VACCINE-ASSOCIATED POLIOMYELITIS IN AN INFANT WITH AGAMMAGLO BULINEMIA

Abstract. Sakano, T., Kittaka, E., Tanaka, Y., Yamaoka, H., Kobayashi, Y. and Usui, T. (Department of Paediatrics, Hiroshima University Hospital and Hiroshima City Hospital, Hiroshima, Japan). Vaccine-associated poliomyelitis in an infant with agammagiobulinemia. Acta Paediatr Scand, 69:549, 1980.—We describe a female infant with agammaglobulinemia who contracted vaccine-associated poliomyelitis. Poliovirus type 2 was isolated from the initial stool specimen. In our patient, temporary changes in the cerebrospinal fluid resembled those in patients without immunodeficiencies, although gammaglobulin therapy had not yet been started. Pleocytosis was observed for a short time after viremia, but soon there was a return to normal without antibody production.     

CHERRY RED MACULAR DEGENERATION

ABSTRACT. Cherry red macular degeneration was presented in a 6-month-old Japanese girl suffering from classical features of the diencephalic syndrome. The association of cherry red macular degeneration with the diencephalic syndrome was previously undescribed.     

Early Gastric Lymphoma Coexisting with Reactive Lymphoreticular Hyperplasia (RLH) -A Case Report-

A 34-year-old female visited our hospital because of epigastralgia. We performed an upper gastrointestinal x-ray examination, and both conventional endoscopy and dye-spraying endoscopy (indigo-carmine contrast method). We diagnosed early gastric lymphoma which simulated Borrmann 3 type gastric cancer with IIb type early gastric cancer on the middle body and reactive lymphoreticular hyperplasia (RLH) of cobble stone-like granular pattern by endoscopic appearance on the lower body. Although dye-spraying endoscopy showed the details of the mucosa, it was very difficult to diagnose the lesions correctly by gross appearance alone. Because gastric lymphoma arises from the mucosal or submucosal layer and spreads in the mucosa diffusely, ultrasonic visualization by echo-endoscopy might be useful in the diagnostic procedure. We report a case of early gastric lymphoma coexisting with RLH; both lesions showed uncommon endoscopic features.     

Diastolic Mitral Regurgitation in Patients with First-Degree Atrioventricular Block

Diastolic mitral regurgitation has been observed in patients with DDD pacemakers when the atrioventricular (AV) delay was prolonged. However, diastolic mitral regurgitation associated with first-degree AV block has not been fully studied. We examined transmitral blood flow in 24 patients with first-degree AV block and normal cardiac function (ages 35.3 ± 17.4 years), and in nine patients with DDD pacemakers and normal cardiac function (ages 73.1 ± 8.1 years), using pulsed Doppler echocardiography. Diastolic mitral regurgitation was observed in 19 of 24 patients with first-degree AV block. Although PQ interval was shortened from 0.32 ± 0.06 to 0.20 ± 0.05 seconds (P < 0.01) after 1 mg atropine sulfate IV, the interval between P wave (ECG) and the beginning of diastolic mitral regurgitation did not change, while the duration of diastolic mitral regurgitation was shortened from 0.15 ± 0.03 to 0.05 ± 0.03 seconds (P < 0.01). There was a significant correlation between changes in PQ interval and changes in the duration of diastolic mitral regurgifation (r = 0.92, P < 0.001). Although cardiac output (3.9 ± 0.05 L/min) and pulmonary capillary wedge pressure (5.1 ± 1.5 mmHg) were normal in all patients with pacemakers, diastolic mitral regurgitation was observed when the AV delay was prolonged. The critical PQ interval for the appearance of diastolic mitral regurgitation was 0.23 ± 0.01 seconds. In patients with prolonged PQ intervals, delayed ventricular contraction following atrial contraction may be associated with mitral regurgitation in the presence of a reversed AV pressure gradient. The results of this study suggest that diastolic mitral regurgitation occurs not only in patients with DDD pacemakers, but also with AAIR pacemakers when the PQ interval is prolonged. The occurrence of diastolic mitral regurgitation is associated with the pacing mode or the setting of AV delay.     

Transient Manifestation of Antegrade Conduction Over a Kent Bundle After Ventricular Pacing

Postventricular Pacing Preexcitation. Antegrade conduction over a Kent bundle was transiently manifested after the cessation of ventricular overdrive pacing in two patients; otherwise, the heart behaved as if it had a concealed accessory pathway. Conventional electrophysiologic study suggested either a longitudinal dissociation of an accessory pathway or closely located double accessory pathways. The mechanism of the transient manifestation of ventricular preexcitation was discussed. (J Cardiovasc Electrophysiol, Vol. 3, pp. 423–430, October 1992)     

Prediction of Optimal Atrioventricular Delay in Patients with Implanted DDD Pacemakers

In patients with an implanted DDD pacemaker (PM), the atrial contribution may be interrupted by too short an atrioventricular (AV) delay, and filling time may be shortened by too long an AV delay. The AV delay at which the end of the A wave on transmitral flow coincides with complete closure of the mitral valve may be optimal. The subjects were 15 patients [70.3+/-12.3 (SD) years old] with an implanted DDD PM. Cardiac output (CO) and pulmonary capillary wedge pressure (PCWP) were measured by Swan-Ganz catheter. Transmitral flow was recorded by pulsed Doppler echocardiography. AV delay was prolonged stepwise by 25 msc. When the AV delay was set at 155+/-26 ms, the end of the A wave coincided with complete closure of the mitral valve. When the AV delay was prolonged 25, 50, 75, and 100 ms from this AV delay, the interval between the end of the A wave and complete closure of mitral the valve was prolonged 16+/-5, 39+/-6, 65+/-4 and 88+/-5 ms, respectively (r = 0.97, P<0.0001) and diastolic mitral regurgitation was observed during this period. Thus, the optimal AV delay may be predicted as follows: the slightly prolonged AV delay minus the interval between the end of the A wave and complete closure of the mitral valve. When the AV delay was set at 215 ms, there was a significant positive correlation between the predicted optimal AV delay (166+/-23 ms) and the optimal AV delay (CO: 161+/-26 msec, r = 0.93, P<0.0001, PCWP: 161+/-28 msec, r = 0.95, P<0.0001). In conclusion, optimal AV delay can be predicted by this simple formula: slightly prolonged AV delay minus the interval between end of A wave and complete closure of mitral valve at the AV delay setting.     

LEVELS OF VITAMIN D-METABOLITES IN A CASE OF ACRO-OSTEOLYSIS SYNDROME

ABSTRACT. On the initial examinations of a 17-year-old boy with acro-osteolysis syndrome, the levels of serum 25-hydroxycholecalciferol (25OHD) were low, whereas serum levels of 1,25-dihydroxycholecalciferol (1,25(OH)₂D) were normal. Administration of 1α-hydroxycholecalciferol (1OHD) failed to improve the osteoporosis, although the serum 25OHD level rose to normal. It is unlikely that impaired bone remodeling due to abnormal vitamin D-metabolism was a primary defect in our patient.     

Lack of genetic association between cholesteryl ester transfer protein and Japanese sporadic Alzheimer's disease

Background: Cholesteryl ester transfer protein regulates the plasma high density lipoprotein cholesterol level, which is considered to play an antiatherogenic role in humans. The presence of apolipoprotein E epsilon4 allele is a strong risk factor for developing Alzheimer’s disease (AD). Since apolipoprotein E is a regulator of lipid metabolism, it is reasonable to assume that lipids play important roles in the pathogenesis of AD. Methods: We studied the relationship between polymorphisms of the cholesteryl ester transfer protein gene and risk for AD, analyzing two common polymorphisms of the gene and the relationship between them and plasma cholesterol level control samples. Results: These polymorphisms showed no association with risk for AD. In rs5882, there was no significant difference in the mean plasma cholesterol concentrations found between patients with the A/A, A/G and G/G genotype. For rs2303790, no significant difference in the mean baseline cholesterol concentrations was found between patients with the A/A genotype and carriers of the G allele. Conclusion: Our study indicates that these polymorphisms, rs5882 and rs2303790 were not associated with risk for AD. We also pointed out that these two polymorphisms do not affect plasma cholesterol levels in our Japanese AD samples.     

Nocturnal enuresis and overactive bladder in children: An epidemiological study

AIMS: To investigate the prevalence and characteristics of nocturnal enuresis (NE) and to examine the prevalence of overactive bladder (OAB) symptoms in primary schoolchildren. METHODS: After conducting an anonymous questionnaire survey about voiding habits and bowel habits in primary schoolchildren, a total of 6917 schoolchildren belonging to 11 primary schools were randomly enrolled in the survey. According to the International Continence Society, we defined NE as any involuntary loss of urine during sleep, occurring more frequently than once per month. Children with NE were subdivided into two groups, monosymptomatic NE (MNE) and enuretic syndrome (ES). To evaluate the characteristic differences of MNE and ES, we assessed the relationships between NE and voiding habits, and episodes of cystitis and constipation. Overactive bladder was defined as increased daytime frequency and/or urge urinary incontinence, and its prevalence was investigated. RESULTS: The response rate to the questionnaire was 76.4%. The prevalence of NE was 5.9% and was inversely related to increasing age. Monosymptomatic NE comprised 59.4% of NE cases. The annual spontaneous resolution rate of MNE was higher than that of ES. Increased daytime frequency, a history of cystitis and infrequent bowel habits were not related to MNE, but were significantly related to ES. The prevalence of OAB was 17.8%. Children with a history of cystitis had a significantly higher rate of OAB than children without it. CONCLUSIONS: Overall, NE and OAB were detected in 5.9% and 17.8% of primary schoolchildren, respectively. The link between NE and OAB symptoms, urinary tract infections and constipation deserves more attention.