SUMMARY Arthro-osteitis at the anterior chest wall was found in 12 (9*4 %) out of 128 consecutive patients with pustulosis palmaris et plantaris. This finding indicates that the concomitance of arthro-osteitis with PPP is not incidental but is based on some common aetiological factor. We propose a term 'pustulotic arthro-osteitis' for this condition.Intersterno-costoclavicular ossification is a newly described rheumatic condition which shows unique ossifications between the clavicle and the first rib.

Cerebellar ataxia, mental retardation, and disequilibrium syndrome 4 (CAMRQ4) is early onset neuromotor disorder and intellectual disabilities caused by variants of ATP8A2. We report sibling cases and systematically analyze previous literature to increase our understanding of CAMRQ4. Japanese siblings presented with athetotic movements at 1 and 2 months of age. They also had ptosis, ophthalmoplegia, feeding difficulty, hypotonia, and severely delayed development. One patient had retinal degeneration and optic atrophy. Flattening of the auditory brainstem responses and areflexia developed. At the last follow-up, neither patient could sit or achieve head control, although some nonverbal communication was preserved. Whole exome sequencing revealed compound heterozygous variants of ATP8A2: NM_016529.6:c.[1741C>T];[2158C>T] p.[(Arg581*)];[(Arg720*)]. The p.(Arg581*) variant has been reported, while the variant p.(Arg720*) was novel. The symptoms did not progress in the early period of development, which makes it difficult to distinguish from dyskinetic cerebral palsy, particularly in solitary cases. However, visual and hearing impairments associated with involuntary movements and severe developmental delay may be a clue to suspect CAMRQ4.

Kocuria kristinae is a catalase-positive, coagulase-negative, Gram-positive coccus found in the environment and in normal skin and mucosa in humans; however, it is rarely isolated from clinical specimens and is considered a nonpathogenic bacterium. We describe a case of catheter-related bacteremia due to K. kristinae in a young adult with propionic acidemia undergoing periodic hemodialysis. The patient had a central venous catheter implanted for total parenteral nutrition approximately 6 months prior to the onset of symptoms because of repeated acute pancreatitis. K. kristinae was isolated from two sets of blood cultures collected from the catheter. Vancomycin followed by cefazolin for 16 days and 5-day ethanol lock therapy successfully eradicated the K. kristinae bacteremia. Although human infections with this organism appear to be rare and are sometimes considered to result from contamination, physicians should not underestimate its significance when it is isolated in clinical specimens.

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We herein report the successful treatment of a 4-year-old girl with left ventricular noncompaction (LVNC) who presented with incessant ventricular fibrillation at 5 months of age. An implantable cardioverter defibrillator (ICD) was implanted, and dual chamber (DDD) pacing was initiated at 7 months of age. At her 10-month follow-up, her left ventricular ejection fraction (LVEF) had decreased from 45% to 20% with mechanical dyssynchrony. After upgrading to cardiac resynchronization therapy (CRT), the LVEF improved to 50%. The usefulness of CRT in pediatric LVNC has not been fully elucidated. However, our case suggests that CRT therapy may be an effective option for LVNC-induced cardiac dysfunction.