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Childhood-onset lymphocytic infundibuloneurohypophysitis (LINH) due to infiltration of autoimmune lymphocyte in the neurohypophysis is rarely reported. Its definitive diagnosis requires a pituitary biopsy, which is an invasive procedure. Recently, anti-rabphilin-3A antibody has been reported as a potential diagnostic marker for LINH in adults; however, only a few cases have been reported in children. Here, we present a case of childhood-onset LINH in a 10-yr-old boy identified as anti-rabphilin-3A antibody positive during chronic phase, 9 yr post-onset of central diabetes insipidus (CDI). T1-weighted magnetic resonance imaging (MRI) revealed pituitary stalk thickening and absence of posterior pituitary bright signal spot, and the hormonal responses of the adenohypophysis to GHRH, TRH, CRH, and LHRH revealed no abnormalities during the first admission. MRI at 5 mo post-onset indicated reduced stalk swelling; however, replacement treatment with intranasal desmopressin was continued to counter unimproved CDI. Additionally, GH replacement therapy was also initiated to counter its deficiency. Pituitary re-enlargement was not observed in the subsequent routine MRI, and no increase was observed in the levels of tumor markers during follow-up, which was considered clinically consistent with LINH. Our case study suggests that anti-rabphilin-3A antibody may be considered as a useful diagnostic marker for LINH in children.

Aims/Introduction Increased concentrations of serum tumor necrosis factor (TNF) receptors (TNFRs; TNFR1 and TNFR2) are positively associated with the urinary albumin‐to‐creatinine ratio (ACR), and negatively associated with the estimated glomerular filtration rate (eGFR) in patients with type 2 diabetes. However, the mechanism underlying this increase and the relationship between TNFRs in serum, and urine and kidney measures (ACR and eGFR) are unclear. Materials and Methods This was a cross‐sectional study that included 499 patients with type 2 diabetes and eGFR ≥60 mL/min/1.73 m2. The concentrations of TNFRs in serum and urine, and their respective fractional excretion, were measured. Results Serum and urinary TNFR levels were positively associated with the ACR, and negatively associated with the eGFR. The fractional excretion of TNFRs did not differ between patients with an eGFR ≥90 and those with an eGFR 60–89 mL/min/1.73 m2, and also did not correlate with eGFR. After adjustment for relevant covariates, the serum TNFRs were associated with a lower eGFR (60–89 mL/min/1.73 m2) and an increased ACR (≥30 mg/gCr), but urinary TNFRs were associated with an increased ACR (≥30 mg/gCr) alone, in the multivariate logistic model. Conclusions The pattern of fractional excretion TNFRs showed that an increase in serum TNFRs might result from their increased systemic production, including in the kidney, rather than being a simple reflection of GFR decline. Kidney measures appear to be strongly associated with serum TNFRs rather than urinary TNFRs in patients with type 2 diabetes and normal renal function., A scatter plot of the relationship between fractional excretion tumor necrosis factor receptors (TNFR) and serum or urinary TNFR levels showed that urinary TNFR levels are strongly associated with fractional excretion TNFR levels. However, serum TNFR levels are not strongly associated with fractional excretion TNFR levels.

Duloxetine has some effect against cancer neuropathic pain (CNP); however, predictors of duloxetine response are unclear. This study sought to identify predictors of duloxetine response in patients with CNP. Patients (N = 70) with CNP unresponsive to or intolerant of opioid–pregabalin combination therapy, with a brief pain inventory-short form (BPI-SF) Item 5 score (average pain) ≥ 4, and with a total hospital anxiety and depression scale score < 20, were randomized to a duloxetine or a placebo group. Multiple linear regression analysis was conducted to identify predictors of duloxetine response as a secondary analysis with the change in the average pain score on day 10 from day 0 as the dependent variable, and the following five covariates; baseline (day 0) average pain score, baseline opioid dose, continuation/discontinuation of pregabalin, and items 20 and 21 score of the short-form McGill pain questionnaire 2 (SF-MPQ-2) as independent variables. Of the four domains (continuous pain, intermittent pain, neuropathic pain, and affective descriptors) score of SF-MPQ-2 on day 0, significant differences were observed in the neuropathic pain domain (p = 0.040) in change on the average pain between day 10 and day 0 in the duloxetine group. Multiple linear regression analysis revealed that patients with a high score for SF-MPQ-2 Item 21 (tingling pain) on day 0 had a significantly greater change in average pain between day 10 and day 0 (p = 0.046). Patients with a high score for SF-MPQ-2 Item 21 might benefit more from duloxetine.

Background: There is currently no established therapy for allodynia, which is a type of neuropathic pain. However, high concentrations of topical anesthetics can anesthetize the skin and i...

Childhood idiopathic nephrotic syndrome (NS) is defined by proteinuria and hypoproteinemia. The incidence of childhood idiopathic NS varies with age, race, residential areas, and social conditions. In Japan, its incidence was estimated to be 6.49 cases/100,000 children. Our study aimed to investigate the incidence, characteristics, and rate of relapse of idiopathic NS in Fukushima between 2006 and 2016. Overall, 158 children aged from 6 months to 15 years old (65.8% male) developed idiopathic NS (median age at onset, 5.3 years). The peak age at onset was three years. The average annual incidence of childhood idiopathic NS was 5.16 (range, 3.47-9.26) cases/100,000 children. The highest incidence was in 2011, which was the year of the Great East Japan Earthquake and nuclear power plant accident, and reportedly caused psychological distress in the children at the time. Conversely, the five-year birth cohort showed minor difference from 2008 to 2012. The rate of incidence in males aged < 5 years was thrice greater than in females of the same age and almost the same for males and females aged 11-15 years. Of 507 total relapses in 115 NS children, common triggers of relapses were steroid discontinuation or reduction and infection. The average annual incidence of childhood NS based on the Fukushima population was lower than previously reported in Japan, and the annual incidence has changed over an 11-year period. These changes may be affected by social or environmental factors, including mental stress associated with lifestyle changes after the disaster.

Background High expectations regarding therapy are reported to have positive effects on future therapeutic course and related behavior. Some individuals are aware of feelings of comfort immediately after a relaxation therapy session. Methods Heart rate variability biofeedback (HRV-BF) therapy using a relaxation technique called resonant breathing was administered to 44 family caregivers who felt burdened by their work caring for family members with cancer. We prospectively evaluated how the level of comfort participants were aware of immediately after an initial therapy session affected their expectations regarding the therapy, as well as future quality of life (QOL) and autonomic function. This study was a secondary analysis of a randomized, open-label study titled “Self-care system for family caregivers of cancer patients using resonant breathing with a portable home device”. Results Among the participants, 56.8% were aware of a feeling of comfort immediately after an initial therapy session. Participants were then divided into two groups according to the presence or absence of their awareness of comfort. Expectation levels regarding the therapy were significantly increased in the awareness group after the therapy session (P = 0.003). No main effect between groups was observed for heart rate variability (HRV) during therapy (P = 0.949). Four weeks after the initial therapy session, QOL improved and HRV increased in the awareness group (P

Delirium in patients with terminal cancer is irreversible and increases treatment resistance, which leads to a deterioration in quality of life.To investigate factors affecting the effectiveness and safety of intravenous chlorpromazine for irreversible delirium in patients with terminal cancer.Multiple regression analysis for factors affecting treatment effectiveness was carried out based on a retrospective comparison between responders and nonresponders to intravenous chlorpromazine.Ninety-seven patients with terminal cancer who were treated with intravenous chlorpromazine for irreversible delirium were included.The rate of patients with ≥50% improvement in mean Nursing Delirium Screening Scale score from pretreatment to day three of chlorpromazine treatment was 0.48 (95% confidence interval [CI]: 0.38-0.58). Factors affecting chlorpromazine treatment effectiveness were hyperactive delirium (odds ratio [OR]: 6.25, 95% CI: 1.14-34.5) and longer survival (OR: 1.096, 95% CI: 1.05-1.14). The mean chlorpromazine dose was low, at 17.9 mg/day. Adverse events were reported in 11 patients (11.3%) by day three of chlorpromazine treatment, and all were observed in patients who survived less than two weeks after chlorpromazine treatment. Patients who died, who had decreased blood pressure during chlorpromazine administration, and who showed acute akathisia all displayed shock index ≥1.Intravenous administration of low-dose chlorpromazine may be an effective and safe treatment option for delirium in patients with terminal cancer who have hyperactive delirium, longer predictive prognosis, and shock index1.

Rationale: Intestinal Behçet disease (BD) with myelodysplastic syndrome (MDS) is a rare condition that is resistant to various immunosuppressive therapies. Several cases in which hematopoietic stem cell transplantation (HSCT) was effective for intestinal BD with MDS accompanying trisomy 8 have been reported. Patient concerns: We report an 18-year-old female with a 7-year history of BD. Colonoscopy demonstrated a huge ulcer in the cecum. Chromosomal examination revealed a karyotype of trisomy 8 in 87% of cells. Bone marrow examination revealed dysplastic cells in multilineages. Diagnoses: A diagnosis of intestinal BD associated with MDS accompanying trisomy 8 was made. Interventions: The patient underwent ileocecal resection due to microperforations of ileocecal ulcers; she then underwent allogeneic peripheral blood stem cell transplantation (PBSCT) with her mother as a donor. Outcomes: After the PBSCT, the patient's symptoms due to BD (fever, oral aphthae, abdominal pain, and genital ulcers) completely disappeared, with no severe adverse events. Lessons: The present case demonstrates that HSCT including PBSCT might be an effective new therapeutic option for refractory intestinal BD with MDS when immunosuppressive therapy has achieved insufficient efficacy.

Context Although opioids and pregabalin are widely used for cancer-related neuropathic pain (CNP), no clinical trials exist to determine which medications are effective when an opioid-pregabalin combination therapy fails. Objectives We investigated the efficacy of duloxetine for CNP nonresponsive or intolerant to opioid-pregabalin combination therapy. Methods A multicenter, randomized, double-blind, placebo-controlled trial was performed at 12 specialized palliative care services in Japan. Patients with CNP average pain scores (Brief Pain Inventory [BPI]–Item 5) ≥ 4 in the previous 24 hours and nonresponsive or intolerant to opioid-pregabalin combination therapy were eligible. Patients with chemotherapy-induced peripheral neuropathies were excluded. Patients were administered duloxetine 20 mg/day titrated to 40 mg/day or placebo for 10 days. The primary endpoint was BPI-Item 5 on Day 10. Responder analysis measured proportions of patients with 30% and 50% pain decreases. Results Seventy patients were enrolled. Complete case analysis revealed mean BPI-Item 5 on Day 10 of 4.03 for Group D vs. 4.88 for Group P (P = 0.053). Baseline observation carried forward analysis revealed mean BPI-Item 5 on Day 10 of 4.06 and 4.91 for Groups D and P, respectively (P = 0.048). Clinically meaningful pain improvement (≥30%) was reported by 44.1% (n = 15) of patients in Group D vs. 18.2% (n = 6) in Group P (P = 0.02); 32.4% (n = 11) vs. 3.0% (n = 1) of patients in Groups D and P, respectively, reported pain reduction ≥ 50% (P = 0.002). Conclusion Adding duloxetine to opioid-pregabalin therapy might have clinical benefit in alleviating refractory CNP. Further studies are needed to conclude the efficacy of adding duloxetine.

The objective of the study was to evaluate effectiveness and safety of intravenous chlorpromazine for the short-term treatment of insomnia in end-stage cancer patients. Insomnia occurs as one of distressing symptoms in 70% of end-stage cancer patients. End-stage cancer patients often have difficulty in oral administration because of disease progress. We retrospectively evaluated 30 end-stage cancer patients with difficulty in oral administration who received intravenous chlorpromazine for the short-term treatment of insomnia. A primary end point was sleep quality based on St. Mary's Hospital Sleep Questionnaire 3 days after the treatment. Improved sleep quality was observed on the day after the treatment and later (P .001), and the effective rate mean was 0.63 (95% confidential interval: 0.45-0.81) 3 days after the treatment. Increased total sleep time and decreased sleep latency time were observed 3 days after the treatment (P .001); however, no improvement in depth of sleep was achieved (P = .231). There was no adverse event except for two delirium cases. The study indicated that intravenous chlorpromazine can be applied safely and effectively for the short-term treatment of insomnia in end-stage cancer patients with difficulty in oral administration.

Clitoral hypertrophy is caused by disorders of sex development and it is observed from birth in most cases. We encountered a patient in whom normal morphology at birth may have acquired deformity and hypertrophy. The patient was a 10-year-old girl with a chief complaint of pudendal deformity. The clitoral hood was enlarged and the clitoris size was 8 x 5 mm on the first examination. Various tests were performed. Sex chromosome or hormonal abnormalities and tumorous lesions were not detected, and the ovaries, uterus, and vagina were normal, indicating that disorders of mullerian development were negative. In surgery, reconstruction of the vulva was performed following the Marberger method. The present case may have been a very rare case of acquired hypertrophy of unknown origin.

Self-care systems for early-stage specialist palliative care for cancer patients and their family caregivers have received much attention recently. Resonant breathing is an established method for maximizing heart rate variability (HRV), but it has not been implemented for home self-care.We aimed to examine the usefulness and ease of implementation for family caregivers to administer resonant breathing using a portable device at home.We divided caregivers into two groups-a home self-care group and a control group-and we conducted a randomized open-label study, with rate of change in HRV being the primary outcome.We administered HRV biofeedback (HRV-BF) using resonant breathing to 54 family caregivers who felt burdened by their nursing care responsibilities.Among the self-care group, 92.6% of participants completed the study in their homes; 28 days after intervention initiation, the resonant breathing implementation rate at home was 86.1%. There was an interaction between time course and grouping in our HRV comparisons: the change rate in the home self-care group was higher during HRV-BF than before HRV-BF.Because family caregivers in our study learned to quickly administer resonant breathing using a portable device at home, resonant breathing improved rapidly, along with autonomic nerve function and quality of life.

Few studies have reported the efficacy of trigger point injection (TPI) to myofascial trigger points (MTrPs) in advanced cancer patients. Factors that are associated with TPI efficacy have not yet been elucidated.The study was aimed at evaluating factors that are associated with TPI efficacy to MTrPs in advanced cancer patients.Factors that are associated with TPI efficacy were retrospectively identified based on a comparison between clinically relevant responders and nonresponders by using multivariate regression analysis.One hundred five advanced cancer patients who visited the Palliative Care Department with a chief complaint of pain and who received TPI treatment to the MTrP at the pain site.The TPI efficacy rate on the day after TPI treatment was 0.59 (95% confidence interval [CI]: 0.50-0.68). Significant factors associated with TPI efficacy were coexistence of cancer pain with MTrP at the pain site (odds ratio [OR]: 3.87, 95% CI: 1.21-12.4), MTrP at areas other than lower back or hip (OR: 6.45, 95% CI: 1.98-21.0), and fewer MTrPs (OR: 0.64, 95% CI: 0.42-0.99). Coexistence of cancer pain at the pain site of the chief complaint was observed in 64% of study subjects (95% CI: 0.55-0.73).The TPI efficacy is likely high when advanced cancer patients have fewer MTrPs together with cancer pain at areas other than the lower back or hip. MTrPs in advanced cancer patients are more commonly observed together with cancer pain rather than independently. Healthcare providers should recognize the relationship between MTrP and cancer pain and proactively perform physical examinations to detect MTrPs for potential TPI.