Progressive calcification of the brain and the spinal cord at early infantile onset was observed in two siblings. They showed growth failure, psychomotor deterioration, deafness, vestibular dysfunction, microcytic hypochromic anemia, abnormal ratios of lymphocyte subpopulations, and slightly decreased bicarbonate on blood gas analysis. Distal renal tubular acidosis was demonstrated in one of them. Carbonic anhydrase II activity was normal. This new hereditary disease might have a defect in a molecule that is present in brain, spinal cord, kidney and hematocytes and is involved in H+/HCO3‐ production or transport.
Present dialysis condition from the annual statistical data from Japanese Society for Dialysis Therapy Number of chronic dialysis patients in Japan Japanese Society for Dialysis Therapy (JSDT) reports the annual statistical data of dialysis patients at the end of December. According to the data from JSDT in 2016, about 330,000 patients were receiving maintenance dialysis treatment, and this corresponds to about 2600 persons per million in the population (Figure 1). The number of dialysis patients continues to increase, but the increasing rate has already peaked. By the way, only 3% of end-stage renal disease (ESRD) patients are peritoneal dialysis (PD) patients (9021 patients) in Japan. 1 Mean age and original disease of dialysis patients Figure 2 shows the transition of average ages of dialysis patients. The patients with newly initiated dialysis and that of patients already on dialysis were 69.4 and 68.2 years at the end of 2016. The average age of both
A 44-year-old man suddenly excreted pink urine (Picture 1) after surgery for hepatoma. The serum biochemical examination did not indicate kidney dysfunction with hyperuricemia, except for metabolic acidosis. The pink substance was easily precipitated in the urine (Picture 2). Urinary sediment analysis showed a considerable amount of pink homogenous substance (Picture 3). Since the concentration of uric acid (UA) in the dissolved liquid and that in the patient's urine were extremely high (UA in pH 6.0 urine, 119.4 mg/dL), we concluded that the pink substance was amorphous UA. The solubility of UA is decreased via reduction of pH (pH 7.0, 200 mg/dL vs. pH 5.0, 15 mg/dL). The aciduria and hyperuricosuria, induced by metabolic acidosis and dehydration after the surgery, caused a deposition of amorphous UA, resulting in pink urine, which disappeared immediately when we treated the patient's dehydration and acidemia.
Dear Sirs, Acquired coagulation factor XIII (FXIII) deficiency is a rare bleeding disorder, in which severe haemorrhagic symptoms develop, often with significant mortality (1, 2). It usually develops in elderly populations.Two types of acquired FXIII deficiency are recognised. The first type is caused by specific inhibitors against FXIII and the second type involves less severe clinical symptoms associated with various conditions, often without any bleeding diathe-sis, from unidentified mechanisms. An IgG subtype of inhibitor against FXIII A subunit (FXIII-A) is a major contributor to the former type.Because of its rarity, management of acquired FXIII deficiency has not been clearly standardised; however, FXIII supplementations and immunosuppressive therapies have been utilised.Here, we report a case of acquired FXIII deficiency with FXIII-specific inhibitor treated with FXIII concentrates, which subsequently resulted in spontaneous disappearance of the inhibitor with satisfactory clinical outcome.A 76-year-old Japanese male was referred to our hospital in 2004 because of extensive haematomas. His history contained no potentially contributive factors, except for lung cancer, which was curatively resected at 60 years old without any haemorrhagic complications, and diabetes mellitus treated with metformin hydrochloride and insulin.A month earlier, a haematoma developed at his buttock after a bruise with spontaneous regression. Four days before,
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.