To investigate whether age at onset of steroid-sensitive nephrotic syndrome (SSNS) is predictive of subsequent relapses, or influences outcome, we retrospectively studied 60 patients who were under 10 years of age at onset and were followed for over 10 years. They were divided into three groups according to age at diagnosis: group 1-3 (1.0-3.9 years at onset, n=24), group 4-6 (4.0-6.9 years at onset, n=22), and group 7-9 (7.0-9.9 years at onset, n=14). In the 51 patients with long-term remission, defined as remaining relapse-free over 3 years, the total number of relapses was significantly more in group 1-3 (n=18) than in group 4-6 (n=19), and the interval between onset and long-term remission was significantly longer. Group 4-6 and group 7-9 had fewer patients with active disease at 10 years, follow-up than group 1-3, as assessed by the Kaplan-Meier method. These data suggest that the age at onset of SSNS influences the clinical course (i.e., frequency of relapses) and the time to reach long-term remission. An age of less than 4 years at onset of SSNS is associated with greater likelihood for frequent relapses and a greater time interval to attain long-term remission.

These data indicate that the prognosis of APSGN during childhood is excellent, when adequately diagnosed and treated.

Arthrogryposis -renal tubular dysfunction -cholestasis (ARC) syndrome is a rare disease that was fi rst reported by LutzRichner and Landolt. 1 Affected infants do not thrive and usually die within the fi rst year of life. Recently, ARC syndrome was mapped to a 7 cM interval on 15q26.1; 2 however, not many physicians, not even pediatricians, have suffi ciently recognized the existence of this syndrome. For the fi rst time, we describe two cases of this syndrome in children born to Japanese parents; the children had lethal ARC syndrome together with other symptoms, including ichthyosis and central nervous system defects. In order to disseminate information on ARC syndrome, we discuss here the clinical fi ndings in the patients presented. Case report Case 1The fi rst subject was a girl born with a weight of 2.7 kg, a length of 49 cm, and a head circumference of 35.2 cm. She was born at the gestational age of 41 weeks and 5 days by cesarean section because of pelvic presentation. On examination, abnormal fi ndings included wide anterior fontanel, a high arched palate and low-set ears. There was generalized hypotonia, hepatomegaly and fl exion contractures of the knees ( Fig. 1). No abnormal fi ndings could be detected in the eyes at birth.Within 72 h, she developed renal tubular acidosis with glycosuria in the absence of hyperglycemia, generalized amin oaciduria, and phosphaturia, and was diagnosed with Fanconi's syndrome. Her skin was dry and scaly, and worsened gradually until it was diagnosed as ichthyosis.On the sixth day of life, she developed progressive jaundice and gray-colored stools. Serum bilirubin levels ranged from 5 to 8 mg/dL, with direct bilirubin levels of 3 -7 mg/dL, although the transaminase and ␥ -glutamyltranspeptidase were repeatedly found to be normal. Alkaline phosphatase was elevated to >2000 IU/L. Viral hepatitis studies, including hepatitis B surface antigen antibody, were negative. Kidney function test results at the fi rst blood examination included the following: blood urea nitrogen, 7.5 mg/dL; creatinine, 0.4 mg/dL; sodium, 139.2 mEq/L; potassium, 4.4 mEq/L; chloride, 111.8 mEq/L; calcium, 9.5 mg/dL; bicarbonate, 18.5 mmol/L. Urine studies also showed hyper-␤ 2-microglobulinuria and increased N -acetylglucosamine. There were few white blood cells with no evidence of urinary tract infection. Hyperlipidemia, with a total cholesterol level of >230 mg/dL and triglyceride levels >250 mg/dL, was observed and was found to persist.Magnetic resonance imaging (MRI) of the brain revealed hypoplasia of the corpus callosum, and deformity of right lateral ventricles. There were no abnormal fi ndings on the fornix or fi ssures. The cerebellum and the formation of the vermis were normal. There was no delay in myelination. The auditory brainstem response test revealed bilateral nerve deafness. Abdominal ultrasound showed hepatomegaly, but no other abnormal fi ndings; the kidneys and gallbladder were normal. Technetium-99 m ( 99m Tc)-labeled pyridoxyl-5-methyln trip) tophan hepatobiliary scintigram revea...

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