Atopic dermatitis (AD) is considered to be Th2 cell-mediated disorder. In most infants with AD, AD may be induced by food allergy. In the early stage of infantile AD, it is unclear whether there are changes in serum Th2 chemokines or in Th2 chemokine production by peripheral blood mononuclear cells (PBMC). Thirty-four patients with AD were examined (mean age, 4.5 months; female:male, 18:16). Ten age-matched infants with no history of allergic disease were used as controls. Thirty of these 34 patients were sensitized with ovalbumin (OVA; radioallergrosolvent score of >2). Serum levels of CCL17, CCL22, and CCL27 were measured with enzyme-linked immunosolvent assay (ELISA) kits and their correlation with the severity of skin lesions, defined by the scoring atopic dermatitis (SCORAD) index, was analyzed. The amounts of TNF-alpha, CCL17, CCL22, and CCL27 in the culture supernatants of PBMC from OVA-sensitized AD infants after stimulation with OVA were estimated with ELISA kits. Elevated serum CCL17, CCL22, and CCL27 levels significantly correlated with SCORAD index (r = 0.7181, p < 0.001; r = 0.5354, p < 0.005; r = 0.8312, p < 0.0001, respectively). CCL22 levels produced by PBMC from OVA-sensitized infants with AD reflected serum CCL22 levels. Only six of 30 OVA-sensitized patients in whom the skin signs increased immediately after OVA intake showed markedly high titers of TNF-alpha produced by PBMC after stimulation with OVA. These high TNF-alpha titers correlated significantly with serum CCL27 levels (r = 0.7181, p < 0.001). Serum concentrations of CCL17, CCL22, and CCL27 correlate well with the extent and intensity of AD in infants. Of the three Th2 chemokines examined, serum CCL27 correlated most significantly with the severity of AD. Thus, the peripheral immune responses of infantile AD patients are skewed to a Th2 dominant bias.
The grass pollen allergen Lol p III (Mr 11,000) is a well-characterized antigen that has been found useful in immunogenetic studies of human immune responsiveness. Since immune responsiveness to this allergen is associated with HLA-DR3, we investigated whether there was any sequence in the HLA-D region that would render a person "susceptible" [antibody (Ab)-positive] to the allergen. By sequence-specific oligonucleotide (SSO) slot-blot and sequence analyses of polymerase chain reaction (PCR)-amplified genomic DNA from Lol p III responder and nonresponder subjects, Ab responsiveness was found to be strongly associated with the sequence Glu-Tyr-Ser-Thr-Ser (EYSTS), present in the first polymorphic regions of DR beta I polypeptide chains of DR3, DR11 (split of DR5), and DRw6. Of the 41 grass-allergic subjects investigated, 19 had the EYSTS sequence, of whom 18 (95%) were Lol p III immunoglobulin G (IgG) Ab responders; among the 22 EYSTS- subjects, ten were Lol p III responders (P = 0.001, relative risk = 21.6). No such association was found with any polymorphic sequences in other DR beta chains, or in DQ alpha I and DQ beta I chains. These findings suggest that the EYSTS sequence is important in the presentation of an epitope of Lol p III; other sequence(s) may be involved in the presentation of other epitope(s). To our knowledge, this is the first demonstration of a strong association between a specific HLA sequence and immune responsiveness to a well-defined antigen. The paper shows that presence of the EYSTS sequence classifies subjects as Lol p III responders in 18/19 cases.
solated noncompaction of the ventricular myocardium (INVM) is a rare disorder characterized by excessively prominent trabeculations and deep intertrabecular recesses, mainly in the left ventricle without other congenital cardiac malformations. 1 Most patients with INVM show various abnormalities on the electrocardiogram (ECG), including Wolff-Parkinson-White (WPW) syndrome, 2,3 which is currently classified into several types based on the localization of the bypass tract. It has not previously been reported which type of WPW syndrome occurs in patients with INVM. 3,4 We report 3 patients with INVM and WPW syndrome type B on ECG, which has not been previoulsy reported. 3,4 Case Reports Patient 1A 9-month-old Japanese girl was admitted to hospital because of increasing dyspnea. She was the first child of healthy nonconsanguineous parents, and the pregnancy and delivery had been uneventful.On admission, she was thin and malnourished: weight 6 kg and height 56 cm, both below the 10th percentile. She was pale and had cyanosis of the lips and nail bed. On auscultation, there was tachypnea and moderate retraction of the chest wall with moist rales over the entire lung field. A grade 3 pansystolic murmur was noted at the apex. Her abdomen was moderately distended, with the liver palpable 3 cm below the right costal margin. Chest X-ray showed marked cardiac enlargement, and pulmonary venous congestion. She died suddenly at 11 months of age. Patient 2The 6-month-old younger sister of Patient 1 was referred for examination of her cardiac function. The pregnancy and delivery had been uneventful and she was currently alert and healthy. Her heart sounds were clear and the rhythm was regular without murmur.Chest X-ray showed mild cardiac enlargement with a cardiothoracic ratio of 0.61. One year later, she still did not have symptoms, but her cardiac performance had deteriorated slightly, with a left ventricular fractional shortening (LVFS) of 24% and an ejection fraction (LVEF) of 57%. She was treated with oral angiotensin-converting enzyme inhibitor. Patient 3A 9-year-old girl was referred for an outpatient examination of an arrhythmia diagnosed previously during a school physical examination. She was alert and healthy with clear heart sounds and no murmur. No cardiac enlargement was noted on the chest roentgenogram. Electrocardiography, Echocardiography and Magnetic Resonance ImagingThe ECG of Patient 1 revealed marked tachycardia of 150 beats/min and WPW syndrome type B, with localization of the accessory pathway in the right anteroseptal area (Fig 1A). Cardiac performance was markedly depressed. A 2-dimensional (D) echocardiogram (Fig 1B) and a T1-weighted magnetic resonance imaging (MRI) scan (Fig 1C) revealed numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses, but there was no evidence of endocardial thrombus over the left ventricular wall.The ECG from Patient 2 revealed a normal sinus rhythm and WPW syndrome type B, with localization of the accessory pathway in the right anteros...
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