase, at 6 months, and at 12 months after injury. Growth hormone and adrenocorticotropin hormone reserves were assessed using the glucagon stimulation test. Baseline serum concentrations of other anterior pituitary hormones were measured. Results were compared with normative data obtained from matched healthy controls.

Results

Nine patients (18%) had growth hormone deficiency in the acute phase; at 6 months, 5 patients recovered function and 2 new deficiencies were detected; at 12 months, 1 patient recovered, leaving 5 patients (10%) with growth hormone deficiency. Eight patients (16%) showed subnormal cortisol response in the acute phase; at 6 months, 4 patients had recovered and 5 new deficiencies were detected; all 9 patients had persistent abnormalities at 2 months. Forty patients (80%) had gonadotropin deficiency in the acute phase, of whom 29 (73%) recovered by 6 months and 34 (85%) recovered by 12 months. Thyrotropin deficiency was present in 1 patient in the acute phase, who recovered by 6 months; 1 new case was diagnosed at 6 months, which persisted at 12 months.

Conclusion

After traumatic brain injury, early neuroendocrine abnormalities are sometimes transient, whereas late abnormalities present during the course of rehabilitation. A follow-up strategy with periodic evaluation is a necessary part of the optimal care for patients with traumatic brain injury.     

Growth hormone replacement improved oocyte quality in a patient with hypopituitarism: A study of follicular fluid

BackgroundGrowth hormone (GH) is known to be involved in ovarian folliculogenesis and oocyte maturation. In patients with poor ovarian response without growth hormone deficiency (GHD), adjuvant GH treatment improves in-vitro fertilization (IVF) results. Improvement of oocyte quality in IVF by GH replacement was reported in only a few patients with GHD. We report on a new case with study of follicular fluid.MethodsA 29-year-old patient with hypopituitarism was referred to our infertility center. She was undergoing hormonal replacement for hypogonadotropic hypogonadism and diabetes insipidus, and did not consider at first GH replacement. Four IVF procedures were performed between 2011 and 2014. Growth hormone replacement (somatotropin 1.1 mg/day) was initiated before the fourth IVF procedure and unmasked central hypothyroidism; levothyroxine (75 mg/day) was introduced. It took 10 months to reach the treatment objectives for insulin-like growth factor 1 (IGF1), free triiodothyronine (fT3) and free thyroxine (fT4). GH, IGF1 and thyroid hormones were measured in the blood and follicular fluid before and after GH and thyroid hormone replacement. Oocyte and embryo quality were also compared.ResultsThe first 3 IVF procedures were performed without GH replacement. 62% to 100% of mature oocytes presented one or more morphologic abnormalities: diffuse cytoplasmic granularity, large perivitelline space with fragments, fragmentation of the first polar body, ovoid shape, or difficult denudation. Embryo quality was moderate to poor (grade B to D), and no pregnancy was obtained after embryo transfer. After GH replacement, hormones levels increased in follicular fluid: GH [7.68 vs. 1.39 mIU/L], IGF1 [109 vs. < 25 ng/mL], fT3 [3.7 vs. 2.5 pmol/L] and fT4 [1.45 vs. 0.84 ng/mL]. Concomitantly, there was dramatic improvement in oocyte quality (no abnormal morphologies) and embryo quality (grade A), allowing an embryo transfer with successful pregnancy.ConclusionsThis is the first report illustrating changes in hormonal levels in follicular fluid and the beneficial effect of GH replacement on oocyte and embryo quality during an IVF procedure in a patient with hypopituitarism. These results suggest that GH replacement is beneficial for oocyte quality in patients with GHD.     

Multidisciplinary protocol of preoperative and surgical management of patients with pituitary tumors candidates to pituitary surgery

The optimal planning of preoperative diagnosis, management and treatment of pituitary tumors (PT) candidates to pituitary surgery (PS) requires a multidisciplinary approach involving a team of endocrinologists, neurosurgeons, ENT, neuro-ophthalmologists and neuroradiologists with experience in pituitary diseases. Such teams improve surgical results, minimize complications and facilitate their correct treatment if occurring, and optimize the hormonal, ophthalmological and radiological preoperative and follow-up evaluation. We have developed a clinical practice protocol for patients with PT who are candidates to PS based on the most recent national and international guidelines and the relevant literature regarding PT published in the last years. The protocol has been elaborated by a multidisciplinary team of a Spanish Pituitary Tumor Center of Excellence (PTCE) that includes at least one neurosurgeon, ENT, neuroradiologist, neuro-ophthalmologist, endocrine pathologist and endocrinologist specialized in pituitary diseases. We elaborated this guideline with the aim of sharing our experience with other centers involved in the perioperative and surgical management of PT thereby facilitating the management of patients undergoing PS.     

Systemic sclerosis associated with generalized vasculitis and hypopituitarism

Systemic sclerosis (SSc) is a progressively evolving multisystemic disorder of unknown etiology. Beyond skin, several other organs can also be affected with a severity of involvement that is often heterogeneous. We describe a 53-year-old female patient who was admitted urgently to the hospital almost collapsed, because of numerous bleeding deep skin ulcers, located all over the body. Clinical findings and autoantibody screening were typical of SSc. Moreover, both histopathology and immunofluorescence findings were compatible with scleroderma and vasculitis as well. In addition, pituitary hormone investigation revealed severely damaged function of the gland. We assume that severe skin ulceration and serious hypopituitarism were both implications of underlying SSc-associated vasculitis. To the best of our knowledge, these peculiar clinical manifestations have not been described in the international literature to date.     

垂体瘤术后垂体功能评估情况的调查

目的 分析垂体瘤患者术后1周及3个月的垂体功能复查情况,为垂体瘤手术患者的后续临床治疗提供参考.方法 收集1993年1月至2010年1月期间解放军总医院内分泌科住院诊断为垂体瘤并进行完整垂体功能评估后行垂体瘤切除术的106例患者,回顾性分析其各项临床资料.结果 患者术后1周与3个月的乖体功能评估率分别为23.6％与16.0％.术前存在垂体功能减退患者,术后1周与3个月的垂体功能评估率分别为23.5％与5.9％,术前垂体功能无减退者垂体功能评估率分别为23.6％和20.8％,术前存在垂体功能减退者并未得到更多的垂体功能评价关注.术后l周因手术导致新发垂体功能减退率为48.0％,术后3个月为35.3％.结论 垂体瘤术后1周内及3个月复查率低,垂体瘤术后可能存在较多漏诊垂体功能减退的患者.