Kazuo Isoda

Anemia and neutropenia due to copper deficiency in enteral nutrition.

Copper deficiency has been regarded a rare complication of total parenteral nutrition. This report describes the first known case of anemia and neutropenia caused by copper deficiency in a patient receiving long-term enteral nutrition. A 34-year-old man presented with bulbar palsy and tetraplegia after an operation for cerebellar hemorrhage on June 7, 1989. Nasopharyngeal tube feeding with a defined-formula diet (Besvion) was instituted on June 19, 1989. He developed normocytic anemia and neutropenia approximately 19 months after the initiation of the tube feeding. Serum copper and ceruloplasmin levels were markedly below normal. There were anisocytosis and erythrocyte deformities in the peripheral blood smear. Bone marrow aspirates revealed a hypocellular marrow with numerous myeloid and erythroid cells with cytoplasmic vacuoles. Because the amount of copper administered was estimated to be 2.6 to 5.1 mumol/d during the tube feeding, copper deficiency was suspected to be the cause of the hematologic disorders. The patients daily oral copper intake was increased to 34 mumol/d. His serum copper and ceruloplasmin concentrations reached a normal level after 16 days and 23 days of copper supplementation, respectively. A marked reticulocytosis occurred after 10 days of copper supplementation, and his anemia gradually improved over the next 3 months. His blood neutrophil count also returned to normal within 2 weeks.

A family with two sisters with collagenofibrotic glomerulonephropathy

Collagenofibrotic glomerulonephropathy is a recently recognized disease entity. Although an autosomal recessive inheritance pattern has been suggested for this condition, there are few reports of familial cases. Only four pairs of child siblings, with histological confirmation of the glomerular lesions, have been reported. The current report describes a family including two sisters with histological evidence of collagenofibrotic glomerulonephropathy. Serum concentrations of the procollagen III peptide were elevated in the affected sisters, whereas their parents and other siblings demonstrated neither proteinuria nor increased blood levels of the procollagen III peptide. Our findings support an autosomal recessive pattern of inheritance for this type of glomerulonephropathy. They also suggest that the serum concentration of the procollagen III peptide is a useful marker for collagenofibrotic glomerulonephropathy.

Eradication of Helicobacter pylori in patients with end-stage renal disease under dialysis treatment

The efficacy and safety of combination therapy with amoxicillin, lansoprazole, and plaunotol for the eradication of Helicobacter pylori in patients on dialysis were evaluated. The study subjects comprised 15 dialysis patients in whom H pylori had been found in the gastric mucosa. The patients were given 500 mg amoxicillin once a day for 3 weeks, 30 mg lansoprazole once a day for 8 weeks, and 80 mg plaunotol three times a day for 24 weeks. Endoscopy was performed on entry and at 4 and 24 weeks after cessation of amoxicillin. The concentrations of serum gastrin and gastric juice ammonia also were measured. Fourteen patients completed the treatment protocol, one having dropped out because of nausea and diarrhea. H pylori was eradicated in 11 of the 14 patients 4 weeks after the end of amoxicillin therapy (eradication rate, 78.6%). All but one patient was free of H pylori 24 weeks after the amoxicillin was discontinued. Patients who became negative for H pylori had significantly decreased serum gastrin and gastric juice ammonia concentrations. Our findings indicate that a combination of amoxicillin, lansoprazole, and plaunotol can be used to eradicate H pylori in patients on dialysis.

T-cell receptor beta-chain gene polymorphism and the prognosis of IgA nephropathy in Japanese patients.

Ryuji Nagasawa, MD, Department of Internal Medicine, Saitama Medical Center, Saitama Medical School, 1981 Kamodatsujido, Kawagoe, Saitama 350 (Japan) Dear Sir, IgA nephropathy (IgAN), which is characterized by predominant IgA deposition in the glomerular mesangial area, is now recognized as the most common form of chronic glomerulonephritis worldwide [1]. The course of IgAN was first considered to be benign, but it has now become apparent that approximately 20-30% of these patients gradually lose their renal function and eventually progress to renal failure over a period of 20 years. At present, no therapy exists to halt this progression. Several clinical factors predict a poor prognosis for patients with IgAN. These factors are advanced age at the onset of disease, heavy proteinuria, hypertension, and severe histological renal damage [2]. However, not all patients with more than one such factor progress to end-stage renal failure, and, conversely, some without any of these factors undergo renal failure. Although the exact mechanism(s) governing the fate of renal function in IgAN is unclear, genetic factors are presumed to participate in its initiation and progression [3,4]. Clinically, a high level of serum IgA is the prominent immunological abnormality in patients with IgAN, in association with au-toantibodies, hyperactivity of helper T cells, high serum levels of T-cell-derived cyto-kines, etc. [5]. Such associated features are commonly observed in patients with systemic lupus erythematosus. Because the T cell receptor beta chain constant region (TCRC-ß) gene has been linked with autoantibody production in patients with systemic lupus erythematosus [6], we analyzed the restriction fragment length polymorphism (RFLP) of the TCRC-ß locus to seek the probable genetic contribution of T cells to IgAN. Thirty-four Japanese patients with biopsy-proven IgAN were investigated. High-molecularweight DNA was extracted from their peripheral blood leukocytes and digested with the restriction enzyme Bglll. After gel electrophoresis, DNA was hybridized to a cDNA probe from the constant region of the TCRC-ß locus. The results of RFLP analysis identified three allelic fragment lengths (type A: 10.0/10.0/0.8 kb; type B: 10.0/9.2/ 0.8 kb; type C: 9.2/9.2/0.8 kb). Four patients belonged to type A (12%), 16 to type B (47%), and 14 belonged to type C, (41%).

Neonatal thymectomy diminishes renal IgA deposition in IgA nephropathy-prone ddY mice

To understand the role of thymus-derived T cells in the development of IgA nephropathy (IgAN), we performed neonatal thymectomies on ddY mice, in which this disease occurs spontaneously. Although these thymectomized mice developed a renal lesion closely resembling that typical of IgAN, the extent of their mesangial IgA deposition was significantly milder than in control sham-operated mice. The immunological mechanisms responsible for curbing this mesangial deposition of IgA were then analyzed. The percentage of splenic T cells and the magnitude of mitogenic responses both decreased markedly in thymectomized compared with control mice. These results suggested the hypofunction of thymus-derived T cells. However, serum IgA levels were almost identical in both groups. Furthermore, sera from both groups contained similar amounts of macromolecular IgA, of the type formerly eluted from the affected glomeruli of patients with IgAN. These results strongly indicate that thymus-derived T cells or their products determine the amount of IgA deposited in the kidneys of ddY mice.

A Case of Water Intoxication with Prolonged Hyponatremia Caused by Excessive Water Drinking and Secondary SIADH

Water intoxication is a life-threatening disorder accompanied by brain function impairment due to severe dilutional hyponatremia. We treated a 22-year-old man without psychotic illness who had been put in a detention facility. He drank 6 liters of water over a 3-hour period at the facility as a games penalty, and he showed progressive psychiatric and neurological signs including restlessness, peculiar behavior and convulsions. On his admission, 15 h after the discontinuation of the water drinking, he was in a coma, showing intermittent convulsions and remarkable hyponatremia (120 mmol/l). Because his laboratory tests showed hypertonic urine and normal sodium excretion, the diagnosis of secondary development of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) was strongly suggested and later confirmed by the suppression of the renin-aldosterone system and the inappropriately elevated secretion of ADH. Saline infusion and an initial administration of furosemide in addition to dexamethasone as treatments for the patients brain edema successfully improved his laboratory data and clinical signs by the 3rd hospital day, and he was returned to the facility without physical or psychiatric abnormalities on the 6th day. The secondary SIADH might have been due to the prolonged emesis, recurrent convulsions and rapid elevation of intracranial pressure.

The Significance of Serum and Urinary Guanidinoacetic Acid Level for the Restoration of Renal Metabolic Function in Patients with Kidney Transplantation

It is well known that guanidinoacetic acid (GAA), precursor of creatine, is mainly producted by the kidney and the concentration of GAA in serum and urine and decreased in patients with chronic renal failure (CRF). However, the mechanism for the excretion of this substance by the kidney still remains to be clarified. Therefore, this study is aimed to assess the restoration of the metabolic function of the transplanted kidney and the excretory manoeuvre by means of measurements of serum and urinary guanidino compounds, especially the GAA levels using fluorometric HPLC.

Assessment of left ventricular diastolic function by pulsed Doppler echocardiography in chronic hemodialysis patients.

慢性血液透析患者の心不全の成因として, 収縮不全のみならず, 心室壁の肥厚や心筋間質の線維化による左室コンプライアンスの低下など, 種々の要因に基づく拡張不全も関与する可能性がある. そこで慢性血液透析患者の左室流入動態を超音波パルス・ドップラー法を用いて解析し, さらに前負荷の左室流入動態に及ぼす影響について, 右室駆出率/心拍出量測定装置 (REF-1) を用いて検討した.慢性血液透析患者 (HD群) 13例 (男5例, 女8例, 年齢55±9歳) および健康成人 (control群) 6例を対象とし, 超音波パルス・ドップラー法を施行し, 左室流入パターンを急速流入最大速度 (R), 心房収縮流入最大速度 (A), A/R比などを指標として検討した. なおHD群では血液透析前後でも同様の検査を行い比較検討し, うち5例にREF-1を用い透析中の血行動態を測定した. Control群に比しHD群ではA/R比の上昇 (0.90±0.09 vs 1.21±0.30, p<0.01) が認められた. HD群では, 透析前に比べ透析後でRは有意に低下 (43.5±7.9 vs 37.0±8.0, p<0.01) したが, Aには有意な変化は認めなかった. Rの変化と除水量には有意 (p<0.01) な相関を認めた. REF-1を用い透析中の血行動態を検討すると, 除水に伴い右房圧 (RAP), 肺動脈楔入圧 (PCWP), 右室容量 (RVEDVI) および心係数 (Cl) の低下を認めたが, 前負荷の指標としてはRVEDVIが優れていた. しかし, Rの変化とRVEDVIの変化には, 症例数が少ないため有意な相関は認めなかった.左室流入パターンは心室の拡張機能のみならず, 左室充満圧に大きく変化をうけるので, 拡張機能の評価には前負荷の影響を考慮に入れる必要があるが, control群に比しHD群では透析前後ともにA/R比は有意の高値を示すことより, 慢性血液透析患者の左室拡張機能は異常を呈することが示唆された.