The syndrome of dysuria and hematuria in pediatric urinary reconstruction with stomach.

Between July 1989 and March 1992 at a single institution 27 male and 30 female patients underwent lower urinary reconstruction with stomach. Mean patient age was 9.9 years (range 1.5 to 28 years). The diagnoses were epispadias/exstrophy complex (19 patients), myelodysplasia (11), cloacal exstrophy (6), posterior urethral valves (6), Hinman syndrome (4), sacral agenesis (3) and other (8). Indications for surgery were urinary incontinence, upper tract deterioration or undiversion. A total of 54 patients underwent augmentation gastrocystoplasty and 3 had total bladder replacement. Mean followup time was 23.2 months (range 12 to 39 months). The syndrome of dysuria and hematuria is defined as 1 or a combination of the following symptoms: bladder spasm or suprapubic, penile or periurethral pain, coffee brown or bright red hematuria without infections, skin irritation or excoriation and dysuria without infections. Telephone and clinic interviews identified 21 patients (36%) with symptoms of the dysuria and hematuria syndrome. The most common symptoms were hematuria (71%) and bladder or suprapubic pain (76%). Of the patients 18 (86%) ranked the severity of symptoms as mild to moderate and 3 (14%) ranked them as severe. No medications were required to control the symptoms in 13 patients (62%) and 3 other patients only required medications on an as needed basis. Overall patients who required no medications had lower symptom scores than those who required medications. Patients with decreased renal function may be more at risk for the dysuria and hematuria syndrome than those with normal renal function. Patients who were wet were more prone to have the dysuria and hematuria syndrome than those who were totally dry. The pathophysiology of the dysuria and hematuria syndrome is currently unknown. Patients who require urinary reconstruction with stomach tissue need to be made aware of the potential of the dysuria and hematuria syndrome.

Marfan syndrome presenting as aortic rupture in a young athlete: sudden unexpected death?

A 20-year-old man had a spontaneous rupture of the ascending aorta while exercising. The diagnosis of Marfan syndrome, established by the gross and microscopic autopsy findings, was further supported by review of the autopsy report of the father, who had died at age 26 of a nearly identical aortic rupture. To avoid occurrences such as the above, the responsibility of the forensic pathologist should extend beyond the autopsy to appropriate counseling of families in instances where medically treatable hereditary conditions may be present in the survivors.