Pathology and Renal Outcome of IgA Nephropathy / 대한내과학회지

BACKGROUND/AIMS: The Oxford classification of immunoglobulin A nephropathy (IgAN) is a pathology-based prognostic classification system. However, further study is needed to determine its validity. We studied the relationships between the Oxford classification and established prognostic factors and renal survival. We also examined associations between electron microscopy findings and these parameters. METHODS: We reviewed and reclassified 213 patients who were diagnosed with IgAN from 1997 to 2007 using the Oxford and World Health Organization (WHO) classification systems. The patients were also categorized by a pathologist using electron microscopy findings, including foot process fusion, glomerular basement membrane thickness, and electron-dense deposits. We examined the correlations between light and electron microscopy data and known prognostic factors (e.g., age, sex, proteinuria, serum creatinine, estimated glomerular filtration rate [eGFR], and blood pressure). The same procedure was applied to renal survival. RESULTS: Patient age increased with the grades of segmental sclerosis (S) and tubular atrophy/interstitial fibrosis (T) (P < 0.05). eGFR decreased significantly with increasing mesangial hypercellularity (M) (p = 0.0034), S (p = 0.0003), endocapillary hypercellularity (E) (p = 0.0411), and T (P < 0.0001). MSET differed significantly by sex (P < 0.0001). The 24-h urine protein/creatinine ratio increased significantly with the degrees of S (p = 0.036), E (p = 0.0155), and T (p = 0.015). The serum creatinine level was significantly higher in patients with T2 than T1 or T0 (P < 0.0001). At the time of biopsy, the degree of tubular atrophy/interstitial fibrosis affected the doubling of serum creatinine or end-stage renal disease. However, the electron microscopy findings did not predict the renal outcome. CONCLUSIONS: Our study suggests that tubular atrophy/interstitial fibrosis is significantly associated with proteinuria and renal progression in IgAN.

Supraclavicular BCG Lymphadenitis Noted at 21 Months after BCG Vaccination Confirmed by a Molecular Method / 소아감염

Bacille Calmette-Guerin (BCG) lymphadenitis is the most common complication of BCG vaccination. It commonly occurs in infants aged <6 months involving ipsilateral axillary lymph nodes. We described BCG lymphadenitis in a 22-month-old boy presenting swelling of left supraclavicular lymph node that was confirmed by real-time polymerase chain reaction (PCR) and the multiplex PCR targeting the region of difference (RD).

Inhaled iloprost for the treatment of patient with Fontan circulation / 소아과

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

An Analysis of Neutropenia after the Administration of High-dose Intravenous Immunoglobulin or Anti-D Immunoglobulin on Acute Immune Thrombocytopenic Purpura Children: Age based Analysis / 임상소아혈액종양

BACKGROUND: The purpose of this study is to evaluate the decreased neutrophil count after the administration of high-dose intravenous immunoglobulin (IVIG) or anti-D immunoglobulin (anti-D Ig) to children with acute immune thrombocytopenic purpura (ITP). In addition, post-treatment changes in absolute neutrophil count (ANC) were also analyzed according to patient age.METHODS: We retrospectively performed a chart review of 83 patients who had received IVIG or anti-D Ig to manage the decreased platelet count due to acute ITP. Pre- and post-treatment ANC were analyzed in the IVIG treatment group and anti-D Ig treatment group, and ANC were compared between each group. In addition, all the subjects were divided into two groups according to age: infantile group and other group, and ANC of these two groups were compared.RESULTS: A decrease in ANC in just a day and recovering to its pre-treatment value on the 7th post-treatment day was observed for the IVIG treatment group. Especially, the decrease in ANC observed in the infantile group was found to recover in 14 days, where-as ANC recovery in the other group was seen within 2 days after the treatment. There was no statistically significant decrease in ANC in the anti-D Ig treatment group.CONCLUSION: IVIG used for childhood acute ITP caused a decrease in ANC more severely than with the administration of anti-D Ig. Subjects aged less than 12 months who were treated with IVIG for acute ITP showed more ANC decrease and needed longer time to recover normal ANC compared to those over 12 months of age.

An Analysis of Neutropenia after the Administration of High-dose Intravenous Immunoglobulin or Anti-D Immunoglobulin on Acute Immune Thrombocytopenic Purpura Children: Age based Analysis / 임상소아혈액종양

BACKGROUND: The purpose of this study is to evaluate the decreased neutrophil count after the administration of high-dose intravenous immunoglobulin (IVIG) or anti-D immunoglobulin (anti-D Ig) to children with acute immune thrombocytopenic purpura (ITP). In addition, post-treatment changes in absolute neutrophil count (ANC) were also analyzed according to patient age. METHODS: We retrospectively performed a chart review of 83 patients who had received IVIG or anti-D Ig to manage the decreased platelet count due to acute ITP. Pre- and post-treatment ANC were analyzed in the IVIG treatment group and anti-D Ig treatment group, and ANC were compared between each group. In addition, all the subjects were divided into two groups according to age: infantile group and other group, and ANC of these two groups were compared. RESULTS: A decrease in ANC in just a day and recovering to its pre-treatment value on the 7th post-treatment day was observed for the IVIG treatment group. Especially, the decrease in ANC observed in the infantile group was found to recover in 14 days, where-as ANC recovery in the other group was seen within 2 days after the treatment. There was no statistically significant decrease in ANC in the anti-D Ig treatment group. CONCLUSION: IVIG used for childhood acute ITP caused a decrease in ANC more severely than with the administration of anti-D Ig. Subjects aged less than 12 months who were treated with IVIG for acute ITP showed more ANC decrease and needed longer time to recover normal ANC compared to those over 12 months of age.

Using the High Frequency Ventilation during Neonatal Transport

PURPOSE: To present and evaluate a system of high-frequency oscillatory ventilator (HFOV) during intra-/inter-hospital neonate transport. METHODS: The system includes a charged HFOV (SOPHIE, Fritz Stephan GmbH, Dusseldorf, Germany), an incubator, and E-oxygen/air-cylinders with connections to the HFOV. The test lung was evaluated at the high and medium ventilator settings used for infants to determine the operating time of HFOV. The time required to exhaust the gas supply was checked, and the HFOV was operated until the low-battery alarm sounded to determine the operating time of the batteries. RESULTS: The batteries provided electrical power for at least 60 mins, and the oxygen and air-cylinders lasted at least 20 mins. The system has been used frequently for the intra-hospital transport, from delivery rooms to ICU and from ICU for surgery. The system has been used twice for the inter-hospital transport of infants with bronchopulmonary dysplasia and pulmonary hypertension to another hospital 45 km away (one hour distance). In one case, the ambulance's electrical power supply failed, causing the system failure during the last 5 mins of transport. However, with the complete check and simulation of the system and the ambulance bulk oxygen/electric supply, the second patient was transported successfully in stable condition. CONCLUSION: The system was useful for intra-/inter-hospital transport of the neonates on HFOV. For the transport time of 60 mins, fully charged HFOV, 2 E-oxygen-cylinders, and 3 E-air-cylinders seemed to be sufficient. H-oxygen-cylinder and ambulance electrical power supply should also be provided for safe and efficient transport between hospitals.