deamino-arginine-vasopressin and Remission--Spontaneous

Xanthoma disseminatum (XD) is a rare and potentially progressive non-Langerhans-cell histiocytosis. To date, a few cases of XD with spontaneous complete resolution have been described. The present report describes a 16-year-old girl who presented with yellow to red-brown papules and nodules on her eyelids, cheeks, axillae, back and buttocks. Indirect laryngoscopy showed multiple xanthomatous plaques on the larynx, posterior pharynx, epiglottis, and vocal cords. Additional findings were polyuria, polydipsia, and amenorrhea. Skin biopsy and electron microscopy results confirmed the diagnosis of XD. The patient was treated with fenofibrate, simvastatin, desmopressin, and sex-hormone replacement therapy. Her skin lesions began to slowly fade 6 years after disease onset, eventually resolving spontaneously and completely, but leaving an atrophic scar, frank anetoderma, and persisting diabetes insipidus. This case report together with a review of the English-language literature on the long-term follow-up of XD patients provides additional information on the natural history of this disease.

Topics: Adolescent; Amenorrhea; Anetoderma; Antidiuretic Agents; Biopsy; Cicatrix; Deamino Arginine Vasopressin; Dermatologic Agents; Diabetes Insipidus; Female; Fenofibrate; Gadolinium DTPA; Histiocytosis, Non-Langerhans-Cell; Hormone Replacement Therapy; Humans; Radionuclide Imaging; Remission, Spontaneous; Simvastatin

Nocturnal enuresis (bedwetting) is a common inherited medical condition affecting both children and adults. It is not due to laziness. It can be distressing, embarrassing, humiliating and has a negative impact on self esteem and mood especially in children and young people over the age of 10 years.. To assist busy general practitioners develop a better understanding of bedwetting and the importance of its diagnosis and treatment.. Spontaneous remission rates over the age of 10 years are low at 5% per year and approaches often tried at home such as waking to void, reward charts or fluid restriction are not effective. However, treatment with alarm systems or desmopressin for those who fail to respond to alarms is successful in over 90% of cases.

Topics: Adolescent; Adult; Behavior Therapy; Child; Child, Preschool; Deamino Arginine Vasopressin; Enuresis; Female; Humans; Male; Remission, Spontaneous; Renal Agents

Topics: Adolescent; Age Factors; Antidepressive Agents, Tricyclic; Behavior Therapy; Case Management; Child; Child, Preschool; Deamino Arginine Vasopressin; Enuresis; Female; Humans; Male; Managed Care Programs; Primary Health Care; Remission, Spontaneous; Renal Agents; Treatment Outcome

Bleeding contributes to the high mortality of venovenous extracorporeal membrane oxygenation (vvECMO). The development of acquired von Willebrand syndrome (AVWS) has been identified as relevant pathology during ECMO. This study was performed to determine the onset of AVWS after implantation and the recovery of von Willebrand factor (VWF) parameters after explantation of ECMO in a large cohort of patients.. VWF parameters of 59 patients treated with vvECMO at a university ECMO center were obtained before ECMO implantation, during therapy, and after explantation. In a subgroup of patients, light transmission aggregometry of platelets and flow-cytometric quantification of platelet granule secretion were performed.. All patients developed severe AVWS hours after implantation of vvECMO. After explantation, AVWS recovered within 3 hours in 60%, within 6 hours in 86%, and in all patients within 1 day. Aggregometry showed hypoaggregability of platelets after stimulation with ADP, ristocetin, collagen, and epinephrine. Flow-cytometric platelet analyses revealed severely reduced expression of CD62 and CD63.. All patients during vvECMO support rapidly develop AVWS and platelet dysfunction, resulting in severe impairment of coagulation. After explantation, AVWS overwhelmingly recovers within hours, resulting in a hypercoagulative state. These findings augment the need for novel extracorporeal technologies with reduced shear stress, and shift the emphasis for intense anti-coagulation during ECMO instead to a time-point after explantation.

Topics: Adult; Aged; Anticoagulants; Blood Platelet Disorders; Deamino Arginine Vasopressin; Drug Combinations; Erythrocyte Transfusion; Extracorporeal Membrane Oxygenation; Factor VIII; Female; Follow-Up Studies; Humans; Male; Middle Aged; Platelet Aggregation; Platelet Transfusion; Remission, Spontaneous; Thrombocytopenia; von Willebrand Diseases; von Willebrand Factor

To describe the natural history of patients with nocturnal enuresis (NE) during a 10-year period and to evaluate possible impact of comorbid conditions on the persistence of NE.. Ninety-five children (male to female ratio [M:F] 65:30), aged at first visit between 6 and 21 years were included in this study. Of study subjects 75 had primary monosymptomatic nocturnal enuresis (PMNE), 3 had secondary monosymptomatic nocturnal enuresis (SMNE) and 17 had non-mono­symptomatic nocturnal enuresis (NMNE). Demographic and NE-related details were assessed from electron­ic medical records and by telephone interview at the times 3, 6, 12 months and 3, 5, 10 years after the first examination. Sixty-seven of 95 patients were enrolled, of whom 57 had PMNE (M:F ratio 39:18, mean age 9.35 ± 2.81 years, mean age at improvement 11.5 ± 4.08 years), 8 had NMNE (M:F ratio 4:4, mean age 10.1 ± 2.64 years, mean age at improvement 12.6 ± 1.68 years) and 2 had SMNE (M:F ratio 1:1, mean age 12 years, mean age at improvement 13.5 ± 2.12 years).. The mean duration of follow up was 7.2 ± 2.5 years. All of the 67 children had 5 years follow up. Only 29 of 67 patients (19 with PMNE, 8 with NMNE and 2 with SMNE) had 10 years follow up and 4 of 19 with PMNE were still affected by NE. Out of 57 patients with PMNE 12 (2/12 with language disorders, 1/12 varicocele and 1/12 cryptorchidism) and out of 8 patients with NMNE 1 were still enuretic while all patients with SMNE were in remission.. We observed that language disorders and testicular pathology in NE children could be comor­bidities associated with persistence of NE and treatment resistance.

Topics: Adolescent; Antidiuretic Agents; Child; Chronic Disease; Comorbidity; Cryptorchidism; Deamino Arginine Vasopressin; Female; Follow-Up Studies; Functional Laterality; Humans; Language Disorders; Male; Nocturnal Enuresis; Remission Induction; Remission, Spontaneous; Time Factors; Varicocele; Young Adult

Central nervous system (CNS) sarcoidosis is a crucial disease and has a poor prognosis. A 58-year-old woman had acute development of polydipsia and polyuria. Her pituitary MRI demonstrated a swelling of pituitary gland and hypophyseal stalk. She was diagnosed as central diabetes insipidus (CDI) due to CNS sarcoidosis based on the examinations and pituitary MRI findings as well as a result of cutaneous biopsy. Uveitis and bilateral hilar lymphadenopathy were observed mildly throughout. However, CDI and pituitary MRI findings were getting recovered spontaneously without steroid treatment in a couple of months, suggesting an atypical clinical course of CNS sarcoidosis.

Topics: Antidiuretic Agents; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Middle Aged; Remission, Spontaneous; Sarcoidosis

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Remission, Spontaneous; Time Factors