Extended septal myectomy for midventricular obstruction in hypertrophic cardiomyopathy.

Midventricular obstruction (MVO) is a rare form of hypertrophic cardiomyopathy (HCM). While surgical treatment for HCM is among the most technically challenging cardiac operations for acquired disease, surgery for MVO is rarely reported. A 38-year-old man was admitted to our hospital with a cough and dyspnea. Transthoracic and transesophageal echography and computed tomography revealed extensive left ventricular hypertrophy, extending from the anteroseptal wall to the apex, and marked papillary muscle hypertrophy. We underwent septal myectomy via aortotomy (Morrow procedure) and apical surgery. Extended myectomy provides the best exposure to the hypertrophied septum and improves the functional status of patients.

Does preoperative enhanced CT predict requirement of intestinal resection in the patients with incarcerated myopectineal hernias containing small bowel?

PURPOSE: Bowel wall enhancement on CT imaging is considered one of the useful features for the prediction of the presence of irreversible ischemic change in patients with small bowel obstruction. However, the applicability of CT imaging in patients with incarcerated hernias has not been investigated in detail. The aim of this retrospective study was to evaluate the feasibility of preoperative CT findings for the prediction of the presence of irreversible ischemic change in patients with incarcerated hernias containing small bowel. METHODS: Included in this study were 76 patients who underwent surgery for preoperatively diagnosed incarcerated hernias containing small bowel (27 inguinal hernias, 37 femoral hernias and 12 obturator hernias) at our hospital between January 2011 and June 2020. The preoperative clinicoradiological features were compared between the groups, and predictors for intestinal resection were evaluated. RESULTS: Nineteen patients required intestinal resection (Resection group), and the other 57 patients did not require intestinal resection (Nonresection group). Multivariate analyses revealed that age ≥ 80 years (p = 0.018, odds ratio = 6.604) and the absence of bowel wall enhancement (p = 0.032, odds ratio = 51.200) were independent predictors for intestinal resection. In resected specimens, all patients with an absence of bowel wall enhancement on preoperative enhanced CT had ischemic changes extending beyond the muscularis propria. CONCLUSIONS: Preoperative enhancement CT yields useful information for the prediction of the presence of irreversible ischemic change in patients with incarcerated hernias containing small bowel.

Japanese civilian and US military interaction in the evacuation of casualties from Camp Fuji.

Historically, if US soldiers at Camp Fuji become severely ill or suffer trauma, they are transported by the ground ambulance, as the doctor-led air ambulance in eastern Shizuoka has never been permitted to land at Camp Fuji. However, it is widely recognised that severely ill or traumatised patients require time-dependent medical management. It was therefore agreed to undertake a joint exercise between the US medical assets of Camp Fuji and the doctor helicopters in eastern Shizuoka prefecture in evacuating a simulated severely ill or traumatised US soldier. The aim of this article is to describe the background and rationale between this collaboration between the civilian Japanese air ambulance and the US medical assets in Camp Fuji.

Characteristics of IgG subclasses and complement deposition in BP230-type bullous pemphigoid.

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease. BP180 is the primary autoantigen of BP, and in a portion of BP cases, BP230 is the only target of autoantibodies. Such BP is called BP230-type BP. BP230-type BP tends to show milder clinical phenotypes than conventional BP, but the reason is unclear. The pathogenic roles of autoantibodies and complement activation have been shown in conventional BP, but the distribution of IgG subclasses and the degree of complement deposition in BP230-type BP remain unclear. OBJECTIVE: To compare the distribution of IgG subclasses and the degree of complement deposition in BP230-type BP with those in conventional BP with autoantibodies to BP180 and BP230 (BP180-BP230-type BP). METHODS: The diagnosis of BP was confirmed by the histopathology of the lesions, the deposition of IgG and complement in the perilesional skin and the presence of circulating autoantibodies to BP180 and BP230. The disease severity was determined by bullous pemphigoid disease area index. The deposition of IgG subclasses and complement deposition were examined by direct immunofluorescence of the perilesional skin in 6 BP230-type BP cases and 11 BP180-BP230-type BP cases. RESULTS: Sixty seven percent of BP230-type BP cases show a mild clinical phenotype. All BP230-type BP cases and 82% of BP180-BP230-type BP cases were found to demonstrate the clear deposition of IgG4 at the basement membrane zone of skin specimens. Notably, the deposition of IgG1 and IgG3 was faint or negative in all of the BP230-type BP cases, whereas they were clearly detected in 91% and 64% of the BP180-BP230-type BP cases, respectively. The deposition of complement C3 tended to be weaker in BP230-type BP than in BP180-BP230-type BP. CONCLUSION: The mild clinical phenotype of BP230-type BP may correlate with the weaker deposition of IgG1, IgG3 and complement in the skin lesions.

NDUFAF3 variants that disrupt mitochondrial complex I assembly may associate with cavitating leukoencephalopathy.

Genetic abnormalities in mitochondrial complex assembling factors are associated with leukoencephalopathy. We present a 1-year-old girl with consciousness disturbance after a respiratory infection. Brain MRI revealed leukoencephalopathy with bilaterally symmetrical hyperintensity in the substantia nigra, medial thalamic nuclei, and basal nuclei, as well as cavities in the cerebral white matter and corpus callosum. Lactate levels in the spinal fluid were high, while magnetic resonance spectroscopy of the cerebral white matter and basal nuclei showed high peak lactate levels, suggesting mitochondrial dysfunction. The respiratory enzyme activity of complex I was reduced to 17% to 21% in skeletal muscle. Whole exome sequencing identified compound heterozygous variations in NDUFAF3, involved in the assembly of mitochondrial complex I (c.342_343insGTG:p.117Valdup, c.505C > A:p.Pro169Thr). Two-dimensional, blue-native polyacrylamide gel electrophoresis (PAGE) and sodium dodecyl sulfate-PAGE revealed reductions in Q-module (NDUFS2, NDUFS3, and NDUFA9) and P-module (NDUFB10 and NDUFB11) subunits, indicating disruption of mitochondrial complex I assembly. Our report expands the spectrum of clinical phenotypes associated with pathogenic variants of NDUFAF3.

The question of whether or not to perform therapeutic hypothermia: A case of neonatal spinal cord injury.

Neonatal spinal cord injury is an extremely rare perinatal complication that often occurs concurrently with hypoxic ischemic encephalopathy (HIE), further complicating diagnosis of spinal cord injury. Although therapeutic hypothermia for moderate to severe HIE is widely recommended in Japan, it is difficult to determine whether it satisfies the neurological findings-related entry criteria in some patients.We describe a female infant with neonatal spinal cord injury after forceps delivery, who underwent therapeutic hypothermia upon diagnosis of HIE. The Apgar scores were 5 at 1 min, 6 at 5 min, and not recorded at 10 min. Blood gas analysis of her umbilical artery was not performed. Since respiratory failure, hypotonia and the absence of primitive reflexes were found at 2 hours after birth, she was initially diagnosed with moderate HIE and underwent a therapeutic hypothermia. Magnetic resonance imaging after therapeutic hypothermia revealed the spinal cord was narrowed from the lower medulla oblongata to the upper cervical cord. Thus she was diagnosed with an upper spinal cord injury at that time.Some patients with neonatal spinal cord injuries satisfy the criteria for therapeutic hypothermia. When neonates with asphyxia present with prolonged respiratory failure and hypotonia, spinal cord injury should be considered in the differential diagnosis. Thus, an early MRI is vital for the diagnosis of spinal cord injury.

Longitudinal association of dentition status with dietary intake in Japanese adults aged 75 to 80 years.

Limited information is available on the temporal association between dentition status and dietary intake. The aim of this 5-year prospective cohort study was to investigate whether impaired dentition was associated with subsequent decline in dietary intake in older Japanese adults. Two hundred and eighty-six community-dwelling Japanese individuals, all aged 75 years at baseline, were included in the study. Functional tooth units (FTUs), defined as a pair of opposing natural or prosthetic teeth excluding third molars (range: 0-14), were counted on the basis of baseline dental examinations. Individuals with ≤5 FTUs were defined as having impaired dentition. Dietary intake was assessed at baseline and 5 years later, using a validated dietary questionnaire. Robust regression analyses were used to evaluate the differences in change in dietary intake between participants with and without impaired functional dentition, after adjustment for potential confounders. Sixty-one study participants (21·3 %) were defined as having impaired dentition. Overall, mean values for all estimated dietary variables (energy, nutrients and food groups) declined over time. Notably, individuals with impaired dentition demonstrated a significantly (P < 0·05) greater degree of decline in the intake of multiple nutrients (protein, sodium, potassium, calcium, vitamin A, vitamin E and dietary fibre) and food groups (vegetable and meat) than those without impaired dentition, after adjusting for potential confounders. The results of this study describe the temporal association of impaired dentition with the decline in selected nutrient and food group intake among older Japanese adults.

Molecular genetic analysis of 30 families with Joubert syndrome.

Joubert syndrome (JS) is rare recessive disorders characterized by the combination of hypoplasia/aplasia of the cerebellar vermis, thickened and elongated superior cerebellar peduncles, and a deep interpeduncular fossa which is defined by neuroimaging and is termed the 'molar tooth sign'. JS is genetically highly heterogeneous, with at least 29 disease genes being involved. To further understand the genetic causes of JS, we performed whole-exome sequencing in 24 newly recruited JS families. Together with six previously reported families, we identified causative mutations in 25 out of 30 (24 + 6) families (83.3%). We identified eight mutated genes in 27 (21 + 6) Japanese families, TMEM67 (7/27, 25.9%) and CEP290 (6/27, 22.2%) were the most commonly mutated. Interestingly, 9 of 12 CEP290 disease alleles were c.6012-12T>A (75.0%), an allele that has not been reported in non-Japanese populations. Therefore c.6012-12T>A is a common allele in the Japanese population. Importantly, one Japanese and one Omani families carried compound biallelic mutations in two distinct genes (TMEM67/RPGRIP1L and TMEM138/BBS1, respectively). BBS1 is the causative gene in Bardet-Biedl syndrome. These concomitant mutations led to severe and/or complex clinical features in the patients, suggesting combined effects of different mutant genes.

Spatiotemporal fluctuations in natural selection acting on the gall-parasitic aphid Tetraneura sorini.

The measurement of the selection gradient is crucial for understanding the magnitude of selection acting directly on a trait and predicting the evolutionary trajectory of that trait. This study evaluated the selection gradient acting on the morphology of the gall-parasitic aphid Tetraneura sorini during the galling process and compared the strength among populations. Gall formers (first instars) frequently fight with conspecifics or heterospecifics for usurping incipient galls using their well-developed hind legs. First instars that successfully acquired galls were found within galls, whereas those that failed were found dead on leaf surfaces. Selection gradients were estimated using logistic stepwise regression and partial least square (PLS) regression. Calculated selection differentials indicated that first instars that secured galls were larger in body size than failed individuals through all populations. However, selection gradients on weapon traits varied largely among populations or among years in the same population. We confirmed microevolutionary changes in the relationship between traits, which accorded with the expectation from changes in the selection gradients. When gall formers were transferred onto developing buds individually, individuals that successfully induced galls had smaller body size than failed individuals. Available evidence suggests that the selection gradient on body size becomes higher with an increasing proportion of T. sorini in the Tetraneura species community. Thus, we concluded that more intense fighting with conspecifics leads to stronger selective pressure on body size, but that selective pressure for each trait is variable depending on differences in the tactics and species composition among populations.

Effects of different limestone particle sizes in the diet of broiler breeders post molting on their performance, egg quality, incubation results, and pre-starter performance of their progeny.

An experiment was conducted to test the hypothesis that a coarse limestone diet improves productivity, reproductive performance and the calcium utilization of molted broiler breeders. In total, 640 broiler breeder females, 73-week-old and sixty-four 27-week-old cockerels, Cobb 500, were evaluated during 10 weeks, according to a randomized block design composed of 4 treatments with 8 replicates each. Treatments consisted of diets with the inclusion of 100% fine limestone-fine PS (0.2 mm GMD-geometric mean diameter); PS1: 30% fine limestone+70% limestone with 1.0 mm GMD; PS2: 30% fine limestone+70% limestone with 2.0 mm GMD; and PS3: 30% fine limestone+70% limestone with 3.0 mm GMD. Calcium retention in the gizzard of the breeders, bone characteristics, and breeder performance, egg characteristics, eggshell quality, incubation performance, chick quality and yield, chick pre-starter live performance, and chick bone characteristics were determined. There was no significant difference (P>0.05) in the rate of lay, percentage of non-settable eggs, egg weight, egg shape index, egg specific gravity, eggshell weight, thickness, and percentage hatchability and egg weight loss of broiler breeders fed with diets with different limestone particle sizes. The chick quality and yield, chick pre-starter live performance, and chick bone characteristics were not affected (P>0.05) by any of the limestone particle sizes. It was concluded that live and reproductive performance parameters of broiler breeders post molting is not affected by limestone particle size in the feed.

Impact of obesity on outcomes of paediatric acute pancreatitis based on a national administrative database.

BACKGROUND: Insufficient information is available on the relationship between obesity and outcome of paediatric patients with acute pancreatitis. OBJECTIVES: This study aimed to investigate the effect of obesity on outcomes of paediatric patients with acute pancreatitis based on a national administrative database. METHODS: A total of 500 cases in 416 paediatric patients with acute pancreatitis (aged 5-17 years) were referred from 260 hospitals between 2010 and 2012 in Japan. Patients were divided into two groups according to the presence of obesity: with obesity (n = 65) and without obesity (n = 435). Patient data were collected from the administrative database to compare the prevalence of severe acute pancreatitis, in-hospital mortality, length of stay (LOS) and medical costs between the groups. RESULTS: Both prevalence of severe acute pancreatitis and in-hospital mortality were significantly higher in paediatric patients with obesity than those without (36.9% vs. 16.3% and 3.1% vs. 0.0%; P < 0.001, respectively). Longer LOS and higher medical costs were also observed in paediatric patients with obesity (25.7 vs. 15.2 days, P < 0.001 and 14 169.5 vs. 7457.7 US dollars, P < 0.001, respectively). CONCLUSION: This study demonstrated that obesity significantly influenced the outcomes of paediatric acute pancreatitis.