Acute Tubulointerstitial Nephritis due to Human Papillomavirus Vaccination.

Acute tubulointerstitial nephritis (ATIN), a rare cause of acute kidney injury in children, is caused by various factors such as drugs, infection, and systemic inflammation. We herein present a case of ATIN with mild encephalitis/encephalopathy with reversible splenial lesion (MERS)-like findings on head magnetic resonance imaging (MRI), which was associated with human papillomavirus (HPV) vaccination. A 14-year-old girl presented to our hospital with a high fever for 5 days. Results of common laboratory tests were normal except for increased C-reactive protein (CRP) levels and erythrocyte sedimentation rate (ESR). Antibiotics were administered, and the fever promptly resolved after admission. After 7 weeks, she was readmitted due to a high fever for 4 days. In addition to increased CRP levels and ESR, urine test revealed high urine N-acetyl-ß-D-glucosaminidase and ß-2-microglobulin levels, and a renal scintigram showed mild bilateral uptake of 67Ga-citrate, consistent with the pathology of ATIN. Furthermore, head MRI, which was performed because the patient experienced prolonged headaches, revealed MERS-like lesions, although she did not have other neurological symptoms. Detailed examination of her medical records revealed that she had developed high fever 10 days after the third HPV vaccination and another previous episode of high fever 12 weeks after the second HPV vaccination. Based on these findings, we concluded that the ATIN and MERS-like lesions could have been associated with HPV vaccination. Although HPV vaccination is important for preventing uterine cancer, physicians must be vigilant about its various potential adverse effects, including ATIN.

Mucolipidosis â ¡ and III with neurological symptoms due to spinal cord compression.

In mucopolysaccharidoses (MPS), spinal cord compression (SCC) resulting from glycosaminoglycan (GAG) accumulation is a critical complication that can cause significant neurological and respiratory morbidities. However, clinically similar disorders such as mucolipidosis types II and III (ML) with SCC have been scarcely reported. Herein, we report four patients with ML who had SCC. Brain MRI revealed progressive spinal canal stenosis and SCC. In addition, T2-weighted high signal changes in the cervical cord were detected in two cases. Severe cases of SCC were detected as early as 1 year of age. All cases had respiratory problems. One case showed severe hypoxia and another, severe sleep apnea. In two cases, respiratory insufficiency and tetraplegia rapidly progressed as SCC progressed. Then, the patients became bedridden and needed artificial ventilation. In addition, two of the four patients died of respiratory failure. The autopsy of one patient revealed a compressed cervical cord and marked dura mater thickening due to GAG accumulation. These findings suggest that the accumulation of substrates in the dura mater caused SCC in the patients with ML. Our cases indicate that SCC is expected to be a common and critical complication of ML and MPS. MRI evaluation of cervical involvements and careful clinical observation are required in patients with ML.

Trends in descriptions of palliative care in the cancer clinical practice guidelines before and after enactment of the Cancer Control Act (2007): content analysis.

BACKGROUND: Palliative care was a priority issue in the Cancer Control Act enacted in 2007 in Japan, and this has resulted in efforts being made toward educational goals in clinical settings. An investigation of how descriptions of palliative care for the treatment of cancer have changed in clinical practice guidelines (CPGs) could be expected to provide a better understanding of palliative care-related decision-making. This study aimed to identify trends in descriptions of palliative care in cancer CPGs in Japan before and after enactment of the Cancer Control Act. METHODS: Content analysis was used to count the lines in all relevant CPGs. We then compared the number of lines and the proportion of descriptions mentioning palliative care at two time points: the first survey (selection period: February to June 2007) and the second survey (selection period: February to December 2015). Descriptions from the CPGs were independently selected from the Toho University Medical Media Center and Medical Information Network Distribution Service databases, and subsequently reviewed, by two investigators. RESULTS: Descriptions were analyzed for 10 types of cancer. The proportion of descriptions in the first survey (4.4%; 933/21,344 lines) was similar to that in the second survey (4.5%; 1325/29,269 lines). CONCLUSIONS: After the enactment of the Cancer Control Act, an increase was observed in the number, but not in the proportion, of palliative care descriptions in Japanese cancer CPGs. In the future, CPGs can be expected to play a major role in helping cancer patients to incorporate palliative care more smoothly.

A novel PLP1 mutation F240L identified in a patient with connatal type Pelizaeus-Merzbacher disease.

Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive hypomyelination disorder caused by mutations in the proteolipid protein 1 gene (PLP1) located on chromosome Xq22. A male patient showed severe developmental delay, pendular nystagmus and laryngeal wheezing. The auditory brain stem response showed only the first wave and brain magnetic resonance imaging showed white matter hypomyelination, suggesting typical PMD. A novel PLP1 mutation, F240L, which was inherited from his mother, was identified.

Prescribing pattern of anti-Parkinson drugs in Japan: a trend analysis from 2005 to 2010.

OBJECTIVE: Therapeutic options for Parkinson's disease mainly consist of L-dopa and dopamine agonists. However, in Japan, the product labeling of the ergot dopamine agonists, cabergoline and pergolide, was revised in April 2007 due to the risk of developing cardiac valvulopathy. Here, we describe the prescribing trends of anti-Parkinson drugs from 2005 through 2010 in Japan, and examined whether these trends changed after the drug safety measures in 2007. METHODS AND PATIENTS: We used medical claim data from January 2005 to December 2010 for Parkinson's disease patients older than 30 years who were prescribed anti-Parkinson drugs. We calculated the proportion of patients prescribed each drug for each year, and compared the proportions of first-line drugs prescribed before and after April 2007. We also examined the prescription variations of cabergoline/pergolide users one year before or after April 2007. RESULTS: L-dopa was the most frequently prescribed drug for Parkinson's disease (2005, 58%; 2010, 51%). The proportion of patients prescribed ergot dopamine agonists markedly decreased and non-ergot dopamine agonists increased after 2007. Among first-line drugs, the proportion of non-ergot agents increased after April 2007. Among 54 cabergoline/pergolide users, 24 (44%) discontinued these drugs, nine of whom switched to non-ergot agents. CONCLUSION: L-dopa was the mainstay of Parkinson's disease treatment between 2005 and 2010 in Japan. There was a decrease in ergot agents and an increase in non-ergot agents prescribed after the regulatory actions in 2007.

A de novo interstitial deletion of 8p11.2 including ANK1 identified in a patient with spherocytosis, psychomotor developmental delay, and distinctive facial features.

The contiguous gene syndrome involving 8p11.2 is recognized as a combined phenotype of both Kallmann syndrome and hereditary spherocytosis, because the genes responsible for these 2 clinical entities, the fibroblast growth factor receptor 1 (FGFR1) and ankyrin 1 (ANK1) genes, respectively, are located in this region within a distance of 3.2Mb. We identified a 3.7Mb deletion of 8p11.2 in a 19-month-old female patient with hereditary spherocytosis. The identified deletion included ANK1, but not FGFR1, which is consistent with the absence of any phenotype or laboratory findings of Kallmann syndrome. Compared with the previous studies, the deletion identified in this study was located on the proximal end of 8p, indicating a pure interstitial deletion of 8p11.21. This patient exhibited mild developmental delay and distinctive facial findings in addition to hereditary spherocytosis. Thus, some of the genes included in the deleted region would be related to these symptoms.

Echocardiography for the detection of valvulopathy associated with the use of ergot-derived dopamine agonists in patients with Parkinson's disease.

OBJECTIVE: The ergot-derived dopamine agonists, cabergoline and pergolide, are associated with valvulopathy risk. In Japan, product labels were revised in April 2007 to recommend periodic echocardiography for patients taking these dopamine agonists, however, the compliance of physicians to follow through with this recommendation is unknown. This study assessed changes in echocardiography evaluation of patients with Parkinson's disease (PD) taking cabergoline or pergolide before and after the label revision and examined the factors related with echocardiography performance. METHODS AND PATIENTS: Medical claim data from January 2005 to December 2008 were used. Patients were divided into a C-P group (prescribed either cabergoline or pergolide) or reference group (prescribed other anti-PD drugs), and further classified based on whether they were prescribed these drugs "pre-revision" or "post-revision." The Cochran-Armitage trend test was used to compare the proportion of echocardiograms obtained amongst these groups before and after the revision. The frequencies of echocardiograms performed among the treatment groups for each period were compared by Fisher's exact test. RESULTS: A total of 222 subjects (C-P, 73; reference, 149) were assessed. The proportion of C-P patients undergoing echocardiography increased from 4.8% to 27.9% after revision of product labels (p=0.001), which was higher than those in the reference group following label revisions (11.0%) (p=0.014). Prescription duration of C-P after the revision was longer in the patients with echocardiography than without echocardiography (p=0.026). CONCLUSION: Although echocardiography evaluations increased, more than 70% of PD patients prescribed cabergoline or pergolide did not undergo such assessment despite the product label recommendation. Adherence to drug safety recommendations should be facilitated with more feasible and effective measures.