Saccular abdominal aortic aneurysm in adolescence with tuberous sclerosis.

Key clinical message: Abdominal aortic aneurysm complicated by tuberous sclerosis is rare, particularly in patients over the age of 10. It is important to screen for abdominal aortic aneurysm in adolescents diagnosed with tuberous sclerosis regularly. Abstract: A 15-year-old girl who was diagnosed with tuberculous sclerosis complicated with a saccular aortic abdominal aneurysm (AAA), measuring 19 × 18 mm in diameter. The patient underwent open repair of AAA using a 11 mm straight prosthetic graft. It is important to screen for AAA in adolescents diagnosed with tuberous sclerosis regularly.

Tolvaptan Reduces Extracellular Fluid per Amount of Body Fluid Reduction Less Markedly than Conventional Diuretics.

Objective Tolvaptan, a vasopressin V2 receptor antagonist, is a water diuretic, removing electrolyte-free water from the kidneys and affecting the water balance between the intracellular and extracellular fluid. We previously reported that tolvaptan efficiently reduced the intracellular fluid volume, suggesting its utility for treating cellular edema. Furthermore, tolvaptan is known for its low incidence of worsening the renal function, with conventional diuretics use associated with worsening of the renal function Methods In this retrospective observational study, five chronic kidney disease (CKD) patients with fluid retention were assessed by the bioelectrical impedance (BIA) method twice (before and after tolvaptan therapy). Tolvaptan was used with conventional diuretics. The post/pre ratio of extracellular water (ECW)/total body water (TBW) in the tolvaptan group was compared with that in 18 CKD patients undergoing body fluid reduction with conventional diuretics alone (conventional diuretics groups), taking the reduced amount of body fluid into consideration. Results Removing body fluid, either by tolvaptan or by conventional diuretics alone, decreased the ECW/TBW ratio. Of note, the reduction in extracellular fluid was milder in the tolvaptan group than in the conventional diuretics group. Conclusion Tolvaptan reduces the extracellular fluid per amount of body fluid reduction less markedly than conventional diuretics.

[EFFICACY AND SAFETY OF 25 AND 50 µg DESMOPRESSIN ORALLY DISINTEGRATING TABLETS IN NOCTURIA DUE TO NOCTURNAL POLYURIA IN JAPANESE MALE PATIENTS].

(Purpose) To conduct a prospective study on the efficacy and safety of desmopressin for nocturnal polyuria. (Materials and methods) We selected 51 Japanese men, aged ≥50 years, with complaints of nocturia and a nocturnal polyuria index of ≥0.33. We administered 25 or 50 µg desmopressin (Minirinmelt Orally Disintegrating Tablet®), once daily at bedtime. We evaluated the nighttime urinary frequency and urine volume, nocturnal polyuria index, time to the first urination after falling asleep, and International Prostate Symptom Score (IPSS) at baseline and at 4, 8, and 12 weeks after administration. In addition, they underwent clinical examinations and blood tests at 1, 4, and 12 weeks to evaluate the safety of the drug. (Results) We observed a decrease in the nighttime urinary frequency and urine volume, and nocturnal polyuria index, increased prolonged time to the first urination after falling asleep, and improved IPSS at and after 4 weeks, compared to baseline data. Furthermore, the drug remained effective even at 12 weeks for all parameters. We observed adverse events in 31.3% of the patients. The incidence of hyponatraemia was particularly high in 15.7% of the patients. Those with a lower serum sodium level and lesser body weight at baseline were more likely to develop hyponatraemia. (Conclusion) Desmopressin was identified as a potential drug for the treatment of nocturnal polyuria. However, hyponatraemia, an important adverse event, resulted in treatment discontinuation in several patients. A sodium level lower than the normal level and low body weight at baseline were the risk factors for hyponatraemia.

[INITIAL TREATMENT OF NOCTURIA CAUSED BY NOCTURNAL POLYURIA WITH LOW-DOSE DESMOPRESSIN].

(Objective) Nocturia, an important male lower urinary tract symptom (LUTS), is often difficult to treat. Herein, we report our experience of the initial treatment of nocturia with the novel drug desmopressin. (Subjects and methods) Subjects included 25 patients with LUTS treated with desmopressin who had the chief complaint of nocturia. Before treatment, the frequency of nocturnal urination (≥2) and nocturnal polyuria index (≥0.33) were confirmed based on the urination diary for ≥ 72 h. Before sleep, 25 or 50 mg desmopressin (Minirin® Melt OD tablets) was administered once daily. The frequency of nocturnal urination, volume of nocturnal urine, time from falling asleep to first urination, first urinary volume after falling asleep, nocturnal polyuria index, International Prostate Symptom Score (IPSS), quality of life index, Overactive Bladder Symptom Score, and residual urine volume were comparatively evaluated before and 4 weeks after treatment. Treatment effect was self-evaluated by patients 4 weeks after the treatment. Safety was evaluated by interview and blood testing 1 and 4 weeks after the treatment. (Results) Decrease in the frequency of nocturnal urination and improvement in IPSS were observed. According to self-evaluation of the treatment, 72.6% of the patients considered the treatment efficacious. Regarding safety, adverse events were observed in 28% of the patients, particularly hyponatremia (12% of the patients). (Conclusion) Desmopressin is a potential key drug for the treatment of nocturia caused by nocturnal polyuria.

Lactate predicts the 28-day survival rate in patients with septic shock treated with the combination of PMX-DHP and rTM.

We have previously reported that combination therapy with polymyxin-B direct hemoperfusion (PMX-DHP) and recombinant thrombomodulin (rTM) is effective in patients with septic shock accompanied by disseminated intravascular coagulation (DIC). Two previous studies reporting the favorable effect of early initiation of PMX-DHP for septic shock did not focus on the combination therapy of PMX-DHP and rTM. This retrospective study included 47 consecutive patients who underwent the combination therapy of PMX-DHP and rTM for septic shock with DIC from August 2011 to August 2016. Main exposure was early or late initiation of PMX-DHP. PMX-DHP initiated within 12 hours after catecholamine administration was designated as early group (N = 25) and later than 12 hours as late group (N = 22). Main outcome was 28-day survival rate. The patient characteristics were age median 73 (IQR 68-78) years, 26 men (55%), APACHE II score 32.7 ± 7.7 and lactate 26.0 (18.0-41.0) mg/dL. The 28-day survival rate after PMX-DHP initiation was 76.6% and was not significantly different in the two groups. In the early group, APACHE II score was lower (P = .02), and lactate was higher (P = .005) than in the late group. Lactate was the only predictor of 28-day mortality [odds ratio (95%CI) per 1 mg/dL, 1.08 (1.03-1.19); P = .037] in multivariate logistic regression analysis adjusted with age, sex, APACHE II score, lactate and timing of PMX-DHP initiation. Late PMX-DHP initiation did not lead to statistically worse 28-day survival rate in this combination therapy. The combination therapy of PMX-DHP and rTM may improve the therapeutic effect of PMX-DHP and modify the effect of early PMX-DHP on the prognosis. Lactate may be an appropriate indicator rather than time after catecholamine administration if we discuss when to start PMX-DHP in this combination therapy.

A Case of Thoracic Endovascular Aortic Repair Using Carotid Access with Axillary-Carotid Bypass for Descending Aortic Aneurysm in a Patient with Aortoiliac Occlusive Disease.

The authors report a 71-year-old male with descending thoracic aortic aneurysm and multiple risk factors (aortoiliac occlusive disease, obesity, ascending aorta dilatation, and history of left ventriculoperitoneal shunt for hydrocephalus) who was treated with thoracic endovascular aortic repair (TEVAR) via left common carotid artery (LCCA) access and left axillary-carotid artery (Ax-CA) bypass; this approach shortened the LCCA clamp time during the procedure. The patient was discharged without any complications. TEVAR via LCCA access with left Ax-CA bypass is a useful and safe procedure for patients in whom conventional femoral artery access is not feasible.

[A CASE OF RENAL CELL CARCINOMA WITH INFERIOR VENA CAVAL TUMOR THROMBUS WHICH THE REDUCTION OF TUMOR IN SPITE OF DRUG DISCONTINUANCE AFTER THE FULMINANT HEPATITIS ONSET WITH THE SUNITNIB].

A 70-year-old man presented with right renal cell carcinoma with inferior vena caval tumor thrombus into the right atrium. CT Scan presented local invasion and lymph node metastasis. We estimated inoperative case, so he was started sunitinib. After 5 month he had general fatigue and admitted to our hospital. He diagnosed serious adverse events of fulminant hepatitis and left ventricular systolic dysfunction and discontinued sunitnib. After drug discontinuance reduction of tumor and tumor thrombus were detected. 7-months later, we showed the increase of tumor and the improvement of the left ventricular systolic dysfunction. We performed right renal nephrectomy and it passes now in 14 months after surgery, but doses not show a recurrence, metastasis.

Esophageal necrosis after endovascular repair for ruptured aortic dissection.

A 78-year-old man presented with back pain and shock and was transferred to our hospital. Computed tomography showed a ruptured aortic dissection in which the false lumen was thrombosed with an ulcer-like projection, and the mid-esophagus was shifted to the right due to a mediastinal hematoma. He underwent emergency thoracic endovascular aortic repair of the descending thoracic aorta. One week later, esophageal necrosis occurred, and he died of mediastinitis and sepsis on postoperative day 16. Although esophageal necrosis is a rare and fatal complication after thoracic endovascular aortic repair, a management strategy has not yet been established.