Study of the profile of behavioral problems and quality of life indexes in a pediatric cohort of monosymptomatic enuresis / Estudo do perfil dos problemas de comportamento e dos índices de qualidade de vida numa coorte pediátrica de enurese monossintomática

Abstract Objective: To evaluate and correlate, before and after the therapeutic intervention, the behavioral problem scores evaluated by the CBCL/6-18 questionnaire and the quality of life indexes evaluated by the PedsQL™ 4.0 in patients with monosymptomatic nocturnal enuresis. Method: After the initial evaluation and completion of the CBCL/6-18 questionnaire, a multidisciplinary evaluation and completion of the PedsQL™ 4.0 questionnaire was performed. Of the initially evaluated 140 children and adolescents aged 6-16 years, 58 were excluded due to non-monosymptomatic enuresis or associated comorbidities. Of the initially included 82 patients, who were randomized to three treatment groups, 59 completed the CBCL/6-18 and PedsQL™ 4.0 questionnaires at the end of the treatment and were included in this study. The α error was set at 5% for ruling out the null hypothesis. Results: Of the total of 59 participants, 45.8% responded with total success, 23.7% were partially successful, 23.7% did not reach the improvement criteria, and 6.8% gave up the treatment. There was a significant increase in quality of life indexes and a reduction of post-intervention behavioral problem scores, in the three proposed modalities, in patients who had a total or partial response to treatment. There was no correlation between higher scores of pre-treatment behavior problems and therapeutic failure. Conclusions: Only the participants who successfully responded to interventions showed improvement in quality of life and behavioral problems, which indicates that enuresis is a primary problem that has a negative impact on these parameters. The authors suggest that it is possible to achieve success in the treatment of monosymptomatic enuresis, even in patients with high pre-intervention behavioral problem scores.

Resumo Objetivo: Avaliar e relacionar, pré e pós-intervenção terapêutica, em pacientes com enurese noturna monossintomática, os escores de problemas de comportamento, avaliados pelo questionário CBCL/6-18, e os índices de qualidade de vida, avaliados pelo PedsQL™ 4.0. Método: Após avaliação inicial e preenchimento CBCL6/18, procedeu-se avaliação multidisciplinar e preenchimento do PedsQL™ 4.0. Das 140 crianças e adolescentes de 6 a 16 anos inicialmente avaliados, 58 foram excluídos por enurese não monossintomática ou comorbidades associadas. Dos 82 pacientes inicialmente incluídos e randomizados em três grupos de tratamento, 59 preencheram o CBCL/6-18 e PedsQL™ 4.0 no fim do tratamento e puderam ser incluídos neste trabalho. O erro alfa foi estabelecido em 5% para descarte da hipótese de nulidade. Resultados: Dos 59 participantes 45,8% responderam com sucesso total, 23,7% tiveram sucesso parcial, 23,7% não atingiram critério de melhoria e 6,8% desistiram do tratamento. Verificou-se aumento significativo dos índices de qualidade de vida e redução dos escores de problemas de comportamento pós-intervenção, nas três modalidades propostas, nos pacientes que obtiveram resposta total ou parcial ao tratamento. Não se demonstrou correlação entre maiores escores de problemas de comportamento pré-tratamento e insucesso terapêutico. Conclusões: Apenas os participantes que responderam com sucesso às intervenções melhoraram em sua qualidade de vida e problemas comportamentais, o que indica que a enurese é um problema primário que impacta negativamente esses parâmetros. Sugere-se que é viável obter sucesso no tratamento da enurese monossintomática, mesmo em pacientes com altos escores de problemas de comportamento pré-intervenção.

Tratamiento con desmopresina sublingual en dos lactantes con hidranencefalia y diabetes insípida central / Treatment with sublingual desmopressin in two infants with hydranencephaly and central diabetes insipidus

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.

Desmopressin is an Effective Treatment for Mixed Nocturia with Nocturnal Polyuria and Decreased Nocturnal Bladder Capacity

To investigate the efficacy and safety of desmopressin in patients with mixed nocturia, Patients aged > or =18 yr with mixed nocturia (> or =2 voids/night and a nocturnal polyuria index [NPi] >33% and a nocturnal bladder capacity index [NBCi] >1) were recruited. The optimum dose of oral desmopressin was determined during a 3-week dose-titration period and the determined dose was maintained for 4 weeks. The efficacy was assessed by the frequency-volume charts and the sleep questionnaire. The primary endpoint was the proportion of patients with a 50% or greater reduction in the number of nocturnal voids (NV) compared with baseline. Among 103 patients enrolled, 94 (79 men and 15 women) were included in the analysis. The proportion of patients with a 50% or greater reduction in NV was 68 (72%). The mean number of NV decreased significantly (3.20 to 1.34) and the mean nocturnal urine volume, nocturia index, NPi, and NBCi decreased significantly. The mean duration of sleep until the first NV was prolonged from 118.4+/-44.1 to 220.3+/-90.7 min (P<0.001). The overall impression of patients about their quality of sleep improved. Adverse events occurred in 6 patients, including one asymptomatic hyponatremia. Desmopressin is an effective and well-tolerated treatment for mixed nocturia.

Response of enuretic children with and without hypercalciuria to nasal desmopressin

Nocturnal enuresis is a common diagnosis in patients referred to pediatric and pediatric nephrology clinics. Nocturnal polyuria is an important patho-physiologic factor in enuresis. Hypercalciuria, with altering concentrating capacity of the kidneys, can affect children's response to desmopressin. This is a double blind clinical trial starting September 2007 to March 2008. One hundred and twenty four enuretic children, 76 [61.3%] males, 48 [38.7%] females, mean age 7.7 [ +/- 1.7], were evaluated by measuring random morning urinary calcium to creatinine ratio. Patients were divided into group 1 with a calcium to creatinine ratio equal to or more than 0.2 mg/mg, and group 2 with a ratio less than 0.2 [Hypercalciuric and non hypercalciuric respectively]. All patients received 10 to 40 mcg of nasal desmopressin at bed time. The response was defined as reduction in wet nights, a "full response" [greater than 90% reduction], "partial response" [50% to 90% reduction] and "no response" [less than 50% reduction]. Chi-square method was used to compare the responses and P<0.05 was considered statistically significant. Nineteen patients in group 1 [Hypercalciuric] and 105 patients in group 2 [Non hypercalciuric] were studied. Response to desmopressin was "full" in 47.4% in group 1 and 64.8% in group 2. 42.1% and 26.7% had "Partial response" in group1 and 2 respectively [P < 0.04]. Hypercalciuria can affect negatively the responsiveness to desmopressin therapy

Uso de desmopresina durante el embarazo en enfermedad de Von Willebrand / Desmopressin use during pregnancy in Von Willebrand diseases

La enfermedad de Von Willebrand es el desorden hemorragíparo más frecuente. Las mujeres constituyen una población particularmente sintomática debido al desafío hemostático de las menstruaciones y el parto. Nosotros revisamos las historias médicas de 54 mujeres con niveles disminuidos de factor von Willebrand (VWF) e historia de sangrado, quienes hubieran usado desmopresina durante el embarazo. No se observaron efectos adversos ni en las mujeres ni en los recién nacidos, incluso en los 5 expuestos a la medicación en el primer trimestre de gestación. No se observaron complicaciones locales asociadas a la colocación del catéter epidural. La DDAVP fue efectiva para prevenir el sangrado posparto. La desmopresina merece ser considerada como la primera elección de tratamiento; en aquellas pacientes con bajo niveles de VWF presentan complicaciones hemorrágicas, incluyendo mujeres embarazadas. Aunque el sangrado posparto aparece en una pequeña proporción de mujeres con VWD, no hay un modo apropiado de identificar quiénes van a sangrar. El uso de profilaxis con DDAVP debería ser tenido en cuenta como una alternativa segura y efectiva.

The von Willebrand disease (VWD) is the most frequent hemorrhagic disorder. Women with VWD are more symptomatic than men because the challenged of menses and delivery. We reviewed the records of 54 women with a low plasmatic VWF level and bleeding history, who had used desmopressin during pregnancy. No adverse effects were observed in mothers or newborns, incluiding those exposed to the drug during the first trimester. No local complication of epidural placement was observed. DDAVP was effective to prevent post-partum hemorrhage. DDAVP merits to be considered as the first choice of therapy, when patients with a previous or current low plasmatic VWF level present bleeding complications, including pregnant women. Although post-partum bleeding will appear in a small proportion of VWD women, there is no accurate way to identify who is going to bleed. The use of DDAVP should be regarded as a highly valuable option.

Successful treatment of diabetes insipidus with oral desmopressin

Acute-onset polyuria, with urine volumes exceeding 3 litres per day, is a relatively uncommon clinical condition. If managed on appropriate guidelines, an excellent recovery is expected

Cranial irradiation--an unusual cause for diabetes insipidus.

Central diabetes insipidus frequently occurs due to tumours in the region of pituitary or hypothalamus or following surgical trauma to these regions. Rarely it has been reported following cranial irradiation. We report the case of a middle aged woman who underwent surgical removal of a frontal capillary hemangioblastoma and received cranial irradiation. She presented ten months later with features of diabetes insipidus which was confirmed to be of central origin. She responded well to desmopressin nasal spray. Radiation induced damage to the hypothalmo-pituitary axis presents usually with anterior pituitary hormone deficiencies, most commonly that of growth hormone. Presentation as central diabetes insipidus is very uncommon.

Acetato de desmopresina en niños enuréticos resistentes a terapias convencionales / Desmopressin acetate in children with conventional therapy resistant enuresis

Se describen los resultados obtenidos con acetato de desmopresina en aerosol por inhalación nasal en 29 niños (15 varones) entre 8 y 10 años de edad, que sufrían enuresis nocturna resistente a tratamiento con imipramina sola y/o asociada a ácido oxibutinino. En todos ellos la anatomía y función vesical eran normales y ninguno sufría enfermedades neurológicas o renales. La desmopresina se suministró en dosis diarias de 10 µg que fueron aumentadas semanalmente, si era necesario, en igual proporción, hasta obtener uno o ningún episodio semanal de enuresis o un máximo de 40 µg diarios del fármaco. La dosis así titulada se mantuvo por 3 meses, al cabo de los cuales se redujo progresivamente en 10 µg semanales hasta suspenderla. Los pacientes fueron seguidos hasta un mes después de la supresión del tratamiento. Se obtuvo buen éxito (uno o menos episodios de enuresis por semana) en 65 por ciento de los casos. Un mes después de suspender la desmopresina se registraban 62,2 por ciento de niñoscon uno o menos episodios semanales de enuresis, sugiriendo una baja proporción de recaídas en plazos cortos. No se anotaron efectos colaterales importantes duarnte el estudio en este grupo de niños, salvo cefalea persistente en un paciente con antecedentes de jaqueca

Diabetes insípida central autosómica dominante / Autosomic dominant central diabetes insipidus

Se presenta el caso de una niña de siete meses de edad con diabetes insípida central autosómica dominante, con un síndrome de hipernatremia-hipodipsia secundario. Se solicitó atención médica por fiebre recurrente, que resultó ser secundaria a la deshidratación crónica, y que cedió al corregir la volemia. Así mismo cedieron las alteraciones de motilidad gastrointestinal al desaparecer el desequilibrio hidroelectrolítico. Aún cuando la paciente presentaba una curva térmica poikilotérmica, no se demostró alteración estructural diencéfalo-hipotalámica ni al nivel del cuerpo calloso. Los familiares afectados (padres, dos tías paternas y abuelo paterno) no habían sido diagnosticados previamente y se desconocía el patrón hereditario de la enfermedad. Diabetes insípida central; autosómica dominante