Acute cerebellitis following COVID-19 infection associated with autoantibodies to glutamate receptors: a case report.

While COVID-19 infection by the SARS-CoV-2 virus was initially identified as a respiratory disease, mounting evidence suggests its association with various neurological issues as well. Notably, COVID-19 has been linked to acute cerebellitis (AC) and post-infectious cerebellar ataxia. The precise underlying mechanisms behind these neurological effects remain unclear. Our case report describes AC following COVID-19 infection, associated with autoantibodies to glutamate receptors (GluRs), hinting at immunological involvement. The case is a 56-year-old woman who experienced fever and fatigue due to COVID-19 infection. About 2 weeks after these symptoms improved, she showed cerebellar symptoms such as ocular overshoot and ataxia when presenting to our hospital. Her cerebrospinal fluid (CSF) findings were normal. Brain MRI revealed cerebellar abnormalities. Treatment with methylprednisolone led to symptom improvement. Later tests of CSF yielded positive results for autoantibodies to GluRs. Our findings suggest a possible immune-mediated mechanism in the onset of AC following COVID-19 infection. Clinicians should consider the possibility of immunological pathogenesis when diagnosing cerebellar symptoms after COVID-19 infection.

Evaluation of safety and diagnostic performance for flexible hysteroscopy in 1591 outpatient cases.

BACKGROUND: To date, only few large studies are available concerning the safety and diagnostic concordance rates of outpatient flexible hysteroscopy. In our institution, outpatient hysteroscopy has been routinely and educationally applied Kosuke Tsuji to intrauterine lesions; thus, we retrospectively investigated the institution's outpatient flexible hysteroscopy cases. METHODS: A total of 1591 cases of outpatient flexible hysteroscopy conducted at our institution in 2012-2016 were retrospectively analyzed in terms of their clinical background, complications and diagnostic concordance rates. RESULTS: A total of 1591 cases included 546 cases of benign tumors (317 endometrial polyps, 168 myomas and 61 endometrial hyperplasia), 361 cases of atypical endometrial hyperplasia, 571 cases of endometrial cancers and 113 cases of other diagnoses. No major complications, including uterine perforation, occurred. However, one patient (0.06%) was diagnosed with septic shock caused by intrauterine infection that required prolonged immunosuppressive drug administration. Meanwhile, 335 patients diagnosed with benign tumors through outpatient flexible hysteroscopy underwent operation, and the diagnostic concordance rate was 74.6% (250 cases). However, this rate included 14 cases (4.2%) diagnosed with malignant tumors postoperatively. In preoperative endometrial cancer cases, the sensitivity and specificity for cervical invasion diagnosis were 39.4 and 90.8%, respectively. In addition, only one patient manifested positive ascites cytology intraoperatively, possibly caused by outpatient hysteroscopy. CONCLUSIONS: Outpatient flexible hysteroscopy is highly safe, with a slight negligible effect on ascites cytology. However, the diagnosis should be determined by multidisciplinary approaches, as hysteroscopy alone can miss malignancy.

Renal abscess with bacteremia caused by extended-spectrum ß-lactamase-producing Escherichia coli: a case report.

BACKGROUND: Renal abscess in children is a rare and severe form of infectious kidney disease that is responsible for several serious complications. In this report, we describe a previously healthy 5-year-old girl with a renal abscess caused by extended-spectrum ß-lactamase (ESBL)-producing Escherichia coli (E. coli), which led to bacteremia and renal scarring. CASE PRESENTATION: The patient presented to our department with high fever, headache, vomiting for 2 days and high inflammatory response. We diagnosed her with a urinary tract infection and initiated treatment with ampicillin and cefotaxime. Gram-negative bacilli bacteremia was noted on day 3. On day 4, her fever persisted, and a computed tomography (CT) scan revealed a renal abscess in the left kidney. After identifying the bacteria as ESBL-producing E. coli from the blood culture, we switched to the antibiotic meropenem and continued treatment for 3 weeks. The renal abscess was not drained. Although the renal abscess was successfully treated and it disappeared, a low-density area remained in same lesion on subsequent CT scans and a dimercaptosuccinic acid renal scan performed 4 months after onset revealed renal scarring. CONCLUSION: Given the increasing prevalence of ESBL-producing microorganisms, clinicians should be aware of the possibility of renal abscesses caused by community-acquired ESBL-producing organisms even in previously healthy children. Once a renal abscess is suspected, early diagnosis and management are important for reducing the risk of life-threating complications and renal scarring.

Cerebrospinal fluid 5-HIAA concentrations correlate with cardiac uptake of 123I-MIBG during myocardial scintigraphy in drug naïve Parkinson's disease.

We examined the correlations between cerebrospinal fluid (CSF) concentrations of homovanillic acid (HVA) and 5-hydroxyindole acetic acid (5-HIAA) and imaging assessment scores, using 123I-Ioflupane SPECT and 123I-MIBG myocardial scintigraphy in 23 drug naïve PD patients. The CSF 5-HIAA concentration correlated with the H/M ratio of the delayed image (r = 0.458, p < 0.05) and the washout rate (r = - 0.642, p < 0.01) of 123I-MIBG myocardial scintigraphy. These correlations suggest some unclarified pathophysiological links between the central serotonergic and cardiac sympathetic systems.

Effect of operating temperature on anaerobic digestion of the Brazilian waterweed Egeria densa and its microbial community.

To develop an effective treatment for the globally invasive Brazilian waterweed Egeria densa, anaerobic digestion was observed at 37 °C, 55 °C, and 65 °C. The average methane production rate at 55 °C was 220 mL L-1 day-1, which was two-fold that at 37 °C and 65 °C. Volatile fatty acid accumulation was detected under thermophilic conditions; however, although there was methane production, the system did not shutdown. The microbial communities differed between mesophilic (37 °C) and thermophilic (55 °C and 65 °C) conditions. A bacterial community consisting of the phyla Bacteroidetes (43%), Firmicutes (37%), Proteobacteria (9%), Synergistetes (5%), Spirochaetes (1%), and unclassified bacteria (5%) were detected under mesophilic condition. In contrast, the phylum Firmicutes was dominant under thermophilic conditions. In the archaeal community, Methanosaeta concilii (40%), Methanolinea sp. (17%), and unclassified euryarchaeota (43%) were detected under mesophilic condition. Methanosarcina thermophila (87% at 55 °C, 54% at 65 °C) and Methanothermobacter thermautotrophicus (13% at 55 °C, 46% at 65 °C) were detected under thermophilic conditions. At both 37 °C and 55 °C, acetoclastic methanogenesis likely occurred because of the lower abundance of hydrogenotrophic methanogens. At 65 °C, the growth of the acetoclastic methanogen Methanosarcina thermophila was limited by the high temperature, therefore, acetate oxidation and hydrogenotrophic methanogenesis may have occurred.

Relationship between epicardial adipose tissue thickness and early impairment of left ventricular systolic function in patients with preserved ejection fraction.

Epicardial adipose tissue (EAT) is metabolically bioactive fat. The present study aimed to clarify the relationship between EAT amount and early impairment of left ventricular (LV) systolic function in patients with preserved ejection fraction (EF), all evaluated echocardiographically. Participants comprised 62 elderly women (mean age ± standard deviation, 68 ± 11 years) with lifestyle-related diseases and EF ≥ 60 %. EAT amount was evaluated as thickness. Parameters suggesting early impairment of systolic function such as decreases in systolic mitral annular velocity (S') and tissue mitral annular displacement percentage (TMAD %) were evaluated along with EF. Correlations between EAT thickness and these LV systolic functions were assessed. Influences of various factors on the resultant significant relationships were also assessed. EAT thickness correlated inversely with S' and TMAD % (r = -0.402, p = 0.001 and r = -0.585, p < 0.001, respectively), but did not correlate with EF (r = 0.054, not significant). These significant relationships were maintained after considering factors such as body mass index, age, presence of lifestyle-related diseases and blood test results. A significant relationship existed between EAT amount and early impairment of LV systolic function in patients with preserved EF. Accumulation of EAT might contribute to the initial development of LV systolic dysfunction.

Relationship between Pericardial Adipose Tissue Thickness and Early Impairment of Left Ventricular Function, Both Evaluated on Echocardiography.

Epicardial adipose tissue (EAT) is metabolically bioactive, and accumulation of this tissue is related to early impairment of left ventricular (LV) systolic function as well as diastolic function. However, pericardial adi- pose tissue (PAT), located outside the EAT, has recently been demonstrated to be more closely associated with metabolic risk factors than EAT. The present study aimed to clarify whether PAT thickness is related to early impairment of LV function in a similar manner to EAT thickness, with both evaluated echocardio- graphically. Subjects were 49 women (mean age, 68ill years) composed of both patients with metabolic diseases and those with other diseases, and ejection fraction (EF) >55%. Systolic function was assessed by measuring EF, systolic mitral annular velocity (S'), and tissue mitral annular displacement percentage (TMAD%). Diastolic function was assessed by measuring early rapid filling wave velocity (E)/late filling wave velocity due to atrial contraction (A) ratio (E/A), peak early diastolic mitral annular velocity (e'), and E/e' ratio. Correlations between EAT or PAT thickness and LV systolic or diastolic function were assessed. EAT thickness correlated with S' and TMAD%(r=-0.399, p=0.005 and r=-0.570, p<0.001, respective- ly), but not with EF. However, PAT thickness was not correlated with any of these. EAT thickness corre- lated with E/A, e' and E/e'(r=-0.382, p=0.007; r=-0.493, p<0.001; and r=0.331, p=0.020, respective- ly). Again, PAT thickness was not correlated with any of these. PAT thickness appears unrelated to early impairment of LV function.

Diagnostic performance of the classic symptom "abdominal pain before vomiting" for pediatric acute appendicitis.

BACKGROUND: Acute appendicitis is the most common type of acute abdomen that requires surgical intervention in children. According to general pediatric textbooks, the presence of vomiting before abdominal pain is considered a classic patient history item for excluding acute appendicitis. However, its diagnostic performance in the pediatric population has yet to be investigated. METHODS: This was a single-center retrospective observational study involving 134 children who were admitted to the hospital with both abdominal pain and vomiting. The reference standard for appendicitis was defined by computed tomography scanning. The diagnostic performance of "abdominal pain before vomiting" was calculated and compared to those of the Alvarado score and pediatric appendicitis score. RESULTS: The diagnostic performance of "abdominal pain before vomiting" was as follows: sensitivity of 75.3% (95% confidence interval [CI], 64.7-83.6), specificity of 25.0% (95% CI, 15.5-36.7), positive likelihood ratio of 1.00 (95% CI, 0.82-1.22), negative likelihood ratio of 0.99 (95% CI, 0.54-1.79), and diagnostic odds ratio of 1.02 (95% CI, 0.46-2.25). In contrast, the Alvarado score and pediatric appendicitis score (with a threshold of 4 points) demonstrated favorable sensitivity (98.3% [95% CI, 92.4-99.6]), negative predictive value (94.6% [95% CI, 78.4-98.8]), negative likelihood ratio (0.04 [95% CI, 0.01-0.23]), and diagnostic odds ratio (49.9 [95% CI, 6.88-243.2]). CONCLUSION: In this study, "abdominal pain before vomiting" was associated with poor diagnostic performance for ruling out acute pediatric appendicitis. Thus, the diagnosis of acute appendicitis in the pediatric population should be made based on existing validated scoring systems such as the Alvarado score and pediatric appendicitis score.

Case report: Recovery from refractory myasthenic crisis to minimal symptom expression after add-on treatment with efgartigimod.

Myasthenic crisis, a life-threatening exacerbation of myasthenia gravis, is a significant clinical challenge, particularly when refractory to standard therapies. Here, we described a case of myasthenic crisis in which the patient transitioned from refractory myasthenic crisis to minimal symptom expression after receiving add-on treatment with efgartigimod, a novel neonatal Fc receptor antagonist. A 54 years-old woman who was diagnosed with anti-acetylcholine receptor antibody-positive myasthenia gravis experienced respiratory failure necessitating mechanical ventilation. Despite aggressive treatment with plasmapheresis, intravenous immunoglobulins, and high-dose corticosteroids, her condition continued to deteriorate, culminating in persistent myasthenic crisis. Efgartigimod was administered as salvage therapy. Remarkable improvement in neuromuscular function was observed within days, allowing for successful weaning from mechanical ventilation. Over the subsequent weeks, the patient's symptoms continued to ameliorate, ultimately reaching a state of minimal symptom expression. Serial assessments of her serum anti-acetylcholine receptor antibody titer showed a consistent decline in parallel with this clinical improvement. This case highlights efgartigimod's potential as an effective therapeutic option for refractory myasthenic crisis, offering new hope for patients facing this life-threatening condition.

The right amygdala and migraine: Analyzing volume reduction and its relationship with symptom severity.

This study aimed to explore the relationship between gray matter volume changes and various clinical parameters in patients with migraine, focusing on symptom severity, quality of life, and states of depression and anxiety. Using a case-control design, we examined 33 patients with migraine, with or without aura, and 27 age-matched healthy subjects. We used magnetic resonance imaging to assess the volumes of 140 bilateral brain regions. Clinical evaluations included the Migraine Disability Assessment, the Migraine Specific Quality of Life Questionnaire, the Center for Epidemiologic Studies Depression scale, Spielberger's State and Trait Anxiety scales, and the Japanese version of the Montreal Cognitive Assessment. We compared the scores of these measures between migraine patients and healthy controls to examine the interplay between brain structure and clinical symptoms. Significant volumetric differences were observed in the pallidum and amygdala between migraine patients and healthy individuals. The reduction in the right amygdala volume correlated significantly with migraine severity as measured by the Migraine Disability Assessment. Path analysis revealed a model where Migraine Disability Assessment scores were influenced by Migraine Specific Quality of Life Questionnaire outcomes, which were further affected by depression, anxiety, and a low right pallidum volume. Our findings suggest that the chronicity and severity of migraine headaches specifically affect the right amygdala. Our path model suggests a complex relationship whereby migraine disability is strongly influenced by quality of life, which is, in turn, affected by psychological states, such as anxiety and depression.

Archaeal community structures in the solfataric acidic hot springs with different temperatures and elemental compositions.

Archaeal 16S rRNA gene compositions and environmental factors of four distinct solfataric acidic hot springs in Kirishima, Japan were compared. The four ponds were selected by differences of temperature and total dissolved elemental concentration as follows: (1) Pond-A: 93°C and 1679 mg L(-1), (2) Pond-B: 66°C and 2248 mg L(-1), (3) Pond-C: 88°C and 198 mg L(-1), and (4) Pond-D: 67°C and 340 mg L(-1). In total, 431 clones of 16S rRNA gene were classified into 26 phylotypes. In Pond-B, the archaeal diversity was the highest among the four, and the members of the order Sulfolobales were dominant. The Pond-D also showed relatively high diversity, and the most frequent group was uncultured thermoacidic spring clone group. In contrast to Pond-B and Pond-D, much less diverse archaeal clones were detected in Pond-A and Pond-C showing higher temperatures. However, dominant groups in these ponds were also different from each other. The members of the order Sulfolobales shared 89% of total clones in Pond-A, and the uncultured crenarchaeal groups shared 99% of total Pond-C clones. Therefore, species compositions and biodiversity were clearly different among the ponds showing different temperatures and dissolved elemental concentrations.

Calcitonin gene-related peptide enhances experimental autoimmune encephalomyelitis by promoting Th17-cell functions.

T(h)17 cells, an inflammatory T helper cell subset, are involved in the pathogenesis of various inflammatory, autoimmune and allergic diseases. Recent evidence supports the idea that immune cell functions and the inflammatory response are finely regulated by various physiological substances. Calcitonin gene-related peptide (CGRP), a neuropeptide released from the sensory nerve endings, is one of these mediators. By binding to its receptor composed of receptor activity-modifying protein 1 (RAMP1) and calcitonin receptor-like receptor, CGRP modulates various immune cell functions, but the function of CGRP in T(h)17 cells is largely unknown. Here, we investigated the effect of CGRP signaling on T(h)17 cells and T(h)17 cell-mediated inflammation and observed that CGRP activates nuclear factor of activated T cells c2 through cAMP/PKA to increase IL-17 production in vitro. In vivo, IL-17 production is suppressed in RAMP1-deficient mice in the experimental autoimmune encephalomyelitis (EAE) model and RAMP1-deficient mice are completely resistant to EAE. Furthermore, T(h)17 cell function and EAE induction are also suppressed in T cell-specific RAMP1-deficient mice. Taken together, our findings indicate that CGRP promotes T(h)17 cell-mediated autoimmune inflammation through the regulation of IL-17 expression.

Calcitonin gene-related peptide is an important regulator of cutaneous immunity: effect on dendritic cell and T cell functions.

Some cutaneous inflammations are induced by percutaneous exposure to foreign Ags, and many chemical mediators regulate this inflammation process. One of these mediators, calcitonin gene-related peptide (CGRP), is a neuropeptide released from nerve endings in the skin. CGRP binds to its receptors composed of receptor activity-modifying protein 1 and calcitonin receptor-like receptor to modulate immune cell function. We show that CGRP regulates skin inflammation under physiological conditions, using contact hypersensitivity (CHS) models of receptor activity-modifying protein 1-deficient mice. CGRP has different functions in CHS responses mediated by Th1 or Th2 cells; it inhibits Th1-type CHS, such as 2,4,6-trinitrochlorobenzene-induced CHS, but promotes Th2-type CHS, such as FITC-induced CHS. CGRP inhibits the migration of Langerin(+) dermal dendritic cells to the lymph nodes in 2,4,6-trinitrochlorobenzene-induced CHS, and upregulates IL-4 production of T cells in the draining lymph nodes in FITC-CHS. These findings suggest that CGRP regulates several types of CHS reactions under physiological conditions and plays an important role in cutaneous immunity.

A red-emissive aminobenzopyrano-xanthene dye: elucidation of fluorescence emission mechanisms in solution and in the aggregate state.

We have designed and synthesized a new class of rhodamine dyes with an extended π-conjugated system and named them 3',3''-bis(oxospiroisobenzofuran)-3,7-bis(diethylamino)benzopyrano-xanthene (ABPX01) dyes. ABPX01 exhibits fluorescence emission in both dilute solution and the aggregate state, whereas conventional rhodamine dyes show aggregation-induced quenching (AIQ). The chemical species of ABPX01 in solution were determined by spectrophotometric measurements and density functional theory (DFT) calculations to study the relationship among chemical species, color, and fluorescence emission. ABPX01 has various forms: the spirolactone form (ABPX01(0)), which is colorless; and the monocationic form (ABPX01H(+)) and the dicationic form (ABPX01H(2)(2+)), which are colored. By orienting a pair of spirolactone benzene moieties differently, the stereoisomers of trans- and cis-ABPX01(0) were separated and their crystal structures determined. ABPX01H(2)(2+) was identified to be a red fluorescent species. Detailed spectroscopic and electron microscopic investigations led to the assumption that the ABPX01H(2)(2+) formed ion associates with Cl(-) as counter anions in HCl aqueous solution, and the nano- and submicrometer-sized colloidal aggregates of ABPX01 hydrochloride exhibit fluorescence emission. To further verify the aggregation-induced emission enhancement (AIEE) mechanism, ABPX01 hydrochloride was synthesized and its fluorescence was similarly checked in the powder state. AIEE in ABPX01 might be attributed to the synergistic combination of the restriction of dye-dye interaction induced dimer formation by sterically hindered ion associates and carboxylic benzene moieties, and the structural rigidity and intermolecular arrangement of the xanthene moiety. We expect that the design strategy of ABPX dyes will be extended to the development of a wide variety of functional organic-dye-based fluorophores (ODFs) with suitable fluorescence-emission controlled mechanisms for many useful applications in new electroluminescent devices.

Relationship between underreporting of energy intake and blood ketone levels in Japanese women with obesity: A retrospective study.

Underreporting is a problem in dietary surveys, and data on Japanese individuals with obesity are lacking. In addition, in dietary surveys of individuals with obesity, underreporting and extreme energy restrictive practices for short periods of time have been reported, and blood total ketone levels (ketone bodies) may be able to distinguish between these factors. The present study aimed to examine the relationship between underreporting [energy intake (EI)/basal metabolic rate estimate (BMR)] and ketone bodies in obese Japanese women. The participants included 91 women with obesity aged 47±9 years with a body mass index (BMI) of 29.8±3.9 kg/m² who met the exclusion criteria out of 164 individuals who participated in an institutional cohort study baseline survey between September 2006 and September 2015. The current study defined the relationship between EI/BMR, BMI and the participants' ketone body levels. EI/BMR <1.35 and ketone body level <1.0 mmol/l was defined as underreporters, while EI/BMR <1.35 and ketone body level ≥1.0 mmol/l was defined as energy-restricted reporters based on previous research. The EI/BMR of the participants was 1.44±0.32, and 25.3% had an abnormally high level of ketone bodies. Multiple regression analysis indicated that ketone bodies were explanatory variables for EI/BMR. Analysis using EI/BMR and ketone bodies estimated that 26.4% were underreporters and 12.1% were energy-restricted reporters. There were no significant differences in reported energy intake, carbohydrate intake (g/day), and percentage carbohydrate (%) between the underreporters and energy-restricted reporters. In conclusion, low EI/BMR was associated with high ketone body levels in Japanese women with obesity. The combination of EI/BMR and ketone bodies may distinguish between or screen for underreporters and energy-restricted reporters during a dietary survey.

Risk factors for anemia of prematurity among 30-35-week preterm infants.

BACKGROUND: The risk factors for anemia of prematurity (AOP) among late preterm infants are unelucidated. We identified risk factors for declining hemoglobin (Hb) concentration and triggering factors for AOP treatment in infants born at 30-35 gestational weeks. METHODS: From 2012 to 2020, we conducted a single-center retrospective study of infants born at 30-35 weeks of gestation without congenital anomalies or severe hemorrhage. The primary outcome was AOP development, defined by initiation of treatments including red blood cell transfusion, subcutaneous injections of erythropoietin, and iron supplementation. A multivariable logistic regression model was used to investigate potential risk factors for AOP. RESULTS: A total of 358 infants were included. Lower gestational age (odds ratio, 0.19; 95% confidence interval 0.11-0.32), small for gestational age (SGA; 7.17, 2.15-23.9), low maternal Hb level before birth (0.66, 0.49-0.87), low Hb at birth (0.71, 0.57-0.89), and multiple large blood samplings (1.79; 1.40-2.29) showed significantly higher odds for AOP development. CONCLUSIONS: Gestational age, SGA, low maternal Hb before birth, Hb at birth, and high number of large blood samplings were positively associated with AOP development in infants born at 30-35 gestational weeks.

Acute Focal Bacterial Nephritis Caused by Staphylococcus simulans.

A toddler girl presented to our hospital with a fever that lasted for five days. She had no prior history of urinary tract infections or contact with farm animals. Investigations revealed a diagnosis of acute focal bacterial nephritis (AFBN), and we initiated antimicrobial therapy with ampicillin and cefmetazole. On day five, methicillin-resistant coagulase-negative staphylococci were detected in her urine culture, and we changed the antibiotics to vancomycin. Antibiotic therapy was continued for 21 days, with no recurrence of fever. Finally, the bacteria were identified as Staphylococcus (S.) simulans, which is a common farm animal pathogen. Clinicians should be aware of the possibility of AFBN caused by S. simulans, even if the patient has no prior history of close contact with farm animals. If a rare organism is detected in urine culture during AFBN treatment, the patient should be treated with appropriate antibiotics for the pathogen.

Recurrence of Myopericarditis Following mRNA COVID-19 Vaccination in a Male Adolescent.

COVID-19 is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The disease has spread worldwide, resulting in health and economic crises. Vaccination against SARS-CoV-2 infection is considered a valid prevention measure to control this pandemic. There have been reports of cases of myopericarditis following mRNA COVID-19 vaccination. We present a case of a 20-year-old man with recurrent myopericarditis following an initial episode of influenza virus-induced myopericarditis and after a second dose of the mRNA-1273 Moderna COVID-19 vaccine. Careful attention should be paid to patients with a history of myocarditis following COVID-19 vaccination.

COVID-19 est causée par le coronavirus du syndrome respiratoire aigu sévère 2 (SRAS-CoV-2). La maladie qui s'est répandue dans le monde a entraîné des crises sanitaire et économique. La vaccination contre l'infection à SRAS-CoV-2 est considérée comme une mesure de prévention valide pour juguler la pandémie. Des cas de myopéricardite ont été déclarés après le vaccin à ARNm contre la COVID-19. Nous présentons le cas d'un homme de 20 ans qui a eu une myopéricardite récurrente après un épisode de myopéricardite induite par le virus de l'influenza et après une deuxième dose du vaccin à ARNm-1273 contre la COVID-19 de Moderna. Il faudrait porter une attention particulière aux patients qui ont des antécédents de myocardite après la vaccination contre la COVID-19.

Simultaneous assay of urine sepiapterin and creatinine in patients with sepiapterin reductase deficiency.

OBJECTIVES: Sepiapterin reductase deficiency (SRD) causes central nervous system symptoms due to dopamine and serotonin depletion because sepiapterin reductase plays an important role in tetrahydrobiopterin biosynthesis. SRD cannot be detected by newborn screening because of the absent hyperphenylalaninemia. To diagnose SRD biochemically, confirmation of reduced monoamine metabolites and elevated sepiapterin in the cerebrospinal fluid (CSF) has been considered necessary, because a past study showed no elevation of urine sepiapterin. Recently, however, the elevation of urine sepiapterin in SRD was reported. METHODS: We developed a fast method to measure sepiapterin and creatinine simultaneously using high-performance liquid chromatography with fluorescence and ultraviolet detection. Urine sepiapterin and creatinine were measured in three SRD patients, two SRD carriers, four SRD siblings, and 103 non-SRD patients. RESULTS: In the three SRD cases, concentrations of urine sepiapterin were 1086, 914, and 575 µmol/mol creatinine (upper limit: 101.7 µmol/mol creatinine), and were markedly higher than those in other groups. CSF sepiapterin concentration was also measured in one SRD case and it was 4.1 nmol/L (upper limit: 0.5 nmol/L). CONCLUSIONS: The simultaneous determination of urine sepiapterin and creatinine appears helpful for the diagnosis of SRD. This assay system can also be used to measure sepiapterin in the CSF.

Sonographically Measured Adipose Tissue Thickness Correlates with Laboratory Test Abnormalities Reflecting Metabolic State in Elderly Women.

Background: Accumulation of adipose tissue progresses to metabolic diseases. Sonography is a convenient modality for measuring the thickness of adipose tissue. The present study aimed to clarify the site of adipose tissue thickness that correlated best with laboratory test values reflecting metabolic abnormalities. Methods: Subjects comprised 37 elderly women with metabolic diseases or an almost healthy state (median age, 71 years; interquartile range, 62-78 years). Abdominal visceral adipose tissue (VAT), subcutaneous adipose tissue, peritoneal adipose tissue, perirenal adipose tissue, and epicardial adipose tissue (EAT) thicknesses were measured. Correlations were evaluated between laboratory test values and these adipose tissue thicknesses. Results: VAT thickness measured at the level of the umbilicus correlated positively with values of triglycerides (TGs) (r = 0.593, P = 0.0009) and hemoglobin A1c (r = 0.490, P = 0.0081) and negatively with the value of high-density lipoprotein cholesterol (r = -0.521, P = 0.0045), even after adjusting for body mass index. Significant positive correlations were also found between EAT thickness and TGs (r = 0.542, P = 0.0029). Conclusions: Among the adipose tissue thicknesses measured at several sites by sonography, VAT thickness correlated most closely with laboratory test values representing metabolic abnormalities in elderly women.