Safety evaluation of a stepwise tracheostomy decannulation program in pediatric patients.

PURPOSE: In the event of failed tracheostomy decannulation, patients might have a tragic course of events. We retrospectively evaluated our stepwise tracheostomy decannulation program and examined its safety. METHODS: A 12-year retrospective study of pediatric patients was conducted. The decannulation program was performed on patients who had airway patency by laryngobronchoscopy and whose cannula could be capped during the day. A stepwise decannulation program was performed: continuous 48-h capping trial during hospitalization (Phase 1), removal of the tracheostomy tube for 48 h during hospitalization (Phase 2), and outpatient observation (Phase 3). If a persistent tracheocutaneous fistula existed, the fistula was closed by surgery (Phase 4). RESULTS: The 77 patients in the study underwent 86 trials. The age at the first time of the decannulation program was 6.5 ± 3.6 years. Sixteen trials failed (18.6%): 8 trials in Phase 1, 2 trials in Phase 2, 4 trials in Phase 3, and 2 trials in Phase 4. Most decannulation failures were due to desaturation in Phase 1/2 and dyspnea in Phase 3/4. The time to reintubation after decannulation was 15-383 days in Phase 3/4. CONCLUSIONS: Patients could fail at every phase of the program, suggesting that a stepwise decannulation program contributes to safety.

Pancreaticobiliary maljunction without biliary dilatation in pediatric patients.

PURPOSE: Pancreaticobiliary maljunction (PBM) without biliary dilatation is a condition in which dilatation of the bile duct is not seen in patients with PBM. Recently, the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) published new diagnostic criteria for PBM. In these criteria, biliary dilatation is defined according to the standard diameter at each age. We reviewed cases of pediatric patients with PBM without biliary dilatation. METHODS: From 1992 to 2019, 134 patients with PBM were treated in our institution. Among these, 7 patients were retrospectively diagnosed with PBM without biliary dilatation. The clinical information was retrospectively assessed in these patients. RESULTS: Of the seven patients, six were female. All patients had symptoms similar to those of patients with congenital biliary dilatation. In all seven patients, the diagnosis of PBM was made before definitive surgery. Six patients had type B PBM, and one had type D PBM. All patients underwent extrahepatic bile duct resection and hepaticojejunostomy, and their symptoms resolved. One patient experienced postoperative complications of anastomotic leakage followed by anastomotic stricture. CONCLUSION: The present report revealed important clinical features of this entity. However, there are still some issues that need to be discussed, and further research is needed.

Mantle cell lymphoma with EBV-positive Hodgkin and Reed-Sternberg-like cells in a patient after autologous PBSCT: Phenotypically distinct but genetically related tumors.

The case of 70-year-old man with mantle cell lymphoma (MCL) carrying t(11;14) translocation that relapsed as nodal lymphoma combining MCL and classic Hodgkin lymphoma (cHL) 9 years after autologous peripheral blood stem cell transplant (auto-PBSCT) is reported. Lymph nodes contained two separate areas of MCL and cHL-like components. Hodgkin and Reed-Sternberg (HRS)-like cells were accompanied by a prominent histiocyte background. HRS-like cells were CD5- , CD15+ , CD20- , CD30+ , PAX5+ , Bob.1- , Oct2- and EBER+ . The MCL component expressed cyclin D1 and SOX11, whereas cyclin D1 and SOX11 expressions were reduced and lost, respectively, in HRS-like cells. Polymerase chain reaction results showed a single clonal rearrangement of the IGH gene in MCL and cHL-like components. CCND1 break apart fluorescence in situ hybridization showed split signals in both MCL and HRS-like cells, suggesting that MCL and cHL-like components were clonally related. Acquisition of p53 expression and Epstein-Barr virus (EBV)-positivity was seen in HRS-like cells. The patient died of disease progression with elevated hepatobiliary enzymes. The autopsy showed both MCL and cHL-like components around the bile ducts, splenic white pulp and bone marrow. The two components were phenotypically distinct, but genetically related, suggesting that transformation of MCL to HRS-like cells during the course of MCL in association with EBV infection.

Long-Term Outcomes After Pediatric Tracheostomy-Candidates for and Timing of Decannulation.

BACKGROUND: Although pediatric tracheostomy has been a widely performed, life-saving procedure, its long-term outcomes have remained unclear. This study aimed to review outcomes after tracheostomy at a Japanese tertiary hospital and clarify candidates for and timing of decannulation. MATERIALS AND METHODS: Hospital records of critically ill children who underwent tracheostomy from 2001 to 2014 were retrospectively reviewed, subsequently analyzing outcomes according to demographics, complications, and decannulation. After excluding those who were lost to follow-up or had irreversible neuromuscular impairment, the remaining patients were divided into the decannulation (D group) and nondecannulation (ND group) groups and compared. RESULTS: In total, 184 patients who underwent tracheostomy were analyzed (median age at operation: 0.5 y). The major indication for tracheostomy was irreversible neuromuscular impairment (46%). Surgery-related and overall mortality rates were 1% and 25%, respectively, while the successful decannulation rate was 21%. No significant difference in surgical indications or comorbidities was observed between the D (n = 39) and ND (n = 50) groups, except for infection (7 in D group versus 0 in ND group; P = 0.002) and chromosome-gene disorder (15% versus 34%; P = 0.04). The ND group had a significantly higher mortality rate than the D group (46% versus 3%; P < 0.0001). The median time to decannulation was 3.6 years, while that for infection was 0.7 y. CONCLUSIONS: Patients who underwent tracheostomy at our institution due to temporary infections achieved more successful and earlier decannulation compared to other indications. Chromosome-gene disorder as a comorbidity can negatively affect decannulation.

Clinical prediction of solitary pulmonary nodule after a curative resection for gastric cancer.

BACKGROUND: Gastric cancer metastasis to the lung can rarely manifest as solitary pulmonary nodule. The surgical strategies are different between metastasis from gastric cancer and primary lung cancer; therefore, the clinical prediction of solitary pulmonary nodule is important. METHODS: Between September 2006 and September 2016, 38 patients with a history of curative resection for gastric cancer who subsequently underwent lung resection for solitary pulmonary nodule at our institution were evaluated. We retrospectively reviewed clinical characteristics in order to investigate clinical predictors for diagnosis and appropriate operative strategies. RESULTS: Nodules were shown to be 9 metastasis from gastric cancer, 26 primary lung cancer and 3 benign tumors. Between metastasis from gastric cancer and primary lung cancer, there was a significant difference in age (P = 0.013), surgical procedure used for gastrectomy (P = 0.018) and pathological stage of gastric cancer (P = 0.031). All eight patients who had undergone endoscopic resection for gastric cancer and all 10 patients whose pulmonary nodules were detected more than 5 years after gastrectomy were diagnosed as primary lung cancer. Regarding the prognosis after lung resection, at a median follow-up of 29.3 months, the median survival time in patients with metastasis from gastric cancer was 19.7 months (range, 10.2-63.7 months), whereas that was not reached in patients with primary lung cancer. CONCLUSIONS: Solitary pulmonary nodule in patients with a history of pathological stage I gastric cancer may be suspected as primary lung cancer, and an anatomical lung resection should be considered positively. In the case of solitary pulmonary nodule suspected metastasis from gastric cancer from clinical characteristics, a limited resection may be considered, and metastasectomy can potentially result in long-term survival.

Pleuroparenchymal Fibroelastosis as a Late-Onset Pulmonary Toxicity after Treatment with Anticancer Chemotherapy for High-Risk Neuroblastoma.

Pleuroparenchymal fibroelastosis (PPFE) is a rare, progressive, restrictive lung disease characterized by hypercarbic respiratory failure. In pediatrics, it has been described in patients with a history of malignancy who have received a bone marrow transplant, chemotherapy, or radiotherapy. It is characterized by pleural thickening, fibrosis, subpleural elastosis, and intraalveolar collagen deposits. Survival is poor, and the only therapy is lung transplantation. Here, we report a patient who developed PPFE as a late-onset pulmonary toxicity after treatment with anticancer chemotherapy for high-risk neuroblastoma (NB).

Postoperative intravenous patient-controlled analgesia improves pain management after subcutaneous implantable defibrillator implantation.

Objective: Postoperative pain is a major issue with subcutaneous implantable cardioverter defibrillators (S-ICD). In 2020, we introduced intravenous patient-controlled analgesia (IV-PCA) in addition to the conventional, request-based analgesia for postoperative pain control in S-ICD. To determine the effect and safety, we quantitatively assessed the effect of IV-PCA after S-ICD surgery over conventional methods. Methods: During the study period, a total of 113 consecutive patients (age, 50.1 ± 15.5 years: males, 101) underwent a de novo S-ICD implantation under general anesthesia. While the postoperative pain was addressed with either request-based analgesia (by nonsteroid anti-inflammatory drugs, N = 68, dubbed as "PCA absent") or fentanyl-based IV-PCA in addition to the standard care (N = 45, dubbed as "PCA present"). The degree of postoperative pain from immediately after surgery to 1 week were retrospectively investigated by the numerical rating scale (NRS) divided into four groups at rest and during activity (0: no pain, 1-3: mild pain, 4-6: moderate pain, 7-10: severe pain). Results: Although IV-PCA was removed on Day 1, it was associated with continued better pain control compared to PCA absent group. At rest, the proportion of patients expressing pain (mild or more) was significantly lower in the PCA present group from Day 0 to Day 4. In contrast to at rest, a better pain control continued through the entire study period of 7 days. No serious adverse events were observed. A few patients experienced nausea in both groups and the inter-group difference was not found significant. Conclusion: IV-PCA suppresses postoperative pain in S-ICD without major safety concerns.

The impacts of preoperative frailty on readmission after cardiac implantable electrical device implantation.

Cardiac implantable electrical devices (CIED) such as pacemakers, implantable cardioverter defibrillators, and cardiac resynchronization therapies are generally recommended for older patients and those with severe heart failure (HF). However, there is currently a lack of evidence on the relationship between frailty and readmission rates among patients with CIED. This study investigated whether preoperative frailty influenced readmission rates among patients with CIED over a one-year period following implantation. The study retrospectively analyzed 101 patients who underwent CIED implantations. To compare frailty-based differences in their characteristics and readmission rates, these participants were categorized into frailty and non-frailty groups via the modified frailty index (mFI). The frailty group had a significantly higher readmission rate than the non-frailty group (non-frailty group vs. frailty group = 1 vs. 8 patients: P < 0.05). Further, a multivariate analysis showed that frailty was a significant readmission factor. Based on individual analyses with/without histories of HF, the readmission rate also tended to be higher among individuals considered frail via the mFI (readmission rate in HF patients: non-frailty group vs. frailty group = 1 vs. 5 patients: P = 0.65; non-HF patients: non-frailty group vs. frailty group = 0 vs. 3 patients: P = 0.01). Participants with preoperative frailty showed higher readmission rates within a one-year period following implantation compared to those without preoperative frailty. This tendency was consistent regardless of HF history. The mFI may thus help predict readmission among patients with CIED.

Obstructive Fecalomas in an Infant Treated with Successful Endoscopic Disimpaction.

A fecaloma is a mass of accumulated feces with a consistency much harder than that of a fecal impaction. It is most frequently observed in the rectum and sigmoid area, and associated complications include colonic obstruction, ulceration, bleeding, and perforation. A one-year-old, previously healthy boy with no history of chronic constipation was admitted because of vomiting and abdominal distension. An abdominal computed tomography scan showed small and large bowel distension due to multiple obstructive fecalomas in the transverse colon. As the fecalomas could not be resolved by laxatives, enemas, or colonic lavage, endoscopic disimpaction under general anesthesia was attempted. Repeatedly shaving the fecalomas with biopsy forceps finally resulted in gradual fragmentation with subsequent passage. Gastrointestinal food allergy was later suggested as the cause because eosinophilic infiltration was found in a biopsy specimen of the colon wall. Endoscopic disimpaction is an effective treatment approach for addressing fecalomas to avoid more invasive surgical intervention.

A 5-year-old boy with acute neurological disorder from anteflexion-induced cervical cord compression after tracheal surgery: Radiological findings similar to Hirayama disease.

Constant neck flexion has been considered crucial to reducing anastomotic tension after tracheal resection. However, in rare cases, anteflexion can cause cervical cord damage, leading to acute neurological disorders such as tetraplegia. Here, we report a case of 5-year-old boy presenting with acute neurological disorder triggered by a chin-to-chest position over 4 days of deep sedation after cricotracheal resection. The radiological findings would suggest a mechanism similar to Hirayama disease, in which a shift of the dura leads to chronic muscular weakness and atrophy in young populations.

Exclusive Cerebellar and Leptomeningeal Metastases from Early Gastric Cancer 14 Months after Proximal Gastrectomy: An Autopsy Case Report.

We report a rare autopsy case in which the patient received gastrectomy after an endoscopic diagnosis of early gastric cancer, and had deteriorated due to exclusive metastatic cerebellar tumors identified 14 months after surgery. A 65-year-old male was diagnosed as having a 0-IIc-type early gastric cancer on the posterior wall of the upper stomach by gastrointestinal endoscopy in search of a cause of epigastralgia, and thus received proximal gastrectomy and pyloroplasty. Although the tumor was in the early stages and limited within the mucosal layer, adjuvant chemotherapy was started by using S-1 80 mg daily due to evidence of metastasis into lymph node #3 at the lesser curvature. Evidence of both recurrence and metastases was not detected by CT scans of the chest, abdomen, and pelvis, and the chemotherapy was completed 12 months after surgery. However, the patient was admitted to hospital 14 months postoperatively due to dizziness and gait disturbance. Cranial MRI (Magnetic Resonance Imaging) revealed multiple tumors in the bilateral cerebellar hemispheres with additional leptomeningeal involvement. The patient died 2 weeks after admission. An autopsy revealed metastatic cerebellar tumors and leptomeningeal lesions from the early gastric cancer, and obstructive hydrocephalus due to metastatic cerebellar tumors. To our knowledge, this case is the first report of metastasis exclusive to the cerebellum and leptomeninges from early gastric cancer limited to the mucosal layer.

A case of primary lung squamous cell carcinoma mimicking malignant mesothelioma producing granulocyte colony stimulating factor with chemotherapy (cisplatin and gemcitabine)-associated thrombotic thrombocytopenic purpura (TTP); An autopsy case report.

OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy. In recent years, an extensive variety of drugs, including certain cytotoxic agents, have been reported to be associated with TTP. Additionally, several studies have reported that granulocyte colony-stimulating factor (G-CSF) was produced by lung carcinoma. G-CSF-producing carcinoma also produces various other cytokines, which may cause vascular endothelial damage and trigger TTP development. However, there has been no report describing G-CSF-producing carcinoma combined with TTP. We report a rare case of pseudomesothliomatous squamous cell lung carcinoma producing G-CSF along with chemotherapy associated TTP. MATERIALS AND METHODS: A 66-year-old man with pseudomesotheliomatous primary squamous cell lung carcinoma was treated with chemotherapy consisting of cisplatin and gemcitabine as the first line treatment. However, thrombocytopenia, acute renal dysfunction and acute respiratory failure occurred after starting the first chemotherapy cycle. As a result, the patient died, and an autopsy was performed. RESULTS: According to the autopsy findings, a diagnosis of primary lung squamous cell carcinoma producing G-CSF associated with TTP was made. CONCLUSION: Chemotherapy-related TTP should be considered when anemia and thrombocytopenia progress rapidly in patients who are under chemotherapy treatment. Furthermore, the current case may provide a possible link between TTP and G-CSF-producing tumor.

Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-ß in the bone marrow: a case report.

We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20+), T lymphocytes (CD3+, CD8+), and plasma cells (CD138+). Immunohistochemical analysis revealed increased expression of transforming growth factor-ß (TGF-ß) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient's pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-ß in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases.

Cholangitis as an initial manifestation of polyarteritis nodosa.

A previously healthy 89-year-old man was admitted to our hospital with right upper quadrant pain and mild fever. A diagnosis of cholangitis was suspected based on the patient's physical findings and imaging features. Although he received treatment typical for cholangitis, he suddenly died of shock for unknown reasons two months after disease onset. An autopsy revealed a ruptured hepatic artery aneurysm, which had caused lethal intra-abdominal bleeding. In addition, systemic necrotizing vasculitis of small- and medium-sized arteries was detected, and polyarteritis nodosa (PAN) was diagnosed after the autopsy. Biliary symptoms as the initial manifestation of PAN are extremely rare.

The tight-junction protein claudin-6 induces epithelial differentiation from mouse F9 and embryonic stem cells.

During epithelialization, cell adhesions and polarity must be established to maintain tissue assemblies and separate the biological compartments in the body. However, the molecular basis of epithelial morphogenesis, in particular, a role of cell adhesion molecules in epithelial differentiation from stem cells, remains unclear. Here, we show that the stable and conditional expression of a tight-junction protein, claudin-6 (Cldn6), triggers epithelial morphogenesis in mouse F9 stem cells. We also demonstrate that Cldn6 induces the expression of other tight-junction and microvillus molecules including Cldn7, occludin, ZO-1α+, and ezrin/radixin/moesin-binding phosphoprotein50. These events were inhibited by attenuation of Cldn6 using RNA interference or the C-terminal half of Clostridium Perfringens enterotoxin. Furthermore, similar results were obtained in mouse embryonic stem cells. Thus, we have uncovered that the Cldn6 functions as a novel cue to induce epithelial differentiation.