Case of localized scleroderma successfully treated with bath psoralen and ultraviolet A therapy

The patient was a 12-year-old girl with linear scleroderma distributed on the right abdomen, dorsal aspect of the right thigh, lower leg and foot. The initial regimen of oral prednisolone and methotrexate, or i.v. methylprednisolone failed in the treatment of the scleroderma. Then bath psoralen and ultraviolet A therapy (bath-PUVA) therapy of 0.2 J–4.0 J/cm² daily to total doses 62.8 J/cm² combined with oral prednisolone was started. After bath-PUVA therapy, regression of the skin sclerosis was observed, the possible mobile range of the right ankle was increased and histological examination confirmed improvement of the sclerosis. The successful results of bath-PUVA therapy in this case suggest its utility for localized scleroderma.     

13C-phenylalanine breath test correlates with liver fibrosis in postoperative biliary atresia

BACKGROUND: Values derived from the (13)C-phenylalanine breath test (PBT) may serve as an index for liver fibrosis and clinically predictive readings for liver diseases in adults. In the present study the PBT was conducted in postoperative biliary atresia (BA) children to evaluate phenylalanine metabolism in the liver, and the results based on biochemical data, especially the index on liver fibrosis, were compared with PBT findings. METHODS: Hepatofunctional evaluations were conducted in 10 postoperative BA children with moderate (group B; n = 4) and severe (group A; n = 6) liver dysfunction, and the PBT results were compared with those of 13 normal healthy children (group C). Subjects were orally given single-bolus (13)C-phenylalanine at 3.5 mg/kg (maximum dosing: 100 mg) in the morning. Time-related exhaled gas was periodically collected until 120 min after dosing. The (13)CO(2) levels were monitored with gas chromatography-mass spectrometry before and after administration, and the (13)C excretion rate, (13)C cumulative excretion and time of maximum (13)C excretion rate were monitored accordingly. RESULTS: Total bile acid, hyaluronic acid, type IV collagen 7S, total bilirubin or albumin and the PBT findings were significantly correlated. The PBT findings in group A were significantly lower those of group B, indicating that phenylalanine metabolism was markedly attenuated in the former. CONCLUSION: The PBT values correlated well with liver fibrosis in postoperative BA children. Because PBT is a non-invasive approach, results from this method may serve as a useful and reliable index for post-surgical monitoring of children operated on for liver fibrosis.     

Effects of Silodosin on Lower Urinary Tract Symptoms in Patients with Benign Prostatic Hyperplasia: Evaluation by Frequency/Volume Chart

Objectives: To prospectively evaluate the efficacy of silodosin, a new α_1A‐adrenoceptor selective antagonist, for the treatment of lower urinary tract symptoms suggestive of benign prostatic hyperplasia (LUTS/BPH) on the basis of a frequency/volume chart. Methods: Forty male patients (71.1 ± 6.6 years old) with LUTS/BPH were treated with silodosin (4 mg twice daily). The effects of the drug were assessed by changes in International Prostate Symptom Score, uroflowmetry, and frequency/volume chart at 1 and 3 months after therapy. Results: The mean total International Prostate Symptom Score, the mean total storage and voiding scores and the mean quality of life score decreased significantly at 1 and 3 months after therapy (all P < 0.01). Average and maximum flow rates increased significantly, and postvoid residual volume decreased significantly after 1 and 3 months (all P < 0.05). The frequency/volume chart showed that daytime frequency in those who initially voided over eight times/day (n = 12) decreased significantly (P = 0.0391) after 1 month, and nighttime frequency in those who initially voided over two times (n = 16) tended to decrease (P = 0.0833) after 3 months. Mean voided volume in those who initially voided less than 250 mL (n = 31) increased significantly after 1 and 3 months (P = 0.0446 and P = 0.0138, respectively), and maximum voided volume in those who initially voided less than 300 mL (n = 18) tended to increase (P = 0.0833) after 1 month. Conclusion: Silodosin appears to be effective for both storage and voiding symptoms by increasing bladder capacity in patients with LUTS/BPH.     

Teratogenicity of Azosemide, a Loop Diuretic, in Rats, Mice and Rabbits

Teratogenic effects of azosemide, a loop diuretic, were investigated in rats, mice and rabbits. Azosemide was given orally to pregnant rats, mice and rabbits during organogenesis. The pregnant animals were killed at term and their fetuses were examined for external, visceral and skeletal abnormalities. In rats, azosemide at 10–30 mg/kg/day did not affect intrauterine growth, resorptions and rates of external and visceral malformations. Treatment with 90 mg/kg/day resulted in a significant increase in skeletal abnormalities such as wavy ribs, bent scapula and bent humerus. However, the skeletal abnormalities observed in term fetuses could not be found in adult offspring, indicating that they were temporary. In mice, 1250 mg/kg/day of azosemide caused maternal death, abortion, and retarded maternal and fetal weight. Treatment with 200–500 mg/kg/day did not induce fetal mortalities, external and visceral malformations. Skeletal abnormalities increased in dose-dependent fashion. The type of abnormalities was identical to that encountered in rat fetuses. Furosemide as a positive control also produced similar types of skeletal abnormalities in mouse fetuses. In rabbits, azosemide did not have embryolethal or teratogenic effects even at the highest dose (6 mg/kg/ day), which caused maternal death. Treatment for a different 3-day period and then a different day during organogenesis in rats and mice showed that the sensitive period was days 15–17 of gestation with a peak on day 16 in rats, and days 12–15 with a peak on day 13 in mice.     

Factors affecting the efficiency of peripheral blood stem cell collection in children treated with chemotherapy and G-CSF

This retrospective study attempts to clarify the optimal timing for peripheral blood stem cell (PBSC) collection after conventional chemotherapy followed by granulocyte-colony stimulating factor (G-CSF) administration. Leukapheresis was performed 32 times in nine children with various cancers during bone marrow recovery phase following transient pancytopenia after chemotherapy. (On two occasions, leukapheresis was excluded because many leukemic blasts were included.) When the number of white blood cells (WBC) exceeded 1.8 times 10¹⁰/L after administration of G-CSF (200 μg/m², continuous infusion), many more CD34⁺ cells were contained in the collected peripheral mononuclear cells (P > 0.02) and a sufficient number of PBSC for transplantation (≥ 10 times 10⁸ CD34⁺ cells/kg) was obtained after one run in 15 of 17 leukapheresis sessions. In contrast, sufficient PBSC were obtained only in one of 13 runs of leukapheresis when the number of WBC was < 1.8 times 10¹⁰/L. The number of WBC on the day when PBSC were collected correlated with collected nuclear cell number (r = 0.60), but not with the CD34⁺ cell ratio. The ratio was higher only when both platelets and reticulocytes increased in parallel with WBC. We conclude that sufficient PBSC collection is possible after conventional chemotherapy using G-CSF, when hematopoietic recovery is parallel, without the use of high-dose chemotherapy.     

Electrolyte Excretion in 12-hour Urine and in Spot Urine

ABSTRACT. Plasma renin activity (PRA) and urinary electrolyte excretion were measured in 137 healthy children aged 6 to 14 years. Plasma aldosterone concentration (PAC) was measured in 52 of the children. Nocturnal 12-hour urine was collected in 110 of the children. Spot urine was collected on two occasions, once just before lying down, once after 90-min supine rest in another 27. Na/K ratio and fractional Na excretion rate (FENa) in 12-hour urine showed a significant inverse correlation with PRA or PAC. Na excretion (mmol/min, mmol/mmol creatinine), Na/K ratio and FENa in the spot urine following 90 min in a supine position showed a significant inverse correlation with PRA or PAC, but they failed to show a significant relationship to PRA or PAC in the spot urine preceding supine rest. A spot urine after 90 min in the supine position is collected easily and hence most appropriate to study the relationship between Na excretion and PRA or PAC clinically.     

Effects of Different Absorption Enhancers on the Permeation of Ebiratide,an ACTH Analogue,across Intestinal Membranes

The permeation of ebiratide (H-Met(O₂)-Glu-His-Phe-d -Lys-Phe-NH(CH₂)₈NH₂), a novel ACTH analogue, across the intestinal mucosae has been examined by use of isolated intestinal membranes from rats in a modified Ussing chamber. Regional differences were observed in the permeation of ebiratide across intestinal membranes; the order of membrane permeability was jejunum > ileum > duodenum > colon. Overall, the permeation of ebiratide was relatively poor. The effects of various absorption enhancers were examined to increase the intestinal permeability to ebiratide. Sodium glycocholate and sodium caprate had no significant enhancing effect on the permeability of the jejunal membrane, but significantly enhanced the permeation of ebiratide through the colonic membrane. On the other hand, N-dodecyl-β-d -maltopyramoside (LM) significantly enhanced the permeation of ebiratide through both jejunal and colonic membranes. In general, the absorption-enhancing effects of these agents were more predominant in the colon than in the jejunum. Membrane damage by the absorption enhancers was evaluated by measuring the amount of protein released from the intestinal membrane. It was found that all the absorption enhancers slightly increased the amount of protein released, but that the amounts of protein released in the presence of these enhancers were much less than in the presence of ethylenediaminetetraacetic acid (EDTA), used as a positive control. These findings suggest that the absorption enhancers, especially LM might be useful adjuvants for improving the intestinal absorption of peptide and protein drugs, including ebiratide.     

Effect of fentanyl on awakening concentration of sevoflurane

This study was designed to determine if fentanyl altered MAC-awake(the end-tidal concentration of sevoflurane associated witheye opening to verbal command) in 30 healthy, ASA I patients.During anaesthesia, no other anaesthetics or drugs were givenwith the exception of sevoflurane. After surgery, end-tidalanaesthetic concentration was maintained constant for at least15 min. If patients failed to respond to command, the end-tidalconcentration was decreased and again maintained constant for15 min. The anaesthetic concentration midway between the valuepermitting the response and that just preventing the responsewas recorded as MAC-awake. Fentanyl was administered at predictedplasma concentrations of 1 and 2 ng mg^–1 using a computer-controlledcontinuous infusion and plasma concentrations of fentanyl weremeasured at the time of MAC-awake measurements. MAC-awake ofthe control group in which fentanyl was not administered wasmean 0.67 (SD 0.12)% or 0.36 (0.03) MAC, being significantlyhigher than that of the fentanyl 2-ng ml^–1 group (0.57(0.09)% or 0.30 (0.04) MAC). In the fentanyl 1 -ng ml^–1group, MAC-awake (0.65 (0.10)% or 0.34 (0.05) MAC) did not differfrom that in the control group. Logistic regression analysisshowed that increasing plasma concentration of fentanyl andincreasing age significantly reduced the MAC-awake of sevoflurane.Because the reduction was very small relative to the overallscatter of the MAC-awake, a low plasma concentration of fentanyldid not significantly reduce the MAC-awake of sevoflurane.     

Melatonin treatment for circadian rhythm sleep disorders

Abstract We administered 1–3 mg melatonin to 11 patients (eight men, three women, aged 16–46 years) with circadian rhythm sleep disorders; nine with delayed sleep phase syndrome and two with non-24-hour sleep-wake syndrome. Sleep logs were recorded throughout the study periods and actigraph and rectal temperature were monitored during treatment periods. Melatonin was administered 1–2 h before the desirable bedtime for expected phase-shifting, or 0.5-1 h before habitual bedtime for gradual advance expecting an hypnotic effect of the melatonin. Melatonin treatments were successful in 6/11 patients. Timing and dose of melatonin administration, together with its pharmacological properties for circadian rhythm sleep disorders, should be further studied.