IgD lambda multiple myeloma causing hyperammonaemia with possibly reduced ammonium excretion.

A man in his 70s presented with impaired consciousness due to hyperammonaemia while investigating multiple tumours in his brain, chest, spine and right adrenal gland. He did not have any disorders causing hyperammonaemia, such as liver dysfunction, urea-producing bacterial infection or any medications interfering with ammonium metabolism. Blood and urine tests in addition to tumour biopsy specimens confirmed immunoglobulin D lambda multiple myeloma. His general status responded to chemotherapy using bortezomib, dexamethasone and daratumumab, and he subsequently regained full consciousness and a normalised serum ammonia level. He, unfortunately, died of refractory multiple myeloma with hyperammonaemia. The autopsy specimen revealed lambda light-chain deposits in the distal tubule epithelium with cast precipitation and intact liver cells. Urine osmolality gap analysis suggested possibly reduced urinary ammonium excretion, but further investigation is necessary to elucidate the significance of pathological renal characteristics in multiple myeloma with hyperammonaemia.

A comprehensive analysis of the clinical characteristics and laboratory features in 179 patients with autoimmune autonomic ganglionopathy.

The clinical importance of autoantibodies against the ganglionic acetylcholine receptor (gAChR) remains to be fully elucidated. We aimed to identify the clinical characteristics of autoimmune autonomic ganglionopathy (AAG) in patients with gAChR autoantibodies. For this cohort investigation, serum samples were obtained from patients with AAG between 2012 and 2018 in Japan. We measured the levels of autoantibodies against gAChRα3 and gAChRß4 and evaluated clinical features, as well as assessing the laboratory investigation results among the included patients. A total of 179 patients tested positive for antibodies, including 116 gAChRα3-positive, 13 gAChRß4-positive, and 50 double antibody-positive patients. Seropositive AAG patients exhibited widespread autonomic dysfunction. Extra-autonomic manifestations including sensory disturbance, central nervous system involvement, endocrine disorders, autoimmune diseases, and tumours were present in 118 patients (83%). We observed significant differences in the frequencies of several autonomic and extra-autonomic symptoms among the three groups. Our 123I-metaiodobenzylguanidine myocardial scintigraphy analysis of the entire cohort revealed that the heart-to-mediastinum ratio had decreased by 80%. The present study is the first to demonstrate that patients with AAG who are seropositive for anti-gAChRß4 autoantibodies exhibit unique autonomic and extra-autonomic signs. Decreased cardiac uptake occurred in most cases, indicating that 123I- metaiodobenzylguanidine myocardial scintigraphy may be useful for monitoring AAG. Therefore, our findings indicate that gAChRα3 and gAChRß4 autoantibodies cause functional changes in postganglionic fibres in the autonomic nervous system and extra-autonomic manifestations in seropositive patients with AAG.

[Chemotherapy with MTX, 5-FU and CDGP for treatment of newly diagnosed head and neck cancer].

Combination chemotherapy including 5-fluorouracil (5-FU) and nedaplatin (CDGP) with methotrexate (MTX) and leucovorin(LV)as biochemical modulators was performed on patients with newly diagnosed head and neck cancer. We treated 45 patients with MTX, 5-FU and CDGP consisting of 150 mg/body of MTX on day 1 followed by a 3-day continuous infusion of 5-FU at 3,500 mg/m2 and 17 injections of LV at 15 mg and infusion of CDGP at 100 mg/m2. Forty-three patients were concurrently treated with radiotherapy. Seven of the 45 had distant metastasis. In spite of the metastasis, 6 of the 7 underwent radiation therapy only on the primary or cervical metastatic mass because their primary tumor was bulky. Treatment-associated toxicities, e. g., mucositis and myelosuppression were significant but acceptable. The overall response rate was 97.6% with 61.9% of CR.

[Docetaxel (TXT) and irinotecan (CPT-11) as a second-line chemotherapy for platinum-pretreated squamous cell carcinoma of the head and neck].

Cisplatin(CDDP), combined with 5-fluorouracil, is considered one of the most active chemotherapeutic combinations in the treatment of patients with squamous cell carcinoma of the head and neck(SCCHN)and is accepted today as a standard regimen. But second-line chemotherapy for platinum-pretreated patients has not yet been established. Eighteen patients with recurrent SCCHN were treated using docetaxel (TXT) and irinotecan (CPT-11). All of them had been pretreated with platinum included regimen. In principle, our regimen consisted of TXT(50-60 mg/m(2), day 1) and CPT-11(60-90 mg/m(2), day 1, 8, 15). The adverse events were significant, including 13(72.2%)of grade 3 and 4 neutropenia, but acceptable. Seventeen patients were eligible for evaluation of response. Two complete responses (CR; 11.8%)and 6 partial responses (PR; 35.3%)were observed with an overall response rate of 47.1%. Patients pretreated with TXT had a 25.0% response rate (1 PR and 3 progressive disease). We conclude that the combination of TXT and CPT-11 is a worthwhile treatment for platinum-pretreated SCCHN as a 2nd-line-chemotherapy.

[Epidemiological survey of allergic diseases in first-year junior high school students in Wakayama Prefecture in 2003].

Allergic diseases have been increasing in prevalence in developed countries, including Japan. The aim of the present report was to determine the prevalence of allergies in Wakayama prefecture using an epidemiological study. In total, 759 first-year students attending junior high school in Hidaka country, Wakayama prefecture, were surveyed. The results for 699 cases were then analyzed. A questionnaire regarding allergic diseases, specific IgE measurements performed using a MAST26 system (Hitachi Co., Ltd.), and total serum IgE levels measured using RIST (Pharmacia Co., Ltd.) were performed. The prevalence (present + past) of various allergic diseases was 37.9%. The prevalence of rhinitis, including pollinosis, was 31.0%, while that of atopic dermatitis was 26.2% and bronchial asthma was 11.3%. The positive rates for specific IgE antibodies against Japanese cedar pollen was 48.6%. The positive rate for Dermatophagoides farinae, timothy and housedust II were 44.2%, 29.6%, and 28.9%, respectively. Statistically significant differences were recognized between the students with allergic rhinitis, atopic dermatitis, allergic conjunctivitis or bronchial asthma and positive results for D. farinae, housedust II, cedar pollen, Penicillium, Cladosporium, or Aspergillus-specific antibodies. Regarding family history (two generations), a statistical significant difference between family history and positivity for specific IgE antibodies like D. farinae, housedust II, ragweed, cedar pollen or Cladosporium was observed. The total IgE titer was correlated with the number of positive allergen items. The increasing prevalence of various allergic diseases in developed countries remains a mystery, but the hygienehypothesis has attracted some attention. The findings of this epidemiologic study will contribute to basic data on the increasing prevalence of various allergic diseases.