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Biomedical subjects

Nozomu Sasaki

Publications and source records attributed to Nozomu Sasaki.

At least 19 recordsLinked to original sources

Comprehensive genetic analysis of relevant four genes in 49 patients with Marfan syndrome or Marfan-related phenotypes.

In order to evaluate the contribution of FBN1, FBN2, TGFBR1, and TGFBR2 mutations to the Marfan syndrome (MFS) phenotype, the four genes were analyzed by direct sequencing in 49 patients with MFS or suspected MFS as a cohort study. A total of 27 FBN1 mutations (22 novel) in 27 patients (55%, 27/49), 1 novel TGFBR1 mutation in 1 (2%, 1/49), and 2 recurrent TGFBR2 mutations in 2 (4%, 2/49) were identified. No FBN2 mutation was found. Three patients with either TGFBR1 or TGFBR2 abnormality did not fulfill the Ghent criteria, but expressed some overlapping features of MFS and Loeys-Dietz syndrome (LDS). In the remaining 19 patients, either of the genes did not show any abnormalities. This study indicated that FBN1 mutations were predominant in MFS but TGFBRs defects may account for approximately 5-10% of patients with the syndrome.

Activin Receptors, Type I↗

Cardiac rest and reserve function in patients with Fontan circulation.

OBJECTIVES: In the present study, we systematically tested cardiac rest and reserve function in patients with Fontan physiology to check for inherent limitations of this circulation. BACKGROUND: Details of the mechanisms of cardiac performance that could account for adverse outcome after Fontan surgery are not well understood. METHODS: The subjects were 17 Fontan patients with good functional status (Fontan group) and 20 patients with normal two-ventricle circulation (control group). We examined baseline ventricular contractility, diastolic function, and loading factors, and examined changes in those parameters in response to increased heart rate (HR) due to atrial pacing and in response to beta-adrenergic stimulation, using ventricular pressure-area relationships during preload reduction. RESULTS: At baseline, the Fontan patients exhibited minimal abnormality of cardiac properties, but the significant increase in afterload resulted in decreased cardiac index. In addition, Fontan circulation was associated with limited inotropic response and worsening of diastolic filling with increased HR, leading to decreased systolic pressure and elevation of central venous pressure at higher HRs (p < 0.05 vs. control). Furthermore, beta-adrenergic reserve was markedly decreased in the Fontan group, compared with controls, owing to limited preload reserve rather than limited contractile reserve. CONCLUSIONS: Because normal ventricular-vascular interaction and augmentation of cardiac performance during increased HR and adrenergic stimulation are important for maintaining cardiac output and exercise capacity, the present results may have important implications for the mechanisms underlying adverse outcome after Fontan surgery. Thus, improvement of long-term prognosis of patients after Fontan surgery requires the development of medical interventions that can overcome such limitations inherent in Fontan circulation.

Child↗

Comparison of PCR for sputum samples obtained by induced cough and serological tests for diagnosis of Mycoplasma pneumoniae infection in children.

Passive agglutination (PA) and immunoglobulin M (IgM), IgA, and IgG enzyme-linked immunosorbent assays (ELISAs) for the diagnosis of Mycoplasma pneumoniae were compared with PCR testing of sputum samples obtained from children with lower respiratory tract infections. The sensitivity and specificity of PA were 80.3% and 92.3% at a titer of 1:80. ELISA was found to be less sensitive than PA.

Agglutination↗

The inhibitory effect of disodium cromoglycate on the growth of Chlamydophila (Chlamydia) pneumoniae in vitro.

Chlamydophila (Chlamydia) pneumoniae is associated with asthma and several other respiratory illnesses. Disodium cromoglycate (DSCG) is known to inhibit both immediate and late asthmatic responses. In this study, the inhibitory effect of DSCG on the growth of C. pneumoniae was examined by minimum inhibitory concentration (MIC) and pre-inoculation minimal cidal concentration (MCC) assays using HL cells and C. pneumoniae AR-39. DSCG below the clinically relevant concentration inhibited the growth of C. pneumoniae in a dose-dependent manner in both the MCC and MIC assays. The inhibitory effect was also time-dependent in the MCC assay at 20 mg/ml of DSCG. These results warrant further clinical study on the connection between C. pneumoniae infections and use of DSCG.

Cell Line↗

[Childhood Graves' disease].

Because of the low frequency of childhood Graves' disease, detailed evidence-based clinical studies have not been reported. Practical clinical work has been performed on the basis of adult clinical references. Therapeutic management includes antithyroid drugs, surgical thyroidectomy and radiologic therapy. Recently in the U.S.A. radiotherapy has become the recommended course of action, even for childhood Graves' disease, whereas in Japan, antithyroid drug therapy is the primary course of action for childhood Graves' disease. In some cases, thyroidectomy is performed following drug therapy. Methimazole (MMI) and propylthiouracil (PTU) have been used, however, MMI is the preferred drug treatment. Compared to PTU, MMI is administered once a day and the frequency of side effects is lower than that of PTU. Evaluation of the TSH receptor antibody value before administration of antithyroid drugs is very useful in estimating the duration of the treatment. No appropriate index has been established guiding when to quit antithyroid drug therapy.

Antithyroid Agents↗

Arterial hemodynamics in patients after Kawasaki disease.

BACKGROUND: Histopathological findings in the acute stage of Kawasaki disease (KD) indicate widespread vascular inflammation that involves not only coronary arteries but also systemic arteries. This may cause changes in systemic arterial wall properties after KD, which could have adverse effects on arterial hemodynamics (an important predictor of cardiovascular morbidity and mortality). METHODS AND RESULTS: Systemic arterial hemodynamics were investigated by measuring aortic input impedance during cardiac catheterization in 42 KD patients who had developed coronary artery lesions (CALs) in the acute stage of KD. The KD patients were subdivided into 2 groups according to the angiographic findings (group 1A, 26 patients with persistent CALs; group 1B, 16 patients with regressed CALs), and results were compared with those of 36 referents (group 2). Compared with referents, characteristic impedance was significantly higher for KD patients (137.0+/-5.1, 125.7+/-8.2, and 97.9+/-4.1 dyne x s x cm(-5) x m2 for group 1A, group 1B, and group 2, respectively), and total peripheral arterial compliance indexed to age-specific values was significantly lower for KD patients (group 1A 72.9+/-4.2% of normal; group 1B 70.6+/-5.9% of normal; group 2 97.7+/-4.0% of normal; for both variables, P<0.05 for each KD group versus group 2; P=NS between KD groups), which suggests that both central and peripheral arterial wall stiffness increase after KD regardless of persistence of CALs. Also, indices of arterial wave reflection (reflection coefficient, reflection factor, and augmentation index) were all significantly higher in KD patients than in referents (P<0.05), with the result that the aortic pressure waveforms of the present KD patients resembled those generally observed in the elderly. In addition, levels of circulating markers of endothelial dysfunction (ACE and von Willebrand factor) were associated with increased vascular stiffening in KD patients but not in referents. CONCLUSIONS: These results indicating abnormal arterial hemodynamics after KD highlight the importance of regular monitoring of the systemic arterial bed and potentially relevant cardiovascular events in long-term follow-up of KD.

Adolescent↗

Past 10-year status of insulin therapy for preschool-age Japanese children with type 1 diabetes.

The aim of this study was to evaluate the past 10-year status of insulin therapy for preschool-age children with type 1 diabetes in Japan. One-hundred and forty-two patients who had been diagnosed at less than 5 years of age within the past 10 years (1993-2002) at 36 hospitals were registered in this study on April 2003. The methods of daily insulin therapy and episodes of severe hypoglycemia during the preschool period were investigated. Eighty-six (60.6%) children were treated with a pen-type device and 56 (39.4%) were treated with a syringe-type device. The once-a-day insulin regimen was used for 2, a twice-a-day regimen for 104, a three-times-a-day for 28 and a four-times-a-day for 8. Episodes of severe hypoglycemia were recorded in nearly half of the subjects, and one-fourth of the subjects had repeated episodes. One hundred and eleven of their parents were questioned regarding the degree of psychosocial stress experienced during the care of their children. Most parents worried about the glycosylated hemoglobin value at each hospital visit. They were next very afraid of nocturnal severe hypoglycemia, independent of any actual experience. These results suggest that although insulin therapy can involve various methods, the important point is to simultaneously provide good glycemic control and prevent severe hypoglycemia, especially during this age.

Child, Preschool↗

Vasopressin in the treatment of vasodilatory shock in children.

BACKGROUND: Many recent studies suggest that vasopressin deficiency is an important cause of catecholamine-resistant hypotension with vasodilation in adults, but little is known about vasopressin deficiency in children. METHODS: To clarify the usefulness of vasopressin administration in pediatric cathecolamine-resistant hypotension with preserved ventricular contractility, urinary output and blood pressure response to vasopressin were retrospectively analyzed in 12 consecutive patients (15 instances) who were treated with vasopressin. The causes of vasodilation were central nervous system disturbance (n = 5), side-effect of drug (n = 5), and infection (n = 5). Plasma vasopressin concentration was measured six times before vasopressin administration and five times during vasopressin administration. RESULTS: Patients were divided into four groups according to their response to vasopressin administration. In group 1 (n = 5), urinary output increased to > 3 mL/kg per h within 3 h after vasopressin administration. In group 2 (n = 4), urinary output increased to > 3 mL/kg per h from 3 to 5 h after vasopressin administration. In group 3 (n = 4), urinary output did not increase to > 3 mL/kg per min within 5 h after vasopressin administration, but systolic blood pressure increased to > 120% of the level at the time of vasopressin administration. All remaining patients were classified into group 4 (n = 3). Plasma vasopressin concentration were low considering the markedly hypotensive state in all six instances. Plasma vasopressin concentration during vasopressin administration were significantly increased compared with before administration (P < 0.05). No apparent side-effects were observed in this series. CONCLUSION: Vasopressin deficiency may occur in catecholamine-resistant hypotension of pediatric patients due to various causes including central nervous system disturbance, drug induced hypotension and sepsis. Small doses of vasopressin administration seems to be very effective in such conditions by increasing blood pressure and urinary output.

Adolescent↗

Biosynthesized tea polyphenols inactivate Chlamydia trachomatis in vitro.

Biosynthesized tea polyphenols showed antichlamydial activity against Chlamydia trachomatis D/UW-3/Cx and L2/434/Bu using cell culture. The most active compounds were (-)-epigallocatechin gallate and (-)-epicatechin gallate, followed by (-)-epicatechin (EC). (+)-Epicatechin and (-)-epigallocatechin were intermediate. EC was the least toxic. These results warrant evaluation of tea polyphenols as topical antichlamydial agents.

Anti-Bacterial Agents↗

Distribution of Chlamydia trachomatis serovars among female prostitutes and non-prostitutes in Thailand, and non-prostitutes in Japan during the mid-90s.

The distribution of Chlamydia trachomatis serovars in Thailand and Japan during the same period of the mid-90s was determined. Seventy-one C . trachomatis specimens isolated from female patients who visited the Venereal Diseases Center at Bangkok, Thailand in 1994 were used in this study. Of these, 56 patients were prostitutes. Forty-seven specimens obtained from female non-prostitutes who attended the Department of Obstetrics and Gynecology, Saitama Medical School, Japan during the period from 1993 to 1995 were also used in this study. DNA was extracted from these specimens and typing of C. trachomatis serovars was performed by the polymerase chain reaction-restriction fragment length polymorphism method. The identified serovars among prostitutes of Thailand (n = 56)/non-prostitutes of Thailand (n = 15)/non-prostitutes of Japan (n = 47) were as follows: Ba 1/0/2, D 8/1/15, E 11/2/8, F 16/9/8, G 4/0/7, H 3/2/3, I 1/0/1, J 3/0/0, and K 10/1/4. Serovar F was most prevalent (35.2%) in both prostitutes and non-prostitutes from Thailand, followed by serovar E (18.3%). On the other hand, serovar D was the most frequent serovar in non-prostitutes in Japan (31.9%) followed by serovars F (17.0%) and E (17.0%). A difference in the distribution of C. trachomatis serovars of Thailand and Japan was noted.

Adolescent↗

Clinical usefulness of culturing H. pylori from gastric juice in patients with severe neurological impairment.

OBJECTIVES: To clarify the diagnostic usefulness of culturing Helicobacter pylori from gastric juice for the diagnosis of H. pylori infection in patients with neurologic impairment. METHODS: Seventy-five patients (44 males, 31 females) with severe neurologic impairment admitted to Tokyo Children's Rehabilitation Hospital were included in this study. Samples of gastric juice obtained from these patients were put into Helicoporter, a transport medium for H. pylori. Samples were then centrifuged and sediment was inoculated onto a culture medium. Polymerase chain reaction assays were performed on 41 gastric juice samples for comparison with results of culture. Tests for occult blood in gastric juice and serum anti-H. pylori immunoglobulin G were tested for all patients. RESULTS: H. pylori was isolated from 33 of 75 gastric juice samples by culture (positive rate, 44%). There was agreement between results of culture and polymerase chain reaction assays in 38 samples (93%). Twenty-two samples were positive and 16 were negative by both methods. In H. pylori immunoglobulin G-positive patients, occult blood was more frequently found in the culture-positive group (92%) than in the culture-negative group (59%) (P <0.025). The rate of detection of occult blood in immunoglobulin G-negative patients was similar in the culture-positive group (44%) and culture-negative group (50%). CONCLUSIONS: The combination of a culture method using gastric juice and a serum H. pylori immunoglobulin G assay was not invasive and clinically useful for evaluating H. pylori infections among handicapped patients.

Adolescent↗

A questionnaire survey on the use of quick-acting insulin analog in Japanese children and adolescents with type 1 diabetes.

BACKGROUND: The aim of this study was to investigate the actual condition of quick-acting insulin analog (Q) in Japanese children and adolescents with type 1 diabetes. METHODS: Forty-seven physicians who managed 944 patients under 18 years of age with type 1 diabetes, were requested to complete questionnaires in May 2002 regarding the use of Q. RESULTS: Q was administered to 417 out of 944 (44%) patients. The details of the daily insulin regimens were as follows: three doses of Q with basal insulin in 116 of 403 (29%), three doses of Q with more than twice the basal insulin in 69 of 403 (17%), and other modifications using either Q or regular insulin (R) depending on the patients' lifestyles in 213 of 403 (53%). Q dose was equal to R after the change of insulin regimens in 232 of 290 (80%). After switching to Q from R, the basal insulin dosage increased in 113 of 341 (33%) and the number of basal insulin injections increased in 42 of 341 (12%). After the transfer to Q from R, glycemic control was judged to improve in 179 of 372 (48%), to have no significant change in 162 of 372 (44%), and to aggravate in 31 of 372 (8%). The majority of patients, (305/389; 78%), noted an improvement of quality of life (QOL) following the administration of Q. CONCLUSIONS: Q was considered to be useful in flexibility of daily insulin therapy and improvement of QOL in children and adolescents with type 1 diabetes. Insulin therapy for type 1 diabetes during childhood and adolescence should be tailored to meet individual needs.

Adolescent↗

Decreased hepatic uptake of gallium-67 citrate in haemophagocytic syndrome occurring concomitantly with capillary leak syndrome.

We describe a 4-year-old girl with haemophagocytic syndrome (HPS) in whom hepatic gallium-67 citrate (Ga-67) uptake was suppressed when the disease was in its acute phase and returned to normal when the disease was in remission. The prominent clinical feature of this case was the occurrence of systemic capillary leak syndrome (CLS). Because extravasation of plasma proteins may be the result of vascular endothelial injury in CLS, loss of hepatic Ga-67 uptake may reflect insult to the hepatic sinusoidal endothelium. This case suggests a possible role of sinusoidal endothelial cells in the mechanism of hepatic Ga-67 uptake and indicates the need for further study of Ga-67 uptake in patients with HPS.

Capillary Leak Syndrome↗