scholarly journals Anterior Pituitary Dysfunction in Children with Idiopathic Central Diabetes Insipidus

1995 ◽  
Vol 4 (Supple6) ◽  
pp. 109-114
Author(s):  
Yoko Nomura ◽  
Kazumichi Onigata ◽  
Ryo Ogawa ◽  
Tomohisa Nagashima ◽  
Hiroshi Miyake ◽  
...  
2020 ◽  
Vol 105 (11) ◽  
Author(s):  
Deborah Bianco ◽  
Flavia Napoli ◽  
Giovanni Morana ◽  
Angela Pistorio ◽  
Anna Elsa Maria Allegri ◽  
...  

Abstract Context The etiology of central diabetes insipidus (CDI) in children is often unknown. Clinical and radiological features at disease onset do not allow discrimination between idiopathic forms and other conditions or to predict anterior pituitary dysfunction. Objective To evaluate the evolution of pituitary stalk (PS) thickening and the pattern of contrast-enhancement in relation with etiological diagnosis and pituitary function. Methods We enrolled 39 children with CDI, 29 idiopathic and 10 with Langerhans cell histiocytosis (LCH). Brain magnetic resonance images taken at admission and during follow-up (332 studies) were examined, focusing on PS thickness, contrast-enhancement pattern, and pituitary gland size; T2-DRIVE and postcontrast T1-weighted images were analyzed. Results Seventeen of 29 patients (58.6%) with idiopathic CDI displayed “mismatch pattern,” consisting in a discrepancy between PS thickness in T2-DRIVE and postcontrast T1-weighted images; neuroimaging findings became stable after its appearance, while “mismatch” appeared in LCH patients after chemotherapy. Patients with larger PS displayed mismatch more frequently (P = 0.003); in these patients, reduction of proximal and middle PS size was documented over time (P = 0.045 and P = 0.006). The pituitary gland was smaller in patients with mismatch (P < 0.0001). Patients with mismatch presented more frequently with at least one pituitary hormone defect, more often growth hormone deficiency (P = 0.033). Conclusions The PS mismatch pattern characterizes patients with CDI, reduced pituitary gland size, and anterior pituitary dysfunction. The association of mismatch pattern with specific underlying conditions needs further investigation. As patients with mismatch show stabilization of PS size, we assume a prognostic role of this peculiar pattern, which could be used to lead follow-up.


2019 ◽  
Vol 104 (9) ◽  
pp. 4051-4057 ◽  
Author(s):  
Nat Nasomyont ◽  
Andrew W Lindsley ◽  
Amal Assa'ad ◽  
D Brian Dawson ◽  
Derek E Neilson ◽  
...  

Abstract Context Deficient anterior pituitary with variable immune deficiency (DAVID) syndrome is a recently described, rare disorder characterized by anterior pituitary hormone deficiencies and common variable immunodeficiency associated with NFKB2 mutations. Posterior pituitary hormone deficiencies have not been reported in patients with DAVID syndrome. Case Description We report a pediatric patient who initially presented with hypogammaglobulinemia and alopecia totalis, who was identified to have a de novo NFKB2 mutation at one year of age. He developed central diabetes insipidus and central adrenal insufficiency at three and four years of age, respectively. At seven years of age, he had not developed GH or TSH deficiencies. Whole exome sequencing ruled out known genetic causes of central diabetes insipidus, adrenal insufficiency, and hypopituitarism. Conclusion This is a report of central diabetes insipidus in a patient with DAVID syndrome caused by an NFKB2 mutation. This case report expands the evolving endocrine phenotype associated with NFKB2 mutations beyond anterior pituitary deficiencies.


2000 ◽  
Vol 142 (4) ◽  
pp. 365-372 ◽  
Author(s):  
C Bullmann ◽  
M Faust ◽  
A Hoffmann ◽  
C Heppner ◽  
F Jockenhovel ◽  
...  

OBJECTIVES: We retrospectively reviewed 5 patients with neurosarcoidosis, who all presented with central diabetes insipidus and hypogonadism. DESIGN: This was a single-centre, retrospective analysis of 5 cases with a minimum follow-up of 2 years. METHODS: Case analysis included clinical, biochemical, and endocrinological evaluation and frequent CT/MRI scans of involved organs as primary evaluation and in response to immunosuppressive therapy. RESULT: Neurosarcoidosis was diagnosed in all patients. Two patients had no proven extracerebral manifestation and had a stable disease over 3 and 5 years. One patient showed deterioration with corticosteroids alone but partial remission after additional cyclophosphamide. Pituitary dysfunction remained unchanged in all patients, despite total clinical and radiological remission in two patients. However, one of these patients died of acute granulomatous meningoencephalitis after two years of follow-up. CONCLUSION: Although the presenting symptoms of neurosarcoidosis may vary, the occurrence of central diabetes insipidus associated with typical radiological features is suggestive of neurosarcoidosis. However, there is an increasing number of case reports on lymphocytic hypophysitis. Without the bioptic diagnosis, the differentiation between potentially lethal isolated neurosarcoidosis and lymphocytic hypophysitis is difficult. These cases demonstrate the difficulties in diagnosing neurosarcoidosis and reflect experiences with follow-up parameters.


1982 ◽  
Vol 146 (1) ◽  
pp. 102-102 ◽  
Author(s):  
J. M. Gatell ◽  
E. Esmatjes ◽  
C. Serra ◽  
M. Aymerich ◽  
J. G. SanMiguel

Author(s):  
Misaki Aoshima ◽  
Koji Nagayama ◽  
Kei Takeshita ◽  
Hiroshi Ajima ◽  
Sakurako Orikasa ◽  
...  

Summary Patients treated with immunosuppressive drugs, especially methotrexate (MTX), rarely develop lymphoproliferative disorders (LPDs), known as MTX-related LPD (MTX–LPD). The primary site of MTX–LPD is often extranodal. This is the first reported case of MTX–LPD in the pituitary. A 65-year-old woman was admitted to our hospital with symptoms of oculomotor nerve palsy and multiple subcutaneous nodules. She had been treated with MTX for 11 years for rheumatoid arthritis. Computed tomography showed multiple masses in the orbit, sinuses, lung fields, anterior mediastinum, kidney, and subcutaneous tissue. Brain magnetic resonance imaging revealed a sellar mass. She was diagnosed with hypopituitarism and central diabetes insipidus based on endocrine examination. Although pituitary biopsy could not be performed, we concluded that the pituitary lesion was from MTX–LPD, similar to the lesions in the sinuses, anterior mediastinum, and subcutaneous tissue, which showed polymorphic LPD on biopsy. MTX was discontinued, and methylprednisolone was administered to improve the neurologic symptoms. After several weeks, there was marked improvement of all lesions, including the pituitary lesion, but the pituitary function did not improve. When pituitary lesions are caused by MTX–LPD, the possibility of anterior hypopituitarism and central diabetes insipidus needs to be considered. Further studies are needed to investigate the effectiveness of early diagnosis and treatment of MTX–LPD in restoring pituitary dysfunction. Learning points Pituitary lesions from MTX–LPD may cause hypopituitarism and central diabetes insipidus. Pituitary metastasis of malignant lymphoma and primary pituitary lymphoma, which have the same tissue types with MTX–LPD, have poor prognosis, but the lesions of MTX–LPD can regress only after MTX discontinuation. In cases of pituitary lesions alone, a diagnosis of MTX–LPD may be difficult, unless pituitary biopsy is performed. This possibility should be considered in patients treated with immunosuppressive drugs. Pituitary hypofunction and diabetes insipidus may persist, even after regression of the lesions on imaging due to MTX discontinuation.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
S. B. Smedegaard ◽  
J. O. Jørgensen ◽  
N. Rittig

Pituitary apoplexy (PA) is a rare endocrine emergency that occasionally presents with sodium disturbances. Here we present a rare case with a previously healthy 41-year-old female who presented with acute onset headache and nausea without visual impairment or overt pituitary dysfunction. Plasma sodium concentrations declined abruptly during the first two days of admission to a nadir of 111 mmol/l. Urine and blood chemistry were consistent with syndrome of inappropriate antidiuretic hormone secretion (SIADH). Magnetic resonance imaging revealed recent bleeding into a pituitary cystic process. Hyponatremia was successfully corrected with fluid restriction and both visual function and anterior pituitary function remained intact. Subsequently, the patient developed central diabetes insipidus (CDI), which responded well to desmopressin substitution. To our knowledge, this is the first case of PA presenting predominantly with posterior pituitary dysfunction that transitioned from SIADH to permanent CDI.


2021 ◽  
Vol 9 ◽  
pp. 232470962110121
Author(s):  
D. Sofia Villacis-Nunez ◽  
Amit Thakral

Neurosarcoidosis is a rare phenomenon in the pediatric population, with only a few cases reported in the literature worldwide. While hypothalamo-pituitary involvement is known to occur, direct infiltration of the pituitary gland and isolated anterior pituitary dysfunction without diabetes insipidus is seldom observed. A high index of suspicion is required for diagnosis of neurosarcoidosis, and treatment can be challenging due to lack of standardized guidelines. We present the case of a 17-year-old female with known sarcoidosis of the lacrimal glands, who developed severe headache and neurologic symptoms secondary to granulomatous infiltration of the pituitary gland and infundibulum due to neurosarcoidosis. She was successfully treated with corticosteroids, methotrexate, and adalimumab, with complete radiologic resolution. This is the first documented pediatric case of neurosarcoidosis with radiologic granulomatous infiltration of the pituitary gland, manifesting as partial anterior hypopituitarism, in the form of central hypothyroidism, without diabetes insipidus.


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