This study aimed to illustrate the importance of regular followup and pituitary function monitoring in pediatric cdi. Answer nephrogenic diabetes insipidus is caused by problems related to. Central di, the most common form of diabetes insipidus, is caused by insufficient levels of circulating antidiuretic hormone adh. Diabetes insipidus refers to the condition where the kidneys are unable to retain water. Diabetes insipidus di is a condition in which the kidneys are unable to concentrate urine. The use of hypertonic saline infusions in the differential diagnosis of diabetes insipidus and psychogenic polydipsia. The major symptoms of central diabetes insipidus di are polyuria, nocturia, and polydipsia due to the concentrating defect. Learn more about this unusual disorder that disrupts the bodys fluid balance, causing excessive urination and possibly leading to dehydration. Diabetes insipidus is an uncommon condition that causes excessive production of very diluted urine and, as a result, intense thirst. The incidence of diabetes insipidus in the general population is 3 in 100,000, with a slightly higher incidence among males 60%. Diabetes insipidus di is part of a group of hereditary or acquired polyuria and.
Polydipsia and polyuria with dilute urine, hypernatremia, and dehydration are the hallmarks of diabetes insipidus in infants and children. The kidneys normally produce 12 quarts of urine per day, but with diabetes insipidus, they may produce 3. The division of pediatric endocrinology and diabetes center at massgeneral hospital for children is an international referral center for the management of pediatric diabetes and endocrine disorders in. Infants consume most of their calories as liquids which with desmopressin puts them at risk for hyponatremia and seizures. Disorder characterized by polydipsia, polyuria, and formation of inappropriately hypotonic dilute urine. Diabetes insipidus symptoms, diagnosis and treatment.
Central diabetes insipidus cdi is a disorder of the neurohypophyseal system caused by a partial or complete deficiency of vasopressin adh, which results in excessive, dilute urine. Our multidisciplinary pediatric diabetes clinic opened its doors in september of 2017. There are few cases reporting chlorothiazide as a temporizing measure for central diabetes. Describe the treatments of choice for central diabetes insipidus and nephrogenic diabetes insipidus. We report the case of an adolescent female with a history of severe an, restricting subtype, treated aggressively with multiple hospitalizations. Diabetes insipidus occurs in the acute phase of tbi in 20% of cases, 2, 3 and in 15% of patients with sah. Management of diabetes insipidus in children garima mishra, sudha rao chandrashekhar department of pediatrics, division of pediatric endocrinology, bai jerbai wadia hospital for children, parel. The estimated incidence of xlinked nephrogenic diabetes insipidus in boys is 4 cases per million.
Vasopressin helps the kidneys recognize and regulate the amount of water that is in the body. The various forms of desmopressin, oral, subcutaneous, and intranasal, have variability in the duration of action. Diabetes insipidus di is a heterogeneous clinical syndrome of disturbance in water balance, characterized by polyuria urine output 4 mlkghr, polydypsia water intake 2 lm 2 d and failure to thrive. Diabetes insipidus in children childrens hospital of. Diabetes insipidus is a relatively rare disorder where the body does not create enough vasopressin or stops responding to this antidiuretic hormone. Cdi is due to the relative or absolute lack of the posterior pituitary hormone vasopressin avp, also known as antidiuretic hormone. Diagnostic testing for diabetes insipidus endotext ncbi bookshelf. Even though the patient may be dehydrated, the kidneys cannot balance the fluid and produce large amounts of insipid urine dilute and odorless. Nephrogenic and central diabetes insipidua is more common in boys compared to girls. Diabetes insipidus is a disease characterized by polyuria and polydipsia due to inadequate release of arginine vasopressin from the posterior pituitary gland neurohypophyseal diabetes insipidus. Diabetes insipidus is a rare entity, it is estimated in the literature that has a prevalence of 1 per 25000 inhabitants, being most often central diabetes insipidus, which nephrogenic diabetes insipidus, with a smaller percentage etiology of hereditary nature less than 10%.
Xlinked nephrogenic diabetes insipidus is very rare, with arginine vasopressin receptor2 avpr2 gene mutations among males estimated to be 4 in 1,000,000. Pediatric diabetes center massachusetts general hospital. Diabetes insipidus an overview sciencedirect topics. The main objective of management for diabetes insipidus is decreased urine output and thirst control, allowing adequate control of levels of sodium and water homeostasis, the management of primary forms of diabetes insipidus focuses on dietary modification to reduce the salt load. Diabetes insipidus and nonobstructive dilation of urinary tract. Cranial diabetes insipidus cdi is a treatable chronic condition that can potentially develop into a lifethreatening medical emergency. We present a case of a 27monthold boy who presented with polyuria and polydipsia. Use of chlorothiazide in the management of central. Family history was significant for multiple maternal relatives with diabetes insipidus di, including an older sibling with nephrogenic diabetes insipidus ndi and a mother who was a known carrier. It is the most frequent pediatric intracranial neoplasm, accounting for nearly 54% of cases. Nephrogenic diabetes insipidus ndi is caused by inability of the kidneys to respond to arginine vasopressin and concentrate urine by reabsorption of water in. Weight loss is also a feature of chronic renal failure and may also be due to dehydration in diabetes insipidus. It results either from a deficiency of arginine vasopressin avp, termed central di cdi, or from renal resistance to the.
The full text of this article is available in pdf format. The effects of treatment with hydrochlorothiazide 5075 mgday p. Diabetes insipidus can be divided into four different types that are caused by any one of four fundamentally different defects fig. In children, nephrogenic di ndi is more common than central di cdi, and is often acquired. Pdf on sep 7, 2018, irina suley tirado perez and others published pediatric diabetes insipidus find, read and cite all the research you need on researchgate. In pediatric central diabetes insipidus cdi, etiology diagnosis and pituitary function monitoring are usually delayed. Diabetes mellitus in children lecture for 5th year medical student type i dm. Mainly diabetes insipidus is characterized by clinical symptoms such as polyuria and polydipsia, secondary to absolute or relative. Diagnosis and management of central diabetes insipidus in. Pediatric cohorts of central diabetes insipidus cdi have shown varying prevalences for the different causes of cdi, including idiopathic. Adh is made in a part of the brain called the hypothalamus. Di is due either to 1 deficient secretion of adh by the pituitary gland central or neurogenic di or to 2 renal tubular unresponsiveness to vasopressin nephrogenic di.
Di is due either to 1 deficient secretion of adh by the pituitary gland central or neurogenic di or to 2 renal tubular unresponsiveness to. Diabetes insipidus and siadh reference sheet clinical manifestations associated with hyponatremia serum sodium levels associated symptoms 140 meql impaired taste, anorexia, dyspnea with exertion, fatigue, dulled sensorium. Central di insipidus and multiple pituitary hormone. Primary polydipsia is differentiated from psychogenic polydipsia by the lack of a clear psychotic disturbance.
Pituitary morphology and function in 43 children with. As these authors pointed out, however, the diagnosis in many of these is questionable, owing to inadequate laboratory studies. Treatment of this disorder is primarily aimed at decreasing the urine output, usually by increasing the activity of antidiuretic hormone adh. Diabetes insipidus, also called di, is a rare condition that leads to frequent urination passing a lot of clear urine and excessive thirst. The clinical, hormonal, and neuroradiological characteristics of children with cdi at diagnosis. The occurrence of diabetes mellitus and diabetes insipidus in the same patient is rare. Diabetes insipidus is a condition caused by not enough antidiuretic hormone adh in the body. Delineate the inheritance pattern of central diabetes insipidus and nephrogenic diabetes insipidus. Diabetes insipidus di is characterized by polydipsia and polyuria with a dilute urine having a specific gravity less than 1. Central diabetes insipidus associated with refeeding in. It results either from a deficiency of arginine vasopressin avp, termed central di cdi, or from renal resistance to the action of avp, called nephrogenic di ndi.
Diabetes insipidus symptoms and causes mayo clinic. Columbia university irving medical center, new york, ny diabetes insipidus di is characterized by polydipsia and polyuria with a dilute urine having a specific gravity less than 1. Research article causes and followup of central diabetes. The condition may be caused by problems with your pituitary gland andor your kidneys. Pathophysiology, diagnosis and management of nephrogenic. The 2 most common types of diabetes insipidus are central and nephrogenic. During hospitalization for severe weakness and lethargy, her course of medical stabilization was complicated by significant polyuria, ultimately diagnosed as central diabetes insipidus di. Diabetes insipidus di, which is characterized by polyuria and polydipsia due to excessive urinary loss of solutefree water, can be either central cdi or nephrogenic. Adh is secreted by the hypothalamus a small gland located at the. Diabetes insipidus di is a heterogeneous clinical syndrome of disturbance in water balance, characterized by polyuria urine output 4 mlkghr, polydypsia water intake 2 lm2d and. What are mechanisms by which nephrogenic diabetes insipidus works. Diabetes insipidus di is part of a group of hereditary or acquired polyuria and polydipsia diseases in which the kidneys pass large amounts of water irrespective of the bodys hydration state.
Research article causes and followup of central diabetes insipidus in children wendong liu,1 jing hou,1 xiuqin liu,1 limin wang,1 and guimei li 2 1department of pediatrics, qingdao municipal hospital a. The objective of the study was to determine the causes of cdi at a pediatric tertiary care center and to characterize their clinical outcomes. Children with type 1 diabetes are at higher risk of other autoimmune disorders, particularly thyroid disease and celiac disease. In this clinic, the only one of its kind in the boston area, patients and their families meet with a certified diabetes educator, nutritionist, child psychologist and pediatric endocrinologist over the course of a single twohour visit. Xlinked nephrogenic diabetes insipidus is a very rare condition. Diabetes insipidus di is a disease that causes frequent urination. Diabetes insipidus di is part of a group of hereditary or acquired polyuria and polydipsia diseases in which the kidneys pass large amounts of. Vasopressin stimulation test confirmed likely nephrogenic rather than central diabetes insipidus. Close relatives are at increased risk of diabetes about 15 times the risk of the general population, with overall incidence 4 to 8% 30 to 50% in monozygotic twins. Diabetes insipidus the journal of clinical endocrinology. Treatment of neurogenic diabetes insipidus emconsulte. Management of central diabetes insipidus in infancy is challenging.
1302 806 1572 1583 1413 268 241 66 521 1335 671 987 1212 52 1567 1086 1463 1147 129 48 1083 570 95 1492 871 140 219 423 1259 995 172 1276 1198 1270 912