Masaru Takamizawa

Failure of pre-diarrheal antibiotics to prevent hemolytic uremic syndrome in serologically proven Escherichia coli O157:H7 gastrointestinal infection

A girl had hemolytic uremic syndrome after Escherichia coli O157:H7 infection, despite pre-diarrheal administration of an antibiotic that prevented detectable intestinal colonization. This report casts doubt on the advisability of antibiotic therapy for E coli O157:H7 infections and has implications for our understanding of the mechanism of this disorder.

Long-Term Survival of a Baby with Body Stalk Anomaly: Report of a Case

Body stalk anomaly is characterized by severe scoliosis, severe pulmonary hypoplasia, and giant omphalocele. The prognosis of the disease is poor and most obstetricians consider it fatal. Very few patients with body stalk anomaly survive. We report the case of a baby diagnosed with body stalk anomaly in fetal life, who was saved by intensive care after birth. We closed the giant omphalocele successfully by placing karaya gum sheets over it, which created a humidified environment and promoted natural skin epithelization over the skin defect.

Survival in a Neonate with Complete Urorectal Septum Malformation Sequence after Fetal Vesico-Amniotic Shunting for a Prominently Dilated Cloaca

Complete urorectal septum malformation sequence (URSMS) is usually a lethal anomaly that is characterized by urethral obstruction, imperforate anus, ambiguous genitalia, renal agenesis or dysplasia, and mullerian duct maldevelopment. This anomaly is thought to be caused by the cessation of urorectal septum migration toward the caudal cloacal membrane. Teratogenic factors or a genetic abnormality is postulated as the etiology. To date, only 4 patients with URSMS have survived the neonatal period; however, 2 of these infants died before the age of 1 year. We report the survival in a case with complete URSMS who had moderate pulmonary hypoplasia and preserved left renal function. The cloacal remnant wasdilated more than expected because the wall of the muscle layer was torn, perhaps in early fetal life, and timely placement of vesico-amniotic shunts prevented severe pulmonary hypoplasia caused by oligohydramnios.

Elevated production of interleukin-4 and interleukin-5 by T cells in a child with idiopathic hypereosinophilic syndrome

T h e id iopa th i c hype reos inoph i l i c synd rome ( I H E S ) is cha r ac t e r i z ed by pe r s i s t en t eos inoph i l i a of m o r e than 1500/mm 3 and a var ie ty of r e su l t an t o rgan damage , and these p a r a m e t e r s a l low classif icat ion of pa t i en t s in to four cl inical ca tegor ies . This s y n d r o m e is ex t r eme ly r a re in chi ldren . The mos t c o m m o n immuno log ic a b n o r m a l i t y in pa t ients with I H E S is an e l eva ted se rum immunog lobul in level ( IgG, IgM, and /o r IgE) . However , no cons i s ten t abno rma l i t y has b e e n d e t e c t e d in T-cel l funct ion . 1 W e descr ibe a child with I H E S whose T cells p r o d u c e d e l eva ted levels of in ter l euk in ( IL) -4 and IL-5.

Fever pattern and C-reactive protein predict response to rescue therapy in Kawasaki disease

Evidence to guide rescue therapy in refractory Kawasaki disease (KD) is lacking. The aim of this study was to determine the most important variables in predicting non‐response to rescue therapy in refractory KD.

Novel Risk Assessment Tool for Immunoglobulin Resistance in Kawasaki Disease: Application Using a Random Forest Classifier

Background: Resistance to intravenous immunoglobulin (IVIG) therapy is a risk factor for coronary lesions in patients with Kawasaki disease (KD). Risk-adjusted initial therapy may improve coronary outcome in KD, but identification of high risk patients remains a challenge. This study aimed to develop a new risk assessment tool for IVIG resistance using advanced statistical techniques. Methods: Data were retrospectively collected from KD patients receiving IVIG therapy, including demographic characteristics, signs and symptoms of KD and laboratory results. A random forest (RF) classifier, a tree-based machine learning technique, was applied to these data. The correlation between each variable and risk of IVIG resistance was estimated. Results: Data were obtained from 767 patients with KD, including 170 (22.1%) who were refractory to initial IVIG therapy. The predictive tool based on the RF algorithm had an area under the receiver operating characteristic curve of 0.916, a sensitivity of 79.7% and a specificity of 87.3%. Its misclassification rate in the general patient population was estimated to be 15.5%. RF also identified markers related to IVIG resistance such as abnormal liver markers and percentage neutrophils, displaying relationships between these markers and predicted risk. Conclusions: The RF classifier reliably identified KD patients at high risk for IVIG resistance, presenting clinical markers relevant to treatment failure. Evaluation in other patient populations is required to determine whether this risk assessment tool relying on RF has clinical value.

Fever pattern and CRP predict response to rescue therapy in Kawasaki disease

Evidence to guide rescue therapy in refractory Kawasaki disease (KD) is lacking. The aim of this study was to determine the most important variables in predicting non‐response to rescue therapy in refractory KD.

Hemoglobin Seattle detection based on low capillary oxygen saturation: First reported case in Japan

Peripheral capillary oxygen saturation (SpO2), or pulse oximetry, is commonly used in modern medical practice as a basic vital index of health. Although low SpO2 is generally caused by pulmonary, cardiac, or vascular disease, hemoglobin (Hb) abnormalities can also cause reduced pulse oximetry. Hb alterations, however, are sometimes overlooked as the cause of low SpO2, especially when they are congenital. Hemoglobin Seattle (Hb-Seattle) is an inheritable hemoglobinopathy that involves the substitution of aspartic acid for alanine at codon 70 of the b-globin chain, a side chain of which is located in the heme contact region of the protein. Because mutations in this region could affect molecular stabilization and oxygen affinity, patients with this variant have hemolytic chronic anemia and hypoxia symptoms. Since it was first identified in 1970, Hb-Seattle has been reported in three families, with a total of nine individuals reported to date (Table S1). We herein describe the first reported case of Hb-Seattle in Japan, the diagnosis of which was prompted by low SpO2. A 13-year-old Japanese girl was referred to Saitama Citizens Medical Center because of normocytic anemia and low SpO2. She was incidentally diagnosed with anemia at the age of 12 at a local clinic. The parent reported that the patient had displayed facial pallor intermittently since early childhood. Her father, who had died of renal cancer, had a history of pediatric anemia of undetermined etiology persisting into adulthood. The patient was ethnically pure Japanese and had no other family history of hematological disease. Although she was otherwise in good health on admission, the patient had facial pallor with anemic palpebral conjunctiva. Although SpO2 was 89%, no intolerance to physical exercise was observed. No jaundice, hepatomegaly, or splenomegaly was observed. Laboratory test indicated normocytic normochromic anemia with hemolysis: Hb, 8.9 g/dL; mean corpuscular volume, 90.7 fL; reticulocyte count, 3.25%; serum haptoglobin, 8 mg/dL (reference range, 19–170 mg/dL); and fetal Hb (HbF), 2.7% (reference range, 0–1.7%). Congenital cyanotic heart disease and respiratory disease were ruled out based on imaging and physical examinations. Peripheral blood smear showed target cells (Fig. 1a) and inclusion bodies in red blood

Effect of i.v. immunoglobulin in the first 4 days of illness in Kawasaki disease

Although early treatment of Kawasaki disease (KD) with i.v. immunoglobulin (IVIG) is expected to prevent coronary artery abnormalities, the effectiveness of IVIG by day 4 of illness remains to be determined.

Enhanced theophylline metabolism in patients with bronchial asthma at age 4 and under

The plasma levels of theophylline (TP) and its metabolites were measured in patients with bronchial asthma who were treated with a slow-release preparation of TP. The ratios of the plasma levels of these metabolites to TP levels in the group aged 1-4 years were larger than those in the group aged 5 years and older, suggesting enhanced activity of drug-metabolizing enzymes during infancy.