Adult umbilical hernia with vertical dislocation

We present a case of adult umbilical hernia with vertical dislocation along the abdominal wall. The hernial sac arose from the internal ring and connected to the umbilicus 20?mm below the internal ring. The postoperative course was uneventful. Two years and four months after the operation there was no evidence of recurrent hernia even when abdominal pressure was increased, and the umbilicus looked acceptable. An umbilical hernia is usually within the umbilicus. The hernial sac arose from the internal ring so should be called an umbilical hernia not an epigastric hernia. It is unusual that the umbilical hernia dislocates vertically along the abdominal wall, while the umbilicus stays depressed. This atypical form of umbilical hernia has not been described previously as far as we know.     

Diagnostic and management difficulties in a case of multiple intracranial juvenile xanthogranuloma

Introduction

Juvenile xanthogranuloma (JXG) preferentially occurs in childhood, and its characteristics have been thought to be benign and with slow growth. JXG is classified as an inflammatory disease, which forms multiple lesions in the patients’ head, neck, and other organs and typically skin. JXG is rare, and few case reports have been published in the past, particularly with multiple intracranial lesions, which as in our case, is an extremely rare finding.

Case

Patient is a 4-year-old boy who presented with polydypsia and polyuria for 1 year. He had been followed up only under l-deamino-8-d-arginine vasopressin until he started vomiting and his level of consciousness deteriorated. Then, magnetic resonance imaging (MRI) revealed multiple intracranial lesions. He underwent biopsy via small craniotomy, and pathology was confirmed as juvenile xanthogranuloma. The patient received chemotherapy, in complete compliance with JLSG-02 protocol.

Findings

JXG is characteristic with homogenous enhancement with contrast matter and, most importantly, high intensity on diffusion-weighted image (DWI). The DWI was high when the degree of diffusion of water is restricted, as seen in inflammation and, additionally, the change of intensity after administration of steroid would reflect its anti-inflammatory nature. However, though the steroid therapy made high-intensity lesions decrease signal intensity, the size and the number of lesions did not change at all. As we expected, after we stopped steroid administration to the patient, his consciousness deteriorated and we found again the very-high-intensity lesions. We consider the intensity on DWI to reflect progression of the lesions.     

ITIH4 and Gpx3 are potential biomarkers for amyotrophic lateral sclerosis

The diagnosis of amyotrophic lateral sclerosis (ALS) is difficult due to lack of definitive biomarkers. Our aim was to identify characteristic serum protein patterns that could provide candidate biomarkers for ALS. We divided mutant superoxide dismutase-1 (SOD1)^H46R rats into three groups based on disease progression: pre-symptom (90 days), onset, and end-stage. After separation of serum proteins using two-dimensional electrophoresis, we selected clear protein spots and identified two candidate proteins—inter-alpha-trypsin inhibitor heavy chain H4 (ITIH4) and glutathione peroxidase 3 (Gpx3). The 120 kDa ITIH4 increased at the onset of the disease and the 85 kDa ITIH4, a cleaved form, at the end-stage in the sera of the SOD1^H46R rats. Expression of the 85 kDa ITIH4 was substantial in ALS compared with controls or patients with muscular dystrophy, Alzheimer diseases, or Parkinson diseases. The Gpx3 protein levels in the sera of SOD1^H46R rats were upregulated pre-symptom and gradually decreased as the disease progressed. The Gpx3 protein levels were lower in the sera of the patients with ALS than in other diseases. These results indicate that ITIH4 and Gpx3 are potential biomarkers for ALS.     

Reference values of focused assessment with sonography for obstetrics (FASO) in low-risk population

Objective: Hemorrhagic shock is a relatively common occurrence in the postpartum period. In our hospital, we performed abdominal ultrasonography using the focused assessment with sonography for obstetrics (FASO) technique (a modified version of FAST). The aim of the present study was to determine the reference values for the ultrasonographic findings to establish the criteria for the diagnosis of a postpartum hemorrhage and severe shock using the FASO.

Methods: The present prospective cohort study included all postpartum women who vaginally delivered singleton infants. Abdominal ultrasonography was performed after delivery. The observation points of ultrasonography were as follows: (1) the diameter of the intrauterine cavity, (2) the pouch of Douglas, (3) Morison’s pouch, (4) between the spleen and kidney, and (5) the diameter of the inferior vena cava.

Results: One hundred and eighty-two postpartum women were included in this study. The mean uterine cavity was 9.8?±?7.3?mm. An echo-free space in the pouch of Douglas was observed in three cases, in one case in Morison’s pouch, and not observed between the spleen and kidney. A negative correlation was found between the volume of bleeding and IVCi (p?=?0.0008, r²=??0.061) and IVCe (p?<?0.0001, r²=??0.106).

Conclusions: The present study establishes criteria that can be used to diagnose a postpartum hemorrhage or severe shock using the FASO.     

Macroreentrant atrial tachycardia with an isolated pathway mimicking focal activation on three-dimensional electroanatomical mapping

A 76-year-old man with two different sustained atrial arrhythmias that occurred after coronary artery bypass grafting underwent electrophysiological studies. Macroreentrant atrial tachycardias were detected with an isolated slow pathway mimicking focal activation on three-dimensional electroanatomical mapping. The slow conduction pathway in the right atrial free wall was assumed to represent tissue damaged by right atrial cannulation during previous coronary artery bypass grafting.     

Predictive model for recurrence of renal cell carcinoma by comparing pre‐ and postoperative urinary metabolite concentrations

To improve treatment outcomes in real practice, useful biomarkers are desired when predicting postoperative recurrence for renal cell carcinoma (RCC). We collected data from patients who underwent definitive surgery for RCC and for benign urological tumor at our department between November 2016 and December 2019. We evaluated the differences in pre‐ and postoperative urinary metabolites with our precise quantitative method and identified predictive factors for RCC recurrence. Additionally, to clarify the significance of metabolites, we measured the intracellular metabolite concentration of three RCC cell lines. Among the 56 patients with RCC, nine had a recurrence (16.0%). When comparing 27 patients with T1a RCC and 10 with benign tumor, a significant difference was observed between pre‐ and postoperative concentrations among 10 urinary metabolites. In these 10 metabolites, multiple logistic regression analysis identified five metabolites (lactic acid, glycine, 2‐hydroxyglutarate, succinic acid, and kynurenic acid) as factors to build our recurrence prediction model. The values of area under the receiver operating characteristic curve, sensitivity, and specificity in this predictive model were 0.894%, 88.9%, and 88.0%, respectively. When stratified into low and high risk groups of recurrence based on this model, we found a significant drop of recurrence‐free survival rates among the high risk group. In in vitro studies, intracellular metabolite concentrations of metastatic tumor cell lines were much higher than those of primary tumor cell lines. By using our quantitative evaluation of urinary metabolites, we could predict postoperative recurrence with high sensitivity and specificity. Urinary metabolites could be noninvasive biomarkers to improve patient outcome.     

Diagnostic value of abdominal fat tissue aspirate in familial amyloid polyneuropathy

To confirm amyloid deposition we performed aspiration biopsy of abdominal fat tissue in 14 patients with type I familial amyloid polyneuropathy (FAP). All patients, about half of whom were at an early stage of disease and lacking severe neurological disabilities, showed positive amyloid deposition in preparations stained with Congo red. On light and electron microscopic examinations deposits of amyloid were observed around fat cells and small vascular walls, and among collagen bundles. All patients in the study were demonstrated to have a variant transthyretin in their sera by radioimmunoassay. Abdominal fat tissue aspiration can be easily performed with an ordinary syringe and is very sensitive for detecting amyloid deposition. The procedure is valuable in the diagnosis of type I FAP patients with actual deposition of amyloid substance, even at an early stage.