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.

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.

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.

Declining hepatitis C virus (HCV) prevalence in pregnant women: impact of anti-HCV screening of donated blood.

BACKGROUND: Screening blood donors for hepatitis C virus (HCV) antibody has effectively mitigated transfusion transmission of HCV. We conducted a post hoc analysis to clarify the impact of donor screening on a general population of reproductive-age females and their offspring. STUDY DESIGN AND METHODS: Anti-HCV screening in Japan started in late-1989. In a cohort studied between May 1990 and November 2004, a total of 22,664 consecutive serum samples from pregnant women were screened for anti-HCV. Reactive samples were further tested for HCV RNA. Linear structural regression was applied to identify causal relationships. RESULTS: Anti-HCV-reactive rates declined significantly by two measures. First, among women known to have been transfused, rates fell from 14.8% to 3.1% with the implementation of anti-HCV screening (p < 0.01). Nevertheless, this is 10 times higher than the 0.3% reactive rate seen in a similar cohort of nontransfused women. Second, rates fell from 1.8% among women born in 1955 or before to 0.3% for women born in 1966 or later (p < 0.01). Among 103 anti-HCV-reactive women, 31 (30%) had been transfused and another 17 (17%) had other identifiable risk factors. The remaining 55 (53%) had no clear risk factor. Blood transfusion accounted for 19% of anti-HCV acquisition, by path analysis. Only one infant in this cohort was vertically infected with HCV. CONCLUSION: Anti-HCV screening of donated blood and hygienic improvements have markedly decreased HCV infection of pregnant women with a transfusion history; however, 70% of anti-HCV-reactive women were deemed to be infected via routes other than transfusion.

Evolution in the hypervariable region of the hepatitis C virus in two infants infected by mother-to-infant transmission.

BACKGROUND: There is little data on the evolution of hepatitis C virus (HCV) quasispecies in infants infected by mother-to-infant transmission during long-term follow up. The hypervariable region 1 (HVR1) of the HCV genome was investigated in two mother-infant pairs from birth to 7.6 and 10.2 years, respectively. METHODS: Ten cDNA clones of HVR1 generated from HCV-RNA and extracted from serum samples of both pairs were analyzed. The sequences were compared with regard to variability, identity, and hydrophobia profile, and analyzed by phylogenetic studies. RESULTS: The alanine aminotransferase (ALT) level was high with fluctuation in infant A and almost within the normal range in infant B. Sequence diversity was higher in infant A at 7.6 years than in infant B at 9.3 years (sequence identity with the mothers'; 69.3-70.7% vs 85.3-90.7% for nucleotides, and 48% vs 68-72% for amino acids, respectively). Compared to the first samples, amino acid changes greatly increased in infant A (35.2% at 4.9 years and 52% at 7.6 years), but not in infant B (4% at 5.6 years and 27.5% at 9.3 years). Phylogenetic studies revealed that quasispecies in infant A evolved to a greater extent than that in infant B. Hydrophobia profile analyses revealed that dynamic shifts between hydrophilia and hydrophobia occurred in both infants. CONCLUSIONS: As in adults, the evolution of HVR1 and variability of quasispecies increased in infants infected through mother-to-infant transmission for 10 years after birth. A large episode of ALT elevation suggested the emergence of escape mutants and the evolution of new quasispecies.

Low frequency of observed anti-HLA among transfused preterm infants.

BACKGROUND: Although low-birth-weight infants (LBWI) often receive multiple transfusions, there is controversial information on their development of antibodies against WBCs or platelets. STUDY DESIGN AND METHODS: A total of 52 LBWI with birth weights less than 1500 g were randomly assigned to receive either RBCs that had been WBC- reduced (n = 25) or nonfiltered blood (n = 27). Serum samples collected from 37 infants at 3 months of age and from 30 children when they were 5 to 11 years old were tested. Anti-HLA was assayed with an anti-human globulin-augmented lymphocytotoxicity test against a panel consisting of 13 lymphocytes and against parental cells. RESULTS: None of 52 transfused LBWI of either group developed anti-HLA (95% CI, 0%-6.8% for overall, 0%-13.7% for the WBC-reduced group, and 0%-12.7% for the nonfiltered group). CONCLUSION: Multiply transfused LBWI rarely produced antibodies to HLA of blood donors and to noninherited maternal antigens. The benefits of WBC reduction to prevent HLA alloimmunization during infancy were not supported by this study and need further investigation.

Residual WBC subsets in filtered prestorage RBCs.

BACKGROUND: New-generation RBC filters reduce WBC concentrations by 4 to 5 logs and may prevent or decrease transfusion complications such as HLA alloimmunization, nonhemolytic febrile reaction, and transfusion-transmitted infections. The residual level of WBC subsets may influence efficacy of WBC reduction for preventing various complications. This study analyzed subsets of residual WBCs in WBC-reduced RBC components prepared for a large, multicenter prospective study. STUDY DESIGN AND METHODS: The Viral Activation Transfusion Study (VATS) assessed the impact of WBC reduction in HIV-1-infected patients undergoing RBC transfusion. WBC-reduced RBC from 11 clinical sites with variable filtration practices were sorted into "low,""middle," and "high" groups based on residual WBC concentration. Subsets were isolated from units by immunocapture (anti-CD4-, anti-CD8-, anti-CD15-, and anti-CD19-coated magnetic beads) and quantified by PCR amplification. RESULTS: After validation studies confirming test methodology, 105 VATS WBC-reduced RBC samples were analyzed. Concentrations of subsets in low and middle residual WBC groups were very low in contrast to relatively high concentrations in the high group. Although highly significant differences were identified between the middle and high groups for total WBCs and all subsets, no single subset predominated. CONCLUSION: These results suggest that overall efficacy of WBC filtration correlates with removal of WBC subsets.