A Case of Acrodermatitis Enteropathica with Chronic Diarrhea and Disseminated Intravascular Coagulation / 대한소아소화기영양학회지

Acrodermatitis enteropathica, an autosomal recessive disease, usually presents with severe acral and circumorificial dermatitis, diarrhea, alopecia, intercurrent bacterial infection during early infancy, and is eventually fatal if left untreated. We report a case of acrodermatitis enteropathica in a 2-month-old male infant who presented with chronic diarrhea not responsive to conventional therapy and developed disseminated intravascular coagulation (DIC). He showed the characteristic eczematoid skin lesions, chronic diarrhea, failure to thrive, and low serum zinc concenturation. Zn2+ was administered with dramatic improvement of skin lesions, DIC and diarrhea. He rapidly catched up normal growth and development on continuing zinc supplementation.

Assessment of Regional Wall Motion of the Left Ventricle in Children with Congenital Heart Disease

PURPOSE: To assess wall motion of the left ventricle in the various forms of congenital heart disease and to select appropriate methods by which to gauge left ventricular wall motion. METHODS: We evaluated left ventricular function, including volume, global ejection fraction, and regional wall motion(Centerline method, Local Ejection Fraction, Curved Perimeter method and Radial method). The evaluation was done on review mode of 30-degree elongated right anterior oblique projection of left ventriculogram by using cardiac analysis program of the DXC Hiline Digital System(GER, USA) in 53 pediatric patients undergoing diagnostic(or therapeutic) cardiac catheterization from June 1995 to July 1996 at Kyung-pook National University Hospital. RESULTS: Cardiac indices calculated by Dodge's and Simpson's methods were 5.76+/-2.13 L/min/ m2, 5.86+/-2.31 L/min/m2 respectively with significant correlation to each other(r=0.99, P<0.01). Global ejection fractions using both methods were 69.81+/-8.33%, and 69.69+/-8.20%, respectively (r=0.99, P<0.01). After dividing the left ventricular outline contour into five regions such as posterobasal, diaphragmatic, apical, anterolateral and anterobasal portions, all four models could ascertain regional wall motion abnormalities well(P<0.01). And among them, the Centerline method and the Curved Perimeter method correlated well in assessing wall motion(for each portion r=0.67, 0.84, 0.94, 0.91 and 0.61 respectively, P<0.01). CONCLUSION: In conclusion, we confirmed regional wall motion abnormalities in the various forms of congenital heart disease in spite of normal global ejection fractions, showing that the Centerline and Curved Perimeter methods are preferable methods in the assessment of wall motion in patiens with congenital heart disease.

A Case of Childhood Essential Thrombocythemia / 대한소아혈액종양학회지

Essential thrombocythemia in childhood is a rare clonal myeloproliferative disorder in the multipotent stem cell origin and is associated with an increased risk of thrombohemorrhagic complications. The one of diagnostic criteria is a platelet count of more than 600,000/mm3. We diagnosed this disease in 8 year old boy incidentally and treated with hydroxyurea. We report a case of essential thrombocythemia to summarize the current trends in the diagnosis and management with a brief review of related literatures.

A Case of Alloimmune Neonatal Neutropenia

Alloimmune neonatal neutropenia (ANN) is due to maternal IgG neutrophil-specific antibodies that cross the placenta to sensitize fetal neutrophils and cause neutropenia in the neonate. Infection appears within a few days of life. Neutropenia worsens during the very first days of life and persists until 20-50 days in spite of the disappearance of detectable antibody in the infant's serum. We report a case of ANN in female neonate who was admitted on the 5th day of life due to poor oral intake. The diagnosis was made by indirect immunofluorescence with fluorescein-tagged antihuman globulin reagent. The patient was treated successfully with systematic intravenous antibiotics and highdose intravenous immunoglobulin (IVIG).