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diabetes insipidus [keywords]
- Twenty-nine cases of resection of suprasellar meningioma through small bone window: an interhemispheric approach. [Journal Article]
- Contemp Oncol (Pozn) 2013; 17(6):525-9.
The present study aims to discuss the value and the effect of resection of suprasellar meningioma through the interhemispheric approach.Twenty-nine cases of patients with suprasellar meningioma diagnosed through enhanced magnetic resonance imaging (MRI) scans and postoperative histopathological examination underwent resection of tumours (the largest diameter ranged from 3 cm to 6 cm) by the microsurgical technique of a small bone window (about 4 cm × 5 cm) through the interhemispheric approach.Among all cases, 25 (86%) (Simpson I, II) were of total resection of tumours and 4 were of subtotal resection of tumours. 19 (65%) were of improvement of vision and visual field, 2 (7%) were of postoperative diabetes insipidus, and 1 (3%) was of electrolyte imbalance. No operative death occurred.The small bone window interhemispheric approach can be used to expose tumours, lightly stretch brain tissues, reduce the incidence of complications, and improve the total resection rate of tumours of patients with sellae meningiomas growing forward, upward, and into the sella.
- Clinical analysis on 33 patients with hypothalamic syndrome in Chinese children. [Journal Article]
- J Pediatr Endocrinol Metab 2014 Mar 1; 27(3-4):291-7.
Aim:To investigate the etiology and clinical characteristics of hypothalamic syndrome in Chinese children.
Methods:Thirty-three cases of hypothalamic syndrome were analyzed for etiology, initial symptoms, and clinical characteristics.
Results:All of the 33 patients manifested symptoms of hypothalamic dysfunction and disorders of the hypothalamus-hypophysis-target gland axis. Fourteen patients were diagnosed with an intracranial tumor by magnetic resonance imaging (MRI) examination, four patients had postoperative intracranial tumors, one had received radiotherapy for suprasellar germinoma, one was hypothalamic-pituitary dysplasia, one had a history of viral encephalitis, and in 12 patients, the cause was unknown. The most common presenting symptoms were polydipsia/polyuria and eating disorders.
Conclusion:Intracranial tumor is an important cause of hypothalamic syndrome in children, with germinoma the most common. Polydipsia, polyuria, and eating disorders are typical presenting symptoms. Long-term follow-up is needed for patients presenting with central diabetes insipidus, eating disorders or hypothalamic syndrome of unknown etiology. In addition, periodic pituitary MRI scanning is necessary to find potential intracranial tumors that may arise at any time.
- Clinical guidelines for management of diabetes insipidus and syndrome of inappropriate antidiuretic hormone secretion after pituitary surgery. [JOURNAL ARTICLE]
- Endocrinol Nutr 2014 Feb 28.
Changes in water metabolism and regulation of vasopressin (AVP) or antidiuretic hormone (ADH) are common complications of pituitary surgery. The scarcity of studies comparing different treatment and monitoring strategies for these disorders and the lack of prior clinical guidelines makes it difficult to provide recommendations following a methodology based on grades of evidence. This study reviews the pathophysiology of diabetes insipidus and inappropriate ADH secretion after pituitary surgery, and is intended to serve as a guide for their diagnosis, differential diagnosis, treatment, and monitoring.
- Channelopathies. [REVIEW]
- Korean J Pediatr 2014 Jan; 57(1):1-18.
Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.
- [Lithium treatment and potential long-term side effects: a systematic review of the literature]. [English Abstract, Journal Article]
- Riv Psichiatr 2014 Jan-Feb; 49(1):12-21.
Introduction and aim. Lithium is recommended by all treatment guidelines for bipolar disorder (BD) as a first-line maintenance treatment. However, the potential side effects and risks associated with long-term lithium use may at times make the implementation of these recommendations in daily practice challenging. The aim of the study is to review available literature on potential long-term side effects of lithium. Materials and methods. A PubMed/Medline search was performed on papers dealing with long-term treatment with lithium and side effects. Articles published from January 1980 to February 2013 were selected.
Results.Long-term lithium treatment is associated with a reduced urinary concentrating ability, with subsequent polyuria and polidypsia and nephrogenic diabetes insipidus (in 10-40% of patients). Lithium also reduces glomerular filtration rate, and increases risk of renal failure, although the absolute risk is small (0.5% of patients). Lithium treatment is associated with significant higher TSH levels, with a 6-fold greater risk of hypothyroidism in lithium-treated than in control subjects. Less known is the increase of PTH and calcium levels induced by lithium. An exacerbation of psoriasis is also frequently associated with lithium treatment.
Conclusions.Lithium remains a fundamental tool for the treatment of BD. Clinicians should know potential side effects (renal, endocrine and dermatological) associated with long-term treatment with lithium, for a correct management of the patient. A specialist referral is often necessary; the question is how to deal with long-term side effects more than whether or not withdrawing lithium. This decision should remain a psychiatrist's competence.
- Prevalence and Correlates of Renal Disease in Older Lithium Users: A Population-Based Study. [JOURNAL ARTICLE]
- Am J Geriatr Psychiatry 2014 Feb 2.
Lithium is an important treatment for mood disorders, but concern about its association with renal disease has contributed to its limited use, particularly in older adults. Because high-quality evidence examining renal disease in this population is lacking, this study aims to quantify the prevalence and identify clinical correlates of renal disease in geriatric lithium users.In a population-based cross-sectional study on 2,480 lithium users aged 70 or more years, the authors searched the provincial administrative health data from Ontario, Canada between April 1, 2005 and March 31, 2011. Prevalence of chronic kidney disease (CKD), acute kidney injury (AKI), and nephrogenic diabetes insipidus (NDI) was measured using International Classification of Diseases, Tenth Revision codes. Logistic regression analyses were used to identify independent correlates of renal disease.The 6-year prevalence rates of CKD, AKI, and NDI were 13.9%, 1.3%, and 3.0%, respectively. Hypertension (odds ratio [OR]: 2.05; 95% confidence interval [CI]: 1.50-2.79), diabetes mellitus (OR: 1.86; 95% CI: 1.45-2.38), ischemic heart disease (OR: 1.65; 95% CI: 1.24-2.20), NDI (OR: 2.54; 95% CI: 1.47-4.40), AKI (OR: 11.7; 95% CI: 5.26-26.1), lithium use for more than 2 years (OR: 1.71; 95% CI: 1.05-2.81), loop diuretic use (OR: 1.74; 95% CI: 1.26-2.41), hydrochlorothiazide use (OR: 1.48; 95% CI: 1.07-2.05), and atypical antipsychotic use (OR: 1.49; 95% CI: 1.17-1.89) were all independently associated with CKD.Older lithium users have high rates of CKD. Lithium use duration was independently associated with CKD. Longitudinal studies including individuals without lithium exposure will be necessary to confirm whether lithium is indeed a risk factor for CKD in older adults.
- Full Endoscopic Transsphenoidal Surgery for Pituitary Adenoma-emphasized on Surgical Skill of Otolaryngologist. [Journal Article]
- Indian J Otolaryngol Head Neck Surg 2014 Jan; 66(Suppl 1):334-40.
The purpose is to summarize the experience in full endoscopic transsphenoidal resection of pituitary adenoma in 28 patients by rhinologist, and introduce the surgical skill of otolaryngologist, especially skills and cautions when operating inside nose. We removed pituitary adenoma in 28 patients via entirely endoscopic transsphenoidal approach with the help of special-designed instruments; we performed the procedure bloodlessly within limited time. The skill emphasized bilateral nostrils and four hands technique which was as delicate as possible not to scratch nasal mucosa or injure nasal frame. The special instruments included curette with suction, monopolar electrotome and bipolar coagulation forceps with suction, powered surgical equipments (Diamond Bur, Irrigation Tubing for Blades and Burs for nasal endoscopic surgery). Among 28 patients, there were 16 total resections, 8 subtotal resections, 3 partial resections, and 1 only biopsy due to excessive bleeding and hard nature. Of 19 patients with preoperative visual impairment, 12 patients had postoperative improvement in visual acuity and visual field. All the procedures were finished within 60 to 90 min. Complications seldom occurred except transient diabetes insipidus, especially no nasal-related signs or complications but 1 had epistaxis. The full endoscopic transsphenoidal surgery is a promising approach for pituitary adenoma resection. Multidisciplinary collaboration will lead to optimal cure for the patients. New technique and special-designed instruments can facilitate greatly this procedure.
- Surgical biopsies in patients with central diabetes insipidus and thickened pituitary stalks. [JOURNAL ARTICLE]
- Endocrine 2014 Feb 16.
Thickened pituitary stalks (TPSs) on magnetic resonance imaging (MRI) result from diverse pathologies; therefore, it is essential to make specific diagnoses for clinical decision-making. The diagnoses and indications for surgical biopsies in patients with central diabetes insipidus (CDI) and TPSs are thoroughly discussed in this paper. Thirty-seven patients with CDI and TPSs were retrospectively reviewed. The mean age at the diagnosis of CDI was 29.0 ± 15.9 years (range 8.0-63.3), and the median duration of follow-up was 5.5 ± 2.8 years (range 0.7-13.0). Anterior pituitary hormone deficiencies were documented in 26 (70.3 %) patients. All patients had a TPS on MRI at the diagnosis of CDI, and 21 (56.8 %) patients exhibited radiological changes during the follow-up. Of these 21 patients, 11 exhibited increases in the thickness of the stalk, and two patients exhibited reversals of the TPSs. Involvements of the hypothalamus, pituitary gland, basal ganglia or supersellar, and pineal gland were found in four, three, one, and 1 patient, respectively. Ultimately, clear diagnoses were established in 17 patients who underwent biopsies, nine of whom had germinomas, six of whom had Langerhans cell histiocytosis, one of whom had a granular cell tumor, and one of whom had Erdheim-Chester disease. Patients with CDI and TPSs should submit to periodic clinic follow-ups with serial MRI assessments to establish anterior pituitary deficiencies and to detect radiological progressions that are appropriate for surgical biopsies. Endoscopic-assisted microsurgery via the supraorbital keyhole approach is a good choice for the biopsy of pituitary stalk lesions.
- Pharmacological chaperoning: A primer on mechanism and pharmacology. [JOURNAL ARTICLE]
- Pharmacol Res 2014 Feb 14.
Approximately forty percent of diseases are attributable to protein misfolding, including those for which genetic mutation produces misfolding mutants. Intriguingly, many of these mutants are not terminally misfolded since native-like folding, and subsequent trafficking to functional locations, can be induced by target-specific, small molecules variably termed pharmacological chaperones, pharmacoperones, or pharmacochaperones (PCs). PC targets include enzymes, receptors, transporters, and ion channels, revealing the breadth of proteins that can be engaged by ligand-assisted folding. The purpose of this review is to provide an integrated primer of the diverse mechanisms and pharmacology of PCs. In this regard, we examine the structural mechanisms that underlie PC rescue of misfolding mutants, including the ability of PCs to act as surrogates for defective intramolecular interactions and, at the intermolecular level, overcome oligomerization deficiencies and dominant negative effects, as well as influence the subunit stoichiometry of heteropentameric receptors. Not surprisingly, PC-mediated structural correction of misfolding mutants normalizes interactions with molecular chaperones that participate in protein quality control and forward-trafficking. A variety of small molecules have proven to be efficacious PCs and the advantages and disadvantages of employing orthostatic antagonists, active-site inhibitors, orthostatic agonists, and allosteric modulator PCs are considered. Also examined is the possibility that several therapeutic agents may have unrecognized activity as PCs, and this chaperoning activity may mediate/contribute to therapeutic action and/or account for adverse effects. Lastly, we explore evidence that pharmacological chaperoning exploits intrinsic ligand-assisted folding mechanisms. Given the widespread applicability of PC rescue of mutants associated with protein folding disorders, both in vitro and in vivo, the therapeutic potential of PCs is vast. This is most evident in the treatment of lysosomal storage disorders, cystic fibrosis, and nephrogenic diabetes insipidus, for which proof of principle in humans has been demonstrated.
- Endocrine dysfunction in Taiwanese children with human chorionic gonadotropin-secreting germ cell tumors. [Journal Article]
- J Formos Med Assoc 2014 Feb; 113(2):102-5.
Human chorionic gonadotropin (HCG)-secreting germ cell tumors (GCTs) are rare childhood malignancies with unique clinical manifestations but delayed diagnosis is common. The purpose of this study is to investigate the clinical manifestations and endocrine dysfunction of Taiwanese children with HCG-secreting GCTs.From 1991 to 2011, 24 children (19 boys and five girls) with HCG-secreting GCTs were evaluated for their clinical findings and endocrine functions.The mean age at diagnosis of the study patients was 10.8 ± 3.1 years. Of the 24 patients, 20 had central nervous system (CNS) GCTs and four had primary mediastinal GCTs (PMGCTs). The most common pathologic findings were germinomas and mixed type GCTs. The common initial symptoms and signs included polyuria, polydipsia, rapid growth, neurologic deficit,sexual precocity, and growth retardation. There was a delay in diagnosis in about 60% of patients. Diabetes insipidus and hypopituitarism were common endocrine dysfunctions in patients with CNSGCTs. Twelve boys had gonadotropin-independent puberty upon diagnosis, which were related to their high serum β-hCG levels. None of the five girls had this disorder despite their high serum β-hCG levels. Three of the four PMGCTs patients had the classic form of Klinefelter syndrome.Taiwanese children with HCG-secreting GCTs often have clinical manifestations related to endocrine dysfunction. High index of suspicion is important to avoid delayed diagnosis in these children.