Effects of coagulation factor concentrate prophylaxis in moderate and severe hemophilia A patients at a single hemophilia center in Korea / 소아과

PURPOSE: The aim of this study was to investigate prophylactic treatment effects in Korean patients with severe hemophilia A. METHODS: A prospective study of 32 severe hemophilia A patients was conducted with the approval of the Institutional Review Board at the Eulji University Hospital. Two patients received primary prophylaxis; whereas, the other 30 patients were divided into 2 groups-secondary prophylaxis (n=15) and on-demand (n=15)-on the basis of their consent for secondary prophylaxis. A 20-25 IU/kg dose of factor VIII concentrate was administered to the primary and secondary prophylaxis group patients every 3 days for 1 year. The prophylactic effect was evaluated by observing changes in the Pettersson scores, annual number of total and joint bleeds, and factor VIII consumption for 1 year. RESULTS: No moderate or severe bleeding was observed, and the Pettersson scores remained unchanged during the prophylaxis period in the patients who received primary prophylactic treatment. After the treatment was changed from on-demand to secondary prophylaxis, the annual number of total and joint bleeds in the secondary prophylaxis group decreased by 64.4%+/-13.0% and 70.0%+/-15.2%, respectively. The average increase in Pettersson scores within 1 year was 0.5+/-0.8 and 1.3+/-1.1 in the secondary prophylaxis and on-demand groups, respectively. Prophylactic effects were also observed in patients >17 years who had nearly the same initial Pettersson scores. CONCLUSION: Intermediate-dose prophylactic treatment may delay hemarthropathy progression and prevent its occurrence in Korean severe hemophilia A patients.

Platelet Dysfunction in a Patient with Multiple Myeloma: A Case Report with a Literature Review / 대한내과학회지

Multiple myeloma is a monoclonal plasma cell proliferation disorder with various symptoms and signs caused by paraproteinemias. Among these signs, a bleeding tendency is one of the major fatal causes. However, significant severe bleeding is rare in most cases. In this study, we report a case of multiple myeloma in a patient who had a severe recurrent bleeding tendency due to platelet dysfunction caused by paraproteins. After being treated with therapeutic plasma exchange and chemotherapy, the patient's monoclonal protein level decreased and the bleeding stopped.

A Case of Serotonin Syndrome Due to Amantadine and Escitalopram in End-Stage Renal Disease / 대한내과학회지

Serotonin syndrome is a potentially life-threatening adverse drug reaction caused by excessive serotonergic activity in the nervous system. It is characterized by a triad of mental status changes, autonomic hyperactivity, and neuromuscular abnormalities. Escitalopram is a selective serotonin reuptake inhibitor. Amantadine, an anti-influenza agent, is commonly used for the treatment of parkinsonism; it also has serotonergic activity. Amantadine can induce toxicity in patients with renal dysfunction because it is excreted mainly in the urine. We report a rare case of serotonin syndrome in a 73-year-old woman with Parkinson's disease, depression, and end-stage renal disease undergoing maintenance hemodialysis. She presented with confusion, myoclonus, and fever after starting escitalopram for her depression while on amantadine for parkinsonism. Based on this case, amantadine as well as escitalopram should be placed on the list of medications that can precipitate serotonin syndrome. The side effects of these drugs should be monitored, especially in end-stage renal disease.

The Effectiveness of High Dose Dexamethasone for the Initial Treatment of Childhood Acute Idiopathic Thrombocytopenic Purpura

BACKGROUND: Corticosteroids and intravenous immunoglobulin (IV-Ig) have been used asfirst line treatments for acute idiopathic thrombocytopenic purpura (AITP) in children. High dose dexamethasone (HD) has been reported to be effective for chronic refractory ITP and for the initial treatment of AITP in adults. There has been no report about HD as the initial treatment for childhood AITP. We assessed the effectiveness of HD for the initial treatment of childhood AITP, as compared to IV-Ig. METHODS: 25 Patients with newly diagnosed AITP were enrolled. We conducted a prospective, randomized study to compare the two treatment options. 11 patients were treated with IV-Ig and 14 patients were treated with HD. The platelet counts were assessed at 3, 5, 7, 14 and 21 days after the beginning of the treatment. The adverse effects were noted, and the patients were followed for more than 6 months. RESULTS: Both the IV-Ig and HD groups showed a rapid rise of the platelet counts and the platelet counts were maintained at 3, 5, 7, 14 and 21 days. The difference of platelet counts between the two groups was significant at day 5 (P<0.05). During the follow-up period, 5 patients had a recurrence: 2 in IV-Ig group and 3 in HD group. All 5 patients were re-treated with HD and they had a good response. One of the recurred patients in the IV-Ig group had chronic ITP. Some side effects were observed, but they were not severe enough to necessitate the discontinuation of treatment. CONCLUSION: We conclude that HD is as effective and safe as high dose IV-Ig for the initial treatment of childhood AITP. The choice between these treatment options can be made according to the cost-effectiveness and the therapy-related risks.

Effects of ezetimibe on lipid profiles and hemostatic markers in end-stage renal disease / 대한내과학회지

BACKGROUND/AIMS: Dyslipidemia is one of the major causes of cardiovascular disease in end-stage renal disease (ESRD) patients. Most of them are dyslipidemic despite the use of lipid-lowering agents. Ezetimibe is a novel chemical entity that inhibits the intestinal absorption of dietary and biliary cholesterol. This study evaluated the effects of ezetimibe on the lipid profile, inflammation markers, endothelial injury, and thrombogenesis in ESRD patients. METHODS: Sixty-five patients with serum low-density lipoprotein (LDL)-cholesterol levels > or =100 mg/d were recruited: 33 patients were on hemodialysis and 32 patients were on peritoneal dialysis. They were assigned randomly to the ezetimibe (10 mg) monotherapy group and the ezetimibe (10 mg) plus simvastatin (10 mg) combination therapy group. Both drugs were administered for 8 weeks. RESULTS: There were no significant differences in the baseline demographic and laboratory characteristics between the two groups. In the monotherapy group, the total and LDL-cholesterol levels were reduced by 14.7 and 21.9%, respectively. There were no changes in the high-density lipoprotein (HDL)-cholesterol or triglyceride levels. Fibrinogen increased significantly (p=0.04). In the combination therapy group, the total and LDL-cholesterol levels were reduced by 29.8 and 42.4%, respectively. There was an additional 15.1% reduction in total cholesterol and an additional 20.5% reduction in LDL cholesterol compared with monotherapy. Several patients complained of minor adverse effects and only one patient in the ezetimibe monotherapy group discontinued medication, because of diarrhea. CONCLUSIONS: In ESRD patients, ezetimibe used as combination therapy with a statin is more effective than ezetimibe monotherapy in ESRD patients.

Clinical factors causing hyponatremia in patients with mucocutaneous lymph node syndrome / 소아과

PURPOSE: Clinical and laboratory findings predict a severe outcome for mucocutaneous lymph node syndrome. This study aimed to define the clinical characteristics of Kawasaki disease (KD) patients with hyponatremia and to determine the factors associated with its development. METHODS: Retrospective studies were performed on 114 KD patients who received an initial high-dose intravenous immunoglobulin (IVIG, single 2 g/kg/dose) within 10 days of fever onset from January 2006 to February 2008. These patients were divided into 2 groups. Group 1 consisted of 30 (26.3%) patients with hyponatremia, and group 2 consisted of 84 (73.6%) patients without hyponatremia. Clinical manifestations, laboratory results, and echocardiographic findings were compared between the groups. RESULTS: Group 1 patients were more likely to have a coronary artery lesion (53.3% versus 20.2%, P=0.005) and suffered from diarrhea (41.3% versus 14.1%, P=0.007). There was a higher incidence of cardiovascular involvement in group 1 patients, including coronary dilatation (46.6%), valvular regurgitation (13.3%), pericardial effusion (6.7%) and medium-sized aneurysm (6.7%). There were no coronary aneurysms in group 2 patients. Serum C-reactive protein (CRP) was significantly higher in patients with hyponatremia (12.2+/-7.79 mg/ dL versus 7.3+/-4.7 mg/dL, P=0.003) and IVIG-resistant patients were more common in group 1 (13.3% versus 3.6%). CONCLUSION: These results indicate that hyponatremia in KD occurs in patients exhibiting severe inflammation and was significantly associated with the development of coronary disease. Further studies will be necessary to confirm the pathogenic mechanisms of hyponatremia in KD patients.

A Clinical Study of Febrile Convulsions in Children Over 5 Year Olds without Organic Problems / 대한소아신경학회지

PURPOSE: Febrile convulsions are the most common form of childhood seizures, appearing between in children 3 months and 5 years of age and are predominantly of benign nature. Febrile convulsions occurring after 5 years of age might be estimated as epilepsy. Most data for these disorders are from mainly epilepsy populations and might not be relevant to these children, and the requirement of any specific treatment or workup is not yet established either. Thus we tried to obtain a unique perspective. METHODS: Clinical data for episodes of febrile convulsions were collected in Eulji hospital in Deajeon. These were obtained from anonymised copies of a standardised admission note, emergency, nursing, and intensive care unit notes and interviews with parents, medical, nursing, and paramedic staff. RESULTS: Finding of 259 episodes of febrile seizure in over 5 year olds. 121(46.7%) were confirmed epilepsy or afebrile seizure in under 5 year olds, 13(5.0%) were caused by infection, metabolic disorder, leukemia and so on, and 23(8.9%) had previous neurological defect. Lastly, 102(39.4%) were included to febrile convulsion in over 5 year olds. 28(27.5%) of the subjects had their initial febrile convulsions in children over 5 year olds while 74(72.5%) of the subjects were under 5 years of age. The male to female ratio of the seizure group was 2:1. In the duration and type of convulsions, generalized convulsions occurred predominantly within 15mins in both of them. 5(17.9%) of group A and 16(21.6%) of group B showed electroencephalogram abnormality. The most common causes of seizure were common colds. Most of the convulsions occurred less than 24hrs after fever but, there were no abnormal findings in lumbar puncture and brain MRI. CONCLUSION: Febrile seizure in over 5 year olds without organic problems has similar characteristics to febrile convulsion in under 5 year olds. In light of these findings, lumbar puncture and brain MRI do not require a routine checkup for the evaluation of febrile seizure in over 5 year olds. However, encephalography needs to be examined.

A Case of Neonatal Alloimmune Thrombocytopenia due to Anti-HLA-B35

Neonatal alloimmune thrombocytopenia (NAIT) is induced by maternal antibodies to fetal platelet alloantigens. Because the main cause of NAIT is incompatibility to platelet specific antibodies, NAIT due to HLA antibodies are relatively rare. We managed a case of NAIT induced by maternal anti-HLA-B35 antibodies. The patient was a second born male. He had no petechiae or purpura at birth. He was admitted to the hospital due to fever for 5 days and a platelet count of 106x10(9)/L. The fever subsided after admission but on the 2nd day of admission, petechiae developed on the chest wall and the platelet count decreased to 25x10(9)/L. Other laboratory findings included C-reactive protein, prothrombin time, and partial thromboplastin time were normal. His mother's platelet count was normal and she had no history of bleeding. Anti-HLA-B35, B52, B56, C3, and C14 were identified in the mother's serum by a panel reactive antibody test and HLA-B35 antigen was identified in the father's and patient's sera. These finding suggested that maternal Anti-HLA-B35 antibody was a response to neonatal HLA-B35 antigen inherited from the father. The patient received concentrated platelet and intravenous immunoglobulin. The platelet count rose to 248x10(9)/L and was maintained thereafter.

A Case of Sarcoidosis with Pancytopenia as Resulting from Bone Marrow Involvement / 결핵및호흡기질환

Sarcoidosis is a granulomatous disease that can involve any organ, although it primarily involves the lungs, intrathoracic lymph nodes, skin, and eyes. We present a case of sarcoidosis with pancytopenia, resulting from bone marrow involvement. A 35-year-old man was admitted to hospital for chronic cough and blurred vision. On chest computed tomography, there were multiple pulmonary nodules and mediastinal lymph nodes enlargement. As the patient also showed pancytopenia, we performed a bone marrow biopsy, as well as a transbronchial lung biopsy. Both biopsies showed non-caseating granulomas. We diagnosed the patient with sarcoidosis with pulmonary, bone marrow, uvea, liver and spleen involvement. After oral steroid therapy, the patient's symptoms as well as his pancytopenia improved. We present this case to demonstrate the significance of bone marrow biopsy in cases of sarcoidosis with pancytopenia, as well the possibility of clinical improvement with steroid treatment.

Reticulocyte hemoglobin content for the diagnosis of iron deficiency in young children with acute infection / 소아과

PURPOSE: Early identification of iron deficiency in young children is essential to prevent damaging long-term consequences. It is often difficult for the pediatrician to know which indices should be used when diagnosing these conditions especially in hospitalized young children. This study investigated the clinical significances of reticulocyte hemoglobin content in young children with acute infection. METHODS: We studied 69 young children aged from 6 to 24 months admitted with acute infection in a single center. Venous blood was drawn to determine hemoglobin (Hb), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), hemoglobin content (CH), reticulocyte hemoglobin content (CHr), and red blood cell distribution width (RDW) using ADVIA 120 (Bayer Diagnostics, NY, USA). For evaluating iron status, iron, total iron binding capacity, ferritin and transferrin saturation (Tfsat) were determined. Iron deficiency was defined as Tfsat less than 20%, and iron deficiency anemia as Tfsat less than 20% and Hb level less than 11 g/dL. RESULTS: In all, 47 were iron deficient; 17 of these had iron deficiency anemia. CHr was the only significant predictor of iron deficiency (likelihood ratio test=71.25; odds ratio=0.67; P<0.05). Plasma ferritin level had no predictive value (P=0.519). Subjects with CHr less than 27.4 pg had lower Hb level, MCH, CH, Tfsat, and iron levels than those with CHr 27.4 pg or more (P<0.05 for all). CONCLUSION: CHr level was a sensitive screening tool and the strongest predictor of iron deficiency in hospitalized infants with acute infection; it was cost saving and avoiding additional sampling. However its reference range should be established.

Effect of Cooling & Lidocaine on Propofol Induced Vascular Pain / 대한마취과학회지

Propofol containing solvents such as soybean oil, egg phosphatide, glycerol, is known to arouse pain on intravenous injection in most patients. The study was undertaken to evaluate the efficacy of the two methods in preventing pain during injection of propofol on induction of anesthesia. Patients were allocated randomly into three groups. The one to receive unmodified propofol as control, the other to have injection of propofol with lidocaine added O.l mg/kg and another to have administration of propofol cooled in 0-4 degrees C. The results showed that propofol with lidocaine mixed and propofol cooled in 0-4 degrees C significantly lowered the incidence of pain during the intravenous injection of the drug and that addition of lidocaine and cooling had no untoward effect on the cardiovascular system.