PURPOSE:Primary immunodeficiency diseases are disorders in which part of the body's immune system is missing or does not function properly due to intrinsic defects in the immune system. These patients have an increased susceptibility to pulmonary complications as well as primary infections. This study was to investigate pulmonary complications in patients with primary immunodeficiency diseases and characterize their patterns according to specific immune defects. METHODS:We retrospectively reviewed the medical records of 37 different cases of primary immunodeficiency diseases that were admitted to Severance hospital in Seoul between 1990 and 2006. RESULTS:Many patients had a previous history of recurrent respiratory infections and some suffered from pulmonary complications. Eight patients with hypogammaglobulinemia showed peribronchial wall thickening or bronchiectasis, and pneumatoceles or emphysematous changes were characteristic complications in two patients with hyper IgE syndrome. On microbiological examination, various bacteria, including Staphylococcus aureus, Pseudomonas aeroginosa, Streptococcus pneumoniae, were isolated from the hypogammaglobulinemia patients' sputum or ear discharge. In some patients with cell-mediated immune defects, such as chronic granulomatous disease or severe combined immune deficiency, yeast and ameba were isolated from the sputum and bronchial washing fluid respectively. CONCLUSION:Infections account for most of these complications, but the host reaction to infection seems to cause characteristic findings that could be helpful for diagnosis. The physician should be alert for the early diagnosis of children with primary immunodeficiency in order to prevent pulmonary complications.
Agammaglobulinemia ; Amoeba ; Bacteria ; Bronchiectasis ; Child ; Diagnosis ; Ear ; Early Diagnosis ; Granulomatous Disease, Chronic ; Humans ; Immune System ; Immunologic Deficiency Syndromes ; Job Syndrome ; Lung Diseases ; Medical Records ; Pseudomonas ; Respiratory Tract Infections ; Retrospective Studies ; Seoul ; Sputum ; Staphylococcus aureus ; Streptococcus pneumoniae ; Yeasts

PURPOSE:Primary immunodeficiency diseases are disorders in which part of the body's immune system is missing or does not function properly due to intrinsic defects in the immune system. These patients have an increased susceptibility to pulmonary complications as well as primary infections. This study was to investigate pulmonary complications in patients with primary immunodeficiency diseases and characterize their patterns according to specific immune defects. METHODS:We retrospectively reviewed the medical records of 37 different cases of primary immunodeficiency diseases that were admitted to Severance hospital in Seoul between 1990 and 2006. RESULTS:Many patients had a previous history of recurrent respiratory infections and some suffered from pulmonary complications. Eight patients with hypogammaglobulinemia showed peribronchial wall thickening or bronchiectasis, and pneumatoceles or emphysematous changes were characteristic complications in two patients with hyper IgE syndrome. On microbiological examination, various bacteria, including Staphylococcus aureus, Pseudomonas aeroginosa, Streptococcus pneumoniae, were isolated from the hypogammaglobulinemia patients' sputum or ear discharge. In some patients with cell-mediated immune defects, such as chronic granulomatous disease or severe combined immune deficiency, yeast and ameba were isolated from the sputum and bronchial washing fluid respectively. CONCLUSION:Infections account for most of these complications, but the host reaction to infection seems to cause characteristic findings that could be helpful for diagnosis. The physician should be alert for the early diagnosis of children with primary immunodeficiency in order to prevent pulmonary complications.
Agammaglobulinemia ; Amoeba ; Bacteria ; Bronchiectasis ; Child ; Diagnosis ; Ear ; Early Diagnosis ; Granulomatous Disease, Chronic ; Humans ; Immune System ; Immunologic Deficiency Syndromes ; Job Syndrome ; Lung Diseases ; Medical Records ; Pseudomonas ; Respiratory Tract Infections ; Retrospective Studies ; Seoul ; Sputum ; Staphylococcus aureus ; Streptococcus pneumoniae ; Yeasts

PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. METHODS: We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. RESULTS: The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. CONCLUSION: This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.
Bone Marrow ; Epstein-Barr Virus Infections ; Etoposide ; Female ; Ferritins ; Fever ; Follow-Up Studies ; Humans ; Lymphohistiocytosis, Hemophagocytic ; Male ; Multivariate Analysis ; Neurologic Manifestations ; Pancytopenia ; Prognosis ; Retrospective Studies ; Splenomegaly ; Survival Rate

PURPOSE: Due to its rapidity and easy accessibility, the fingerstick blood glucometer has been used in almost all hospitals and private clinics, and even by patients themselves. We also have used it even in shock patient care, but shock shows global tissue hypoperfusion, especially in peripheral tissue. The changes of peripheral circulation have an influence on the results for fingerstick glucose. To evaluate the accuracy of the glucometer for patients with poor peripheral perfusion, we designed this study. METHODS: A prospective, nonrandomized comparison group study was done. A hypotensive group and a normotensive group were compared. We obtained three data from each patient: venous blood glucose level (clinicopathologic laboratory), venous blood glucose level (by glucometer) and fingerstick glucose level (by glucometer). RESULTS: We saw a significant difference between the fingerstick glucometer results and the laboratory glucose levels in hypotensive patients: 131.67+/-55.33 mg/dl vs. 1 4 7 . 2 3+/-62.06 mg/dl (paired t-test, p<0.05). There was no significant difference between fingerstick and laboratory glucose in normotensive patients: 101.75+/-20.14 mg/dl vs. 1 0 5 . 6 0+/-21.95 mg/dl (paired t-test, p>0.05). There was no significant difference between the results of venous glucometer and laboratory test in either group: 142.37+/-61.27 mg/dl vs. 147.23+/-62.06 mg/dl (paired t-test, p>0.05) and 102.98+/-17.02 mg/dl vs. 105.60+/-21.95 mg/dl (paired t-test, p>0.05). Although some statistical differences existed between the results, all of the error rates were in an acceptable range (within 15%, accepted by American Diabetes Association consensus). CONCLUSION: These results suggest that the blood glucose level of the glucometer with venous blood is more accurate than that with peripheral blood in patients with poor peripheral circulation.
Blood Glucose* ; Glucose ; Humans ; Patient Care ; Perfusion ; Prospective Studies ; Shock

PURPOSE: GFR(glomerular filtration rate) is a fundamental parameter in detecting renal impairment and predicts the progression of renal disease. Because serum creatinine has several disadvantages, serum cystatin C has been recently proposed as a new endogenous marker for GFR. We compared serum cystatin C with creatinine and creatinine clearance to investigate the clinical usefulness of cystatin C. METHODS: We retrospectively analyzed 46 patients(60 case numbers) who had various renal diseases and classified them into 3 groups according to creatinine clearance(Group 1 : CrCl <40 mL/min/1.73m2, Group 2 : CrCl 40-60 mL/min/1.73m2, Group 3 : CrCl >60 mL/min/1.73 m2). We measured serum creatinine, cystatin C and creatinine clearance and also analyzed the correlations among them. RESULTS: Serum cystatin C and creatinine showed a similar correlation to creatinine clearance (r=0.685, r=0.640, respectively) and showed similar diagnostic accuracy in detecting decreased GFR(AUC, cystatin C 0.829 vs. creatinine 0.826, P=0.848). Serum cystatin C showed a greater sensitivity for detecting a decreased GFR than creatinine in Group 2 and 3(Group 1 : 100% vs. 100%, Group 2 : 70% vs. 35%, Group 3 : 46% vs. 15%). CONCLUSIONS: Serum cystatin C could be a useful endogenous marker for GFR and would be superior to serum creatinine in early detection of renal impairment in pediatric patients with renal diseases.
Child* ; Creatinine ; Cystatin C* ; Filtration ; Glomerular Filtration Rate ; Humans ; Retrospective Studies