Pycnodysostosis is an autosomal recessive disorder characterized by osteosclerosis, small stature, acro-osteolysis of the distal phalanges, loss of the mandibular angle, separated cranial sutures with open fontanels, and frequent fractures. One identified cause of the disease is reduced activity of the cysteine protease cathepsin K. A 48-year-old woman with a history of frequent fractures presented with a severe gait disturbance. Radiography, computed tomography, magnetic resonance imaging, and gene analysis were performed. Physical examination revealed open fontanels, and radiographs showed increased bone density. DNA sequence analysis revealed a deletion mutation of the cathepsin K gene. We diagnosed pycnodysostosis based on these findings. The magnetic resonance and computed tomography images demonstrated multilevel spinal canal stenosis due to ossification of the yellow ligament. We performed a laminectomy, and the patient's neurological signs and symptoms improved. To our knowledge, this is the first case of pycnodysostosis with ossification of the yellow ligament.
Acro-Osteolysis ; Bone Density ; Cathepsin K ; Constriction, Pathologic* ; Cranial Sutures ; Cysteine Proteases ; Female ; Gait ; Humans ; Laminectomy ; Ligaments* ; Magnetic Resonance Imaging ; Middle Aged ; Osteosclerosis ; Physical Examination ; Pycnodysostosis* ; Radiography ; Sequence Analysis, DNA ; Sequence Deletion ; Spinal Canal*

An investigation was performed about clinical histories before hospitalization in 1142 cases of gastric cancer during 16 years from 1969 to 1984.
The average term from onset of the disease to hospitalization was 4.53 months which tends to decrease becoming 3.49 months in the latest 5 years. The patients had visited 0.72 other doctor in average before coming to our hospital, 0.35 in early cancer cases and 0.83 in advanced cases. The sources of patients of our surgery were as follows ; 60.5% were introduced from medical department of our hospital, 20.2% were introduced from other clinics or hospitals, 10.6% visited our surgical department directly, and 8.7% came to us after visiting one or some other doctors. The rate of early cancer cases were high and unresectable cases were low relatively in cases from our medical department and direct visitors to our surgical department.
The causative factors of delay of hospitalization more than one month were considered from both sides of patient and doctor. The results were ; no delay 55.3%, delay due to patient's fault 28.2%, delay due to doctor's fault 19.9%. The delay of hospitalization due to either side's fault was one factor of decreasing early cancers and increasing advanced cases. Among those with no delay, however, 22.6% were unresectable cases. Gastric cancers are too malignant to be cured by visiting hospitals with complaints. Gastric mass survey among symptomeless people is the only reasonable way to come out of this difficult situation.

Medical therapies, adapted to local requirements, are necessary in the field of community medicine. Generalized medical treatment is often all there is in Japan's Konsen Area, due to the large number of elderly people and a paucity of medical institutions. Kampo medical treatment is useful for elderly patients with plural diseases, and the demand for such treatment has been increasing. Therefore, we collected data on Kampo prescriptions prescribed by the doctors at the Nakashibetsu Municipal Hospital, which is the main local hospital in the Konsen Area, from 2010 to 2013. We conducted similar investigations at the Kushiro Red Cross Hospital in Kushiro city, which is centrally located in the Konsen Area, and at Asahikawa Medical University Hospital in Asahikawa, central Hokkaido. The aim of these investigations was to compare Kampo medicine use based on hospital location and size. The results can be used in community medicine to enhance the use of Kampo medicine with increase for its demand. Medical students and doctors should be educated in Kampo medicines, since their prescriptions have a significant impact on community health.

The diagnosis of pituitary carcinoma cannot be made easily histologically, and most cases of pituitary carcinoma are diagnosed only after the clinical detection of metastasis. Distant metastasis of pituitary tumor occurs in 0.1% to 0.2% of cases and has been reported in the liver, bone and central nervous system, with only one case of metastasis to the cauda equine reported. This study describes a rare case of the drop metastasis of adrenocorticotropic hormone-producing pituitary adenocarcinoma to the cauda equina, causing cauda equina syndrome.
Adenocarcinoma ; Adrenocorticotropic Hormone ; Cauda Equina* ; Central Nervous System ; Diagnosis ; Liver ; Neoplasm Metastasis* ; Pituitary Neoplasms* ; Polyradiculopathy

Purpose: Despite widespread adoption of open abdominal management (OAM), there is currently no threshold criterion for OAM duration for non-trauma patients. Moreover, there is a positive relationship between morbidity and the duration of OAM, but an uncertain relationship with patients’ age. Therefore, a novel clinical index for the duration of open abdominal management (IDOM) was developed based on the patient’s age and risk of severe complications following OAM to indicate the maximum tolerable number of days of OAM based on the individual’s age. The utility of this new index was evaluated. Methods: This retrospective study included 65 non-trauma patients managed with an open abdomen (OA) from August 2015 to August 2018. The IDOM was developed based on the patient’s age. The result indicated the maximum number of OA days. Patients’ demographic and operative variables were examined and patient data was assigned to one of two groups according to whether the actual number of OA days was above or below the calculated IDOM. Prevalence of complications between these groups was compared. Measures of validity were employed to assess the utility of the IDOM for patient complications. Results: Sixty-five patients were included. The above-the calculated IDOM group exhibited a significantly longer OA and higher rates of wound complications and postoperative respiratory complications compared with the below the calculated IDOM group. The IDOM predicted the incidence of OA-related complications with a sensitivity of 72.4%, and a specificity of 80.6%. Conclusion The IDOM is a potentially useful tool for appropriate duration at the outset of OA.

　　Safe introduction of laparoscopic partial liver resection (LPLR) requires the selection of appropriate cases not exceeding the surgeon's skills as well as standardization of surgical procedures. After introduction at our institution, 60 LPLR procedures were performed between April 2010 and May 2016. To identify indices for case selection, short-term perioperative parameters were analyzed, including operative time, blood loss, postoperative complications, and postoperative hospital stay. Operative time was significantly shorter in the last 30 cases compared with the first 30 cases (182.5 min vs. 253 min; p＝0.023) and in 16 cases involving the left lobe (S2-4) compared with 44 cases involving the right lobe (S1, S5-8; 148.5 min vs. 246 min; p＝0.004). Blood loss was significantly less (0 mL vs. 50 mL; p＝0.028) and operative time was significantly shorter (185 min vs. 250 min; p＝0.048) in 27 cases with tumor diameter ＜2.5 cm compared with 33 cases with tumor diameter ≥ 2.5 cm. Operative time tended to be longer in 9 cases of multiple-site resection compared with 51 cases of single-site resection (207 min vs. 260 min; p＝0.085). BMI, pathology, and hepatitis virus status showed no significant difference in perioperative short-term results. For the introduction of LPLR, it may be preferable to select cases located in the left lobe with a tumor diameter ＜2.5 cm and to accumulate a certain amount of experience in similar cases first.

Vitamin D (VD) is essential for bone health, and VD or its analogues are widely used in clinics to ameliorate bone loss. The targets and mode of VD anti-osteoporotic actions appear to be different from those of other classes of drugs modulating bone remodeling. VD exerts its biological activities through the nuclear VD receptor (VDR)-mediated transcriptional regulation of target mRNA and non-coding RNA genes. VD-induced gene regulation involves epigenetic modifications of chromatin conformation at the target loci as well as reconfiguration of higher-order chromosomal organization through VDR-mediated recruitment of various regulatory factors. Enhancer RNAs (eRNA), a class of non-coding enhancer-derived RNAs, have recently emerged as VDR target gene candidates that act through reorganization of chromatin looping to induce enhancer-promoter interaction in activation of mRNA-encoding genes. This review outlines the molecular mechanisms of VD actions mediated by the VDR and suggests novel function of eRNAs in VDR transactivation.
Bone Remodeling ; Chromatin ; Epigenomics ; Metabolism ; Receptors, Calcitriol ; RNA ; RNA, Messenger ; RNA, Untranslated ; Transcriptional Activation ; Vitamin D ; Vitamins

PURPOSE: Intracranial administration of lipopolysaccharide (LPS) is known to elicit a rapid innate immune response, activate glial cells in the brain, and induce depression-like behavior. However, no study has focused on the changes in glial cells induced by intraperitoneal injection of LPS in vivo.METHODS: Ten adult male Fischer F344 rats underwent [¹¹C]PK11195 PET before and 2 days after intraperitoneal injection of LPS to evaluate the changes in glial cells. The difference in standardized uptake values (SUV) of [¹¹C]PK11195 between before and after injection was determined.RESULTS: There was a cluster of brain regions that showed significant reductions in SUV. This cluster included the bilateral striata and bilateral frontal regions, especially the somatosensory areas.CONCLUSIONS: Changes in activity of glial cells induced by the intraperitoneal injection of LPS were detected in vivo by [¹¹C]PK11195 PET. Intraperitoneal injection of LPS is known to induce depression, and further studies with [¹¹C]PK11195 PET would clarify the relationships between neuroinflammation and depression.
Adult ; Animals ; Brain ; Depression ; Electrons ; Humans ; Immunity, Innate ; Injections, Intraperitoneal ; Lipopolysaccharides ; Male ; Neuroglia ; Positron-Emission Tomography ; Rats ; Rats, Inbred F344

PURPOSE: Intracranial administration of lipopolysaccharide (LPS) is known to elicit a rapid innate immune response, activate glial cells in the brain, and induce depression-like behavior. However, no study has focused on the changes in glial cells induced by intraperitoneal injection of LPS in vivo. METHODS: Ten adult male Fischer F344 rats underwent [¹¹C]PK11195 PET before and 2 days after intraperitoneal injection of LPS to evaluate the changes in glial cells. The difference in standardized uptake values (SUV) of [¹¹C]PK11195 between before and after injection was determined. RESULTS: There was a cluster of brain regions that showed significant reductions in SUV. This cluster included the bilateral striata and bilateral frontal regions, especially the somatosensory areas. CONCLUSIONS Changes in activity of glial cells induced by the intraperitoneal injection of LPS were detected in vivo by [¹¹C]PK11195 PET. Intraperitoneal injection of LPS is known to induce depression, and further studies with [¹¹C]PK11195 PET would clarify the relationships between neuroinflammation and depression.

Objective: IgG4-related sclerosing cholecystitis is generally associated with IgG4-related sclerosing cholangitis and presents with diffuse, circumferential thickening of the gallbladder wall. We report a rare case of localized IgG4-related sclerosing cholecystitis without IgG4-related sclerosing cholangitis, which was difficult to differentiate from gallbladder cancer preoperatively.Patient: A 56-year-old man with suspected IgG4-related disease or gallbladder cancer was admitted to our ward. The serum IgG4 level was elevated at 721 mg/dL. Computed tomography (CT) demonstrated focal wall thickening of the gallbladder fundus. Drip infusion cholecystocholangiography with CT revealed no dilation, stenosis, or border irregularity of the bile duct.Results: For diagnostic and treatment purposes, cholecystectomy with wedge resection of the gallbladder bed was performed. The pathological diagnosis was IgG4-related sclerosing cholecystitis.Conclusion: It is difficult to differentiate IgG4-related sclerosing cholecystitis from gallbladder cancer in cases involving localized thickening of the gallbladder wall. In similar cases, surgical resection with cancer in mind might be performed based on present clinical knowledge.