Gallstones in acromegalic patients undergoing different treatment regimens

Summary The frequency of gallstones during longterm treatment with the somatostatin analogue octreotide reported in different studies varies from 0% to 50%, the reason for this variation being unknown. Therefore, we examined 58 acromegalic patients undergoing different treatment regimens for the frequency of gallstones. Thirteen were treated with octreotide, 20 with bromocriptine, and 25 had no medical treatment after successful neurosurgery. Also, 58 patients without known gallbladder disease served as controls. The postprandial gallbladder contraction was also investigated in 27 acromegalic patients (10 with octreotide, 10 with bromocriptine, and 7 with no medical therapy). Ten of the 58 acromegalic patients were found to have gallstones, 4 of 25 receiving no medical treatment, 4 of 20 treated with dopamine agonists, and 2 of 13 treated with octreotide. In 9 of the 58 control patients, gallstones were detected. Although in the octreotide group the gallstones were newly formed under therapy, there was no difference in gallstone prevalence between the different treatment regimens and the control group. However, the postprandial gallbladder contraction was significantly more often inhibited during octreotide therapy, and this effect was most pronounced during the first hours following injection. Differences in the timing of injections therefore may be an explanation of the variable incidence of cholelithiasis in the different studies.Abbreviations SD standard deviation - Sm-C somatomedinC     

Long-term treatment of acromegaly with the somatostatin analogue SMS 201-995 over 6 months

Summary This study examined the effects of the long-acting selective mini somatostatin analogue (SMS) 201-995 in two acromegalic patients who were treated for 3 and 6 months, respectively. During treatment the mean growth hormone levels (25.3 and 20.8 ng/ml vs 5.9 and 10.6 ng/ml) and somatomedin C levels (6.2 and 6.2 IU/ml vs 3.3 and 3.8 IU/ml) decreased and the patients reported an improvement in their symptoms. The main side effect was an increase in stool fat excretion which did exceed the normal range (<7 g/day) in one patient. Five acromegalics who received 2 × 50 µg SMS 201-995/day for 5 days showed a significant increase of stool fat excretion (1.7 vs 3.5 g/day;p<0.05). Fasting blood glucose levels, glucose tolerance, and glycosylated hemoglobin were not essentially effected. It is concluded that SMS 201-995 offers new possibilities in the treatment of acromegaly. The gastrointestinal and diabetogenic side effects of this substance, however, should be carefully monitored.Abbreviations GH growth hormone - HbA₁ glycosylated hemoglobin - OGTT oral glucose tolerance test - SMS selective mini somatostatin - TRH thyrotropin-releasing hormone - TSH thyroid-stimulating hormone     

Early treatment of Menkes disease with parenteral Cooper-Histidine: Long-term follow-up of four treated patients

We report on the long-term clinical course of 4 boys with Menkes disease, treated from early infancy with parenteral copper-histidine, with follow-up over 10–20 years. Three of the 4 had male relatives with a severe clinical course compatible with classical Menkes disease. As a consequence of early treatment, our patients have normal or near-normal intellectual development, but have developed many of the more severe somatic abnormalities of the related disorder, occipital horn syndrome, including severe orthostatic hypotension in 2. In addition, 1 boy developed a previously unreported anomaly, namely, massive splenomegaly and hypersplenism as a consequence of a splenic artery aneurysm. Previously reported molecular studies in 2 of these patients had shown gene defects which would have predicted a truncated and probably nonfunctional gene product. Despite the favorable effects on the neurological symptoms, parenteral copper treatment for Menkes disease should still be regarded as experimental. The development of more effective treatments must await a more precise delineation of the role which the Menkes protein plays in intracellular copper trafficking. Am. J. Med. Genet. 76:154–164, 1998. © 1998 Wiley-Liss, Inc.     

腹腔镜诊治不孕症患者盆腔病变临床分析

目的探讨应用腹腔镜手术诊治不孕症的临床价值。方法对126例女性不孕症患者行腹腔镜诊断和治疗,观察术后妊娠率。结果 126例中发现盆腔病变117例,占92.86%;慢性盆腔炎是不孕症的第1位病因;其次为子宫内膜异位症、多囊卵巢综合征。腹腔镜手术术后妊娠率为47.50%,1例发生异位妊娠。结论腹腔镜手术对女性不孕症盆腔病变的诊治具有重要的价值。     

Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients

Ornithine transcarbamylase deficiency is a very heterogeneous urea cycle disorder resulting in hyperammonemia with various presentations from the neonatal period through adulthood. We performed a retrospective study in nine patients (four male/five female, age at diagnosis ranging from 6 days to 14 years) to evaluate the safety and efficacy of sodium phenylbutyrate (Ammonaps) in long-term treatment. All patients were diagnosed by DNA mutational analysis and/or liver enzyme measurement. They had previously been treated with sodium benzoate (median dose 248 mg/kg/day; range 106-275) and low protein diet (median 0.84 g/kg/day) and were switched to sodium phenylbutyrate (median dose of 352 mg/kg/day) at 8.9 and 4.9 years of age (median) in males and females, respectively. We analyzed clinical and biochemical data and the median follow-up duration was 26 months. During that time, there were no hyperammonemic episodes requiring hospitalization. Median plasma ammonia and glutamine levels were 30 and 902 micromol/L, respectively. Total protein intake could be increased to 0.95 g/kg/day after 18 months. No side effects related to therapy were observed. Further prospective studies should be performed to define the optimal dosage of sodium phenylbutyrate and the requirements for protein diet at different ages.     

甲亢孕妇妊娠结局的临床分析

目的探讨妊娠合并甲亢规范化治疗对妊娠结局的影响。方法选取四年间于我院建档并分娩、资料完整的妊娠合并甲亢患者77例,对其妊娠结局进行临床分析。结果甲亢未治疗组甲状腺激素水平明显增高,妊娠并发症增多,与治疗组相比,差异有显著性（P〈0.01）;未治疗组新生儿体重明显低于治疗组,差异有显著性（P〈0.05）;但两组的剖宫产率及新生儿甲状腺功能差异无统计学意义（P〉0.05）。结论及时诊断并规范治疗妊娠甲亢,可降低妊娠并发症发生率,改善妊娠结局。     

Long-term treatment of patients with mild and classical phenylketonuria by tetrahydrobiopterin

Tetrahydrobiopterin (BH4), the natural cofactor of phenylalanine hydroxylase (EC 1.14.16.1), can reduce blood phenylalanine (Phe) in BH4 sensitive patients with hyperphenylalaninemia (McKuisick 261600). We report on the long-term treatment of eight patients with mild and classical phenylketonuria (blood Phe levels maximum blood Phe levels between 771 and 1500 micromol/L) using BH4 at a dosage of 8-12 mg/kg BW per day. In all patients reduction of blood Phe was >30% after BH4 loading test. Three patients were treated from birth by BH4 only, five after initial low Phe dietary treatment. Seven of them continue to be on BH4 treatment only, one has a relaxed low protein diet. No side effects could be observed (longest observation time 5 years), somatic and psychomotor development were normal. The main problem of BH4 treatment is finding an optimal dosage at different ages and an under special conditions like infectious diseases. There is evidence that in some patients BH4 treatment may allow a more relaxed low protein diet showing positive effects on weight gain and quality of life. Further controlled studies are necessary not only to rule out any side effects but also for optimizing treatment strategies with BH4 treatment in mild phenylketonuria.     

Long-term treatment with aspirin desensitization in asthmatic patients with aspirin-exacerbated respiratory disease

BACKGROUND: Aspirin desensitization treatment is an option to decrease disease activity and reduce the need for systemic corticosteroids in patients with aspirin-exacerbated respiratory disease (AERD). OBJECTIVE: This study was designed to determine whether the clinical courses of patients with AERD improved as early as 6 months after starting aspirin desensitization and to compare this with follow-up evaluations after at least a year. METHODS: Between 1995 and 2000, 172 patients with AERD were admitted to our General Clinical Research Center, were desensitized to and treated with aspirin, were discharged to their home communities, and participated in follow-up interviews and written assessments of their clinical courses. RESULTS: By the first 6 months of aspirin treatment, there were significant reductions in sinus infections and numbers of short courses of prednisone and improvements in sense of smell and general assessment of nasal-sinus and asthma symptoms (P <.0001). These results persisted for 1 to 5 years (P <.0001). Mean prednisone doses decreased from 10.8 mg/d to 8.1 and 3.6 mg/d at 6 months and greater than 1 year, respectively. Of the 172 patients, 24 (14%) discontinued aspirin treatment because of side effects, and 115 (67%) responded to aspirin treatment. After eliminating those who discontinued aspirin treatment because of side effects, the improvement rate was 115 (78%) of 148 patients. Of the 126 patients who completed a year or more of aspirin treatment, 110 (87%) experienced improvement. CONCLUSION: Aspirin desensitization followed by daily aspirin is efficacious by at least the first 6 months of treatment and continues to be effective for up to 5 years of follow-up.     

妊娠期烧伤护理与治疗

妊娠期烧伤是临床上罕见但伤害极大的损伤。妊娠期孕妇由于胎儿的存在,身体会发生许多特殊的生理变化,烧伤的发生会进一步加重孕妇的身体负担,危及孕妇及胎儿的健康,因此妊娠期烧伤患者需要特殊的护理。本文对国内外相关的报道进行整理,并结合北京积水潭医院对此类患者的治疗经验,对妊娠合并烧伤的临床特点进行了总结,并提出了系统的护理与治疗原则。     

Assessment of fragmented QRS formation and its relationship with left ventricular hypertrophy in nonhypertensive acromegaly patients

Background/aim It is known that the presence of fragmented QRS (fQRS) on electrocardiography (ECG) is associated with cardiovascular events. The aim of this study was the evaluation of fQRS formation and its relationship with the left ventricular hypertrophy (LVH) parameters in acromegaly patients. Materials and methods In total, 47 previously diagnosed with non-hypertensive acromegaly patients and 48 control subjects were included in the study. ECG and transthoracic echocardiography (TTE) were performed for each participant. Acromegaly patients were divided into two groups according to the fQRS formation on the ECG. Left ventricular wall thicknesses, and left atrial diameter (LAD), left ventricular mass (LVM), left ventricular mass index (LVMi), and relative wall thickness (RWT) were obtained. Results In control group 5 (10.4%) and in acromegaly group 17 (36.2%) patients had fQRS on ECG (p = 0.003). LAD [36.0 (34.0–38.0) vs. 38.0 (35.0–41.0) mm, p < 0.001], LVM [155.27 ± 27.00 vs. 173.0 (153.0–235.0) g, p < 0.001], LVMi [83.12 ± 13.19 vs. 92.0 (83.0–118.0) g/m², p < 0.001] and RWT [0.39 ± 0.03 vs. 0.43 (0.41–0.45), p = 0.001] were significantly higher in patients with acromegaly. Disease duration was significantly higher (11.59 ± 1.3 vs. 8.2 ± 1.8 years, p < 0.001) in the fQRS (+) group. LAD [41.0 (39.0–42.5) vs. 37.0 (34.7–38.0) mm, p < 0.001], LVM [219.0 (160.5–254.5) vs. 164.0 (153.0–188.0) g, p = 0.017], LVMi [117.0 (92.5–128.5) vs. 86.0 (82.0–100.2) g/m², p = 0.013] and RWT [0.44 (0.42–0.49) vs. 0.43 (0.40–0.44), p = 0.037] were significantly higher in fQSR (+) acromegaly patients. In multivariate logistic regression analysis, disease duration (odds ratio: 10.05, 95% CI: 1.099–92.012, p = 0.041) and LAD (odds ratio: 2.19, 95% CI: 1.030–4.660, p = 0.042) were found to be the independent predictors of fQRS formation.Conclusion The results of our study revealed that fQRS (+) acromegaly patients had increased LVH parameters compared to fQRS (-) patients.     

Correlations between forced oscillation technique parameters and pulmonary densitovolumetry values in patients with acromegaly

The aims of this study were to evaluate the forced oscillation technique (FOT) andpulmonary densitovolumetry in acromegalic patients and to examine the correlationsbetween these findings. In this cross-sectional study, 29 non-smoking acromegalicpatients and 17 paired controls were subjected to the FOT and quantification of lungvolume using multidetector computed tomography (Q-MDCT). Compared with the controls,the acromegalic patients had a higher value for resonance frequency [15.3 (10.9-19.7)vs 11.4 (9.05-17.6) Hz, P=0.023] and a lower value for meanreactance [0.32 (0.21-0.64) vs 0.49 (0.34-0.96) cmH₂O/L/s², P=0.005]. In inspiratory Q-MDCT, the acromegalicpatients had higher percentages of total lung volume (TLV) for nonaerated and poorlyaerated areas [0.42% (0.30-0.51%) vs 0.25% (0.20-0.32%), P=0.039 and3.25% (2.48-3.46%) vs 1.70% (1.45-2.15%), P=0.001, respectively].Furthermore, the acromegalic patients had higher values for total lung mass in bothinspiratory and expiratory Q-MDCT [821 (635-923) vs 696 (599-769) g,P=0.021 and 844 (650-945) vs 637 (536-736) g, P=0.009,respectively]. In inspiratory Q-MDCT, TLV showed significant correlations with allFOT parameters. The TLV of hyperaerated areas showed significant correlations withintercept resistance (r_s=−0.602, P<0.001) and mean resistance(r_s=−0.580, P<0.001). These data showed that acromegalic patientshave increased amounts of lung tissue as well as nonaerated and poorly aerated areas.Functionally, there was a loss of homogeneity of the respiratory system. Moreover,there were correlations between the structural and functional findings of therespiratory system, consistent with the pathophysiology of the disease.     

Long-term triamcinolone acetonide aerosol treatment in adult patients with chronic bronchial asthma

Eighty percent of 26 steroid-dependent, chronically asthmatic adults eliminated oral steroid dosage after 12 weeks of treatment with triamcinolone acetonide aerosol (800 micrograms/day). After one year of treatment 60% of the patient were free of oral steroids. Pulmonary function (FEV1, FVC and FEF25-75%) and plasma cortisol test results showed significant improvement during treatment. Adverse effects were mild to moderate.     

Long-term follow-up of patients with candiduria

Candiduria is commonly encountered in hospitalized patients, particularly those with indwelling urinary catheters. While risk factors and therapy are well described in previous studies, little is known about long-term outcomes and recurrence rates of candiduria. We studied 188 patients with candiduria in a retrospective chart review at a single institution from January 1999 to December 2000. Data were collected regarding risk factors and underlying disease, therapy, follow-up cultures until December 2003, and mortality. Ninety-one patients with at least one follow-up culture >1 month after the initial culture (range 2–48) were available for further study. In this group, patients receiving antifungal therapy for asymptomatic candiduria were paradoxically more likely to have subsequent positive urine cultures than patients who never received antifungal therapy. Six patients developed candidemia during follow-up, although in none was this considered to represent a consequence of candiduria. Mortality rate at the end of the follow-up period (mean of 18 months) was 43%, including one death attributed to candidemia. Therapy for candiduria does not appear to reduce candiduria recurrence rates through 48 months of follow-up and little evidence of treatment benefit was identified.     

Long-term treatment with oral sustained-release theophylline

The treatment of chronic asthma with a sustained-release rheophylline preparation was evaluated in a placebo controlled, 2 × 4 week, randomised, double-blind study. The dose which would give serum theophylline levels in the range of 10–20 meg/ml had been previously determined for each patient. This individualization of dosage resulted in 80% of the patients having scrum content rations within the 10–20 meg/ml therapeutic range. The average daily close taken by males was 998.55 mg (range 700–1175 mg) and by females 778.1 (range 500–1000 mg).
Of the 33 patients who completed the study 28 had less asthma during the theophylline period, lour did not respond and one did better during the placebo period. No serious side effects were reported. During theophylline therapy. patient's requirements for aerosol bronchodilators were significantly reduced.     

体外受精-胚胎移植治疗PCOS不孕分析

目的评价体外受精-胚胎移植（IVF-ET）治疗多囊卵巢综合征（PCOS）伴不孕的疗效。方法对2002年5月至2006年4月在本中心行IVF-ET或ICSI的PCOS患者资料回顾分析。A组为PCOS患者29周期,B组（对照组）为同期输卵管阻塞性不孕患者155周期。结果A组获卵数、胚胎数显著多于B组（P〈0.01）,OHSS发生率和移植取消率显著高于B组（P〈0.05）;A组年龄、促排卵药总量、优质胚胎率显著低于B组（P〈0.01）;两组不孕年限、HCG日E2水平、刺激时间、受精率、妊娠率、移植日内膜厚度均无显著差异。结论PCOS患者行IVF/ICSI-ET治疗时Gn用量少,获卵数多,胚胎数多,但优质胚胎率低;OHSS发生高危,取消移植率高;临床妊娠率、受精率与输卵管阻塞性不孕患者无显著差异。对于PCOS患者,IVF/ICSI-ET可以有效助孕。     

Long-term pharmacologic treatment of women with hypertension

As reviewed in “Hypertension in Women: What is Really Known?” (Women's Caucus Working Group on Women's Health of the Society of General Internal Medicine [1991] Ann Intern Med 115:287–293), the published literature reveals that hypertension frequently occurs in women and that only some prospective studies have defined specific benefits and risks of long-term pharmacologic treatment. Major U.S. epidemiologic studies show the incidence of hypertension to very between 20% and 50% of the population. It is more common among Afro-Americans than White Americans. Frequency increases with aging. Several studies of at least 3 years duration that have focused on the use of diuretics and B blockers are reviewed: Hypertension Detection and Follow-Up Program (HDFP), Medical Research Council (MRC), MRC trial in the treatment of older adults (MRC-Older), and isolated systolic hypertension in the elderly (SHEP). Both diuretics and B blockers generally lower blood pressure, and decrease stroke incidence and mortality. Diuretics seem to be more effective and better tolerated than B blockers. Long-term studies of newer antihypertensive agents do not yet exist. The sexual side effects of drugs have only been studied to a limited degree in women. © 1995 Wiley-Liss, Inc.