Comparison of the Clinical Characteristics of Infants with Punctate White Matter Lesions and/or Cystic Lesions.

BACKGROUND: We aimed to investigate the differences in the clinical characteristics of preterm infants with punctate white matter lesions (PWMLs) and those with cystic periventricular leukomalacia (cPVL) using term-equivalent age magnetic resonance imaging. METHODS: We conducted a retrospective case-control study to explore the clinical characteristics of infants (< 35 weeks gestation, born between 2007 and 2017 in a single Level III perinatal center) with PWML, cPVL or with PWML plus cPVL and compared them with those of gestational-age-matched controls. RESULTS: Among 602 infants, 29, 5, and 4 were assigned to the PWML group, cPVL group, and PWML plus cPVL group (PWML-cPVL group), respectively. Compared to the control group (n = 87), the PWML group had higher birth weights (p = 0.04), rates of histological chorioamnionitis (p = 0.04), vaginal delivery (p = 0.008), and early heart contraction failure (within 72 hours after birth) (p = 0.003). The cPVL group had lower umbilical blood gas base excess (p = 0.01), higher rate of late-onset circulatory collapse (p = 0.008), and higher hydrocortisone requirements (p = 0.03) than the control group (n = 15). The PWML-cPVL group had a higher rate of intraventricular hemorrhage (p = 0.03) than the control group (n = 12). In the multivariate logistic regression analysis, vaginal delivery (odds ratio [OR] = 3.5; 95% confidence interval [CI] = 1.37-9.40; p = 0.009), higher birth weight (per 1 g) (OR = 1.001; 95% CI = 1.0001-1.002; p = 0.03), and early heart contraction failure (OR = 5.4; 95% CI = 1.84-16.8; p = 0.002), were independent risk factors for PWML. CONCLUSION: Clinical characteristics of infants with PWML compared with gestational-age-matched controls differed from those with cPVL or PWML plus cPVL, as PWML were not related to severe disruption of hemodynamics.

The status of chronic lung disease diagnosis in Japan: Secondary publication.

BACKGROUND: In Japan, the definition and classification of neonatal chronic lung disease (CLD) used for its diagnosis are a combination of those used in Japan and abroad. METHODS: To clarify the current state of CLD diagnosis, a questionnaire survey was conducted. RESULTS: Half of the patients of the medical centers included in the study were diagnosed with CLD in real time, while the other half were diagnosed after discharge. In addition, in approximately 70% of the facilities, diagnosis was made after discussions various among medical teams. In approximately 80% of the centers, the chest radiography used for CLD diagnosis were evaluated by multiple doctors. Furthermore, some centers used chest X-rays that were taken at approximately 28 days of age for CLD diagnosis, whereas at other facilities, diagnosis was made regardless of time at which the chest radiography were obtained. Only a small number of centers have established criteria for determining the necessity of oxygen at the corrected age of 36 weeks, and the target saturation of peripheral oxygen levels also tend to vary for each facility. Whether the conditions wherein the patient receives respiratory support for apnea or respiratory tract diseases should be considered as CLD also differed among the facilities. CONCLUSIONS: It is necessary to reassess the definition and classification of CLD in Japan to accurately evaluate and improve the quality of respiratory management based on the long-term prognosis.

Microdeletion in Xq28 with a polymorphic inversion in a patient with FLNA-associated progressive lung disease.

Lung phenotype was reported as a novel phenotype in patients with mutations in the filamin A gene (FLNA) in 2011. FLNA mutations can result in pulmonary hyperinflation during the neonatal period or early infancy with progressive respiratory failure, culminating in a diagnosis of FLNA-associated progressive lung disease, particularly if the patient has periventricular nodular heterotopia and cardiac complications, such as patent ductus arteriosus, atrial septal defect, and pulmonary hypertension. We report the first Japanese case of FLNA-associated progressive lung disease caused by a microdeletion in Xq28 encompassing the FLNA gene with a polymorphic inversion.

Clinical Usefulness of Magnetic Resonance Imaging in Four Children With Scleroderma.

Children with localized scleroderma may have more serious sequelae than adults with the disease. In this case report, we analyzed four girls with localized scleroderma (generalized morphea) to evaluate the clinical usefulness of magnetic resonance imaging in facilitating disease control and investigating patient responsiveness to various treatments. Two of the children had flexion contracture, and two displayed a clear high-intensity area in the bone marrow under the sclerotic skin on magnetic resonance imaging. Two girls had different bilateral circumferences of the legs or forearms. These abnormalities were detected without any obvious changes in blood chemistry. Our results demonstrated that magnetic resonance imaging is useful for the evaluation of treatment effects for localized scleroderma in children.

Coexistence of diffuse panbronchiolitis and asthma: reciprocity of neutrophilic and eosinophilic inflammation.

Diffuse panbronchiolitis (DPB) and asthma are obstructive airway diseases, the former being characterized by Th1-type and the latter by Th2-type airway inflammation. Differential diagnosis is often a problem, but coexistence has rarely been reported. A 76-year-old man with asthma was admitted to our hospital because of one-month history of dyspnoea on exertion with bilateral diffuse granular shadows. He also had a history of chronic sinusitis. Auscultation of the lungs showed coarse crackles and wheezes. Laboratory data revealed an elevated total serum immunoglobulin E and a high titre of cold agglutinin. Bronchoscopic evaluations of the shadows revealed compatible pathological findings in both DPB and asthma. Low-dose macrolide caused a prompt reduction of symptoms, along with improvements in radiographic findings and pulmonary functions, whereas the eosinophilic airway inflammation transiently worsened. When DPB and asthma coexist, the balance of Th1/Th2 immune response may be reciprocally altered by therapeutic intervention.

Association of sleep-disordered breathing with decreased cognitive function among patients with dementia.

Sleep is known to be essential for proper cognitive functioning. Sleep disturbance, especially respiratory disturbance during sleep, is a risk factor for the development of dementia. However, it is not known whether hypopnoea during sleep is related to severity of cognitive function in patients already diagnosed with dementia. Considering the high prevalence of sleep problems in aged people, it is important to determine if hypopnoea during sleep contributes to dementia. In addition, it would be desirable to develop a feasible method for objectively evaluating sleep in patients with dementia. For this purpose, a simple sleep recorder that employs single or dual bioparameter recording, which is defined as a type-4 portable monitor, is suitable. In this study, a type-4 sleep recorder was used to evaluate respiratory function during sleep in 111 patients with dementia, and data suggesting a possible relationship with cognitive function levels were examined. Multivariate logistic regression was used to investigate the association of severity of dementia with sleep-disordered breathing, age, diabetes, dyslipidaemia and hypertension. It was found that the respiratory disturbance index was associated with severity of cognitive dysfunction in our subjects. Furthermore, patients younger than 80 years were more susceptible to lower cognitive function associated with sleep-disordered breathing than patients 80 years old or over, because an increase in the respiratory disturbance index was associated with deteriorated cognitive function only in the former age group. These results suggest that proper treatment of sleep apnea is important for the preservation of cognitive function, especially in patients with early-stage dementia.

[Promotion of home shifts for terminal cancer patients through intervention of visitor palliative care team].

It has been recommended that terminal cancer patients be shifted from the hospital to their homes. In our hospital, a visitor palliative care team was started for the purpose of the early introduction of palliative care, and home shifts were promoted. The results of home shifts by the visitor palliative care team from 2008 to 2012 were examined. Home shifts were possible for 27 cases out of 108 cases intervened. In 12 cases, there were at-home deaths, and the median at-home period was 55 days. In the group that could not be shifted, the at-home death rate and application rate of nursing care insurance were low. Additionally, the length of stay (median) for patients who died in hospitalization was 8 days for the group that could be shifted and 17 days for the group that could not be shifted. It was felt that effective communication with local health care facilities is important for a successful home shift. Early and adequate preparations for the treatment and care of terminal cancer patients undergoing home shift are important, and in this regard, a review of the current provisions of nursing care insurance is necessary.

[Renal actinomycosis with pneumonia].

A 55-year-old man was hospitalized for pneumonia. His fever did not subside despite administration of antibiotics ; therefore, he was referred to our hospital. A chest radiograph and thoracic computed tomography showed multiple tubercles ; abdominal computed tomography (CT) showed left renal abscess. The patient's temperature fell after antibiotic administration, but inflammation reaction exacerbated. Abdominal CT showed inflammation spreading to the subcutaneous tissues. We considered renal resection, but the patient could not be administered general anesthesia because of low breathing function caused by pneumonia. We attempted open drainage and wedge resection of the left renal under local anesthesia ; but we were not able to identify the infectious bacteria. Four days later, the patient had blood poisoning and died because of deterioration of breathing function. Actinomyces was detected in the lungs and the kidneys by pathological examination.

Arabidopsis mitochondrial protein TIM50 affects hypocotyl cell elongation through intracellular ATP level.

The plant hypocotyl is an excellent model for the analysis of cell elongation. We have characterized a knockout mutant of the Arabidopsis TIM50 gene that showed a reduction in the hypocotyls length of etiolated seedlings. We also found that a knockout of TIM50 caused enlargement and deformation of the mitochondrial structure and a reduction in intracellular ATP levels. TIM50 is a component of the mitochondrial TIM23 inner membrane protein complex and is involved in the import of mitochondrial proteins. The short hypocotyl phenotype was recovered by the addition of Compound C, an inhibitor of AMPK. Thus, the mitochondrial ATP level controls cell elongation in Arabidopsis hypocotyls through possible signaling via AMPK.

Simple construction of plant RNAi vectors using long oligonucleotides.

RNA interference (RNAi) is one of the most important technologies currently available for the analysis of gene function. However, despite the development of various methods, it is still difficult to construct RNAi vectors for plants with the appropriate inverted repeat fragments to produce double-stranded RNA for knockdown experiments. To solve this problem we have developed an easy and simple method to make RNAi constructs using two long oligonucleotides consisting of partially complementary sequences without the need for PCR amplification and multiple cloning steps. CHS RNAi plants generated using this method showed yellow seed color and a decrease in antocyanin content--phenotypes typically observed in CHS loss-of-function mutants. Moreover, we demonstrated specific knockdown of both the PHYA and PHYB genes using a tandem RNAi construct. This method thus represents a powerful tool for gene knockdown in plants.

Accumulation of gallium-67 within mature and immature teratoma in pediatric patients: investigation for the uptake mechanism.

OBJECTIVE: We encountered cases of mature and immature teratoma with positive uptake of (67)Ga. The objective of this study is to investigate the mechanism of (67)Ga accumulation within mature and immature teratomas by comparing the findings of gallium scan, computed tomography (CT), and autoradiography of surgical specimens with the pathological findings. METHODS: The subjects comprised 14 children who underwent surgical resection for intra-abdominal mature and immature teratomas, which were histologically proved to be of the mature and immature subtype. Their age ranged from 24 days to 14 years. The origins of the mature teratomas consisted of seven ovaries including one bilateral case, two retroperitoneal, and two sacrococcygeal regions. The origins of the immature teratomas were retroperitoneum in two cases, an ovary and a sacrococcygeal region. Complete surgical excision was feasible in all children. They underwent both gallium scan and CT prior to surgery. Single-photon emission computed tomography was added in some cases. For two gallium-positive cases, radiography and scintigraphy (autoradiography) of the resected specimen were performed. RESULTS: Of the 14 children, 5 (one with immature and four with mature subtype) showed positive (67)Ga uptake within tumors, which originated from the retroperitoneum in the 3 boys, and from the ovary in the 2 girls. All had typical CT findings of teratoma, including calcifications, fat components, cystic areas, and solid parts. (67)Ga accumulation in the four mature teratomas appeared discretely strong, and was considered to correspond with intralesional calcifications. However, in the remaining one immature teratoma, the gallium distribution was diffuse within the tumor. The comparison between radiography and autoradiography of the resected mature teratomas confirmed the correlation between the intralesional calcifications and areas of (67)Ga accumulation. CONCLUSIONS: A high-uptake ratio of (67)Ga in benign teratoma was indicated. A close correlation between gallium scan and CT helps to ascertain whether (67)Ga uptake results from malignant and/or immature elements, or mature tissue components.

[A case of intractable chronic abdominal pain caused by hepatomegaly associated with primary amyloidosis, successfully treated with celiac plexus block].

A 51-year-old female previously diagnosed as primary amyloidosis suffered from recurrent abdominal pain. The result of thorough examination indicated that the main cause of the pain was severe hepatomegaly. Continuous venous administration of narcotics and other alternative therapies did not provide symptomatic relief, and thus the patient was treated with celiac plexus block, which resulted in effective pain control and improved ADL level. Though the procedure of celiac plexus block is simple and celiac plexus block is applicable without causing severe complication, it is not widely used. From this case, it is considered that celiac plexus block is one of the most effective means to relieve intractable pain associated with both benign malady and abdominal malignant tumor.

Utility of computed tomography in identifying an ectopic thyroid in infants and pre-school children.

In purpose of identifying thyroid tissue in patients with congenital hypothyroidism during childhood, ultrasonography is usually used as a screening examination, and scintigraphy is performed secondarily. Though these methods are useful, it is not easy to identify the accurate location of thyroid tissue by these methods. We previously reported the utility of computed tomography (CT) in identifying thyroid tissue in four cases of congenital hypothyroidism. The purpose of this study is to investigate whether CT is useful in identifying thyroid tissue, compared to ultrasonography or scintigraphy. Nineteen cases (0 month to 18 years of age) that were suspected to have ectopic thyroid tissue or thyroid agenesis on ultrasonography were examined by CT. CT was useful in diagnosing ectopic thyroid tissue or thyroid agenesis in all the cases, whereas ultrasonography or scintigraphy was less accurate in this purpose in seven cases. Plain CT had a difficulty in identifying thyroid tissue in two cases, in which contrasted image was required. This study showed that CT, especially an enhanced CT, is useful in identifying thyroid tissue, when the gland is not identified in the normal position by ultrasonography.