The proportion of the electronegative low density lipoprotein [LDL( ؊ )] subfraction, which is atherogenic, is increased in type 2 diabetes but is not reduced by glycemic control. Therefore, we evaluated the ability of a new technique, capillary isotachophoresis (cITP), to quantify charge-based LDL subfractions and examined the relation between insulin resistance and the cITP fast-migrating (f) LDL levels. Seventy-five 10-year-old boys were included. The two cITP LDL subfractions, fLDL and major LDL subfractions, were proportional to the LDL protein content within the range of 0.1-0.8 mg/ml LDL protein. Levels of cITP fLDL were positively correlated with triglyceride (TG) levels and negatively correlated with LDL size. Insulin resistance as assessed by the homeostasis model assessment (HOMA-IR) was positively correlated ( P Ͻ 0.01) with cITP fLDL levels ( r ‫؍‬ 0.41). The relation between HOMA-IR and cITP fLDL levels depended on TG levels but was independent of body mass index and LDL size. cITP lipoprotein analysis is an accurate and sensitive method for quantifying charge-based LDL subfractions in human plasma, and insulin resistance is related to cITP fLDL independent of LDL size. -Zhang, B., T. Kaneshi, T. Ohta, and K. Saku. Relation between insulin resistance and fast-migrating LDL subfraction as characterized by capillary isotachophoresis. Electronegative low density lipoprotein [LDL( Ϫ )] subfraction in plasma has been shown to have various atherogenic properties [reviewed by Sánchez-Quesada, Benitez, and Ordonez-Llanos (1)]. Although in vitro oxidized LDL can only be taken up by scavenger receptors, LDL( Ϫ ) can also be taken up by LDL receptors to induce vascular cell adhesion molecule-1 expression through the activation of nuclear factor B and adaptor protein 1 (2).Both type 1 and type 2 diabetic patients have been shown to have an increased proportion of LDL( Ϫ ) (3, 4). However, LDL( Ϫ ) in type 1 and type 2 diabetes seems to be of different origins. In type 1 diabetes, nonenzymatic glycosylation has been shown to contribute to the increased proportion of LDL( Ϫ ): glycemic optimization decreased both the glycated LDL and the proportion of LDL( Ϫ ) (3, 4). However, in type 2 diabetes, glycemic control decreased glycated LDL but had no significant effects on the proportion of LDL( Ϫ ) (4, 5). It is not clear whether or not insulin resistance contributes to LDL( Ϫ ) generation in type 2 diabetes.Insulin resistance is known to be associated with increased levels of triglycerides (TGs) (6). Because LDL( Ϫ ) separated by anion-exchange chromatography techniques has been shown to contain higher TG content than the major LDL subfraction (7-9), it is possible that there may be a relation between insulin resistance and LDL( Ϫ ). However, this has not yet been examined. In addition, it would be interesting to know whether or not the relation between insulin resistance and LDL( Ϫ ) depends on TG levels.Insulin resistance is also linked to plasma levels of small, dense LDLs (pattern B lipoprotein phenot...

BW is related to serum concentrations of adiponectin and uric acid. However, weight velocity was a stronger determinant of serum adiponectin and uric acid levels than BW in Japanese schoolchildren. Thus, it may be important to control weight gain to prevent the development of ACHD in children, especially in children with LBW.

Although serum concentrations of CRP are partly regulated by adipocytokines and conventional risk factors for ACHD, high CRP levels were associated with atherogenic profiles of cardiovascular risk factors in children. Our findings suggest that it may be important to control body weight to prevent an increase in serum CRP in children.

Cornelia de Lange syndrome (CdLS) is a cohesinopathy caused by genetic variations. We present a female with SMC1A -associated CdLS with a novel SMC1A truncation mutation (p. Arg499Ter), transposition of the great arteries, and periodic intractable seizures from 40 months of age. A review of the literature revealed that a seizure-free period after birth of at least 15 months is required for these patients to be able to walk, irrespective of the epileptic course.

We describe the case of a male patient with orofaciodigital (OFD) syndrome type XVI with a homozygous variant of TMEM107 (p.Phe106del) and the additional findings of tibial dysplasia, which is a pivotal finding of OFD syndrome type IV. His family history included two fetuses with anencephaly with or without cleft lip/palate and polydactyly with no genetic information. Careful attention should be given to the interpretation of this rare pattern.

Saul-Wilson syndrome (SWS) is a rare congenital skeletal syndrome characterized by postnatal onset of short stature, relative microcephaly, frontal bossing, prominent eyes with shallow orbits, midface hypoplasia, cataract, and generalized skeletal changes, including spondylar dysplasia, overtubulation of the long bones with metaphyseal flaring and megaepiphyses, coxa valga, elbow deformity, and brachydactyly. We describe a boy with the overall clinical and radiological features fitting the characteristics of SWS, although cataract, elbow deformity, and overt brachydactyly were not seen. He presented with painful hip joint due to hip subluxation in late childhood, which exacerbated with age and ultimately, required surgical intervention. Awareness of this orthopedic complication in SWS is essential in the management of patients with SWS.

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