The theoretical model of psychological well-being that encompasses six domains (self-acceptance, positive relations with others, autonomy, environmental mastery, purpose in life, and personal growth) was tested with a Japanese university student population (N = 574) using a Japanese translation of Ryff's 1989 Psychological Well-being Inventory. A factor structure similar to Ryff's original model emerged. Both depression and anxiety correlated only moderately with scores on some subscales of the inventory, suggesting the relative independence of these dimensions of psychological well-being and negative affectivity. With negative affectivity controlled, some early life experiences were significantly linked with psychological well-being: relationships with romantic partners were linked with greater autonomy and experiences which enhance self-esteem were liked with greater personal growth. Careful psychometric work on the Japanese version is required to use the scale; then a replication and extension of the present study would be feasible.

We present a case of a 9-year-old boy with nemaline myopathy and dilated cardiomyopathy. The combination of nemaline myopathy and cardiomyopathy is rare, and this is the first reported case of dilated cardiomyopathy associated with childhood-onset nemaline myopathy. A novel mutation, p.W358C, in ACTA1 was detected in this patient. An unusual feature of this case was that the patient' s cardiac failure developed during early childhood with no delay of gross motor milestones. The use of a b-blocker did not improve his clinical course, and the patient died 6 months after diagnosis of dilated cardiomyopathy. Congenital nonprogressive nemaline myopathy is not necessarily a benign disorder: deterioration can occur early in the course of dilated cardiomyopathy with neuromuscular disease, and careful clinical evaluation is therefore necessary.

Patients with Down syndrome (DS) and a left-to-right shunt often develop early severe pulmonary hypertension (PH) and pulmonary vascular obstructive disease (PVOD); the pathophysiological mechanisms underlying the development of these complications are yet to be determined. To investigate the mechanisms, we evaluated the biosynthesis of thromboxane (TX) A(2) and prostacyclin (PGI(2)) in four groups of infants, cross-classified as shown below, by measuring the urinary excretion levels of 11-dehydro-TXB(2) and 2,3-dinor-6-keto-PGF(1alpha): DS infants with a left-to-right shunt and PH (D-PH, n = 18), DS infants without congenital heart defect (D-C, n = 8), non-DS infants with a left-to-right shunt and PH (ND-PH, n = 12), and non-DS infants without congenital heart defect (ND-C, n = 22). The urinary excretion ratios of 11-dehydro-TXB(2) to 2,3-dinor-6-keto-PGF(1alpha) in the D-PH, D-C, ND-PH, and ND-C groups were 7.69, 4.71, 2.10, and 2.27, respectively. The ratio of 11-dehydro-TXB(2) to 2,3-dinor-6-keto-PGF(1alpha) was higher in the presence of DS (P < 0.001), independently of the presence of PH (P = 0.297). The predominant biosynthesis of TXA(2) over PGI(2), leading to vasoconstriction, was observed in DS infants, irrespective of the presence/absence of PH. This imbalance in the biosynthesis of vasoactive eicosanoids may account for the rapid progression of PVOD in DS infants with a left-to-right shunt.

Group B streptococcus (GBS) is a commonly recognized cause of sepsis and meningitis in neonatal and young infants. Invasive GBS infection is classified into early onset GBS disease (EOD, day 0–6), late onset GBS disease (LOD, day 7–89) and ultra late onset GBS disease (ULOD, after 3 months of age). ULOD is uncommon and recurrence is especially rare. We present the first recurrent case of ULOD GBS sepsis in 3-year-old girl with a past medical history of hydrops fetalis and thoracic congenital lymphatic dysplasia. The first episode presented as sepsis at 2 years 8 months of age. The second episode occurred as sepsis with encephalopathy at 3 years 1 months of age. During each episode, the patient was treated using intravenous antimicrobials and her condition improved. Serotype examination was not performed in the first episode, but GBS type V was serotyped in the second episode. ULOD over 1 year of age is quite rare and may recur.