Microscopic polyangiitis presented with biopsy-confirmed pleuritis

We describe a case of microscopic polyangiitis manifested as pleuritis confirmed by thoracoscopic biopsy. An 80-year-old man presented with a three-day history of shortness of breath and cough. Chest radiography revealed patchy opacities in the lower fields of the bilateral lung and right-sided pleural effusion. Thoracentesis revealed lymphocytic pleural exudates. Thoracoscopic biopsy specimens were compatible with fibrotic pleuritis. He developed rapidly progressive glomerulonephritis with elevated myeloperoxidase anti-neutrophil cytoplasmic antibody titer in blood and pleural effusion. Although the patient was resistant to two weekly courses of pulse steroid therapy, he was successfully treated with a five-day course of intravenous immunoglobulin

Lysosomal integrity requires sphingosine kinase activity

Sphingosine 1-phosphate (S1P) has been implicated in brown adipose tissue (BAT) formation and energy consumption; however, the mechanistic role of sphingolipids, including S1P, in BAT remains unclear. Here, we showed that, in mice, BAT activation by cold exposure upregulated mRNA and protein expression of the S1P-synthesizing enzyme sphingosine kinase 1 (SphK1) and S1P production in BAT. Treatment of wild-type brown adipocytes with exogenous S1P or S1P receptor subtype-selective agonists stimulated triglyceride (TG) breakdown only marginally, compared with noradrenaline. However, genetic deletion of Sphk1 resulted in hypothermia and diminished body weight loss upon cold exposure, suggesting that SphK1 is involved in thermogenesis through mechanisms different from receptor-mediated, extracellular action of S1P. In BAT of wild-type mice, SphK1 was localized largely in the lysosomes of brown adipocytes. In the brown adipocytes of Sphk1-/- mice, the number of lysosomes was reduced and lysosomal function, including proteolytic activity, acid esterase activity, and motility, was impaired. Concordantly, nuclear translocation of transcription factor EB, a master transcriptional regulator of lysosome biogenesis, was reduced, leading to decreased mRNA expression of the lysosome-related genes in Sphk1-/- BAT. Moreover, BAT of Sphk1-/-mice showed greater TG accumulation with dominant larger lipid droplets in brown adipocytes. Inhibition of lysosomes with chloroquine resulted in a less extent of triglyceride accumulation in Sphk1-/- brown adipocytes compared with wild-type brown adipocytes, suggesting a reduced lysosome-mediated TG breakdown in Sphk1-/- mice. Our results indicate a novel role of SphK1 in lysosomal integrity, which is required for TG breakdown and thermogenesis in BAT

Successful laparoscopic repair of uterine and rectal prolapse in an infant

Neonatal or infantile uterine prolapse is a quite rare condition and is usually managed with conservative treatment. There is no standard surgical treatment for infantile uterine prolapse, and to the best of our knowledge, only 2 out of 30 patients suffering from it have been reported to undergo surgical repair in English literature since 1961. We here report the first successful case of laparoscopic repair for uterine prolapse in an infant. The patient was a 2-month old girl who had sacral myelomeningocele and Chiari type 2 malformation. She had undergone closure of myelomeningocele and ventriculoperitoneal shunting within 6 weeks after birth. At 7 weeks of age, the rectum and the uterus prolapsed, and the prolapse gradually deteriorated. Conservative treatments including repeated digital reduction, use of ointment and glycerin enema, and placement of a Foley catheter into the vagina were not effective. At the age of 100 days, she underwent laparoscopic hysteropexy and rectopexy. Three ports were placed on the umbilicus and the bilateral abdomen, and the bilateral mesovaria were sutured to the anterior abdominal wall to improve the visualization of the pelvis. The rectum and the uterine body were directly sutured to the sacral promontory with 2 non-absorbable braided sutures each, not using mesh prosthesis. The postoperative course was uneventful and neither uterine nor rectal prolapse has recurred for 2.5 years. We plan to follow up the patient for a long period since the long-term prognosis is not known