Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency

BACKGROUND: In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, glycerol and fructose may cause severe hypoglycemia and metabolic acidosis in patients with fructose-1,6-bisphosphatase (FBPase) deficiency, even under stable conditions. The aim of the present study was to determine whether glycerol solution was used for brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis in patients with unrecognized FBPase deficiency in Japan and to examine a long-term prognosis of the patients who had this kind of severe metabolic decompensation with or without glycerol therapy. METHODS: A retrospective study of 20 children with FBPase deficiency was conducted, based on their medical records. RESULTS: Six of the 20 children were given glycerol solution for the presence or possibility of brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis; two of the six patients administered with glycerol were given dialysis. In four patients treated with glycerol alone without dialysis, two had no brain edema before glycerol administration but it developed later after the administration. These four patients treated with glycerol alone died or developed severe neurological complications. Fourteen patients who were not treated with glycerol solution had no brain edema and showed good prognosis. CONCLUSIONS: Glycerol solution, which contains fructose in Asian countries including Japan, should not be used as an osmotic agent for treatment of brain edema in patients who have hypoglycemia and retention-type metabolic acidosis, until FBPase deficiency is ruled out by measuring blood concentration of lactate.     

中药对大鼠自发性慢性胰腺炎的干预作用及其方证病态基础

目的探讨桂枝汤、柴胡桂枝汤、小柴胡汤和卡莫司他对大鼠自发性慢性胰腺炎的干预作用及其方证病态基础。方法57只雄性4周龄WBN/Kob大鼠随机分为5组模型组、桂枝汤组、柴胡桂枝汤组、小柴胡汤组和卡莫司他组,每组大鼠均予以相应药物干预,共12周。每4周取大鼠胰腺组织观察胰腺湿重;HE染色观察胰腺组织病理学改变;RT-PCR技术检测大鼠胰腺胰腺炎相关蛋白(pancreatitis-associatedprotein,PAP)mRNA的表达水平;免疫组织化学方法检测胰腺PAP蛋白的表达。结果模型组自12周龄起胰腺组织发生胰腺炎病理学改变;桂枝汤组未观察到胰腺炎病理学改变;小柴胡汤组和卡莫司他组在16周龄时,胰腺组织均发生明显的胰腺炎病理学改变。桂枝汤组抑制PAPmRNA表达的作用大于其他各中药干预组。桂枝汤组和柴胡桂枝汤组在12周龄时,未发现胰腺PAP的表达。结论桂枝汤、柴胡桂枝汤、小柴胡汤和卡莫司他对大鼠自发性慢性胰腺炎具有不同程度的防治作用,其中以桂枝汤的作用最为明显。“太阳表证”可能是大鼠自发性慢性胰腺炎的中医基本证候病机,PAP的表达可能是其证候病机的病态基础之一。     

Physiological changes in the treatment of acrophobia (fear of height)

In order to investigate the physiological changes produced by the treatment of acrophobic patients body movement and Microvibration were measured before and after treatment. Eighteen acrophobic patients were assigned at random to 1 of the 2 groups: a treatment group (n= 8) and a non-treatment group (n= 10). The control group consisted of 16 healthy volunteers. Body movement area while viewing a slide of a high place or imagining a high place in the treatment group decreased significantly after treatment. Body movement of the control group showed almost no changes, and that of the non-treatment group was situated between the above-mentioned 2 groups. Simple body movements of the acrophobic patients without any stimulation of height were bigger than those of the control subjects. MV pattern of the treatment group had a tendency to improve under psychotherapy. Acrophobic patients had more abnormal MV patterns than the normal subjects.     

Cystosarcoma phyllodes of the seminal vesicle

BACKGROUND: We report here an extremely rare case of cystosarcoma phyllodes of the seminal vesicle. METHODS: A 65-year-old man presented with urinary hesitancy, frequency and constipation. Clinical examinations including two needle biopsies were performed, and the patient had undergone open surgery. RESULTS: The final pathological diagnosis was cystosarcoma phyllodes of the seminal vesicle. Seven months after the operation, a chest X-ray showed lung metastasis, and the patient died 11 months after the operation. CONCLUSION: To our knowledge, only one case of cystosarcoma phyllodes of the seminal vesicle has been previously reported.     

Influence of piecemeal necrosis on the compensatory increase of mitochondrial respiratory enzymes of the tumour-bearing liver: Clinical study of 53 surgical cases

The relationships between histological findings, adaptively increased cytochrome a(+a3) levels in chronic liver disease and complications after hepatectomy were studied in order to clarify the mechanism of mitochondrial derangement. The liver specimens of 53 hepatectomized patients were randomly evaluated by three independent hepatopathologists and were compared with cytochrome a(+a3) levels in the biopsied liver, the extent of operation and postoperative complications. The cytochrome a(+a3) concentrations did not show any significant difference between cases of chronic hepatitis and liver cirrhosis nor groups classified by regeneration. Severity of piecemeal necrosis was categorized into three groups: group A--minimal (n = 20); group B--moderate (n = 19); and group C--severe (n = 14). There were significant differences (P less than 0.01) in cytochrome a(+a3) concentrations between the groups (A: 99 +/- 9; B: 135 +/- 6; C: 155 +/- 10 pmol/mg of mitochondrial protein). Extensive hepatectomy, involving segmentectomy or more, was frequently complicated (four of nine, 44.4%) in group C, whereas there were few complications (two of 16, 12.5%) in group A cases in which extensive hepatectomy was performed. Evidence will be presented which will show that deranged liver function, as indicated by cytochrome a(+a3) levels, is closely correlated with piecemeal necrosis. This may be attributed to the damage of periportal hepatocytes which are the main sites of oxidative phosphorylation.     

Solitary psoas muscle metastasis after radical nephrectomy for renal cell carcinoma

Skeletal muscle is a very rare location for the metastasis of renal cell carcinoma (RCC) and only one case of solitary metastasis to the psoas muscle has been reported. We present a 63-year-old male patient with late recurrence (14 years) after left side radical nephrectomy for RCC. He first visited Chikushi Hospital, Fukuoka University, Japan in January 2000 for a postoperative follow-up because he had shifted residence to the area. Follow-up was by abdominal computed tomography (CT) and chest X-ray. In December 2001, a CT scan showed a 1.5 cm enhanced mass in the right psoas muscle without any other metastasis. The mass was resected that month and histological study showed RCC metastasis.     

Persistent chorea following cardiac surgery for congenital heart disease

We describe a 6 year old girl with chorea following cardiac surgery, the first such report in Japan. The radical operation for total anomalous pulmonary venous return was carried out at the age of 11 months under hypothermia. Seven days after the operation, a sudden onset of irritability, dysphagia, chorea, generalized hypotonia, and complete external ophthalmoplegia were seen. These symptoms diminished gradually, but chorea remained. We speculated that the cause of chorea arose from the cardiac surgery under hypothermia. It is necessary to consider ‘cardiac surgery’ as one of the triggers of certain movement disorders including chorea. We tried treatment with haloperidol, pimozide, and several other drugs; only pimozide was effective in decreasing chorea without any side-effects.     

A case of familial thyroxine binding globulin excess associated with growth hormone deficiency

A 7 years 3 months old Japanese boy with familial thyroxine binding globulin (TBG) excess associated with growth hormone (GH) deficiency is reported. The patients height was 106.4 cm (- 2.86 s.d.) and his bone age was 5 years and 3 months. He had no goiter and his developmental milestones were normal. The serum thyroid stimulating hormone (TSH) was 2.8 μU/mL, triiodothyronine (T₃) 3.1 ng/mL, thyroxine (T₄) 23.4 μg/dL and free T₄ 1.8 ng/dL. The serum TBG level was beyond 80.0 μg/mL, with normal TSH response to the thyrotropin-releasing hormone (TRH) test. Familial study revealed that his grandmother, mother, uncle, younger sister and younger brother had high TBG and T₃ levels, thus an X-linked co-dominant transmission was suggested. The peak GH responses to insulin and clonidine hydrochloride were 5.8 and 8.2 ng/mL, respectively. The mean nocturnal GH concentration was 2.5 ng/mL. His growth velocity increased from 4.8 to 8.4 cm/year and his serum TBG levels decreased gradually after human growth hormone (hGH) treatment.     

Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa

We report a case of a 15 year old boy with polyarteritis nodosa associated with antineutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3). After months of steroid and immunosuppressant therapy, the symptoms subsided and the polyaneurysms almost disappeared. The levels of anti-PR3 antibody and of cytokines also decreased. The results indicate that ANCA is a good indicator of this disease activity, and may play some pathogenic role in the disease.     

STUDIES ON PEPTIDES LXXXI. Application of a New Arginine Derivative,NG-Mesitylene-2-Sulfonylarginine,to the Synthesis of Substance P and Neurotensin

A new devised arginine derivative, N^G-mesitylene-2-sulfonylarginine, Arg(Mts), was employed for the synthesis of hypothalamic substance P and neurotensin. The former was obtained in 74% yield by treatment of the protected undecapeptide amide, Z - Arg(Mts) - Pro - Lys(Z) - Pro - Gln - Gln - Phe - Phe - Gly - Leu - Met(O)-NH₂, with methanesulfonic acid in the presence of anisole followed by reduction of the sulfoxide with 2-mercaptoethanol. The latter was obtained in 54% yield by the similar treatment of the protected tridecapeptide ester, Z-Pyr-Leu - Tyr - Glu(OBzl) - Asn - Lys(Z)-Pro-Arg(Mts) - Arg(Mts) - Pro - Tyr-Ile-Leu-OBzl, with methanesulfonic acid. As scavenger, a mixture of anisole-thioanisole-o-cresol (1:1:1, by vol.) was employed to suppress the side reaction, O-mesitylene-2-sulfonation of the Tyr residue.