Adrenal venous sampling criteria for chemiluminescent enzyme immunoassay as a preferable alternative to radioimmunoassay in primary aldosteronism.

Plasma aldosterone concentration (PAC) was routinely measured using radioimmunoassay (RIA); however, the RIA kit was discontinued in March 2021 in Japan. This study examined PAC conversion in adrenal venous sampling (AVS) and AVS criteria when measured using chemiluminescent enzyme immunoassay (CLEIA). PAC of 415 adrenal venous blood samples from AVS (including segmental AVS) of 63 patients with primary aldosteronism was measured using RIA (Spac-S aldosterone kit; Fujirebio Inc.) and CLEIA (Lumipulse Presto Aldosterone; Fujirebio Inc.). PAC of 70 AVS samples was also measured using liquid chromatography-mass spectrometry (LC-MS/MS, ASKA Pharma Medical Co., Ltd.). PAC conversion formulas were determined for each AVS sample assay. PAC measured using CLEIA was significantly correlated with that measured using RIA (correlation coefficient = 0.971). The PAC conversion formula was PAC (CLEIA) = PAC (RIA) × 0.772 - 1,199 pg/mL. The PAC of 14,000 pg/mL in RIA was equivalent to 9,613 pg/mL in CLEIA. PAC measured using CLEIA was also correlated with that measured using LC-MS/MS, and the PAC conversion formula was PAC (CLEIA, pg/mL) = 0.97 × PAC (LC-MS/MS, pg/mL) + 211. The inter-assay coefficient of variability (CV) was 1.1-1.3% and intra-assay CV was 1.0-1.7%, measured using CLEIA. The PAC conversion formula for AVS samples was obtained using CLEIA and RIA, and the conversion formula was different from that for peripheral blood. PAC values measured by CLEIA showed preferable accuracy and high concordance with those measured by LC-MS/MS, even in AVS samples. The study outcomes are useful for interpreting AVS results using non-RIA measurement methods.

Osmotic Demyelination Syndrome in a Patient with Diabetic Ketoacidosis Despite No Rapid Sodium Correction.

Osmotic demyelination syndrome (ODS) occurs in patients with diabetes and hyponatremia. We herein report a case of ODS with chorea detected on serial magnetic resonance imaging (MRI), despite no prompt hyponatremia correction. A 74-year-old man with cirrhosis and uncontrolled type 2 diabetes developed an altered mental status and chorea during treatment for diabetic ketoacidosis (DKA). Despite no rapid sodium correction and normal initial brain MRI findings, serial MRI revealed ODS-related abnormalities. Clinicians should consider ODS in patients with DKA and a hyperosmolar hyperglycemic state displaying unconsciousness and neurological manifestations, including chorea, even without substantial changes in serum sodium levels. An MRI re-examination can help capture missing ODS complications.

Differences between left adrenal vein sampling sites revealed with segmental sampling in primary aldosteronism.

OBJECTIVES: To clarify the differences between two left sampling sites (left adrenal central vein [LCV] and common trunk [CMT], conjunction with LCV and inferior phrenic vein) and their impact on adrenal venous sampling (AVS) in primary aldosteronism by analyzing the results of segmental AVS (sAVS). METHODS: We retrospectively analyzed a final cohort of 432 patients who underwent cosyntropin-stimulated sAVS from 2017 to 2020. Hormone levels in the LCV and the CMT were compared. Subtype diagnosis was based on the lateralization index with LCV and CMT sampling (a cutoff value of 4) and sAVS after excluding patients with a selectivity index (SI) <3. RESULTS: Compared with the LCV, CMT sampling showed significantly lower aldosterone and cortisol levels and a higher proportion of cases with an SI of <3 (2.8% vs 0.5%, p = 0.025) and <5 (6.9% vs 0.5%, p < 0.001), while the aldosterone-to-cortisol ratio and the lateralization index were not significantly different. Subtyping of both sites was concordant in 94.7% (393/415) and discordant in the remainder, which included left and right aldosterone-producing adenoma and idiopathic hyperaldosteronism cases referring to the sAVS. There was no significant difference between the concordance rate of the two sampling sites based on the sAVS diagnosis. CONCLUSIONS: LCV sampling meets the SI criteria for successful AVS more frequently compared with CMT sampling, but neither was better than the other in terms of diagnosis under conditions of meeting the criteria. ADVANCES IN KNOWLEDGE: LCV sampling would decrease the number of cases judged as AVS failure.

High aldosterone levels in the renal capsular vein from the left aldosterone-producing adenoma on adrenal venous sampling.

Summary: A 42-year-old female patient was referred to our hospital with hypertension and hypokalemia and was diagnosed with primary aldosteronism. Dynamic contrast-enhanced computed tomography images revealed a 13-mm nodule on the lateral segment of the left adrenal gland and a fine venous connection between the nodule and the prominent renal capsular vein running nearby. The venograms in the left lateral tributary with a microcatheter confirmed alternative drainage to the left renal capsular vein during adrenal venous sampling, and the left renal capsular vein sampling was added. The patient was diagnosed with a left aldosterone-producing adenoma (APA) using the lateralization index (48.3) and a higher plasma aldosterone concentration (PAC) of the left lateral tributary (66 700 pg/mL) than other tributary samples after adrenocorticotropic hormone stimulation. Furthermore, markedly higher PAC (224 000 pg/mL) was observed in the left renal capsular vein blood than in the left adrenal central vein (45 000 pg/mL) and tributaries, confirming the diagnosis. Laparoscopic left partial adrenalectomy and following histopathological analysis revealed a CYP11B2-positive adrenocortical adenoma. Complete clinical and biochemical success for primary aldosteronism was achieved after 6 months. Direct evidence of APA blood venous drainage into the renal capsular vein has been demonstrated. Sampling from an alternative drainage pathway could be beneficial for APA diagnosis if such APA blood drainage is assumed. Learning points: Aldosterone-producing adenomas may drain blood into an alternative pathway but for the adrenal vein. The presence of alternative venous drainage could be assumed by contrast-enhanced computed tomography or venogram during adrenal venous sampling. Sampling in the alternative drainage veins and demonstrating elevated aldosterone levels could help in diagnosing aldosterone-producing adenoma.

Node-by-node diagnosis for multiple ipsilateral nodules by segmental adrenal venous sampling in primary aldosteronism.

OBJECTIVES: In patients with primary aldosteronism (PA), multiple adrenocortical nodules may be present on the surgical side. The aim of this study was to clarify the pathological diagnosis and the node-by-node diagnostic capability of segmental adrenal venous sampling (sAVS). DESIGN: Retrospective study. PATIENTS: A total of 162 patients who underwent adrenalectomy following sAVS were studied. MEASUREMENTS: Multiple nodules on the surgical side were extracted while referring to contrast-enhanced computed tomography images. We also performed a detailed histopathological analysis of the resected specimens from patients undergoing sAVS, which included immunohistochemistry for CYP11B2. RESULTS: In 11 (6.8%) patients, two to three nodules were detected on the surgical side. All patients were diagnosed by sAVS with at least one aldosterone-producing adenoma (APA) for localized aldosterone elevation in tributaries. Seven patients showed a lateralization index value of ≥4 after ACTH stimulation. Histopathologically and clinically, two patients had two or three CYP11B2-positive APAs, and the other nine patients both APAs and non-APAs. The positive predictive value of the most suspected APA, that is, the drainer that showed the highest aldosterone level by sAVS, was 11/11 (100%, 95% confidence interval [CI]: 71.5%-100%), while that for the second and third suspected APA was 3/7 (42.9%, 95% CI: 9.9%-81.6%), and they were significantly different (p = .01). Further, the positive predictive value of non-APA was 4/4 (100%, 95% CI: 39.8%-100%). CONCLUSIONS: The sAVS could correctly diagnose the aldosterone production in multiple ipsilateral adrenal nodules.

Infective Arteritis Manifesting as an Acute Subdural Hematoma.

We encountered a 60-year-old man who had been diagnosed with heart failure of valvular disease and infective endocarditis; he was being treated with intravenous antibiotics and diuretics. On the 12th hospital day, he suddenly lost consciousness; computed tomography showed a right-sided huge subdural hematoma (SDH) with brain herniation. He died after invasive care was discontinued. A massive SDH was noted at autopsy, and microimaging demonstrated ruptured infective arteritis, without aneurysm, on the surface of the culprit lobe. Acute SDH usually occurs after head trauma, but an area of nonsaccular aneurysmal arteritis can also result in acute SDH.

Anatomical Variations of the Left Adrenal Vein Encountered During Venous Sampling.

PURPOSE: To identify anatomical variations in the left adrenal vein (LAV) and to evaluate the role of preprocedural contrast-enhanced computed tomography (CT) planning. METHODS: The length of the left adrenal central vein (LACV), the vessel that receives blood from all tributaries of the left adrenal gland, was measured using venograms of patients who had undergone adrenal venous sampling (AVS) for the diagnosis of primary aldosteronism between October 2017 and December 2019. The anatomical variants of the LAV were described and classified. Contrast-enhanced CT was used to evaluate the detection rate of the following: (a) confluence of the left inferior phrenic vein and the LAV and (b) the last tributary flowing into the LAV. RESULTS: In total, 311 patients (143 men, 168 women; mean age: 49.3 years ± 11.0) were enrolled. Of them, 9 (2.9%) patients had anatomical variants lacking a LACV. In patients with a LACV (n = 302), the venographic LACV length was 9.0 mm ± 3.9 (<1 mm in 9 patients). The detection rate of the confluence of the left inferior phrenic vein and LAV, as determined using contrast-enhanced CT, was high (96.2%), whereas that of the last tributary flowing into the LAV was low (0.8%). In 4 of 18 patients with short or absent LACV, the variant was visualized using contrast-enhanced CT. CONCLUSIONS: In some patients, the LACV is absent or short, which is an anatomical variation. Understanding venographic anatomical variations can help avoid misleading results resulting from a suboptimal sampling site in AVS. For some subtypes, contrast-enhanced CT may also help in planning the AVS procedure.

Radiological characteristics and diagnostic impact of duplicated right adrenal veins on adrenal venous sampling in primary aldosteronism.

PURPOSE: We aimed to analyze the prevalence and radiological characteristics of duplicated right adrenal veins (DRAVs) and evaluate the diagnostic impact of adrenal venous sampling (AVS) in primary aldosteronism. METHODS: DRAVs were retrospectively identified among patients who underwent segmental AVS between April 2017 and March 2020. DRAVs were defined as main or accessory according to the drainage area. The diameter, position, hormone levels, and treatment plan based on AVS were compared between main and accessory RAVs, using the Wilcoxon rank-sum test. RESULTS: Fourteen of 432 patients (3.2%) were diagnosed with DRAVs. On venography, the mean diameters of the main and accessory side were 3±0.63 mm and 2.1±0.41 mm, respectively, and were significantly different (p < 0.001). The mean relative position in craniocaudal direction of main and accessory veins from the adrenal caudal edge on computed tomography was 65.5%±16.0%, and 48.1%±16.8%, respectively, which was significantly different (p = 0.007). The left-right positions and hormone levels were not significantly different. Based on conventional AVS, the treatment plan between DRAVs was not changed in six of eight patients, but changed from surgery to medication in two patients with right aldosterone-producing adenoma (APA)/microadenoma based on segmental AVS findings. CONCLUSION: DRAVs, in which the main RAV was thicker and more cranially located than the accessory RAV were rare. Depending on blood sampled from either of DRAVs, the diagnosis made through conventional AVS might change treatment approach from surgery to medication, especially with right APA. Hence, their identification is important to make an accurate subtyping by AVS.

Pitfalls in chest CT findings of COVID-19 patients infected during hospitalisation.

OBJECTIVE: This study evaluated the differences in the appearance of COVID-19 pneumonia on chest computed tomography (CT) images of outpatient and cases that developed during hospitalisation. METHOD: Chest CT images of 66 patients (median age, 76 years; range, 29-94 years) who underwent the severe acute respiratory syndrome coronavirus-2 reverse-transcription polymerase chain reaction (RT-PCR) test were included in this retrospective study. The chest CT appearance was categorised as "typical," "indeterminate," "atypical," or "negative" in accordance with the recommendations of the Radiological Society of North America for COVID-19 pneumonia and compared among the following four subgroups: PCR-positive outpatient (n = 14); PCR-positive hospitalised (n = 7); PCR-negative outpatient (n = 9); and PCR-negative hospitalised (n = 36). FINDINGS: The frequency of "typical" findings in the PCR-positive outpatient cases (13/14, 92.9%) was significantly higher than that of those in the PCR-positive hospitalised cases (2/7, 28.6%, P = 0.022). There was no significant difference between the frequency of the "typical" appearance in PCR-positive hospitalised cases and that of those in the PCR-negative hospitalised cases (1/36, 2.8%, P = 0.192). CONCLUSIONS: When COVID-19 patients acquire infections while hospitalised, their chest CT images are less likely to show typical findings than those of outpatient cases. Comprehensive and careful assessments of CT findings and consideration of the possibility of concomitant infections with other pathogens and clinical information, such as underlying diseases, background lung structure, and time course of the infection, are required for the management of such cases.

A Rare Independent Left Inferior Phrenic Vein Sampling in a Left Adrenal Aldosterone-Producing Adenoma.

This report presents a case of left adrenal aldosterone-producing adenoma (APA) diagnosed by segmental adrenal venous sampling in a patient with primary aldosteronism and a rare venous anomaly in which the left inferior phrenic vein (LIPV) and adrenal central vein entered the left renal vein separately. The outflow of tumor blood into the LIPV and the specimen from the LIPV that showed much higher aldosterone level than that from the adrenal central vein and tributaries were useful for proving the aldosterone hypersecretion from the APA. Sampling from the LIPV could be of diagnostic value for left APA.

Steroidogenic Activity in Unresected Adrenals Associated With Surgical Outcomes in Primary Aldosteronism.

[Figure: see text].

A Novel Method: Super-selective Adrenal Venous Sampling.

Primary aldosteronism (PA) and subclinical Cushing's syndrome (SCS) are conditions in which the adrenal glands autonomously produce excessive amounts of aldosterone and cortisol, respectively. The conventional adrenal venous sampling (cAVS) method collects blood samples from both adrenal central veins and is useful for identifying the laterality of excess hormone production in a unilateral lesion(s), as documented in PA cases. In cAVS, plasma cortisol concentrations (PCCs) are used to normalize plasma aldosterone concentrations (PACs). A novel "super-selective" adrenal venous sampling (ssAVS) method was developed using a micro-catheter, which collects blood samples from adrenal tributary veins (TVs). PACs in ssAVS samples do not require PCC normalization because samples contain a limited amount of systemic venous blood, if any. The ssAVS method enabled segmental lesion(s) to be detected in both adrenal glands, which may be treated by bilateral adrenalectomy, thereby sparing lesion-free segment(s). Right and left adrenals typically have three TVs each, i.e., the superior, lateral, and inferior TVs in the right adrenal as well as the superior-median, superior-lateral, and lateral TVs in the left adrenal. In the ssAVS method, specific parent catheters and a technique to handle them are required, and have been described herein. Furthermore, ssAVS results from three cases of PA are presented: bilateral aldosterone-producing adenoma (APA) (Case #1), left APA and right possible cortisol-producing adenoma causing SCS (Case #2), and idiopathic hyperaldosteronism in which bilateral adrenal segments produced excessive amounts of aldosterone (Case #3). The ssAVS method is not difficult for expert angiographers, and, thus, is recommended worldwide to treat PA cases for which cAVS does not represent a viable surgical treatment option.

Adrenal hormones before and after venography during adrenal venous sampling: a self-controlled study.

OBJECTIVE: A stress reaction involving increased adrenal hormone release occurs when starting adrenal venous sampling (AVS). The purpose of the present study was to investigate the effect of single shot venography on adrenal hormone production during AVS. SUBJECTS AND METHODS: This was a prospective self-controlled study. We enrolled 54 consecutive patients (21 men, 33 women; mean age 52 ± 11 years) with primary aldosteronism who underwent AVS from May 2014 to February 2015. Under non-stimulated conditions, blood samples were obtained from a common trunk of the left adrenal vein before and after single shot venography. The initial plasma aldosterone and cortisol concentration (PAC and PCC) were compared with those measured after venography for each patient. RESULTS: PAC and PCC were slightly but significantly decreased between before and after venography (after log transformation 2.12 ± 0.73 vs 2.07 ± 0.72, P = 0.00066, 1.89 ± 0.52 vs 1.83 ± 0.53, P = 0.00031, respectively). CONCLUSIONS: During non-stimulated left AVS, adrenal hormone secretion was slightly but significantly decreased after venography, similar to the normal time-related stress reaction. Venography did not increase the adrenal hormone secretion.

Somatic KCNJ5 mutation occurring early in adrenal development may cause a novel form of juvenile primary aldosteronism.

We report a case of non-familial juvenile primary aldosteronism (PA). Super-selective adrenal venous sampling identified less aldosterone production in the right inferior adrenal segment than others. Bilateral adrenalectomy sparing the segment normalized blood pressure and improved PA. Both adrenals had similar histologies, consisting of a normal adrenal cortex and aldosterone synthase-positive hyperplasia/adenoma. An aldosterone-driving KCNJ5 mutation was detected in the lesions, but not in the histologically normal cortex. After taking into account that the two adrenal glands displayed a similar histological profile, as well as the fact that hyperplastic lesions in both glands exhibited a common KCNJ5 mutation, we conclude that the specific mutation may have occurred at an adrenal precursor mesodermal cell, at an early stage of development; its daughter cells were mixed with non-mutant cells and dispersed into both adrenal glands, resulting into a form of the condition known as genetic mosaicism.

Rectal arterio-venous malformation (AVM) with bleeding of an internal hemorrhoid.

A 38-year-old male with no past history of illnesses visited the out-patient clinic of Nerima Hikarigaoka Hospital complaining of dizziness and persistent anal bleeding. There was a significant anemia on a blood test and colonoscopy showed a thrombus in a markedly swollen internal hemorrhoid. Contrast-enhanced computed tomography (CT) showed a poorly demarcated area with early face enhancement on the right side of the rectum and anal canal. Based on these findings, an arterio-venous malformation (AVM) of the rectum was suspected. Abdominal angiography showed abnormal vessels receiving a blood supply from the bilateral superior rectal arteries. We suspected that the AVM in the rectum was the cause of the hemorrhage from the internal hemorrhoid, and therefore performed embolization of the AVM. Thereafter, the hemorrhage from the internal hemorrhoid stopped completely and the anemia improved to the normal level, without the need for treatment for the internal hemorrhoid. Colonoscopy performed 6 months after embolization showed shrinkage of the internal hemorrhoid. To the best of our knowledge, there are no reports stating a relationship between rectal AVM and internal hemorrhoids. However, we consider that contrast-enhanced CT can be used to detect vessel abnormalities related to severe bleeding of the internal hermorrhoids in patients with internal hemorrhoids and severe anemia.

Hounsfield unit values of retropharyngeal abscess-like lesions seen in Kawasaki disease.

CONCLUSIONS: Retropharyngeal abscess-like lesions are occasionally seen in computed tomography (CT) imaging of patients with Kawasaki disease (KD) and these patients often undergo unnecessary surgery. We could distinguish the lesions from true abscesses by measuring their Hounsfield unit values (HUs). OBJECTIVE: To distinguish the retropharyngeal abscess-like lesions from true abscesses without any surgical procedure. METHODS: We investigated six cases of KD showing such lesions on CTs, both with and without contrast enhancement (CE). We measured the HUs of those lesions and compared them with those of 10 true abscesses as controls. RESULTS: Abscess-like lesions of KD were well enhanced by CE, whereas abscesses showed virtually no enhancement. The mean HU in the six KD cases was 20.0 ± 4.65 (mean ± SD) on plain CTs and 35.6 ± 4.49 on contrast CTs. In abscesses, it was 30.3 ± 4.42 on plain CTs and 30.3 ± 3.57 on contrast CTs. The difference in HU values [(HU on contrast CT) - (HU on plain CT)] was defined as ΔHU. The mean ΔHU was 15.6 ± 5.36 in the six KD lesions and 0.0 ± 2.93 in abscesses, with statistical significance of p < 0.0001 by Student's t test. Thus, ΔHU value may potentially be a useful parameter for their distinction.

Mycotic pulmonary artery aneurysm diagnosed 18 years after bacterial endocarditis.

A 21-year-old woman with a history of bacterial endocarditis and ventricular septum defect repair was referred for evaluation of a mass in the right mid-lung field. A contrast-enhanced computed tomography scan of the chest showed an aneurysm of the right lateral pulmonary artery. The aneurysm was considered to be mycotic in origin. The aneurysm was treated by coil embolization using 19 detachable coils measuring 425 cm in overall length.

Mucinous adenocarcinoma arising from fistula in ano: MRI findings.

OBJECTIVE: The purpose of this study was to describe MRI findings in 11 patients with mucinous adenocarcinomas arising from fistula in ano. CONCLUSION: More than half of patients had a mass filled with markedly hyperintense content on T2-weighted fast spin-echo images, enhancing solid components within the mass, mesh-like internal enhancement, fluid collections without a thick fibrous capsule, contrast enhancement of peritumoral areas, and a fistula between the mass and the anus. Regional areas of lymph node enlargement were considered indirect findings of locally advanced mucinous adenocarcinoma. These MRI findings may help diagnose mucinous adenocarcinomas arising from fistula in ano.