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Background We examined the epidemiology, clinical manifestations, and prognosis of pediatric systemic lupus erythematosus (SLE) in Fukushima Prefecture, Japan over a 35 year period. Methods We collected the medical records of 37 patients diagnosed with SLE between 1977 and 2013. These children were divided into two groups. group 1 consisted of 19 patients who were diagnosed between 1977 and 1995, and group 2 consisted of 18 patients diagnosed between 1996 and 2013. The epidemiology, clinical features, and prognosis were retrospectively compared between the two groups. Results The mean number of patients per 100 000 children per year for group 1 and group 2 was 0.33 ± 0.25 and 0.35 ± 0.30, respectively. The duration from onset of symptoms to treatment in group 2 was shorter than that in group 1, but the clinical and laboratory findings at onset did not differ between the two groups. All patients were treated with prednisolone, and 17 patients in group 1 and 18 in group 2 were treated with methylprednisolone pulse therapy. The frequency of cyclophosphamide treatment decreased whereas the frequency of cyclosporine, tacrolimus and mizoribine pulse therapy increased in group 2. SLE disease activity index (SLEDAI) score at the latest follow up in group 2 was lower in group 1. The survival rate was 84% in group 1 and 100% in group 2. Conclusion The frequency and severity of SLE in group 1 were similar to those in group 2, and the prognosis of SLE in group 2 was better than that in group 1.

Several studies have shown that brain hypothermia therapy (BHT) after neonatal hypoxic-ischemic encephalopathy (HIE) can improve neurodevelopmental outcomes. However, there have been no reports of the neurodevelopmental outcomes for the infant with a serum creatine kinase (CK) level above 20,000 IU/L in association with neonatal HIE. We report a female infant with a very high serum CK level (26,428 IU/L) associated with neonatal asphyxia. We diagnosed this infant with moderate HIE, and BHT was achieved by head cooling within 6 hours after birth to an esophageal temperature of 34.5°C. There were no significant adverse events during BHT, and the CK level spontaneously decreased. Although we report only the short-term outcomes for this case, she presents neurodevelopmental delays at the age of 18 months. It may be correlated between high serum CK level and long-term neurodevelopmental delays. Abbreviations: CK, creatine kinase; HIE, hypoxic-ischemic encephalopathy; BHT, brain hypothermia therapy; NRFS, non-reassuring fetal status; CK-MB, creatine kinase-myocardial band; MRI, magnetic resonance imaging; CK-BB, creatine kinase-brain band.

Background There have been a number of reports on large outbreaks of hemolytic uremic syndrome (HUS), but there have been no long-term studies of sporadic HUS in Japan. This study therefore investigated the epidemiology and prognosis of HUS in Fukushima Prefecture over a 26 year period. Methods The medical records of 26 patients with HUS between 1987 and 2012 were collected. These children were divided into two groups: those with HUS following an episode of gastroenteritis, often with bloody diarrhea (D + HUS; n = 24) and those with HUS not associated with prodromal diarrhea (D–HUS; n = 2). The D + HUS group was further subdivided into group A (11 patients requiring dialysis) and group B (13 patients not requiring dialysis). The epidemiological and clinical data, as well as prognosis, were retrospectively investigated for each group. Results Approximately 90% of HUS patients belonged to the D + HUS group. In this group, the mean number of patients per year from 1987 to 1999, and from 2000 to 2012 was 0.92 ± 0.95, and 1.08 ± 0.86, respectively. On admission, lactate dehydrogenase (LDH), alanine aminotransferase (ALT), blood urea nitrogen (BUN), serum creatinine and serum fibrinogen degradation product (FDP) levels in group A were all higher than in group B. Serum albumin level and estimated glomerular filtration rate (eGFR) in group A were lower than in group B. At 6 months after the onset of HUS in the D + HUS group, renal function was normal. Conclusions The frequency of HUS was constant from 1987 to 2012 in Fukushima. and serum LDH, ALT, BUN, creatinine, and FDP levels as well as eGFR might be risk factors for dialysis in D + HUS children.

The purpose was to review our experiences and determine if applying the sky blue method is reliable in confirming gastric tube (GT) placement in neonates.The study population consisted of 44 infants (55 placements) who were admitted to the Takeda General Hospital between April 2012 and March 2013 and who required GT exchange. The sky blue method using indigo carmine (IC) was indicated for planned tube exchange only. Diluted IC was injected into the gastric space via the old GT just before the tube exchange. The tube was exchanged using a standard method. Then, we checked whether the diluted IC could be collected through the new GT or not.The reasons for GT placement were a gestational age of35 weeks in 31 (56.4%), poor sucking or swallowing disorders in 17 (30.4%), and respiratory disorders in 7 (12.7%) of the 55 placements. GT placement using the sky blue method was considered successful in 52 placements (94.4%), with the remaining 3 placements (5.6%) considered to be failures due to the inability to obtain IC from the gastric space. No adverse events were observed during the tube exchange period.Based on the results, the sky blue method can be considered to be reliable method for the confirmation of GT placement. These results also suggest that the number of radiologic evaluations performed to confirm correct replacement of the GT in infants can be reduced in the future.

Recently, there have been a few reports on the decrease in the incidence of severe Henoch-Schönlein purpura nephritis (HSPN) in Japan. To evaluate the incidence and the severity of HSPN, we examined the epidemiology and clinical manifestations of HSPN in Fukushima Prefecture over a 22-year period.We enrolled 61 patients (Group 1) diagnosed with HSPN between 1987 and 1997 and a further 59 patients (Group 2) diagnosed with HSPN between 1998 and 2008. Epidemiology, clinical features, laboratory data, pathological findings, treatment and outcome were retrospectively compared between the two groups.The mean number of patients per 100,000 children per year was 3.5 ± 1.2 in Group 1 and 3.6 ± 0.8 in Group 2. The clinical manifestations and laboratory findings at onset were comparable between Groups 1 and 2. Pathological findings in Group 1 and Group 2 were classified as ISKDC grade IIIa in 16 (26%) and 14 cases (24%), grade IIIb in 26 (43%) and 27 cases (46%), grade IV in 5 (8%) and 6 cases (10%)), grade V in 1 (2%) and 5 cases (8%), and grade VI in 2 (3%) and 2 cases (3%), respectively. There were no inter-group differences in ISKDC classification or rate of crescentic formation.Our findings suggest that the incidence of HSPN and the severity of HSPN in patients between 1987 and 1997 were similar to those in patients between 1998 and 2008 and that the number of patients with severe HSPN has not decreased.

小児の上・下気道感染症ならびに気管支喘息に対する漢方療法を述べた。漢方療法とはいえ，医の東西を問わず患児を丁寧に観察・診察して，適切な治療を行うことに変わりはない。そのためには，まず患児の疾患が内科的なものであることを診断する必要がある。そして，この際，重度な緊急疾患や細菌感染症が疑われる場合は，漢方療法での対処は困難であることが多いので，西洋医学療法を第一に選択し，漢方療法は補助的なものとする。その上で，漢方療法の対象となる疾患が，いずれの病位にあるのかを証とともに決定し，方剤を選択する。そして，患児の服薬コンプライアンスをよく観察し，悪ければ他の方剤への変更を考慮することが大切である。小児の呼吸器疾患に対して，漢方療法的なアプローチをすることにより，個々の患児に応じたオーダーメイドの医療が可能になると思われる。

We examined the epidemiology, clinical manifestations, and prognosis of pediatric systemic lupus erythematosus (SLE) in Fukushima Prefecture, Japan over a 35 year period.We collected the medical records of 37 patients diagnosed with SLE between 1977 and 2013. These children were divided into two groups. group 1 consisted of 19 patients who were diagnosed between 1977 and 1995, and group 2 consisted of 18 patients diagnosed between 1996 and 2013. The epidemiology, clinical features, and prognosis were retrospectively compared between the two groups.The mean number of patients per 100,000 children per year for group 1 and group 2 was 0.33 ± 0.25 and 0.35 ± 0.30, respectively. The duration from onset of symptoms to treatment in group 2 was shorter than that in group 1, but the clinical and laboratory findings at onset did not differ between the two groups. All patients were treated with prednisolone, and 17 patients in group 1 and 18 in group 2 were treated with methylprednisolone pulse therapy. The frequency of cyclophosphamide treatment decreased whereas the frequency of cyclosporine, tacrolimus and mizoribine pulse therapy increased in group 2. SLE disease activity index (SLEDAI) score at the latest follow up in group 2 was lower in group 1. The survival rate was 84% in group 1 and 100% in group 2.The frequency and severity of SLE in group 1 were similar to those in group 2, and the prognosis of SLE in group 2 was better than that in group 1.

Chronic active Epstein-Barr virus (EBV) infection (CAEBV), characterized by persistent infectious mononucleosis-like symptoms, can lead to cardiovascular complications including coronary artery aneurysm or myocarditis. Here, we present the case of an 11-year-old boy with pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia associated with CAEBV. The patient did not have any major symptoms attributed to CAEBV, such as fever, lymphadenopathy or splenomegaly when the PAH developed. Mild liver dysfunction was found at the first examination, and it persisted. Two years after the PAH symptoms appeared, CAEBV was evident, based on deteriorated liver function, hepatosplenomegaly, and coronary artery aneurysms. CAEBV should be considered as a cause of secondary PAH, particularly when liver dysfunction coexists.

There have been a number of reports on large outbreaks of hemolytic uremic syndrome (HUS), but there have been no long-term studies of sporadic HUS in Japan. This study therefore investigated the epidemiology and prognosis of HUS in Fukushima Prefecture over a 26 year period.The medical records of 26 patients with HUS between 1987 and 2012 were collected. These children were divided into two groups: those with HUS following an episode of gastroenteritis, often with bloody diarrhea (D + HUS; n = 24) and those with HUS not associated with prodromal diarrhea (D-HUS; n = 2). The D + HUS group was further subdivided into group A (11 patients requiring dialysis) and group B (13 patients not requiring dialysis). The epidemiological and clinical data, as well as prognosis, were retrospectively investigated for each group.Approximately 90% of HUS patients belonged to the D + HUS group. In this group, the mean number of patients per year from 1987 to 1999, and from 2000 to 2012 was 0.92 ± 0.95, and 1.08 ± 0.86, respectively. On admission, lactate dehydrogenase (LDH), alanine aminotransferase (ALT), blood urea nitrogen (BUN), serum creatinine and serum fibrinogen degradation product (FDP) levels in group A were all higher than in group B. Serum albumin level and estimated glomerular filtration rate (eGFR) in group A were lower than in group B. At 6 months after the onset of HUS in the D + HUS group, renal function was normal.The frequency of HUS was constant from 1987 to 2012 in Fukushima. and serum LDH, ALT, BUN, creatinine, and FDP levels as well as eGFR might be risk factors for dialysis in D + HUS children.

Because the role of eosinophils in neonates is not well understood, the clinical significance of eosinophilia in neonates is unclear. We encountered a rare case of cow's milk allergy in a premature male infant with severe eosinophilia in the neonatal period. The peripheral blood eosinophil count in this infant was 7,404/μL at birth, and he produced stools with fresh blood immediately after birth and prior to the first feedings with regular cow's milk. Although the patient's eosinophil count normalized without specific treatment within 6 weeks after birth, it is possible that the causes of the eosinophilia in this infant prior to the first feedings with regular cow's milk were different from those after the first feedings. Cow's milk allergy was diagnosed on the basis of the patient's positivity for this allergy in the challenge test and subsequent allergen-specific lymphocyte stimulation test performed at 6 months of age.