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      One too many diabetes: the combination of hyperglycaemic hyperosmolar state and central diabetes insipidus

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          Summary

          The combination of hyperosmolar hyperglycaemic state and central diabetes insipidus is unusual and poses unique diagnostic and therapeutic challenges for clinicians. In a patient with diabetes mellitus presenting with polyuria and polydipsia, poor glycaemic control is usually the first aetiology that is considered, and achieving glycaemic control remains the first course of action. However, severe hypernatraemia, hyperglycaemia and discordance between urine-specific gravity and urine osmolality suggest concurrent symptomatic diabetes insipidus. We report a rare case of concurrent manifestation of hyperosmolar hyperglycaemic state and central diabetes insipidus in a patient with a history of craniopharyngioma.

          Learning points:
          • In patients with diabetes mellitus presenting with polyuria and polydipsia, poor glycaemic control is usually the first aetiology to be considered.

          • However, a history of craniopharyngioma, severe hypernatraemia, hyperglycaemia and discordance between urine-specific gravity and osmolality provide evidence of concurrent diabetes insipidus.

          • Therefore, if a patient with diabetes mellitus presents with severe hypernatraemia, hyperglycaemia, a low or low normal urinary-specific gravity and worsening polyuria despite correction of hyperglycaemia, concurrent diabetes insipidus should be sought.

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          Most cited references10

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          Hyperosmolar Hyperglycemic State: A Historic Review of the Clinical Presentation, Diagnosis, and Treatment

          The hyperosmolar hyperglycemic state (HHS) is the most serious acute hyperglycemic emergency in patients with type 2 diabetes. von Frerichs and Dreschfeld described the first cases of HHS in the 1880s in patients with an “unusual diabetic coma” characterized by severe hyperglycemia and glycosuria in the absence of Kussmaul breathing, with a fruity breath odor or positive acetone test in the urine. Current diagnostic HHS criteria include a plasma glucose level >600 mg/dL and increased effective plasma osmolality >320 mOsm/kg in the absence of ketoacidosis. The incidence of HHS is estimated to be <1% of hospital admissions of patients with diabetes. The reported mortality is between 10 and 20%, which is about 10 times higher than the mortality rate in patients with diabetic ketoacidosis (DKA). Despite the severity of this condition, no prospective, randomized studies have determined best treatment strategies in patients with HHS, and its management has largely been extrapolated from studies of patients with DKA. There are many unresolved questions that need to be addressed in prospective clinical trials regarding the pathogenesis and treatment of pediatric and adult patients with HHS.
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            Central diabetes insipidus in children and young adults.

            Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. The clinical presentation and the long-term course of this disorder are largely undefined. We studied all 79 patients with central diabetes insipidus who were seen at four pediatric endocrinology units between 1970 and 1996. There were 37 male and 42 female patients whose median age at diagnosis was 7.0 years (range, 0.1 to 24.8). All patients underwent magnetic resonance imaging (MRI) and periodic studies of anterior pituitary function. The median duration of follow-up was 7.6 years (range, 1.6 to 26.2). The causes of the central diabetes insipidus were Langerhans-cell histiocytosis in 12 patients, an intracranial tumor in 18 patients, a skull fracture in 2 patients, and autoimmune polyendocrinopathy in 1 patient; 5 patients had familial disease. The cause was considered to be idiopathic in 41 patients (52 percent). In 74 patients (94 percent) the posterior pituitary was not hyperintense on the first MRI scan obtained, and 29 patients (37 percent) had thickening of the pituitary stalk. Eighteen patients had changes in the thickness of the pituitary stalk over time, ranging from normalization (six patients) or a decrease in thickness (one patient) to further thickening (seven patients) or thickening of a previously normal stalk (four patients). Anterior pituitary hormone deficiencies, primarily growth hormone deficiency, were documented in 48 patients (61 percent) a median of 0.6 year (range, 0.1 to 18.0) after the onset of central diabetes insipidus. Most children and young adults with acquired central diabetes insipidus have abnormal findings on MRI scans of the head, which may change over time, and at least half have anterior pituitary hormone deficiencies during follow-up.
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              Measurement of urinary concentration: a critical appraisal of methodologies.

              The measurement of urine concentration provides information concerning the kidney's ability to appropriately respond to variations in fluid homeostasis. It also assists in the interpretation of other tests performed on the same urine specimen. The gold standard of estimating urinary concentration is the measurement of its osmolality; however, this procedure is not readily available to the practicing physician. Therefore, urine concentration is usually determined by measurement of its specific gravity (SG), which provides a fair estimate of urine osmolality. Over the years numerous tests have been developed to measure urine SG in a simple, quick, reliable and easily available method. These tests measure SG either directly (e.g., gravimetry) or by indirect methods (e.g., refractometry and reagent strip). All these tests have certain limitations based on their underlying physical principles. Specific gravity as measured by refractometry is influenced by proteinuria, such that for each 10 g/l protein the SG increases by 0.003. SG is also influenced by glucosuria such that it increases by approximately 0.002 per 10 g/l glucose when compared with urinary osmolality. Unlike osmolality, which is only affected by the number of particles, refractometry is affected by number, mass and chemical structure of the dissolved particles; hence large molecules like radiographic contrast or mannitol will increase SG relative to osmolality. The reagent strip is minimally affected by glucose, mannitol or radiographic contrast. However, it is affected by urinary pH such that only urine in the pH range of 7.0-7.5 can be correctly interpreted. The measurement of SG by reagent strip is based on the ionic strength of the urine and thus is significantly affected by the ionic composition of the urine and by proteins which have an electric charge in solution. In our experience, SG measured by the refractometer is consistently more accurate than the reagent strip. For the clinician who is interpreting urine SG results, it is important to be aware of these limitations and understand the reasons for possible potential errors of each particular method.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                11 April 2018
                2018
                : 2018
                : 18-0029
                Affiliations
                [1 ]Department of Intensive Care Medicine , Lucerne Cantonal Hospital, Lucerne, Switzerland
                [2 ]Department of Internal Medicine and Endocrinology , Lucerne Cantonal Hospital, Lucerne, Switzerland
                [3 ]Australian and New Zealand Intensive Care Research Centre , School of Public Health and Preventive Medicine, Monash University, Prahran, Australia
                Author notes
                Correspondence should be addressed to L Cioccari Email: luca.cioccari@ 123456monash.edu
                Article
                EDM180029
                10.1530/EDM-18-0029
                5900797
                456bded6-647d-4897-ad56-c79a450ae3f2
                © 2018 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 12 March 2018
                : 23 March 2018
                Categories
                Unique/Unexpected Symptoms or Presentations of a Disease

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