Rituximab, a chimeric anti-CD20 IgG1 monoclonal antibody, has been used as a rescue therapy for steroid-dependent or refractory nephrotic syndrome. However, the adverse effects of rituximab are yet to be investigated. We report a case of a 9-year-old boy with steroid-dependent nephrotic syndrome who developed Kikuchi-Fujimoto disease after several cycles of rituximab therapy. Kikuchi-Fujimoto disease is a benign, self-limited necrotizing histiocytic lymphadenitis of unknown etiology. In the present case, Kikuchi-Fujimoto disease developed when the peripheral blood B-cell count of the patient was at nadir, and the lesion regressed slowly but spontaneously after recovery of the B-cell count. To our knowledge, although the pathologic diagnosis of Kikuchi-Fujimoto disease was unavailable, this is the first report of Kikuchi-Fujimoto disease with clinical diagnosis as a possible adverse effect of rituximab.
Antibodies, Monoclonal, Murine-Derived ; B-Lymphocytes ; Child ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Immunoglobulin G ; Lymphadenitis ; Nephrotic Syndrome ; Rituximab

Ki-1 positive anaplastic large cell lymphoma is a newly described high-grade lymphoma and is defined by histopathological and immunologic criteria. We experienced a case of systemically involving Ki-1 positive anaplastic large cell lymphoma in a 44 year- old female which initially manifested as pleural effusion. Abdominopelvic CT scan showed the evidence of marked lymphadenopathy in retroperitoneal and both external and inguinal lymph nodes. On cytologic examination of pleural fluid, tumor cells revealed pleomorphic large isolated cells with prominent nucleoli and abundant cytoplasms. The nuclei were large with irregular profiles including some deep invaginations. Also, occasional multilobed/ multinucleated and binucleated nuclei were seen. Immunohistochemical examination was performed to differentiate from the undifferentiated adenocarcinoma, Hodgkin's disease, non-Hodgkin's lymphoma and malignant histiocytosis. The neoplastic cells were positive for leukocyte common antigen, CD3, CD30(Ki-1) but negative for cytokeratin, epithelial membrane antigen, and CD15. A histologic diagnosis of Ki-1 positive anaplastic lymphoma was made by biopsies of the inguinal lymph node, polypoid lesions of the stomach and cecum.
Adenocarcinoma ; Amyloid ; Antigens, CD45 ; Biopsy ; Cecum ; Child* ; Cytoplasm ; Diagnosis ; Female ; Histiocytic Sarcoma ; Hodgkin Disease ; Humans ; Keratins ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Non-Hodgkin ; Mucin-1 ; Multiple Myeloma ; Pleural Effusion ; Stomach ; Tomography, X-Ray Computed

In order to investigate the role of adenovirus in diarrheal children, we evaluated 907 children with diarrhea and 193 children without diarrhea for a 22-month. Stools were tested for group A rotavirus antigen and for adenovirus types 40/41 (Ad 40/41) by using ELISA, cell technique and indirect immunofluorescent method. Adenovirus was detected in 10.1% of the diarrheal children and 3.1% of the non-diarrheal children. Ad40/41 was detected in 4.85% of the diarrheal children. Other nonenteric adenovirus was detected in 5.29% of the diarrheal children. In addition, 24% of the adenovirusinfected children excreted rotavirus simultaneously. Rotavirus was found in 57.1% of the diarrheal patients. Among the diarrheal children. 88% of those with adenovirus were younger than 24 months. Although peaks of adenovirus infection were detected in July and Autumn in the study, there is no apparent seasonal trend with adenovirus. The average duration of admission caused by adenoviral infection was 4.9 days and 88% of the cases accompanied by fever. Stool occult blood test revealed positive reaction in 66.3% of the cases and serum aminotransferase activities transiently elevated in 5.4% of the cases. Diarrhea with more than 10 stools per day, vomiting, or fever in adenovirusinfected children were similar with ratavirus, where as the first two manifestions were associated with confection of rotavirus and adenovirus. Nine out of 92 cases(9.8%) of the gastroenteritis caused by adenovirus revealed intussusception. We conclude that Ad40/41 is an important cause of diarrhea among infants and children, and non-enteric adenovirus is considered to be a cause of diarrhea, but their role in diarrheal children should be further studied.
Adenoviridae Infections ; Adenoviridae* ; Candy ; Child* ; Diarrhea ; Enzyme-Linked Immunosorbent Assay ; Fever ; Gastroenteritis ; Humans ; Infant ; Intussusception ; Occult Blood ; Rotavirus ; Seasons ; Vomiting

The Russell-Silver syndrome (RSS) is a disease characterized by intrauterine growth retardation with preserved head circumference, facial dysmorphism and short stature. Reported renal and urinary manifestations of RSS include horseshoe kidney, renal tubular acidosis, hydronephrosis, ureteropelvic obstruction and vesicoureteral reflux. Here we report a case of end-stage renal disease associated with RSS, which, to the best of our knowledge, has not been reported yet.
Acidosis, Renal Tubular ; Fetal Growth Retardation ; Head ; Hydronephrosis ; Kidney ; Kidney Failure, Chronic ; Silver-Russell Syndrome ; Vesico-Ureteral Reflux

Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.
Adult ; Age of Onset ; Capillaries ; Capillary Leak Syndrome* ; Child* ; Female ; Humans ; Hypoalbuminemia ; Hypotension ; Multiple Myeloma ; Paraproteinemias

PURPOSE: To evaluate the diagnostic accuracy of MR imaging in posterior cruciate ligament(PCL) injuries, to describe the variety of these injuries and to assess the type and frequency of associated knee injuries. MATERIALS AND METHODS: We retrospetively analysed 75 cases with knee injuries. In all cases, the prescence of a PCL tear was determined by arthroscopy or surgery and the type of tear and patterns of associated knee injuries were described. RESULTS: Twenty-eight PCL tears were identified with arthroscopy or surgery. Figures for the sensitivity, specificity, and accuracy of MR diagnosis for the PCL injury were 86%, 94% and 91% respectively. Twenty-one cases(75%) had complete PCL tears and seven (25%) had partial tears. The commonest tear site was midsubstance(18 cases, 64.3%). Twenty-seven cases(96%) had associated knee injuries ; the remaining patient(4%) had isolated PCL injuries. There were 19 cases(68%) of ligamentous injuries, ten (36%) of meniscal tear, ten (36%)of bony injuries, and 20 (71%) of joint effusions. Of the ligamentous injuries, those involving the medialcollateral ligament were most often seen(10 cases, 36%). CONCLUSIONS: MR imaging is an accurate method for the detection and evaluation of PCL injury and associated knee abnormalities.
Arthroscopy ; Diagnosis ; Joints ; Knee ; Knee Injuries ; Ligaments ; Magnetic Resonance Imaging* ; Posterior Cruciate Ligament* ; Sensitivity and Specificity

Congenital aniridia is a rare ocular malformation that presents with severe hypoplasia of the iris and various ocular manifestations. Most cases of congenital aniridia are known to be related to mutations in the paired box gene-6 (PAX6), which is an essential gene in eye development. Herein, we report a familial case of autosomal dominant congenital aniridia with four affected members in 3 consecutive generations and describe the detailed ophthalmologic findings for one of these members. As expected, mutational analysis revealed a nonsense mutation (p.Ser122*) in the PAX6 gene. Thus, our findings reiterate the importance of PAX6 mutations in congenital aniridia.
Aniridia* ; Codon, Nonsense ; Family Characteristics ; Genes, Essential ; Humans ; Iris ; WAGR Syndrome ; Wilms Tumor