Stent implantation to relieve secondary pulmonary venous stenosis in total anomalous pulmonary venous connection: case report / 소아과

Pulmonary venous stenosis may be congenital or acquired. Regardless of its origin, the prognosis for patients affected with PVS remains poor. There have been many attempts to palliate PVS with little success. This report describes two patients with PVS which became evident after repair of total anomalous pulmonary venous connection. Intravascular stents were successfully implanted, but progressive restenoses in the stents occurred and eventually both of the patients died. The pertinent literature is reviewed.

Comparison of defect size measured by transthoracic and transesophageal echocardiography with balloon occlusive diameter measured during transcatheter closure of atrial septal defect / 소아과

PURPOSE: Accurate measurement of defect size is important in transcatheter closure of atrial septal defect (ASD). We performed this study to analyze the difference between the measured ASD size and balloon occlusive diameter (BOD) by transthoracic (TTE) or transesophageal echocardiography (TEE). METHODS: We investigated 78 patients who underwent transcatheter closure of ASD. The defect size and the distance between the surrounding structures were measured by TTE and TEE. The BOD was measured by TEE during cardiac catheterization. Clinical characteristics and echocardiographic data were compared and analyzed. RESULTS: The difference between BOD and diameter by TTE was 4.8+/-3.6 mm on short axis view, 5.4+/-3.2 mm on long axis view. The difference between BOD and diameter by TEE was 3.6+/-2.2 mm on short axis view, 4.2+/-3.1 mm on long axis view. The difference between BOD and the diameter of defects on TTE, TEE had statistically significant positive correlations with the age of the patients, distance between the, defect and posterior atrial septal wall, the distance between the defect and the mitral valve leaflet, and the diameter of defects and the length of the atrial septum on TTE (P<0.05). CONCLUSION: BOD of ASD can be estimated by the diameter on TTE and TEE. BOD is expected to measure larger, depending on the size of defects, the distance from surrounding structures and the location of defects on echocardiography. Our data offers important information on details of transcatheter ASD closure which can be helpful in predicting suitability and judging the procedural appropriateness during the procedure.

Late aortic root dilatation and aortic regurgitation in repaired tetralogy of fallot / 소아과

PURPOSE: Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. METHODS: We investigated 130 repaired TOF patients more than 15 years of age who followed-up by echocardiography from January 2002 to December 2003. Of 130 patients, we identified 17 patients with AoRo dilatation, which was defined as ratio of expected AoRo size by standard nomogram (AoRo ratio) >1.5 (dilator group), and 113 TOF controls, with AoRo ratio <1.5 (non-dilator group). RESULTS: Mean indexed AoRo size (mm/m2) in the first echo was 24+/-3.2 in the dilator group and 18+/-3.4 in the non-dilator group (P<0.0001). AoRo rate of change (mm/year) from the first to latest echo study was 1.6+/-3.8 in dilator group and 0.05+/-1.6 in the non dilator group (P=0.0021). Patients from the dilator group showed a higher prevalence of pulmonary atresia (P=0.031) and a history of aortopulmonary shunt before repair (P=0.048), moderate to severe AR (P=0.0065), and increased left ventricular end-diastolic dimensions (P=0.003). Conclusions:A subset of patients late after TOF repair may show progressive dilatation of AoRo. To identify and prevent long-term sequelae in this patient group, regular follow-up and speculation about AoRo after TOF repair is recommended.

Follow-up Studies and Clinical Significance of Chromosomal Rearrangement in Childhood Acute Lymphocytic Leukemia / 소아과

PURPOSE: Through routine screening for chromosomal defects present in patients with acute lymphocytic leukemia(ALL) by means of reverse transcription-polymerase chain reaction(RT-PCR), we aimed for earlier detection of recurrences, hence evaluating the progress of the disease after treatment, and forecasting the need for further testing. METHODS: We analyzed 30 patients who visited the Pediatrics Department of Severance Hospital, from January 2002 to July 2003, in whom pre- and post-chemotherapy(post remission induction, post consolidateion and during maintenance) bone marrow samples were available. Among them, periodic RT-PCR examinations were performed in five bcr/abl positive cases, five TEL/AML1 positive cases, and seven dupMLL positive cases to follow the changes in genetic markers. RESULTS: In patients with bcr/abl, all five cases reached complete remission in hematologic examination after induction chemotherapy, but bcr/abl RT-PCR was positive in one case after the treatment, with complete remission reached in just four patients. In the group with TEL/AML1, all five cases reached both hematologic and molecular complete remission after induction chemotherapy. In seven cases with dupMLL, hematologic complete remission was reached in all patients, except one patient who was six months old at diagnosis, who exhibited positive findings for abnormal precursor after induction chemotherapy. CONCLUSION: Earlier detection of recurrence was possible through hematologic and chromosomal anaylsis of patients during follow-up. The most essential factor to detect recurrence considered the timing of bone marrow biopsy. So the procedure must be performed at critical intervals in a patient's course of treatment. In patients with ALL, recurrences by drug-resistant cells occur primarily after one year from the initiation of treatment, so we propose that bone marrow acquisitions to detect recurrences are recommended at one year after the start of treatment, and just before the discontinuation of treatment.

An Experience of Cord Blood Transfusion in Cold Hemagglutinin Disease Associated with Mycoplasma pneumoniae Infection / 대한진단검사의학회지

We report a case of cold hemagglutinin disease associated with Mycoplasma pneumoniae infection treated with cord blood transfusion. Cold hemagglutinin disease is a hemolytic anemia most commonly associated with cold-reactive autoantibody with anti-I specificity. On the basis of the fact that the level of I antigen on cord red blood cells is extremely low, a six year old male patient was transfused with 60 mL of ABO blood type-matched, cord blood. No complication from the transfusion was observed. Due to the deficiency in cord blood supply, filtered irradiated RBC 100 mL was transfused three times thereafter. The hemoglobin level began to increase from the fifth hospital day. The patient was discharged without additional transfusion on the eleventh hospital day. No remarkable complications were noted at the time of discharge.

A Clinical Study on Childhood Hemolytic Anemia According to Etiological Classification

PURPOSE: The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. METHODS: Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance Hospital were analyzed retrospectively. They were divided into two groups based on the types of red cell defects(group I : erythrocytic defect, group II : extraerythro cytic defect). RESULTS: Twenty one cases were included in group I, thirty four cases in group II, and three cases were unclassified. In group I, nineteen cases(90.5%) were diagnosed as hereditary spherocytosis and were proved to have red cell membrane disorders while two cases(9.5%) were shown to have red cell enzyme deficiencies. In group II, thirteen cases(38.2%) were noted as autoimmune hemolytic anemia, eleven cases(32.4%) as traumatic or microangiopathic hemolytic anemia, four cases(11.8%) as drug induced hemolytic anemia, two cases(5.9%) were related with systemic lupus erythematosus and one case(2.9%) with malignancy. Hemoglobin at the time of diagnosis(7.5 g/dL vs. 6.2 g/dL, P< 0.05) and the incidence of splenomegaly(85.7% vs. 18.2%, P<0.05) were higher in group I though blood urea nitrogen(9.0/0.4 mg/dL vs. 27.8/1.6 mg/dL, P<0.05) was higher in group II. CONCLUSION: Comparing the clinical features of pediatric hemolytic anemia, we concluded as following: In cases associated with extraerythrocytic defect, blood tests revealed significant initial lower hematocrit with higher level of BUN and Cr while cases with erythrocytic defect, splenomegaly were more common noted.

A Case of Membranoproliferative Glomerulonephritis Type II: Dense Deposit Disease, DDD

Type II membranoproliferative glomerulonephritis (Dense deposit disease ) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomrulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago.

A Case of Microscopic Polyangiitis with Pulmonary Hemorrhage and Rapidly Progressive Glomerulonephritis

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN 117 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL.