Renal Involvement in Children With Glycogen-Storage Disease

Abstract

INTRODUCTION: 99m Tc-dimercaptosuccinic acid (DMSA) scintigraphy is accepted as the gold standard in the diagnosis of acute pyelonephritis. OBJECTIVE: In an attempt to reduce the number of investigations after urinary tract infections (UTIs), with this prospective study we aimed to evaluate the diagnostic value of acute-phase reactants in identifying renal involvement in infants with febrile UTI. METHODS: Fifty-four infants (36 male, 18 female) aged 1 to 12 months were studied. For all infants, clinical findings such as duration and height of fever before antibiotic administration and laboratory parameters such as leukocytosis (white blood cell count of Ͼ15.000/ L), elevated erythrocyte sedimentation rate (ESR) (Ͼ20 mm/hour), and high levels of C-reactive protein (Ͼ10 mg/mL) were compared with the results of the DMSA scan obtained within 72 hours after referral. RESULTS: Regarding microbial agents, Escherichia coli was identified in 42 (78%) of the 54 infants, and 16 (29.5%) of the 54 of infants were febrile for Ͼ2 days before diagnosis of UTI. Leukocytosis, elevated ESR, and high levels of C-reactive protein were present in 14 (26%), 41 (76%), and 38 (70%) infants, respectively. Acute-phase DMSA showed renal involvement in 10 (18.5%) infants. Vesicoureteral reflux was found in 16 (29.5%) infants. Gender, duration of fever before antibiotic administration, leukocytosis, elevated ESR, and high levels of C-reactive protein were not related to the severity of renal damage, as shown by DMSA. Only fever of Ͼ39°C was slightly correlated with an abnormal DMSA scan result (r ϭ 0.3; P ϭ .032). CONCLUSIONS: Acute-phase DMSA scintigraphy remains superior to clinical and laboratory data for predicting renal involvement in infants with febrile UTIs. IMMUNE FINDINGS IN CHILDREN OBJECTIVE:The aim of our study was to investigate prospectively the immune disturbances in steroid-sensitive (SS) and steroid-resistant (SR) NS and identify whether these immune disturbances may predict the response to steroid therapy. METHODS: Thirty children with SS NS and 7 children with SR NS (aged 2 to 14 years) were studied. To evaluate the possible relationship between immune disturbances and response to treatment, patients were evaluated during different disease stages. Data were compared with those obtained from 25 age-matched controls. The following parameters were assessed: basic B-and T-cell populations, percentages of CD23 ϩ , CD3 ϩ /CD69 ϩ /interferon ␥ ϩ (IFN-␥ ϩ ) cells, and CD3 ϩ /CD69 ϩ /interleukin 4 ϩ (IL-4 ϩ ) T cells, and serum levels of IL-13 and IL-18. RESULTS: In patients with SS NS percentages of CD23 ϩ and CD19 ϩ B cells, CD3 ϩ /CD69 ϩ /IL-4 ϩ T cells and serum levels of IL-13, IL-18 were significantly higher in the active stage compared with the remission stage on steroids, remission off steroids, and controls (P Ͻ .05). On the contrary, percentages of CD3 ϩ /CD69 ϩ /IFN-␥ ϩ T cells were significantly decreased (P Ͻ .05). In patients with SR NS, percentages of CD23 ϩ B cells, CD3 ϩ /CD69 ϩ / IL-4 ϩ T cells, and serum levels...