DIDMOAD syndrome with megacystis and megaureter.
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چکیده
منابع مشابه
DIDMOAD syndrome with megacystis and megaureter.
A case of DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy and nerve deafness) is described. There was unusually severe urinary tract dilatation which led to an ileal conduit diversion. Immunohistological study of the bladder wall and ureter revealed a marked diminution in nerve fibres, which may have been primary or secondary to the muscle hypertrophy. The possible pathog...
متن کاملA Study of the Vesical Ganglia in Children and the Relationship to the Megaureter Megacystis Syndrome and Hirschsprung's Disease.
A method for the assay of vesical ganglion cells is described and the number and distribution of the neurones in three normal bladders was studied. Vesical ganglion counts were done in cases of the megaureter-megacystis syndrome, megaureter, bladder neck obstruction, megacystis associated with absent abdominal muscles, Hirschsprung's disease, and lumbar myelomeningocoele. A normal complement of...
متن کاملSyndrome of the month Wolfram (DIDMOAD) syndrome
Wolfram syndrome (MIM 222300) is the association ofjuvenile onset diabetes mellitus and optic atrophy, also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness). Patients present with diabetes mellitus followed by optic atrophy in the first decade, cranial diabetes insipidus and sensorineural deafness in the second decade, dilated renal outflow tracts early in t...
متن کاملThe Wolfram or DIDMOAD syndrome
Seven patients with a rare syndrome of diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract, and other abnormalities (Wolfram or DIDMOAD syndrome) are reported. Of the seven patients, three siblings were followed up for 10-17 years. All seven patients had diabetes mellitus and optic atrophy; six had diabetes insipidus; and in...
متن کاملMegacystis-Microcolon-Intestinal Hypoperistalsis Syndrome
We report a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a newborn female infant who presented with an abdominal mass, absent bowel sounds, and feeding intolerance with bilious emesis. MMIHS is a rare congenital bowel and bladder defect requiring surgery and chronic total parenteral nutrition in an attempt to sustain life. With few exceptions, it is predominately...
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ژورنال
عنوان ژورنال: Postgraduate Medical Journal
سال: 1986
ISSN: 0032-5473
DOI: 10.1136/pgmj.62.731.859