Spontaneous Regression of Mismatch Repair-Deficient Colon Cancer: A Case Series.

Spontaneous regression of cancer is a rare phenomenon, with 33 colorectal cancer cases reported between 1900 and 2020.1-4 Spontaneous regression is defined as the partial or complete disappearance of a tumor without treatment.1,3 Several factors may be involved in this process, including biopsy, mechanical stress, humoral factors, and infection.1,5 However, no concrete evidence for the mechanistic insights has been indicated.

Efficacy of CO2 Infusion for Preoperative Computed Tomographic Angiography with Computed Tomographic Colonography.

BACKGROUND Computed tomographic colonography (CTC) is useful for patients for whom colonoscopy may be difficult to perform and is widely employed to examine the vasculature prior to colorectal cancer surgery. Computed tomographic angiography (CTA) was shown to be beneficial intraoperatively to manipulate blood vessels and prevent vascular injury. Three-dimensional (3D)-CTA combined with CTC (3D-CTA with CTC) is useful for preoperative evaluations of the anatomy of mesenteric vessels, colon, and lymph nodes. We observed that when the intestine was dilated with carbon dioxide (CO2), the arteriovenous delineation was often more pronounced than without CO2. To clarify the effects of gas injection with and without CO2 on hemodynamics and vascular passage, we compared the effect of contrast for blood vessels. MATERIAL AND METHODS Thirty patients with resectable colorectal cancer who underwent a preoperative CT examination at our institution from January to October 2019 were study participants. Of these, 15 underwent 3D-CTA and 15 had 3D-CTA with CTC. Three board-certified radiologists independently and blindly evaluated 18 blood vessels. CT values for each blood vessel were measured on each image. RESULTS CT values for 3D-CTA with CTC were significantly higher with CO2 than without CO2. The quality of 3D-CTA with CTC images for visualization of blood vessels was also significantly greater than that of 3D-CTA, especially those of arterial and intramesenteric venous systems. CONCLUSIONS Based on the higher image quality and CT values obtained by 3D-CTA with CTC for vessels, compared with by 3D-CTA imaging, 3D-CTA with CTC imaging might be useful in evaluating colorectal cancers.

Long-term clinical outcomes of endoscopic submucosal dissection for colorectal neoplasms in 423 cases: a retrospective study.

Background and study aim Endoscopic submucosal dissection (ESD) is known as a curative treatment for colorectal superficial neoplasms. There is however a need for more long-term clinical data to establish the full advantages of colorectal ESD regarding very low recurrence rates. The aim of this retrospective study was to determine long-term clinical outcomes of colorectal ESD. Methods A total of 423 lesions treated by ESD for colorectal adenoma/dysplasia or adenocarcinoma between 1998 and 2008 at a single high volume referral center were included. We conducted a retrospective survey on patients with follow-up and obtained complete 1-, 3-, and 5-year outcome data for 358 (85 %), 292 (69 %), and 209 (49 %) lesions, respectively. Curative resection was defined when the pathological specimen had carcinoma-free resection margins, irrespective of piecemeal or en bloc resection, without submucosal deep invasion (≥ 1000 µm), lymphovascular involvement, or a poorly differentiated adenocarcinoma component. Results After a median 4.9 years of follow-up, the 3-year overall cumulative endoscopic recurrence rate and cancerous recurrence rate were 2.9 % (95 % confidence interval [95 %CI] 1.2 - 4.7) and 1.1 % (0 - 2.1), respectively. The 5-year overall cumulative endoscopic recurrence and cancerous recurrence rates were 3.8 % (1.7 - 5.9) and 1.6 % (0.1 - 3.0), respectively. In 361 lesions eligible for endoscopic follow-up, the 3-year endoscopic recurrence and cancerous recurrence rates were 2.4 % (0.8 - 4.1) and 0.4 % (0 - 1.4), respectively. Multivariate analysis revealed that piecemeal resection and submucosal deep tumor invasion were associated with recurrence. Conclusions The current study demonstrated favorable long-term clinical outcomes of colorectal ESD when en bloc curative resection is achieved.

Impact of a Deep Learning-based Super-resolution Image Reconstruction Technique on High-contrast Computed Tomography: A Phantom Study.

RATIONALE AND OBJECTIVES: Deep-learning-based super-resolution image reconstruction (DLSRR) is a novel image reconstruction technique that is expected to contribute to improvement in spatial resolution as well as noise reduction through learning from high-resolution computed tomography (CT). This study aims to evaluate image quality obtained with DLSRR and assess its clinical potential. MATERIALS AND METHODS: CT images of a Mercury CT 4.0 phantom were obtained using a 320-row multi-detector scanner at tube currents of 100, 200, and 300 mA. Image data were reconstructed by filtered back projection (FBP), hybrid iterative reconstruction (HIR), model-based iterative reconstruction (MBIR), deep-learning-based image reconstruction (DLR), and DLSRR at image reconstruction strength levels of mild, standard, and strong. Noise power spectrum (NPS), task transfer function (TTF), and detectability index were calculated. RESULTS: The magnitude of the noise-reducing effect in comparison with FBP was in the order MBIR

Efficacy of 6-mm diameter fully covered self-expandable metallic stents in preoperative biliary drainage for pancreatic ductal adenocarcinoma.

Objectives: Plastic stents (PS) used for preoperative biliary drainage (PBD) of pancreatic ductal adenocarcinomas (PDAC) tend to be associated with a high incidence of recurrent biliary obstruction (RBO). Although 10-mm diameter fully covered self-expanding metallic stents (FCSEMS) have come into use, vigilance is still required to prevent complications, such as cholecystitis and surgical site infection. The present study examined the efficacy and safety of the 6-mm diameter FCSEMS for PBD. Methods: The present retrospective study compared the incidence of complications associated with the use of 6-mm FCSEMS and PS. The inclusion criteria were a diagnosis of PDAC and preoperative endoscopic biliary tract drainage performed at our institution between April 2012 and June 2019. Results: Of the 51 patients enrolled, 25 and 26 patients received a PS and a 6-mm FCSEMS, respectively. The RBO incidence was significantly lower in the 6-mm FCSEMS group (7.7%) than in the PS group (40.0%) (p = 0.009), and time to RBO was significantly longer in the 6-mm FCSEMS group (HR = 6.008, p = 0.021). The patency rate at three months after stent placement was significantly higher in the latter group (83.5% vs. 45.3%, p = 0.009, Log-rank test). The groups did not differ significantly in terms of complications associated with PBD, such as cholecystitis and surgical site infection. Conclusion: The present findings suggested that the 6-mm FCSEMS may be an effective drainage device for use in PBD in PDAC treatment.

Pulmonary hemorrhage associated with Henoch-Schönlein purpura in a child.

Swelling of the right ankle and purpura on the bilateral lower extremities of a 6-year-old boy indicated a diagnosis of Henoch-Schönlein purpura (HSP). The patient was referred to our hospital because of severe abdominal pain that was unresponsive to prednisolone. Respiratory symptoms and pulmonary infiltrates on chest X-ray were absent upon admission. Methylprednisolone pulse therapy gradually improved the abdominal symptoms, but tachypnea developed, and insufficient aeration of the right lung was accompanied by a decline in percutaneous oxygen saturation from 99% to 90% and a rapid decrease in hemoglobin levels from 11.7 to 7.6 g/dL. Chest X-rays and high-resolution computed tomography scans showed widespread consolidation and patchy opacities predominantly in the right lung fields, suggesting acute pulmonary hemorrhage. Intravenous cyclosporin A (CyA) gradually resolved the pulmonary hemorrhage and respiratory insufficiency. Pulmonary hemorrhage, as a complication of HSP, is extremely rare and sometimes fatal. Aggressive steroid and immunosuppressive treatment is required to address severe complications of HSP.

Absence of high-risk stigmata predicts good prognosis even in severely anemic patients with suspected acute upper gastrointestinal bleeding.

BACKGROUND: The need for a blood transfusion, defined as hemoglobin < 70 g/L, is the measure for assessing the risk levels in patients with upper gastrointestinal bleeding (UGIB). However, not all patients with a low hemoglobin level have a poor prognosis. OBJECTIVE: We assessed the clinical predictive factors associated with poor short-term prognosis in patients with a low hemoglobin level. METHODS: In this prospective cohort study, all consecutive patients with suspected acute UGIB at Tokyo Metropolitan Tama Medical Center were enrolled between 2008 and 2015. Then, we extracted those who needed a blood transfusion (hemoglobin < 70 g/L) and explored the variables associated with all-cause mortality within 28 days after presentation. RESULTS: Among 1307 patients, 311 needed a blood transfusion and 13 (4.2%) died from all causes. The presence of high-risk stigmata requiring endoscopic treatment (peptic ulcers; Forrest scores Ia, Ib and IIa; varices with current bleeding or signs of recent bleeding; and spurting or gushing bleeding or visible vessel in other diseases), diagnosed by emergency endoscopy, was a unique factor affecting mortality (odds ratio: 8.47, 95% confidence interval: 1.45-160, P = 0.01). Patients without high-risk stigmata neither died from UGIB nor had rebleeding, irrespective of the hemoglobin levels. CONCLUSIONS: Patients without high-risk stigmata showed a good prognosis even if they needed a blood transfusion. This result could facilitate triage of patients with suspected acute UGIB who only need a blood transfusion.

Successful corpus callosotomy for Doose syndrome.

Doose syndrome (epilepsy with myoclonic-atonic seizures) is an epilepsy syndrome with an incidence of approximately 1-2% of childhood-onset epilepsies. Although this syndrome is associated with multiple types of generalized seizures, the diagnosis is based on the presence of myoclonic-atonic seizures. Eighteen percent of patients have refractory seizures and intellectual disabilities. There have, however, been a few reports on the efficacy of surgical treatment for Doose syndrome. We describe a case of Doose syndrome in a 10-year-old boy. He developed generalized tonic-clonic seizures at 3years 8months of age and subsequently developed myoclonic-atonic, myoclonic, and tonic seizures. The frequent myoclonic seizures were refractory to multiple antiepileptic medications. His cognitive development was moderately delayed. Anterior four fifths corpus callosotomy was performed at 8years 5months of age. His seizures, especially myoclonic seizures, were markedly reduced. He was given vagus nerve stimulation therapy at 9years and 1month of age, which led to complete resolution of the myoclonic seizures. Corpus callosotomy can be a good treatment strategy in patients with Doose syndrome with medically refractory generalized seizures.

Successful hemispherotomy for a patient with intractable epilepsy secondary to bilateral congenital brain malformation with lateralized pyramidal tract of diffusion tensor image tractography.

PURPOSE: To perform an epilepsy surgery on a patient with drug resistant epilepsy secondary to bilateral brain malformation. The patient was a 2-year 9-month-old boy who had congenital bilateral multiple abnormalities. He developed a complex partial seizure at 9 months old. Based on the presurgical evaluations, he underwent a right hemispherotomy. RESULTS: Brain MRI revealed congenital bilateral polymicrogyria, right schizencephaly, and corpus callosum agenesis. The abnormality was noted to be more severe in the right hemisphere. Diffusion tensor image tractography clearly detected a left pyramidal tract. On the contrary, the right hemisphere did not show a clear pyramidal tract. An interictal EEG showed epileptiform discharges over both hemispheres with right frontotemporal area dominancy. The long-term video-EEG recorded his habitual seizures arising from the right frontal area. Right hemispherotomy led to freedom from seizures and a reduced need for anti-epilepsy drugs. CONCLUSIONS: We report a patient with bilateral MRI and EEG abnormalities with clear DTI laterality who achieved seizure freedom with epilepsy surgery.

Wolf-Hirschhorn (4p-) syndrome with West syndrome.

Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

A simple scoring system to assess the need for an endoscopic intervention in suspected upper gastrointestinal bleeding: A prospective cohort study.

BACKGROUND: Assessment of the emergent endoscopy for upper gastrointestinal bleeding (UGIB) patients has important clinical implications. There is no validated criterion to triage. AIMS: To develop a simple score predicting an endoscopic intervention. METHODS: A prospective cohort study was conducted at a tertiary care centre. Primary outcome was the high-risk stigmata which were well-established endoscopic findings to determine the need for an endoscopic intervention. We created a simple score by multivariable logistic regression and compared with the Glasgow Blatchford Score (GBS). External validation was performed in a second cohort. RESULTS: 284 of consecutive 568 patients with suspected UGIB had the high-risk stigmata. Three variables were selected: "no daily use of proton pump inhibitors during one week before examination (+1 point)", "shock index (heart rate/systolic blood pressure)≥1 (+1 point)" and "urea/creatinine≥140 (blood urea nitrogen/creatinine≥30) (+1 point)". The accumulating score (range 0-3) achieved an area under the receiver-operating characteristic curve (AUC) of 0.74 (95% confidence interval [CI], 0.70-0.78), which was superior to the GBS (AUC, 0.63; 95% CI, 0.59-0.68; p<0.001). Validation in an external cohort demonstrated superiority to the GBS (AUC, 0.78 vs. 0.59; p<0.001). CONCLUSIONS: The simple score has greater accuracy than the GBS for assessing the need for an endoscopic intervention in cases of suspected UGIB. Further external validation should be performed to verify generalizability.

[Efficacy of Levetiracetam as an Add-on Therapy in Children with Localization-related Epilepsy].

OBJECTIVE: To clarify the efficacy and safety of levetiracetam (LEV) in children with localization-related epilepsy, we performed a retrospective study in our hospital. METHODS: We reviewed the cases of 70 pediatric patients (mean age, 9.4 years, range 4-15 years) who had undergone LEV add-on therapy between October 2010 and September 2014. The subjects were followed for a period of 24 weeks. RESULTS: The mean starting dose was 9.9 mg/kg/day, and the mean final dose was 39.9 mg/kg/day. The LEV continuation rate was estimated to be 87%. The responder rate (based on ≥ 50% reduction in seizure frequency) was 57%. Twenty-four of the 70 patients experienced side effects, such as drowsiness, fatigue, and aggressiveness. Although most of the adverse events were mild and tolerable, it was necessary to discontinue the medication in four patients. We evaluated the effects of gender, age, duration of illness, secondarily generalized seizure, baseline seizure frequency, MRI abnormality, number of previously administered drugs, number of concomitant drugs, and final dose. MRI abnormality and final dose of LEV had a statistically significant influence on the efficacy. CONCLUSION: LEV can be used as an effective and safe add-on treatment in children with localization-related epilepsy.

[Effectiveness and role of lamotrigine in the paradigm shift towards new antiepileptic drugs: a therapeutic strategy proposed after an investigation of a large number of cases at a single hospital].

Lamotrigine (LTG) has been recognized as one of the best newer antiepileptic drugs (AEDs) used in developed countries since 1991. A retrospective study was carried out to evaluate the efficacy, adverse reactions, and other peculiarities of LTG. Two hundred and eighty-one patients with epilepsy, aged 16 or more, were treated with LTG between December 2008 and December 2012 at the Seirei Hamamatsu General Hospital. One hundred and forty patients were found to be eligible to evaluate the effectiveness of LTG. Of them, 100 patients had localized epilepsy, and 40 patients had generalized epilepsy. Seventy-five out of these 140 (53.6%) patients obtained seizure freedom following LTG use, and 24 patients (17.1%) showed more than 50% seizure reduction, which indicates that a total of 99 patients (70.7%) were good responders. A seizure reduction of less than 50% was seen in 8 patients (5.7%), whereas 30 patients (21.4%) did not display any obvious seizure reduction. Three patients (2.1%) showed a worsening of seizure frequency. Patients with idiopathic generalized epilepsy such as juvenile myoclonic epilepsy showed satisfactory results. Many good responders were also patients with stroke or brain tumor. The number of concomitant AEDs used demonstrated a more distinctive feature. Monotherapy without any other AEDs was used in 20 patients. LTG was used as an add-on with 1 concomitant AED in 59 patients (42.1%) and as an add-on with 2 concomitant AEDs in 37 patients (26.4%). Thus, a large number of patients (68.6%) underwent early add-on treatment. LTG as an add-on in concert with concomitant AEDs contributed to the excellent results in terms of seizure reduction observed in this study. LTG administration requires established titration, particularly to prevent drug eruption. Over 60% of the patients were treated by slower titration than that used in the established titration method. However, seizure freedom was accomplished within 1-5 weeks from the beginning of LTG treatment with doses much lower than 200 mg/day, which indicated the effectiveness of low-dose LTG in the early phase of treatment. The average maintenance dose was 162.7 mg/day; however, half of the patients maintained their dose at more than 200 mg/day. The continuation rate was 77.2%. The main reasons for withdrawal were dissatisfaction with drug effectiveness and drug eruption. However, the incidence of drug eruption was only in 15 out of 281 (5.3%) patients, which is similar to previously reported rates. Therefore, LTG is an effective and safe AED. Moreover, it is a promising drug for promoting a paradigm shift towards newer AEDs. LTG administration as a first add-on AED is strongly recommended to obtain excellent results and to maintain good compliance with the epilepsy treatment course.

Comparison of dense array EEG with simultaneous intracranial EEG for interictal spike detection and localization.

PURPOSE: To evaluate the clinical utility of dense array electroencephalography (dEEG) for the detection yield and localization of interictal spikes in mesial temporal lobe epilepsy. METHODS: We simultaneously recorded 256-channel dEEG and intracranial electroencephalography (icEEG) implanted over the lateral and mesial temporal lobe in patients with intractable epilepsy. We calculated the dEEG spike detection rate for mesial temporal spikes which were confirmed by icEEG and applied source estimation to dEEG to compare noninvasive localization to the invasive recordings. RESULTS: 339 of 760 interictal spikes (45%) were detected by dEEG examining the 256-channel head surface array. The average icEEG amplitude of dEEG detectable spikes was 1083 µV, and that of dEEG undetectable spikes was 780 µV (P<0.05). All spikes detected in dEEG were localized to the temporal lobe. 295 of 339 spikes (87%) were well localized in mesial temporal lobe, close to the position confirmed by subdural electrodes. SIGNIFICANCE: 256-channel dEEG may provide more precise information for the localization of interictal epileptiform discharges than conventional EEG or MEG in patients with deep spike foci. 256-channel dEEG may be clinically useful in the presurgical work-up for epilepsy, providing accurate noninvasive guidance for the placement of intracranial electrodes.

[Clinical efficacy of the novel antiepileptic agent levetiracetam: evaluation in patients with localization-related epilepsy].

In this retrospective study, we evaluated the safety and efficacy of levetiracetam (LEV) as an antiepileptic drug by using data in our hospital database from October 2010, when LEV became available in Japan, through August 2011. Data from patients aged 16 years or more (n=132) with localization-related epilepsy (n=112) and generalized epilepsy (n=19) were reviewed. Among patients with localization-related epilepsy, 53.6% showed a greater than 50% reduction in seizure frequency compared with the pretreatment baseline, and 28.6% of them became seizure free during the treatment period. Adverse events were reported in 27.3% of patients and caused discontinuation of LEV in 10.6%. The most frequent adverse events were somnolence (14.4%), irritability or aggressiveness (6.1%), and depression (4.5%). However, most of the adverse events were of mild to moderate severity. More than 80% of patients continued LEV treatment. The frequency of adverse events and discontinuation rate after LEV treatment were not associated with the starting dose (1,000 mg/day or less). LEV was well tolerated and efficacious as an adjunctive therapy for localization-related epilepsy.

Heterozygous ABCA3 mutation associated with non-fatal evolution of respiratory distress.

A boy without symptoms up to 12 months of age started with persisting cough followed by respiratory failure at 18 months of age, resulting in mechanical ventilation because of alveolar proteinosis. Lung biopsy showed PAS-positive material. PCR was negative for CMV, Pneumocystis jiroveci and adenovirus. BALF showed mature SP-B. Analysis of the ATP-binding cassette transporter A3 (ABCA3; OMIM 601615) gene showed a compound heterozygous mutation from paternal W1148X and maternal T1114A. Alveolar lavage with 720 mg of bovine surfactant allowed weaning from ventilator support. Heterozygous mutation in the ABCA3 gene could be associated with a milder evolution as compared to the homozygous frequently lethal evolution.

Single-dose chloral hydrate for benign convulsions with mild gastroenteritis.

PURPOSE: To reveal the efficacy of single-dose treatment with chloral hydrate (CH) for clustering seizures in benign convulsions with mild gastroenteritis. METHODS: We retrospectively studied the details of treatment in 33 patients with ages ranging from 7 to 39 months. The time-series records of seizures and processes of drug administrations were investigated. RESULTS: A single-dose therapy with CH was effective in 19 of 22 patients (86%), and diazepam in two of 16 (13%). The doses of CH in patients having a successful treatment with single-dose therapy ranged from 41.7 to 62.5 mg/kg (mean 50.2). In two patients, seizures were resistant to single-dose CH therapy, and their doses of CH were 33.8 and 35.1 mg/kg. CONCLUSIONS: An advantage of the single-dose therapy with CH was shown. We recommend treatment with a sufficient dose of not less than 40 mg/kg of CH.