• Tuberculosis and Respiratory Diseases
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• v.72 no.2
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• pp.207-211
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• 2012

Hemolytic uremic syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. HUS arises from a wide spectrum of conditions, and chemotherapeutic agents have been reported to be associated with HUS, including Mitomycin, Cisplatin, Bleomycin, and Gemcitabine. A 76-year-old man treated with Gemcitabine due to non-small cell lung cancer developed clinical and laboratory findings compatible with HUS. Gemcitabine was ceased and hemodialysis and plasma exchange were utilized and he recovered. A high level of suspicion for HUS is necessary when cancer patients are treated with Gemcitabine, and prompt recognition and treatment are also essential.

• Journal of Chest Surgery
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• v.40 no.12
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• pp.867-870
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• 2007

Antiphospholipid syndrome (APS) is defined as venous and/or arterial thromboses, recurrent fetal losses, thrombocytopenia in combination with repeatedly positive tests for the lupus anticoagulant (LAC), and anticardiolipin antibodies (aCL). The pulmonary manifestation is APS are relatively rare. We report a rare case of antiphopholipid syndrome with systemic lupus erythematosus in a patient who presented with pulmonary hypertension secondary to a chronic pulmonary thromboembolism. A bilateral thromboendarterectomy was performed satisfactorily and the incision was extended to the left intrapleural pulmonary artery.

• Korean Journal of Clinical Pharmacy
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• v.14 no.2
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• pp.61-70
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• 2004

Heptaplatin is a new platinum derivative with antitumor activity against gastric cancer. Preclinical studies showed that it is less toxic than other platinum analogues. The purpose of this study is to evaluate the efficacy and toxicity of the combination therapy of heptaplatin and 5-fluorouracil in Korean advanced gastric cancer patients. This study was investigated retrospectively. The patients group consisted of 65 advanced gastric cancer patients with no prior radiotherapy. All patients received heptaplatin $400\;mg/m^2$ by 2-3 hour infusion on Day 1 and 5-FU $1000\;mg/m^2by 12-24 hour continuous infusion for 5 days. After the first cycle, subsequent doses were adjusted according to the toxicity. Courses were repeated every 28 days. As results, objective response occurred in 16 patients $(24.6\%)$. Two were complete and 14 were partial response. Median progression free survival was 32 weeks with $29\%$ of patients progression free at 1 year. The most common hematologic toxicity was anemia. Grade 3 or 4 anemia was seen at $2.7\%$ of treatment cycles. Grade 3 or higher leucopenia was seen at $1.2\%$ of cycles. Grade 3 or 4 neutropenia and thrombocytopenia occurred at $6.1\%\;and\;1.5\%$ of cycles, respectively. The most common nonhematologic toxicity was proteinuria. Though no patients experienced grade 3 or 4 proteinuria, proteinuria was a considerable factor for this chemotherapy. Grade 3 or higher gastrointestinal toxicities were nausea and vomiting ($4.6\%$ of patients) and diarrhea ($1.5\%$ of patients). Grade 2 renal toxicity with elevation of serum creatinine was seen in $0.3\%$ of cycles, which is less than that of other platinum analogues. This study showed that combination therapy of heptaplatin and 5-FU have modest antitumor activity against advanced gastric cancer without severe renal toxicity.

• Journal of Korean Traditional Oncology
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• v.20 no.1
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• pp.31-44
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• 2015

Background : The purpose of this study is to investigate the efficacy and safety of the circadian delivery schedule of fluorouracil or capecitabine based chemotherapy for advanced colorectal cancer. Patients and methods : A meta-analysis was performed using individual data from eight international randomized clinical trials, especially phase II or III trials, comparing 5-fluorouracil, or capeticabine in chronomodulated or conventional schedule. The data from 8 studies was composed of 692 patients receiving chronomodulated chemotheray and 684 patients receiving conventional chemotherapy. The main end point was response rate. Results : Response rate was insignificantly different from each group (RR 1.14, 95%CI 0.74-1.74, p=0.55). Overall survival and progresseion-free survival were not significant either. Chemotherapy induced anemia, diarrhea, and nausea/vomiting were worse in the chronotherapy group, with statistic significance respectively. On the other hand, chemotherapy induced thrombocytopenia, stomatitis, peripheral neuropathy, and dermatotoxicity were better but they were not statistically significant results. Conclusions : Patients lived longer but not significantly on chronomodulated chemotherapy rather than on conventional chemotherapy. Patients on chronomodulated chemotherapy experienced adverse events more. The chronomodulated chemotherapy schedule needs adjustment of its delivery schedule and further research is required.

• Pediatric Infection and Vaccine
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• v.22 no.2
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• pp.117-120
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• 2015

Virus-associated immune thrombocytopenic purpura (ITP) can occur following common viruses, but cases of ITP associated with influenza infection has seldom been reported. In this report we describe a previously healthy 5-year-old boy who admitted with fever, flu-like symptoms and a few bruises on both legs. Severe thrombocytopenia were found. Bone marrow aspirates and biopsy showed no abnormalities and results of coagulation tests were all in normal limit. Real-time polymerase chain reaction was positive for influenza B infection. The patient fully recovered with intravenous immunoglobulins and steroid therapy.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.81-88
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• 2017

Purpose: The American Society for Apheresis provides clinical guidelines for therapeutic apheresis in adults, but there are no guidelines for children. This study aimed to analyze the effect of therapeutic plasma exchange (TPE) in pediatric patients with various kidney diseases in Korea. Methods: We retrospectively reviewed the data of 16 children (up to 18 years of age) who were admitted to Severance Children's Hospital with refractory kidney disease. All patients received TPE between 1994 and 2016. Clinical and laboratory characteristics such as age, weight, sex, change in blood urea nitrogen (BUN), and creatinine level before and after TPE, and complications after TPE were analyzed. Results: The mean age and weight of the 16 patients at the time of TPE was $11.3{\pm}4.0$ years and $34.6{\pm}17.5$ kg, respectively. The BUN level was 35.4 mg/dL before TPE and significantly decreased to 21.5 mg/dL (P=0.025) at 1 week and 20.5 mg/dL (P= 0.01) at 1 month after TPE. The creatinine level significantly decreased from 1.20 mg/dL before TPE to 0.90 mg/dL (P=0.02) at 1 week after TPE. Four complications (hypovolemia, anemia, hypocalcemia, and thrombocytopenia) were reported, but were not fatal. Conclusion: Our findings suggest that TPE is an effective therapeutic modality in children with refractory kidney disease and can be indicated for the treatment of various kidney diseases.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.75-80
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• 2017

Purpose: To investigate the frequency, presentation, management, and outcome of cytomegalovirus (CMV) infection in pediatric patients who underwent renal transplantation. Methods: We performed a retrospective chart review of 70 patients under the age of 18, who underwent renal transplantation between January 1990 and November 2014. A diagnosis of CMV infection was based on serology, molecular assays, antigenemia assays, and culture. CMV infection was defined as detection of virus and CMV disease was diagnosed when clinical signs and symptoms were present. Results: The number of patients with CMV infection was 18 (25.7% of renal transplant recipients). Twelve were male (66.7%), and the $mean{\pm}standard$ deviation (SD) age at infection was $13.3{\pm}3.9$ years. Median time of infection after renal transplantation was 4 months (range 1.0-31.0 months). Pretransplantation CMV status in the infected group was as follows: donor (D)+/recipient (R)+, 11 (61.1%); D+/R-, 7 (38.9%); D-/R+, 0; and D-/R- 0. Nine patients had CMV disease with fever, leukopenia, thrombocytopenia, or organ involvement such as enteritis, hepatitis, and pneumonitis. The age of disease occurrence was $13.1{\pm}3.9$ years and the median time to disease onset after renal transplantation was 8 months (range 1.0-31.0). Immunosuppressive agents were reduced or discontinued in 14 patients (77.8%), antiviral agents were used in 11 patients (61.1%), and all patients with CMV infection were controlled. Conclusions: A quarter of the patients had CMV infection about 4 months after renal transplantation. CMV infection was successfully treated with reduction of immunosuppressants or with antiviral agents.

• Clinical and Experimental Pediatrics
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• v.48 no.5
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• pp.551-556
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• 2005

Wiskott-Aldrich syndrome(WAS) is an X-linked recessive immunodeficiency characterized by thrombocytopenia with small platelet volume, eczema, and recurrent infections, and is also characterized by increased incidence of auto immune diseases and malignancies. The phenotype observed in this syndrome is caused by mutation in the Wiskott-Aldrich syndrome protein(WASP) gene localized to the proximal short arm of the X chromosome and recently isolated through positional cloning. The gene encodes a 502 amino acid protein, which contains 12 exons and spans 9 kb of genomic DNA. The function of the encoded protein is not well understood. The clinical diagnosis of WAS can be difficult and is usually confirmed by the detection of WASP gene mutations and the expression of WSAP in patient blood sample using genetic analysis. We reported a case of a 13-month old boy with WAS who was identified with the novel mutation in exon 2 of WASP gene by direct sequencing and the complete absence of WASP expression by immunoblotting.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.1004-1008
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• 2005

Hemangiomas are the most common benign tumors of infancy. Fifteen to 30% of these patients have multiple hemangiomas. Diffuse neonatal hemangiomatosis (DNH) is a disease that often has a fatal outcome if left untreated, and is characterized by multiple cutaneous and visceral hemangiomas. Corticosteroids are the commonly accepted first line treatment, but if no effect is seen, further treatment is required such as interferon, surgical excision, embolization and radiotherapy. Interferon is effective, but the neurologic sequela including spastic diplegia can be a complication. We experienced a case of DHN in a neonate. In this case, the baby presented with multiple cutaneous and visceral hemangiomas with Kasabach-Merritt syndrome (KMS) that included thrombocytopenia and consumptive coagulophthy. The baby was successfully treated with vincristine after the failure of steroid therapy.

• Clinical and Experimental Pediatrics
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• v.57 no.2
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• pp.96-99
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• 2014

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case.