A clinical study on patients with porencephaly

Porencephaly is relatively rare condition defined by an defect or a defect or cavity in the cerebrum owing to a developmental malformation or to a destructive lesion. Fory-five porencephaly patients diagnosed by Brain CT were clinically analyzed and the following results were obtained. 1) By the age group presenting initial symptoms, the peak incidence was from 1 month to below 3 years old. 2) In initial symptoms, seizure, spastic weakness, headache were showed in order of frequency. But 7 cases (15.5%) were asymptomatic. 3) The latency of diagnosis after presenting initial symptoms from the symptom onset time to 10 years. 4) The subsequent symptoms were as follows: spastic weakness, speech disturbance, gait disturbance, mental retardation, sensory loss and seizure showed independently or combined. 5) As etiologic factor, 21 cases (46.7%) were congenital, 16 cases (35.5%) were post-traumatic or post-operative and 8 cases (17.8%) were perinatal. 6) The prognosis was seen various from mild to severe. Out of 45 cases, 29 cases (64.4%) were no complications. But the prognosis in patients with post-traumatic or postoperative etiological factors was poor. With the advent of brain CT and the resultant capability of detecting structural defect and cerebral lesions responsible for epilepsy or focal neurologic signs, porencephaly was seen to be readily recongizable by CT examination. Since porencephaly is a significant contributor to the spectrum of CNS lesion and benign condition, ist recognition is important in determining prognosis and therapy.

Renal scarring in relation to visicoureternal reflux in urinary tract infection

The vesicoureteral reflux (VUR) is frequently found among the patients with urinary tract infection (UIT) and some patients with VUR progress to reflux nephropathy. The presence of scarring at UIT is an important determinant in the selection of those at risk of progressive damage. 99m Technetium dimercaptosuccinic acid (99m Tc-DMSA) is a renal scanning agent provides a good quality of renal image as a result of preferential cortical accumulation. 99m Tc-DMSA scan and VCUG were performed in 133 patients diagnosed UTI at pediatric department of Kyung Hee University, College of Medicine from May, 1990 to May, 1992 The results were as follows: 1) VUR was seen in 27 children among 133 patients with UTI. The incidence was 20.3%. Under 1 year of age, male to female ratio was higher incidence of VUR. 2) Incidence of renal scarring was higher in patients with VUR than those of without VUR. 3) Grading of VUR in relation to age, the older the age, the lower the grade. 4) There is no correlation between renal scarring and grades of VUR. 5) In distribution of renal scarring, it was found to have tendency to develop at upper polar area, there was diffuse and multiple in patients with VUR. In conclusion the incidence of renal scarring was related to the age of onset, duration of UTI and the severity of the VUR and 99mTc-DMSA scan is mandatory in patient with UTI even without VUR in order to detect early phase of renal scarring.

Three cases of atypical Kawasaki disease with coronary aneurysm

Kawasaki disease is described by fever lasting five days or more, bilateral conjunctival injection, changes of lips and oral cavity, polymorphous exanthema, acute non-purulent cervical lymphadenopathy, and changes of extremities. Atypical Kawasaki disease is defined as fewer than 4 of 6 criteria described above including coronary artery abnormalities. Especially, atypical clinical manifestations of Kawasaki disease appear in infants younger than 6 months old of age. Thus we recommend echocardiography in early infancy who has prolonged fever in order to diagnose atypical Kawasaki disease and treat early. We have experienced three cases of atypical Kawasaki disease with coronary aneurysm who were admitted because of fever and revealed coronary aneurysm on echocardiography.