pituitrin and Dilatation--Pathologic

Vasopressin is a 9-amino acid peptide synthesized by magnocellular neurons of the hypothalamus and released from posterior pituitary gland. The primary physiological role of vasopressin is the maintenance of fluid homeostasis. In this review, the classification of vasopressin receptors, namely V1 vascular, V2 renal, V3 pituitary, oxytocin receptors, and purinergic receptors, and the effects of vasopressin on vascular smooth muscles, the heart, and the kidneys are discussed. Mortality rates of vasodilatory (or distributive), for example septic shock, are high. The use of vasopressin is an alternative therapy for vasodilatory shock with better outcome. Vasopressin is effective in resuscitation of adults after ventricular fibrillation or pulseless tachycardia, when epinephrine is not effective.

Topics: Adult; Clinical Trials as Topic; Dilatation, Pathologic; Heart; Heart Arrest; Homeostasis; Humans; Intensive Care Units; Kidney; Muscle, Smooth, Vascular; Receptors, Vasopressin; Resuscitation; Shock; Shock, Septic; Survival Analysis; Tachycardia; Time Factors; Treatment Outcome; Vasoconstrictor Agents; Vasodilation; Vasopressins; Ventricular Fibrillation

Portal hypertension, a major hallmark of cirrhosis, is defined as a portal pressure gradient exceeding 5 mm Hg. In portal hypertension, porto-systemic collaterals decompress the portal circulation and give rise to varices. Successful management of portal hypertension and its complications requires knowledge of the underlying pathophysiology, the pertinent anatomy, and the natural history of the collateral circulation, particularly the gastroesophageal varices.

Topics: Collateral Circulation; Dilatation, Pathologic; Endoscopy, Gastrointestinal; Esophageal and Gastric Varices; Gastrointestinal Hemorrhage; Hemostatics; Humans; Hypertension, Portal; Ligation; Portasystemic Shunt, Transjugular Intrahepatic; Pyloric Antrum; Risk Factors; Sclerotherapy; Vasopressins

Polydypsia and polyuria are frequent symptoms in patients with sellar masses caused by neurohypophyseal diabetes insipidus. Autosomal dominant familial neurohypophyseal diabetes insipidus (adFNDI), a disorder caused by mutations in the arginine vasopressin (AVP) -neurophysin II (NPII) gene, should be considered as a rare differential diagnosis. A delayed diagnosis bears the risk of life-threatening electrolyte imbalances and permanent urinary tract damage, leading to impaired quality of life.We present a Caucasian kindred of at least 4 generations with FNDI.Clinical histories, endocrine para-meters, and results of molecular analyses of the AVP gene are presented with a review of the literature on diabetes insipidus (DI) related urinary tract dilatation.Polyuria and polydipsia were only reported based on explicit and thorough interrogation after more than 4 years of clinical follow-up. A novel heterozygous mutation in the AVP gene was found in all examined symptomatic subjects (c.1-33_c.4del37nt). A literature review revealed that non-obstructive hydronephrosis (NOH) is a rare but known complication of DI.Since increased fluid intake is often a typical familial pattern in adFNDI, it is frequently missed as being pathologic in affected patients, therefore a detailed clinical history of drinking volumes is of critical importance. AVP gene testing is an important component in the confirmation of the diagnosis. Otherwise unexplainable NOH should lead to further investigations and evaluation of rare diseases like FNDI.

Topics: Arginine Vasopressin; Child; Delayed Diagnosis; Diabetes Insipidus; Dilatation, Pathologic; DNA Mutational Analysis; Follow-Up Studies; Humans; Hydronephrosis; Kidney Function Tests; Kidney Pelvis; Magnetic Resonance Imaging; Male; Neurophysins; Pedigree; Protein Precursors; Sequence Analysis, DNA; Ureter; Urinary Bladder; Urinary Retention; Vasopressins