HLA analysis of immune checkpoint inhibitor-induced and idiopathic isolated ACTH deficiency

Pituitary - Isolated adrenocorticotropic hormone deficiency is a rare disease; however, since immune check point inhibitors (ICIs) have become widely used, many more cases have been reported. In...     

Diagnosis of a case of ectopic parathyroid adenoma on the early image of 99mTc-MIBI scintigram

We report the case of a 64-year-old woman who had a severe hypercalcemia. Serum calcium, intact parathyroid hormone (PTH), 1alpha, 25 (OH)(2 )vitamin D(3) levels were all elevated, and serum phosphorus level was decreased, which were all consistent with primary hyperparathyroidism (PHPT). (201)Tl/(99m)Tc subtraction scintigraphy failed to detect any abnormal accumulation in the neck and chest, while (99m)Tc-MIBI scintigraphy demonstrated the focal accumulation of increased radiotracer uptake in the mediastinum only on the early image, but not on the delayed image. Neck and chest computerized tomography scanning showed a small nodule at the retrosternal region, and a selective venous sampling study of the intact PTH suggested PTH production from the nodule. Together with the observation of the early image of (99m)Tc-MIBI scintigraphy, it was diagnosed that the patient had an ectopic parathyroid adenoma. Video-assisted thoracic surgery was performed. A 15-mm diameter mass, visualized by an intravenous infusion of methylene blue, was excited. The histopathology was consistent with the parathyroid adenoma. The adenoma was composed of mainly chief cells and rarely oxyphil cells. The absence of oxyphil cells would explain the lack of (99m)Tc-MIBI retention on late-phase imaging in our case. Even without uptake on the delayed image of (99m)Tc-MIBI scintigram, the early image was available for the localization of an ectopic parathyroid adenoma.     

Potential risk caused by the lack of recognition of coronary spasm: analysis of the coronary spasm questionnaire in Japan

BACKGROUND: The spasm provocation test (SPT)is no longer widely used in patients with undiagnosed chest pain syndromes in the USA and Europe. Objectives. The clinical significance of the SPT was examined in Japan and compared with the frequency of coronary spastic angina (CSA) in institutions with and without SPT screening. METHODS AND RESULTS: Questionnaires concerning the number of cases of coronary angiography (CAG), percutaneous coronary intervention (PCI), and invasive/non-invasive SPT in 2005 were sent to members of the Japanese Circulation Society in 1,177 cardiology hospitals. Completed surveys were returned from 208 hospitals (17.7%). Non-invasive SPT was performed in only 27 hospitals (13.0%). Invasive SPT was not performed in 50 (24.0%) institutions, and performed in the remaining 158 institutions(< 10 cases/year: 29.8%, > or = 10< 50: 33.7%, > or = 50< 100: 8.7%, > or = 100: 3.8%). There was a close correlation between the number of acetylcholine/ergonovine SPTs and the number of CSA cases finally diagnosed (acetylcholine: r(2)= 0.907, ergonovine: r(2) = 0.76). There was no difference in the number/year of CAG (525+/-451 vs 513 +/-888, NS) and PCI(175+/-156 vs 144+/-225, NS) between institutions with and without SPT screening. However, the number of CSA cases (15.6+/-21.6 vs 4.2 +/-13.0, p < 0.01) and variant angina cases (3.3+/-7.2 vs 1.4+/-2.4)in hospitals with SPT screening was higher than hospitals without SPT screening. CONCLUSIONS. If Japanese cardiologists discontinue use of the SPT as in the USA and Europe, occurrence of CSA may disappear in the near future in Japan.     

Properties of ipsilateral retinogeniculate afferents in albino and hooded rats

By recording single unit activities from the dorsal lateral geniculate nucleus in albino and hooded rats, physiological properties of the ipsilateral retinogeniculate afferents were compared with those of the contralateral ones. The results show that the ipsilateral retinogeniculate pathway was characterized by intermediate conduction velocities, relatively high incidence of the tonic response and the visual field representation of central 30° from the vertical midline on both sides.     

Clinical impact of gastroesophageal reflux disease in patients with subacute/chronic cough

BackgroundWhile gastroesophageal reflux disease (GERD) is one of the commonest causes of subacute/chronic cough along with cough-variant asthma (CVA) and rhinosinusitis, its clinical impact remains unknown. Therefore, we sought to investigate the impact of GERD in patients with subacute/chronic cough.MethodsBetween April 2012 and March 2018, a total of 312 patients presenting subacute or chronic cough lasting for ≥3 weeks [median cough duration, 4.9 (0.7–434) months] underwent diagnostic tests. GERD symptoms and cough-specific QoL were evaluated through the Frequency Scale for Symptoms of Gastroesophageal reflux (FSSG) and the Japanese version of the Leicester Cough Questionnaire (J-LCQ). According to the FSSG domains, patients with GERD were arbitrarily categorized into 3 groups; acid-reflux predominant, dysmotility predominant, and pauci-symptoms groups, respectively.ResultsThe average scores of J-LCQ was 12.5 (SD3.7). One hundred-forty three were diagnosed as having GERD-related cough based on classical reflux symptoms including heartburn and characteristic triggers of cough such as phonation, rinsing, lying, and eating. Most of them (89.8%) had other causative diseases including CVA. Cough lasted longer (p = 0.019) and required a longer time until alleviation (p = 0.003) in patients with GERD than in those without GERD. They also scored lower J-LCQ than counterpart group (p < 0.0001). In terms of symptom stratification, dysmotility predominant group showed significant more response to specific GERD treatments than the remnants (p = 0.002).ConclusionsThese results indicate that GERD is associated with the aggravation of other causes including CVA. Particularly, dysmotility symptoms may be potential therapeutic target for GERD-related cough.     

Isolation of a vascular cell wall-specific monoclonal antibody recognizing a cell polarity by using a phage display subtraction method

Using a strategy consisting of (i) the isolation of cell walls from synchronously differentiating cells of Zinnia, (ii) the generation of mAbs with an antibody phage display method, and (iii) screening with a subtraction method, we isolated mAbs recognizing vascular development-specific cell wall components without prior antigen identification. One of the isolated mAbs, designated CN 8, recognized a cell wall component contained in the hemicellulosic fraction. Immunohistochemical analyses showed that the CN 8 epitope was localized to the cell wall of immature tracheary elements and xylem parenchyma cells. In immature tracheary elements, the CN 8 epitope had a polarized localization pattern regardless of whether the cells are formed as parts of vessels in situ or as single tracheary elements in vitro, suggesting that cell polarity autonomously formed on the cell wall may function in tracheary element differentiation.     

Resuscitation from fulminant myocarditis associated with refractory ventricular fibrillation

Resuscitation was possible in a case of fulminant myocarditis with refractory ventricular fibrillation (Vf) using a percutaneous cardiopulmonary support system (PCPS). A 46-year old Japanese man suddenly experienced cardiopulmonary dysfunction shortly after the onset of flu symptoms, was promptly diagnosed as having fulminant myocarditis and PCPS was immediately initiated. On the second day in the hospital, refractory Vf occurred, which lasted for approximately 2h despite repeated efforts to terminate it. Finally, a large dose of steroids was administered. From the third day of hospitalization and onwards, the Vf disappeared totally. The patient completely recovered from such a serious state in 6 months. During the following 3 years, he has had no clinical symptoms of worsening. As in this case demonstrates, most myocarditis is curable and invasive measures are very helpful in rescuing patients from the fulminant type with refractory Vf.     

The relationship between bone turnover and body weight, serum insulin-like growth factor (IGF) I, and serum IGF-binding protein levels in patients with anorexia nervosa

Malnutrition is one of the risk factors for bone loss in patients with anorexia nervosa (AN). To clarify the effects of nutritional status on bone metabolism, we examined the relationship between serum levels of nutritional indicators [insulin-like growth factor I (IGF-I), IGF-binding protein-2 (IGFBP-2), and IGFBP-3] and markers for bone metabolism [serum osteocalcin and urinary excretion of C-terminal telopeptide of collagen type I (CrossLaps)] in 45 AN out-patients, including 8 severely malnourished patients who required hospitalization and iv hyperalimentation (IVH). Compared to healthy subjects, serum IGF-I and IGFBP-3 were lower, whereas IGFBP-2 was higher in out-patients who had a body mass index (BMI) less than 16.5 kg/m2. In these patients, urinary excretion of CrossLaps, a marker of bone resorption, was higher, whereas serum osteocalcin, a marker of bone formation, was lower than those in control subjects. All of these parameters were normal in patients whose BMI ranged from 16.5-18.5 kg/m2. Serum levels of osteocalcin correlated positively with BMI (r = 0.512; P<0.0001), IGF-I (r = 0.558; P<0.0001), and IGFBP-3 (r = 0.369; P<0.001) in AN out-patients. In the 8 severely malnourished AN patients, serum levels of IGF-I and osteocalcin significantly increased 3 and 7 days, respectively, after the start of a 5-week IVH therapy regimen and reached normal levels within 5 weeks, accompanied by still elevated urinary excretion of CrossLaps. The present study demonstrates that an improvement in nutritional status in AN patients during IVH therapy rapidly increases the serum IGF-I levels, followed by a progressive increase in osteocalcin, suggesting immediate start of bone formation. However, increased bone resorption appears to continue for at least 5 weeks.