Notes

Lasting biodiesel production through microalgae Graesiella emersonii by way of valorization regarding back garden wastes-based vermicompost.
Although the initial treatment of childhood seizures is important, treatment within an appropriate time window is often difficult in resource-limited areas. This study examined childhood seizure treatment in a rural area in Japan.

We retrospectively investigated children presenting to Nakatsugawa Municipal General Hospital emergency department between 2015 and 2018. From the hospital database, we identified children who were diagnosed with seizures, epilepsy, or acute infectious encephalitis/encephalopathy or were given benzodiazepines. We considered etiology, seizure duration, and treatment according to the specialties of the doctors providing initial care.

We extracted 236 seizure events 40 initially treated by pediatricians, 16 by a mobile doctor team, and 180 by other doctors. Twenty patients had continuous seizures for longer than 5min on admission. Two were treated by pediatricians at presentation; it took 4 and 7min after arrival to stop the seizures. Four were treated by a mobile team, and 14 by other doctors; the median response times were 11.5 (range 3-47) and 19 (range 5-60) min, respectively. All patients treated by pediatricians or mobile doctor teams received intravenous or intramuscular diazepam, whereas 50% of those treated by other doctors initially received diazepam suppositories. In three of the 20 events, establishing intravenous access was difficult.

In rural Japan, many children with seizures are initially treated by doctors other than pediatricians or emergency physicians, and they require a longer time to achieve seizure cessation. Non-intravenous benzodiazepine formulas, which have not yet been approved in Japan, would be helpful.
In rural Japan, many children with seizures are initially treated by doctors other than pediatricians or emergency physicians, and they require a longer time to achieve seizure cessation. Non-intravenous benzodiazepine formulas, which have not yet been approved in Japan, would be helpful.
Heterozygous variants in BICD2 cause autosomal dominant spinal muscular atrophy with lower extremity predominance. These variants are also identified in individuals with severe forms of congenital muscle atrophy representing arthrogryposis multiplex.

A girl was born with severe muscle weakness and respiratory distress. A fetal ultrasound had detected polyhydramnios and multiple joint contractures in utero. She was born with severe muscle weakness and respiratory distress. Bilateral hip joint dislocation and multiple bone fractures were also present at birth. Although she depends on medical care, including assisted ventilation and tube feeding, she has reached eight years of age. Her motor developmental skills were reduced owing to muscle weakness and deformity of her lower extremities, whereas her cognitive development slowly but steadily grew. Whole exome sequencing revealed a novel de novo missense BICD2 variant (c.1625G>A, p.(Cys542Tyr)), which was evaluated as likely pathogenic.

This is the first case report of a severe form of spinal muscular atrophy with lower extremity predominance caused by a de novo BICD2 variant in Japan.
This is the first case report of a severe form of spinal muscular atrophy with lower extremity predominance caused by a de novo BICD2 variant in Japan.
We aimed to explore the prevalence and nature of complications associated with Class II correctors in adolescents and their impact on the quality of life (QOL), completion of treatment, and success rate.

The review was registered in PROSPERO, and a comprehensive electronic search was performed without language or date restrictions. Randomized and nonrandomized trials, prospective cohort and cross-sectional studies, case series, and qualitative research were included. The Cochrane Collaboration's risk of bias tool and the Newcastle-Ottawa scale were used to assess the quality of included studies. Data were grouped according to appliances design removable functional, fixed functional, hybrid functional, headgear, and fixed maxillary molar distalization appliances.

Data from 27 studies were included, of which 11 were deemed eligible for meta-analysis. Overall, 1676 adolescents were included related to fixed functional (n=682), removable functional (n=682), hybrid functional (n=84), headgear (n=186), and CaQOL and lack of compliance. More complications were observed with fixed designs, although this did not impact the overall success rates. Further prospective studies are needed to explore patient perceptions and cost-effectiveness to inform treatment decisions better.
Complete cytoreductive surgery with hyperthermic intraperitoneal chemotherapy is an effective treatment for improving prognosis of selected patients with peritoneal carcinomatosis. The addiction of diaphragmatic procedures may increase the incidence of postoperative respiratory complications. Our goal was to evaluate the early postoperative results following diaphragmatic surgery.

Prospectively collected data of patients undergoing diaphragmatic surgery between January 2000 and January 2020 were retrospectively analyzed. Chest drains were routinely placed in all cases. Demographics, clinical and perioperative features were evaluated.

The study included 222 patients. Peritoneal stripping and full-thickness resections were performed in 165 and 57 cases, respectively. Women and ovarian tumours represented the vast majority of cases. Overall postoperative mortality and morbidity rates were 1.3% (3 patients) and 43.2% (96 patients), respectively. Specific diaphragmatic surgery-related complications was 9.4% to obtain complete resection. It may be associated with higher postoperative morbidity, especially bleeding, but routine placement of chest drains may reduce the incidence of adverse respiratory events.Colorectal cancer (CRC) is a rare condition in the pediatric population, but it is usually associated with worse prognosis compared to the adult population. Surgical resection is the gold standard and most effective treatment for CRC. 3-O-Acetyl-11-keto-β-boswellic purchase Cytoreductive surgery (CRS) and Heated Intra-Peritoneal Chemotherapy (HIPEC) is a feasible option in resectable primary tumour with carcinomatosis and non-evidence of extra-abdominal disease. Although it is very uncommon in children when performed, CRS-HIPEC is based on the description by Sugarbaker et al. and the two most common administrated drugs are Cisplatin and Mitomycin-C. We present a review of the cases found in the literature of peritoneal carcinomatosis from CRC treated with CRS and HIPEC in children. A systematic search was performed in the major databases up to February 2020. We included all the reviews and studies reporting clinical data on pediatric patients with peritoneal colorectal carcinomatosis. Nine cases were extracted from the literature. Patient age was between 11 and 16 years old. All patients underwent neoadjuvant chemotherapy. All patients were treated with HIPEC and the majority of them received a complete cytoreduction (CC-0). At follow-up, three patients were found free from disease with an average time of follow up of 74 weeks (40-100). In 33% of cases, recurrence was described. No postoperative death within 30 days from surgery was observed. CRS and HIPEC can be a feasible option for CRC peritoneal carcinomatosis in children. Because CRC is unusual among the pediatric population, multi-institutional studies should be done to achieve larger cohorts and a more reliable analysis.Two recent genetic studies reported that loss-of-function mutation of the C-terminal cargo-binding tail domain of the KIF5A gene cause amyotrophic lateral sclerosis (ALS). The aim of this study is to investigate the frequency of KIF5A variants in Japanese patients with sporadic ALS. In total, 807 sporadic ALS patients and 191 normal controls from a multicenter ALS cohort in Japan were included. Whole exome sequencing on an Illumina HiSeq 2000/2500 sequencer was used to identify and select variants within the KIF5A gene. Thirteen patients harbored a nonsynonymous variant in the KIF5A gene; These were considered variants of uncertain significance. One patient harbored a novel splice-site variant (c.2993-3C>A) in the C-terminal cargo-binding tail domain of the KIF5A gene. Functional analysis of this variant revealed that it caused skipping of exon 27. The frequency of KIF5A mutations in Japanese patients with sporadic ALS was 0.12% (1/807). This study reports a novel loss-of-function variant in KIF5A, and indicates that loss-of-function variant in KIF5A is a rare cause of sporadic ALS in Japanese patients.
Hypersensitivity pneumonitis (HP) is an interstitial lung disease due to an immunological reaction to exposure, by inhalation, to a large variety of antigens. The patho-physiological mechanism remains poorly understood. The diagnosis can be challenging and requires a detailed medical history taking especially when the clinical presentation is atypical or when the causal agent remains unknown.

We report the case of a 75-year-old woman with a history of mammary carcinoma who presented with recently identified intramammary adenopathy. Biopsy of the adenopathy revealed non-necrotising, giant cell epithelioid granuloma. A diagnosis of hot tub lung with extra-pulmonary granulomatous lymph node involvement was made based on the clinical, functional, radiological and microbiological investigations. The evolution was favorable following antigen avoidance.

Extrapulmonary lymph node involvement is rare in HP, suggesting a systemic inflammatory involvement.
Extrapulmonary lymph node involvement is rare in HP, suggesting a systemic inflammatory involvement.
Bronchoalveolar lavage (BAL) was previously considered as the standard diagnostic procedure to investigate pneumonia occurring in immunocompromised patients, and it is probably still widely used. However, the development of new microbiological diagnostic tools, applicable to samples obtained non-invasively, leads to questioning of the predominant place of BAL in this situation.

The available studies agree on the acceptable tolerance of BAL performed in immunocompromised patients. Although imperfect, the diagnostic yield of BAL in immunocompromised patients is well established, but it may vary between studies depending on the underlying disease. However, it must also be compared to the yield of non-invasive microbiological tools, now widely available and effective. The position of BAL remains important both for the diagnosis of fungal infections (invasive aspergillosis, pneumocystis pneumonia) and non-infectious lung diseases both of which occur frequently in immunocompromised patients.

The place of BAL in the diagnostic work-up of pneumonia occurring in immunocompromised patients must be considered in the framework of a structured consideration, taking into account the diagnostic performance of non invasive microbiological tests and the broad spectrum of lung diseases occurring in this context.
The place of BAL in the diagnostic work-up of pneumonia occurring in immunocompromised patients must be considered in the framework of a structured consideration, taking into account the diagnostic performance of non invasive microbiological tests and the broad spectrum of lung diseases occurring in this context.
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