Congenital Cataract in a Triple X Syndrome Patient

Purpose@#To report a case of bilateral congenital cataract in a patient with triple X syndrome.Case summary: A 9-month-old female was referred to our clinic because of poor fixation and white pupils in both eyes. The patient underwent a performed chromosomal study in the pediatric department due to developmental delay and torticollis. As a result, she was diagnosed with triple X syndrome (47, XXX). Under general anesthesia, the patient underwent lens aspiration with intraocular lens insertion and anterior vitrectomy was performed followed by posterior capsulotomy with optic capture. Intraoperatively, remnant pupillary membrane, posterior embryotoxon, and anterior lenticonus with anterior capsular defect in both eyes were observed. But there was no specific finding on fundus examination. @*Conclusions@#We report the first case of bilateral congenital cataract in a patient with triple X syndrome. It is recommended that clinicians check for cataracts as soon as possible when this chromosome abnormality is confirmed.

The Clinical Usefulness of a Repeat Urine Culture 48 Hours after Antimicrobial treatment in Anatomically Normal and Abnormal Urinary Tract Infection

PURPOSE: We aimed to compare the frequency of positive repeat urine cultures 48 hours after antimicrobial treatment between anatomically normal and abnormal urinary tract Infection (UTI) groups to determine the potential clinical usefulness of the tests. METHODS: We reviewed medical records of 930 patients under age 14, who had been admitted for UTI at Gyeongsang National University Hospital from January 1, 1998 to August 1, 2008. The eligible patients were divided into two groups the anatomically normal UTI group and the anatomically abnormal UTI group. Statistical analyses were performed with variables consisting of the sex ratio, age distribution and the frequency of positive repeat urine cultures of each group. RESULTS: The sex ratio of the anatomically normal UTI group was M:F=1.9:1, whereas that of the anatomically abnormal UTI group was M:F=3.5:1 (P=0.019). For age distribution, it was found that the mean age of the anatomically normal UTI group was 0.82+/-1.83 years, whereas that of the anatomically abnormal UTI group was 1.18+/-2.57 years (P=0.113). The frequency of positive repeat urine cultures in the anatomically normal UTI group was 3/279 (1.1%), whereas that of the anatomically abnormal UTI group was 1/90 (1.1%) (P=0.675). CONCLUSION: We conclude that performing a repeat urine culture is not justified in terms of clinical usefulness, and it is unreasonable to use the results as an index of therapeutic success. A follow-up urine culture is unnecessary in patients with both the anatomically normal and abnormal UTI group.

Correlaton between soluble transferrin receptor concentration and inflammatory markers / 소아과

PURPOSE: The concentration of soluble transferrin receptor (sTfR) is estimated as an iron parameter to evaluate erythropoiesis and iron status. The aim of our study is to evaluate the correlation between sTfR concentration and inflammatory parameters and to distinguish iron deficiency anemia from anemia of inflammation. METHODS: One hundred and forty-four infants younger than two years of age who visited Gyeongsang University Hospital for 7 years from 2000 to 2006 were enrolled. Patients who had hemoglobin (Hb) <11 g/dL and ferritin <12 mg/L were excluded. Routine hematologic lab, serum ferritin, sTfR, and inflammatory markers [C-reactive protein(CRP), interleukin-6(IL-6), and absolute neutrophil count (ANC)] were investigated. RESULTS: In all patients, the sTfR concentration showed a correlation with Hb, ferritin, MCV, and ANC, but not with CRP and IL-6. In multiple regression models, positive correlations were found between sTfR concentration and IL-6 (r=0.078, P=0.043), and negative correlations were found between sTfR concentration and ANC (r=-0.117, P=0.033) and MCV (r=-0.027, P=0.009). CONCLUSION: sTfR concentration was influenced by inflammatory parameters. Therefore, sTfR does not appear to be a useful parameter for discriminating between iron deficiency anemia and anemia of inflammation in infants.

Relationship between Helicobacter pylori infection and iron-deficiency anemia in infants and children / 소아과

PURPOSE: To elucidate a potential association between Helicobacter pylori (HP) infection and iron-deficiency anemia (IDA) in infants and children in terms of the other factors related to iron utilization and storage although the association of ferritin was previously studied. METHODS: We evaluated 135 infants (aged 6-24 months) admitted at Gyeongsang National University Hospital from 2000 to 2006. Western blot assays using the HP CagA antigen (120 kD) were conducted to identify infections. The concentrations of six parameters were measured: hemoglobin (Hb), serum ferritin, soluble serum transferrin receptors, interleukin-6, prohepcidin, and C-reactive protein. In addition, the infants were classified into IDA, anemia from inflammation (AI), unclassified anemia (UCA), and normal groups on the basis of Hb and ferritin concentrations. RESULTS: In the IDA group (n=20), seven infants were infected with HP, with the other infants showing no evidence of infection. The mean Hb levels in the IDA group were significantly lower in HP-infected infants than those uninfected (7.1 vs. 8.2 g/dL, respectively); the mean ferritin levels were also significantly lower in the infected infants (3.2 vs. 6.8 microgram/L). The other four parameters did not differ significantly among the IDA infants. No correlations were found between the six parameters and HP infection status in the other groups. CONCLUSION: There were no significant differences in the HP infection rates among the study groups. However, in the IDA group, the HP-infected infants had significantly lower serum ferritin and Hb levels than the HP-negative infants (P<0.05).

Relationship between Helicobacter pylori infection and iron-deficiency anemia in infants and children / 소아과

PURPOSE: To elucidate a potential association between Helicobacter pylori (HP) infection and iron-deficiency anemia (IDA) in infants and children in terms of the other factors related to iron utilization and storage although the association of ferritin was previously studied. METHODS: We evaluated 135 infants (aged 6-24 months) admitted at Gyeongsang National University Hospital from 2000 to 2006. Western blot assays using the HP CagA antigen (120 kD) were conducted to identify infections. The concentrations of six parameters were measured: hemoglobin (Hb), serum ferritin, soluble serum transferrin receptors, interleukin-6, prohepcidin, and C-reactive protein. In addition, the infants were classified into IDA, anemia from inflammation (AI), unclassified anemia (UCA), and normal groups on the basis of Hb and ferritin concentrations. RESULTS: In the IDA group (n=20), seven infants were infected with HP, with the other infants showing no evidence of infection. The mean Hb levels in the IDA group were significantly lower in HP-infected infants than those uninfected (7.1 vs. 8.2 g/dL, respectively); the mean ferritin levels were also significantly lower in the infected infants (3.2 vs. 6.8 microgram/L). The other four parameters did not differ significantly among the IDA infants. No correlations were found between the six parameters and HP infection status in the other groups. CONCLUSION: There were no significant differences in the HP infection rates among the study groups. However, in the IDA group, the HP-infected infants had significantly lower serum ferritin and Hb levels than the HP-negative infants (P<0.05).

Analysis of disease mechanism of subacute necrotizing lymphadenitis in children / 소아과

PURPOSE: The cause of subacute necrotizing lymphadenitis, a rare disease in children, has not been completely clarified. This study was aimed to investigate the disease mechanism by examining clinical, radiologic, and immunohistochemical findings in children diagnosed with subacute necrotizing lymphadenitis after an excisional biopsy. METHODS: We examined 19 lymph node tissue specimens from 17 children diagnosed with subacute necrotizing lymphadenitis at Gyeongsang National University Hospital from March, 1998 to July, 2006. A retrospective survey of the medical records was performed. CT findings were analyzed. Immunohistochemical staining was done on tissues obtained by excisional biopsy from all patients. RESULTS: The patient's age ranged from 5 to 19 years (average age :11.8 years). The main symptoms included a neck mass (17/19), pain in the mass (6/17), and fever (12/19). The palpable lymph nodes were mostly cervical in location; the maximum diameter, which was measured radiologically, was less than 3 cm in all 10 cases. The masses were pathologically divided into proliferative, necrotic, and xanthomatous types. With immunohistochemical staining the masses were divided into lesion (L), perilesion (PL), and necrosis (N). The CD8 staining was stronger than the CD4 staining for all regions in three types. The CD4 staining intensity was mainly increased in the perilesion, and CD8 was mainly increased in the lesion. CONCLUSION: We compared the radiologic findings, clinical symptoms, and pathology to help understand the cause of disease in patients with subacute necrotizing lymphadenitis.