Soluble LR11 as a Novel Biomarker in Acute Kawasaki Disease.

BACKGROUND: Intimal smooth muscle cells (SMCs) play an important role in the vasculitis caused by Kawasaki disease (KD). Lipoprotein receptor 11 (LR11) is a member of the low-density lipoprotein receptor family, which is expressed markedly in intimal vascular SMCs and secreted in a soluble form (sLR11). sLR11 has been recently identified as a potential vascular lesion biomarker. sLR11 is reportedly elevated in patients with coronary artery lesions long after KD, but there is no description of sLR11 in acute KD. Our aim was to determine the sLR11 dynamics in acute KD and to assess its usefulness as a biomarker.Methods and Results: 106 acute KD patients and 18 age-matched afebrile controls were enrolled. KD patients were classified into the following subgroups: intravenous immunoglobulin (IVIG) responders (n=85) and non-responders (n=21). Serum sLR11 levels before IVIG therapy were higher in non-responders (median, 19.6 ng/mL; interquartile range [IQR], 13.0-24.9 ng/mL) than in controls (11.9 ng/mL, 10.4-14.9 ng/mL, P<0.01) or responders (14.3 ng/mL, 11.7-16.5 ng/mL, P<0.01). Using a cutoff of >17.5 ng/mL, non-responders to initial IVIG therapy were identified with 66.7% sensitivity and 78.8% specificity. CONCLUSIONS: sLR11 can reflect the state of acute KD and might be a biomarker for patient response to IVIG therapy.

Clinically Mild Encephalitis/Encephalopathy with a Reversible Splenial Lesion Associated with Rhinovirus.

A 2-year-old girl with fever and seizures was diagnosed as having clinically mild encephalitis/encephalopathy with a reversible splenial lesion, as indicated by magnetic resonance imaging. Virologic analysis identified human rhinovirus A49 in her serum. Although human rhinovirus rarely involves the central nervous system, such involvement could result in mild encephalitis/encephalopathy with a reversible splenial lesion.

Occurrence of severe rotavirus gastroenteritis in children younger than three years of age before and after the introduction of rotavirus vaccine: a prospective observational study in four pediatric clinics in Shibata City, Niigata Prefecture, Japan.

In Japan, rotavirus (RV) vaccines have already been introduced but not used for universal vaccination as of 2018. Therefore, we identified cases of severe rotavirus gastroenteritis (RVGE) in children younger than three years of age and investigated the occurrence of infection before and after the introduction of RV vaccines. An ecological study through prospective surveillance was conducted in four pediatric clinics in Shibata City, Niigata Prefecture, Japan, during the 2011 to 2018 RVGE epidemic seasons. We divided the study period into three eras: pre-vaccine introduction era (2011), low-mid coverage transitional era (2012 to 2014, RV vaccine coverage rate: 32.9-56.5%), and high coverage plateau era (2015 to 2018, 67.7-81.7%). In this study, the incidence rate of severe RVGE was significantly lower in the plateau era than in the pre-vaccine introduction and transitional eras. Furthermore, the hospitalization rate due to RVGE in Shibata City was lower in the plateau era than in the pre-vaccination introduction and transitional eras. The number of hospitalizations due to RVGE in subjects who required or did not require intravenous rehydration at the pediatric clinics significantly decreased with the increase in vaccine coverage rates by more than 70% in the plateau era.

Twins with neonatal pemphigus vulgaris born to a mother with pemphigus vulgaris: a case report.

Dichorionic diamniotic twins were born at 37 weeks of gestation by cesarean section to a 34-year-old primigravid Japanese woman because the first twin was in breech presentation. The mother had been diagnosed with pemphigus vulgaris prior to her pregnancy. In addition to a high antidesmoglein 3 autoantibody titer, flaccid bullae and erosions on both of the twins' lips and in their oral cavities at 13 days of age led to the diagnosis of neonatal pemphigus vulgaris. This case highlights the need for awareness that pemphigus vulgaris may not occur immediately after birth.

Rhabdomyolysis associated with antimicrobial drug-resistant Mycoplasma pneumoniae.

We describe a case of rhabdomyolysis in a patient infected with antimicrobial drug-resistant Mycoplasma pneumoniae The patient's acute-phase serum levels of interleukin-18 and tumor necrosis factor-α were high, which suggests a pathogenic role for M. pneumoniae. In an era of increasing antimicrobial drug resistance, a system for rapidly identifying resistant M. pneumoniae would be beneficial.

Staphylococcal scalded skin syndrome in an extremely low-birth-weight neonate: molecular characterization and rapid detection by multiplex and real-time PCR of methicillin-resistant Staphylococcus aureus.

BACKGROUND: Staphylococcal scalded skin syndrome (SSSS), caused by methicillin-resistant Staphylococcus aureus (MRSA) producing exfoliative toxin (ET), is a life-threatening infection for neonates in neonatal intensive care units (NICUs). SSSS in extremely low-birth-weight (ELBW) neonates is rare. A new class of MRSA (community-acquired MRSA, CA-MRSA) has been emerging in the community. The aim of this study was to characterize MRSA from an ELBW neonate with SSSS, and to develop rapid detection methods for SSSS-associated and emerging pediatric MRSA. METHODS: An ELBW infant in the NICU developed SSSS on day 16 after birth. Isolated MRSA was genetically characterized and compared with CA-MRSA from bullous impetigo (biCA-MRSA), which is positive for the ET and collagen-adhesin (CNA) genes in many cases, and the Panton-Valentine leucocidin (PVL) gene rarely. Specific primers and probes for five virulence genes (for ETA, ETB, ETD, PVL, CNA) were designed for multiplex polymerase chain reaction (PCR) and real-time PCR. RESULTS: MRSA strain H5 from SSSS exhibited the genotype (ST91, spa416[t375], agr3, SCCmecIVa, CoaI), and possessed the ETB and CNA genes, similar to ST91 biCA-MRSA (albeit with a divergence). Multiplex PCR detected the ETB and CNA genes of strain H5, and real-time PCR detected strain H5 at as low as 10(2) CFU/mL. The assays were 100% specific and 100% sensitive, for the five virulence genes. CONCLUSION: ETB-positive ST91 MRSA, which was very similar to ST91 biCA-MRSA, was isolated from an ELBW infant with SSSS. The multiplex and real-time PCR assays specifically or quantitatively detected SSSS-associated and emerging pediatric MRSA.

Fulminant type 1 diabetes mellitus acutely emerged during pregnancy.

A pregnant woman at 32 weeks of gestation was emergently admitted to our hospital with symptoms of nausea, vomiting, and uterine contraction. Cardiotocogram demonstrated a loss of variability and late deceleration in fetal heart rate pattern. Emergency cesarean section was performed, and a male infant weighing 1750 g was born with Apgar scores of 1 at 1 min, and 3 at 5 min after delivery. After cesarean section, the patient developed an acetone breath odor, and blood examination demonstrated remarkable acidemia and an extremely high level of blood glucose. The patient was diagnosed with ketoacidosis with acute onset of fulminant type 1 diabetes mellitus. Intensive care was applied due to the severe diabetes mellitus conditions. The patient's general condition ameliorated during the postoperative period, although there was a possibility of neurological complications in the infant.

Large pseudocyst of the umbilical cord detected in the second trimester.

We present a case of a large umbilical cord cyst detected at 21 weeks of gestation. Serial ultrasonographic examination revealed a single umbilical artery and progression of the cystic mass. A 2,842-g male infant was delivered at 37 weeks of gestation, and we confirmed that the umbilical cord cyst was a pseudocyst in our pathological examination. This case demonstrated an uneventful course of pregnancy despite the large umbilical cord pseudocyst.

Management of patients with pregnancy-associated gastric cancer in Japan: a mini-review.

Gastric cancer associated with pregnancy is quite rare, and is most often diagnosed at an advanced stage. Furthermore, physicians are confronted with two conflicting issues in this condition: the need for early treatment of the maternal gastric cancer and the continuation of the pregnancy. To clarify the characteristics of pregnancy-associated gastric cancer and to obtain useful information that would help us choose the best treatment strategy for pregnancy-associated gastric cancer, we reviewed the existing literature, using the key words "pregnancy" and "gastric cancer". We were able to accumulate 136 cases, including 100 cases reported previously in Japan, and 1 case that we report here. We analyzed a total of 137 cases in the present study. With respect to the stage of gastric cancer, 92.5% of the patients studied had advanced gastric cancer, and only 45.3% of the patients underwent gastrectomy, including incomplete resection. Accordingly, the prognosis was very poor; the 1- and 2-year survival rates were 18.0% and 15.1%, respectively. However, the number of patients found to have early gastric cancer by endoscopic examination has been increasing recently. An endoscopic examination should be conducted immediately in pregnant patients presenting with persistent gastrointestinal symptoms for the differential diagnosis of hyperemesis gravidarum. When an endoscopic examination reveals that pregnant patients have gastric cancer, a therapeutic plan should be promptly formulated, in accordance with the number of weeks of gestation, by a medical team consisting of specialists in perinatal obstetrics and gastric cancer specialists.

Four cases of fetal hypoechoic hepatomegaly associated with Trisomy 21 and transient abnormal myelopoiesis.

OBJECTIVE: Our objective was to determine the clinical significance of fetal hypoechoic hepatomegaly and serial change of liver sizes. METHODS: The liver sizes of four fetuses with hypoechoic hepatomegaly were serially estimated by liver length, as measured from the dome of the right hemidiaphragm to the tip of the right lobe. RESULTS: All cases were associated with trisomy 21 or transient abnormal myelopoiesis (TAM). Two cases were trisomy 21 with TAM, one case was a phenotypically normal newborn, who had developed TAM during the fetal period, and the last case was trisomy 21 without TAM. In the last case, it is speculated that TAM had developed and regressed completely before birth. Two cases, whose hepatomegaly had improved before birth, showed good prognosis and the other two cases, in whom improvement had not been observed, resulted in death after birth by liver failure. CONCLUSION: These experiences show that one of the differential diagnoses of hypoechoic hepatosplenomegaly is TAM and that the change of live size is a predictor of prognosis.

Urinary acidification in extremely low birth weight infants.

Premature infants often present metabolic acidosis without protein load in the early neonatal period, around days 4-6. In order to elucidate the cause of acidosis, we investigated urinary acidification of infants in the early neonatal period. Urine pH, fractional excretion of HCO(3)(-) (FEHCO(3)), excretion of HCO(3)(-) and NH(4)(+) of the appropriate-for-date infants were measured on days 0-2 and on days 4-6 of life. Extremely low birth weight (ELBW) infants showed higher urine pH than more than 1500 g birth weight infants. FEHCO(3) and HCO(3)(-) excretion were of high values in ELBW infants on days 0-2, but decreased on days 4-6. Urine NH(4)(+) excretion rate was lower in ELBW infants than in birth weight more than 1000 g on days 0-2 of life and still remained at a low rate on days 4-6. These data indicated that insufficiency of NH(4)(+) excretion is the main cause for metabolic acidosis of ELBW infants in the early neonatal period.