Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection.

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.

Incidence and prognosis of systemic lupus erythematosus in a 35 year period in Fukushima, Japan.

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.

Incidence and index of severity of hemolytic uremic syndrome in a 26 year period in Fukushima Prefecture, Japan.

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.

Combined Fundal and Segmental Adenomyomatosis of the Gallbladder in a Child: A Rare Case Report.

Adenomyomatosis of the gallbladder (AMG) is characterized by mucosal hyperplasia leading to invagination through the thickened muscle layer, which is relatively common in adults, but is rare in childhood. We report a 12-year-old boy with adenomyomatosis of the gallbladder combined segmental and fundal type. This combined type is rare in adults and is first reported here in childhood. Although initial imaging with computed tomography (CT) suggested the presence of a circular solid mass-like lesion because of its rare morphology, repeated ultrasonography (US) was useful for leading to a correct diagnosis.

Cow's Milk Allergy with Severe Eosinophilia.

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.

BRAIN HYPOTHERMIA THERAPY FOR NEONATAL HYPOXIC-ISCHEMIC ENCEPHALOPATHY WITH A SEVERELY ELEVATED SERUM CREATINE KINASE LEVEL.

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.

Confirmation of gastric tube bedside placement with the sky blue method.

BACKGROUND: 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. METHODS: 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. RESULTS: The reasons for GT placement were a gestational age of < 35 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. CONCLUSION: 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.

Neurodevelopmental outcomes of children with periventricular leukomalacia.

OBJECTIVES: To examine the neurodevelopmental outcomes of children with periventricular leukomalacia (PVL). MATERIALS AND METHODS: Twenty-five children diagnosed with grade 1, 2 or 3 PVL on the basis of magnetic resonance imaging (MRI) findings between January 2002 and December 2011 were enrolled and followed from 15 months to 10 years of age. RESULTS: Of the 25 children, one was a term and 24 were preterm-births. Nine (36%) had spastic diplegia and 12 (48%) had quadriplegia. Ten of the 25 (40%) were able to walk independently at 36 months utilizing short leg braces, whereas 13 children (52%) were unable to walk independently. MRI findings revealed grade 1 PVL in nine (36%), grade 2 in 12 (48%), and grade 3 in four (16%) of the 25 children. Eleven of the 16 children (69%) with grade 2 or 3 PVL had Papile III or IV intraventricular hemorrhage (IVH), and many of these children had severe neurologic motor abnormalities, severe psychomotor delay, and seizures. Five of the nine children (56%) with grade 1 PVL had normal psychomotor development. There were statistically significant differences in the motor impairment and walking ability between the children with grade 1 and those with grade 2 PVL (p = 0.008 and 0.005, respectively). CONCLUSION: Most children with grade 2 or 3 PVL had severe neurodevelopmental delays, but attention should also be paid to the 56% of children with grade 1 PVL who presented with normal psychomotor development. Further studies of larger populations, including long-term follow-up, are necessary to evaluate the outcomes of children with PVL.

The incidence and severity of Henoch-Schönlein purpura nephritis over a 22-year period in Fukushima Prefecture, Japan.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.