ABSTRACT
La diabetes insípida central es una patología infrecuente en pediatría ocasionada por un déficit de vasopresina. Sus manifestaciones clínicas principales son poliuria y polidipsia. Las malformaciones cerebrales son una de las principales causas. La desmopresina es la droga sintética de elección para el tratamiento. Una de las vías de administración es la sublingual y su uso en lactantes es muy limitado. Se describe a dos lactantes con hidranencefalia y diabetes insípida central que fueron tratados satisfactoriamente con desmopresina sublingual.
Central diabetes insipidus is a rare disease in children caused by a deficiency of vasopressin. Its main clinical manifestations are polyuria and polydipsia. Brain malformations are one of the main causes. Desmopressin is the synthetic drug of choice for the treatment. One of the routes of administration is sublingual and its use in infants is very limited. We describe two infants with central diabetes insipidus and hydranencephaly who were successfully treated with sublingual desmopressin.
Subject(s)
Humans , Male , Female , Infant , Diabetes Insipidus, Neurogenic/drug therapy , Deamino Arginine Vasopressin/administration & dosage , Antidiuretic Agents/administration & dosage , Hydranencephaly/drug therapy , Administration, SublingualABSTRACT
CONTEXT: Central diabetes insipidus (CDI) is a rare cause of hypernatremia during the neonatal period. The diagnosis is particularly difficult in very low birth weight (VLBW) newborns. CASE REPORT: We report on a preterm newborn who presented CDI soon after birth. On the third day of life, signs of dehydration were present despite normal fluid supply. The diuresis rate was 4.4 ml/kg/h. Although the fluid supply was then increased, the dehydration continued, with hypernatremia, normal glycemia, diuresis of 7.4 ml/kg/h and urine density of 1005 mOsmol/l. Thus, a diagnostic hypothesis of diabetes insipidus was raised. A test with a nasal vasopressin analogue (dDAVP) was performed and CDI was confirmed. Reduction of the fluid supply became possible through appropriate treatment. CONCLUSION: The diagnosis of CDI is rarely made during the neonatal period, especially in VLBW newborns, because of the difficulty in detecting elevated diuresis. Persistent hypernatremia, usually accompanied by hyperthermia despite abundant fluid supply, weight loss and low urine osmolality are important signs of alert. .
CONTEXTO: Diabete insípido central (DIC) é uma rara causa de hipernatremia durante o período neonatal. O diagnóstico é difícil, particularmente em recém-nascidos (RN) de muito baixo peso (RNMBP). RELATO DE CASO: Relatamos um RN que apresentou DIC logo após o nascimento. No terceiro dia de vida, apresentava sinais de desidratação, embora estivesse recebendo aporte adequado de líquidos. A diurese aferida era de 4,4 ml/kg/h. Apesar do aumento do aporte hídrico, manteve-se desidratado, com hipernatremia, valores normais de glicemia e diurese de 7,4 ml/kg/h com densidade urinária de 1005 mOsmol/l. Desta forma, a hipótese diagnóstica de diabete insípido foi considerada. O teste com análogo da vasopressina (dDAVP) foi realizado e DIC foi confirmado. A redução do aporte de líquidos foi possível com o tratamento adequado. CONCLUSÃO: O diagnóstico de DIC raramente é realizado durante o período neonatal, particularmente em RNMBP, devido à dificuldade em detectar diurese aumentada. Hipernatremia persistente, geralmente acompanhada de hipertermia, apesar do abundante aporte de água, perda de peso e osmolaridade urinaria baixa, são importantes sinais de alerta. .
Subject(s)
Female , Humans , Infant, Newborn , Male , Dehydration/etiology , Diabetes Insipidus, Neurogenic/complications , Administration, Intranasal , Deamino Arginine Vasopressin , Dehydration/drug therapy , Diabetes Insipidus, Neurogenic/diagnosis , Diabetes Insipidus, Neurogenic/drug therapy , Diuresis , Early Diagnosis , Hemostatics/therapeutic use , Hypernatremia/blood , Infant, Very Low Birth Weight , Osmolar Concentration , Premature Birth , Treatment OutcomeABSTRACT
We report a rare case of diabetes insipidus following fire burn injury. Meticulous fluid balance and the use of carbamazepine resulted in her survival.