腹腔镜和开放去顶减压术治疗多囊肾的临床效果比较

目的:探讨腹腔镜去顶减压术治疗常染色体显性遗传性多囊肾(ADPKD)的临床价值。方法:开放手术68例,分期行双肾去顶减压术;腹腔镜手术54例,一期行双肾去顶减压术。观察两组患者在囊肿处理的数量以及手术效果方面的差异。结果:开放手术组单侧肾脏囊肿处理数量为(268±93)个,术后平均住院7.9d;腹腔镜手术组单侧肾脏囊肿处理数量(197±76)个,术后平均住院5.1d。开放手术组1例发生切口感染,腹腔镜手术组1例发生尿外渗。术后两组患者疼痛明显改善,部分患者的高血压缓解,肾功能无明显变化。随访3年患者疼痛与高血压有复发。组间比较显示,两组患者的治疗效果差异无显著性(P>0.05)。结论:腹腔镜去顶减压术手术创伤小并取得了与开放手术相同的治疗效果。     

Reduction of cyst volume for symptomatic management of autosomal dominant polycystic kidney disease

A total of 11 patients with refractory pain secondary to autosomal dominant polycystic kidney disease underwent ultrasound guided percutaneous aspiration of cyst fluid on the affected side. Surgical reduction of cyst volume was performed if pain recurred. Dramatic relief of pain was observed after both procedures. The probability of a patient being free of renal pain at 18 months was 33 +/- 17 per cent for aspiration and 81 +/- 12 per cent for an operation. Individual patients had relief of pain for more than 4 years. There was no deleterious effect on renal function after either aspiration or an operation. Blood pressure improved in the 5 patients with hypertension. There were no complications of percutaneous cyst aspiration. One patient required neurolysis of the drain site after cyst reduction.     

Laparoscopic nephrectomy for autosomal dominant polycystic kidney disease

Background The authors reviewed their experience with laparoscopic nephrectomy for autosomal dominant polycystic kidney disease to evaluate whether patient-related or surgery-related factors influence operative outcomes.Methods A retrospective review was carried out of 22 consecutive laparoscopic nephrectomies performed by one surgeon in a university setting between March 1998 and March 2003. The impact of patient factors (body mass index, preoperative hemoglobin level, preoperative blood urea nitrogen and creatinine, kidney size and side, prior abdominal surgery, dialysis) and surgical factors (surgeon experience and preoperative embolization) on short-term outcomes (estimated blood loss, transfusion requirements, operative time, conversion, intra- and postoperative complications and length of stay) was analyzed using the Students t-test, Pearson correlation, and Mann–Whitney and Fisher tests.Results A total of 19 patients underwent 22 nephrectomies. The average patient age was 49 years (range, 36–65 years) and the average body mass index was 31.4 kg/m² (range, 20.4–64.5 kg/m²). Fourteen patients (68%) were receiving dialysis. Fifteen right (68%) and 7 left (32%) nephrectomies were performed. The median kidney size was 22 cm (range, 8–50 cm). Five patients (23%) had preoperative embolization. The median operative time was 255 min (range, 95–415 min). There were no mortalities. The intraoperative complication rate was 18% (1 vena cava laceration, 1 cecal perforation, 1 dialysis fistula thrombosis, 1 intrarenal bleeding requiring conversion), and the postoperative complication rate was 32% (1 myocardial infarction, 1 urgent laparotomy for clinical peritonitis, 1 minor bile fistula, 1 AV fistula thrombosis, 2 incisional hernias, 1 urinary retention). Four procedures (18%) were converted (1 for vena cava laceration, 1 for cecal perforation, 1 for intrarenal bleeding, 1 for adhesions). The median blood loss was 400 ml (range, 100–5000 ml). Eight patients (36%) received transfusions (median, 2 units). The median length of stay was 4 days. The patients who required blood transfusions had lower preoperative hemoglobin levels. Preoperative embolization did not affect surgical outcome. However, surgeon experience significantly reduced operative time.Conclusions Laparoscopic nephrectomy for autosomal dominant polycystic kidney disease is a safe procedure, providing patients with a short hospital stay. Complication and conversion rates are relatively high.Presented at the 11th International Congress of the European Association for Endoscopic Surgery and other Interventional Techniques (EAES), Glasgow, 15–18 June 2003     

Laparoscopic cyst decortication in autosomal dominant polycystic kidney disease: impact on pain,hypertension, and renal function

BACKGROUND AND PURPOSE: In patients with autosomal dominant polycystic kidney disease (ADPKD), laparoscopic cyst decortication (LCD) has been proposed as a means to relieve chronic cyst-related pain. We present our 7-year experience with LCD for ADPKD with regard to pain relief, hypertension, and renal function. PATIENTS AND METHODS: Between August 1994 and February 2001, 29 ADPKD patients with chronic pain (N=29), hypertension (N=21), and renal insufficiency (N=10) underwent 35 LCD procedures. Every detectable cyst within 2 mm of the renal surface was treated. Pain relief was assessed using a pain analog scale; relative pain relief (RPR) equaled (preoperative pain score) - (postoperative pain score)/(preoperative pain score). Hypertension was evaluated using the antihypertensive therapeutic index (ATI): [(dose of blood pressure medication 1/max dose 1) + (dose med 2/max dose 2) + etc.] x 10. Renal function was assessed using the Cockcroft and Gault formula for creatinine clearance. RESULTS: The mean operating room time was 4.9 hours (range 2.6-6.6 hours) with no conversions to open surgery. An average of 220 cysts (range 4-692) were treated per patient. The mean follow-up was 32.3 months (range 6-72 months). The RPR was 58%, 47%, and 63% at 12, 24, and 36 months, respectively. At 12, 24, and 36 months, 73%, 52%, and 81% of patients, respectively, noted >50% improvement in their pain compared with the preoperative situation. Five patients became normotensive, and patients improved their ATI by an average of 49% (range 11%-93%). However, six patients had worsening hypertension, with an ATI increase averaging 53% (range 11%-122%), and one patient who was not hypertensive preoperatively has since developed hypertension. The creatinine clearance changed +4%, +7%, and -2% at 12, 24, and 36 months, respectively. Only one patient had a >20% increase in creatinine clearance. The only patients with a >20% decrease in creatinine clearance were those who had a creatinine clearance <30 mg/dL preoperatively (average decrease 34% [range 20%-51%]). CONCLUSIONS: For ADPKD patients with debilitating pain, extensive LCD can provide durable relief. In the majority of patients with pain and hypertension, a marked improvement in blood pressure also occurs. Cyst decortication was not associated with worsening renal function; indeed, renal function remained largely unchanged over the 3-year follow-up period.     

后腹腔镜下介入超声辅助定位治疗成人多囊肾的临床研究

目的：探讨后腹腔镜下应用介入超声辅助定位进行囊肿去顶术治疗成人多囊肾的临床价值。方法：2005年6月～2007年1月对13例成人多囊肾患者采用后腹腔镜进行双侧囊肿去顶减压术（非超声组）,其中6例成人多囊肾患者采用介入超声辅助定位（超声组）。并对两组患者的手术时间、出血量、手术并发症以及术后血压、肾功能等情况进行分析。结果：超声组和非超声组的手术时间分别为3．57±0．51h和3．28±0．68h。术中出血量分别为115±28ml和108±34ml。差异无统计学意义。超声组患者表浅的囊肿和肾实质内直径大于1cm的囊肿均得到有效清除,术后未出现尿外渗等并发症。非超声组7例患者肾脏表浅囊肿得到有效清除,但其中5例肾实质内仍有直径大于3cm的深部囊肿残存,术后2例患者出现尿外渗。所有患者疼痛症状明显缓解,贫血及高血压等得到不同程度的控制。肾功能稳定。结论：后腹腔镜双侧囊肿去顶减压术是一种微创且安全有效的多囊肾治疗方法,应用介入超声辅助定位能够更加彻底地对肾脏囊肿,尤其是深部囊肿进行处理,并且可降低集合系统损伤,从而防止尿外渗等并发症的发生。     

后腹腔镜肾囊肿去顶术治疗成人多囊肾

目的 评估后腹腔镜去顶术治疗常染色体显性遗传性多囊肾(ADPKD)的临床价值.方法 采用后腹腔镜囊肿去顶减压术治疗多囊肾患者83例.结果 83例手术成功.术后平均住院5 d,1例发生尿外渗,术后患者疼痛明显改善,部分患者的高血压缓解,发生肾功能不全的患者术后均有不同程度肾功能改善.结论 腹膜后腹腔镜囊肿去顶术具有创伤小、患者恢复快、操作简便等优点,应作为首选术式.     

后腹腔镜和开放去顶减压术治疗多囊肾的疗效比较

目的：通过与传统开放肾囊肿去顶减压术治疗常染色体显性遗传性多囊肾病（ADPKD）的效果比较,评价后腹腔镜肾囊肿去顶减压术治疗ADPKD的临床价值。方法：回顾性分析后腹腔镜肾囊肿去顶减压术治疗ADPKD25例（A组）及开放肾囊肿去顶减压术29例（B组）的临床资料．就两组手术时间、术中出血量、术后肠道功能恢复时间、术后止疼药用量、术后住院天数、并发症和临床疗效等指标进行比较,根据数据类型选用X^2检验、成组t检验或Mann—Whitey U检验。结果：A组在术中出血量、术后肠道功能恢复时间、术后止疼药用量、术后住院天数方面优于B组,差异有统计学意义（P〈0．01）;并发症和临床疗效等指标与B组相当,差异无统计学意义（P〉0．05）。结论：后腹腔镜肾囊肿去顶减压术是治疗ADPKD微创、安全、有效的疗法。     

Renal cyst infection in autosomal dominant polycystic kidney disease.

A 56-year-old man with a medical history of autosomal dominantpolycystic kidney disease (ADPKD) and right-sided cadaverickidney transplantation with homolateral nephrectomy in 1999was admitted in June 2006 with fever and chills. On physicalexamination, his temperature was 38.6°C and he showed anenlarged and slightly painful left     

Laparoscopic bilateral synchronous nephrectomy for autosomal dominant polycystic kidney disease: the initial experience

PURPOSE: We report our experience with laparoscopic bilateral synchronous nephrectomy for giant symptomatic autosomal dominant polycystic kidney disease (ADPKD) and compare outcome data with open bilateral nephrectomy. MATERIALS AND METHODS: Since March 1998, 10 patients underwent bilateral synchronous laparoscopic nephrectomy for giant symptomatic ADPKD. A 3 port retroperitoneal laparoscopic approach was used to secure the renal hilum and mobilize the kidney. Intact specimen extraction was performed through a midline infraumbilical extraperitoneal incision. The patient was then repositioned for the contralateral retroperitoneoscopic nephrectomy, with the second specimen also delivered through the same infraumbilical incision. Data were retrospectively compared with 10 patients who had undergone bilateral synchronous open nephrectomy for ADPKD between 1981 and 1992. RESULTS: Patients in the laparoscopic and open groups were comparable in regard to age (53 versus 47 years, p = 0.54) and Anesthesiologist Society of America class (3 versus 3, p = 0.84) but patients in the laparoscopic group were significantly more obese (body mass index 35.9 versus 23.8, p = 0.02). For comparable total specimen weights (3 versus 3 kg, p = 0.69) surgical time was longer in the laparoscopic group (4.4 versus 3.8 hours, p = 0.007). However, the laparoscopic group was superior in regard to blood loss (150 versus 325 cc, p = 0.05), postoperative requirement of nasogastric tube (10% versus 100%, p = 0.0001), narcotic analgesics (34.2 versus 120.4 mg. morphine sulfate equivalent, p = 0.03) and hospital stay (1.5 versus 9 days, p = 0.004). Complications occurred in 5 patients (50%) in the laparoscopic group and 4 (40%) in the open group (p = 0.66). No laparoscopic case was converted to open surgery. CONCLUSIONS: Synchronous bilateral retroperitoneal laparoscopic nephrectomy for giant symptomatic adult polycystic kidney disease is feasible, safe and efficacious, and can be performed either before or after renal transplantation. Compared to open surgery, the laparoscopic approach results in significantly shorter hospital stay, decreased morbidity and quicker recovery. Laparoscopy is currently our technique of choice in this setting.     

Therapeutic interventions for autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threatening hereditary disease in the United States and causes end-stage renal failure requiring dialysis and renal transplantation. There is no effective treatment for ADPKD in humans. However, there are now multiple clinical trials testing a host of therapeutic interventions in children and adults with ADPKD. The major therapeutic interventions being tested in patients with ADPKD include Tolvaptan, Octreotide, Sirolimus, Everolimus, and statins, angiotensin converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs).     

Percutaneous cyst puncture in the treatment of cyst infection in autosomal dominant polycystic kidney disease

Cyst infection in autosomal dominant polycystic kidney disease (ADPKD) poses a difficult diagnostic and therapeutic problem. We describe a serious indolent cyst infection due to Staphylococcus aureus, which was successfully diagnosed and treated with repeated percutaneous cyst drainage and intravenous (IV) antibiotic therapy. Cyst aspiration also permitted monitoring of cyst antibiotic levels and evidence of active infection.     

Hypertension in autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) has been shown to be associated with a greater than 50 percent incidence of hypertension prior to deterioration in renal function as assessed by glomerular filtration rate. The present study provides evidence for increased cardiac pre-load, as assessed by plasma atrial natriuretic factor (ANF) and cardiac index, in hypertensive as compared to normotensive ADPKD. The hypertensive ADPKD patients exhibited an increased renal vascular resistance as compared to the normotensive patients in spite of comparable glomerular filtration rates. It is hypothesized that the renal involvement of hypertensive ADPKD patients causes an impaired renal response to the observed increase in cardiac index, and also may release a venoconstrictor (such as angiotensin) which contributes to the enhanced cardiac pre-load and thus the hypertension.     

Myofibroblast depletion reduces kidney cyst growth and fibrosis in autosomal dominant polycystic kidney disease

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Angiogenesis and autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the growth of multiple cysts that in many cases result in end-stage renal disease. Current strategies to reduce cyst progression in ADPKD focus on modulating cell turnover, fluid secretion, and vasopressin signalling; but an alternative approach may be to target pathways providing “general support” for cyst growth, such as surrounding blood vessels. This could be achieved by altering the expression of growth factors involved in vascular network formation, such as the vascular endothelial growth factor (VEGF) and angiopoietin families. We highlight the evidence that blood vessels and vascular growth factors play a role in ADPKD progression. Recent experiments manipulating VEGF in ADPKD are described, and we discuss how alternative strategies to manipulate angiogenesis may be used in the future as a novel treatment for ADPKD.     

Boy with autosomal recessive polycystic kidney and autosomal dominant polycystic liver disease

BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) shows a great phenotypic variability between patients, ranging from perinatal demise to mildly affected adults. Autosomal dominant polycystic liver disease (PCLD) does not manifest in childhood. CASE-DIAGNOSIS/TREATMENT: A boy was reported with the co-occurrence of ARPKD and PCLD. He presented at the age of 16 days with pyelonephritis and urosepsis. Subsequent investigations showed enlarged kidneys and hyperechogenic renal medulla and liver parenchyma. Genetic analysis revealed compound heterozygous mutations in the PKHD1 gene (p.Arg496X and p.Ser1862Leu). After his mother was diagnosed with PCLD, the finding of a liver cyst on ultrasound prompted analysis of the PRKCSH gene, revealing a missense mutation (p.Arg139His). At the most recent follow-up at 13 years of age, the patient's course and clinical examination was uneventful with normal renal and liver function without evidence of portal hypertension. CONCLUSIONS: The patient with ARPKD and PCLD has so far demonstrated a benign clinical outcome, consistent with the great phenotypic variability of ARPKD and, apart from the liver cyst, asymptomatic manifestation of PCLD in childhood. However, close long-term follow-up is mandatory.     

Efficacy of chloramphenicol in refractory cyst infections in autosomal dominant polycystic kidney disease

We previously predicted that highly lipid soluble antibiotics would be very effective in the treatment of infected cysts in autosomal dominant polycystic kidney disease (ADPKD). This study examines the use of chloramphenicol, a lipid soluble antibiotic with a therapeutic spectrum covering most gram negative organisms, in the treatment of patients with infected polycystic cysts who had not responded to initial antibiotic therapy. Intravenous chloramphenicol was used in five hospitalized patients with serious infections of the renal cysts. Three of the five patients had positive blood cultures. E. coli was grown in all five patients, in three from blood, in two from urine, and four of five patients were initially treated with antibiotics to which the organism was sensitive. Despite favorable sensitivities, none of the reported patients showed clinical response to initial antibiotic therapy. Chloramphenicol treatment was subsequently effective in all cases. One patient ultimately developed an infection with a chloramphenicol resistant organism. We conclude that chloramphenicol is effective in the treatment of infected cysts in ADPKD but that care must be taken in the use of this agent as selection of resistant organisms may occur in patients with recurrent cyst infections.     

Renal disease progression in autosomal dominant polycystic kidney disease

Background

Autosomal dominant polycystic kidney disease is a lifelong progressive disorder. However, how age, blood pressure, and stage of chronic kidney disease (CKD) affect the rate of kidney function deterioration is not clearly understood.

Methods

In this long-term observational case study up to 13.9?years (median observation period for slope was 3.3?years), serum creatinine was serially measured in 255 mostly adult patients. The glomerular filtration rate was estimated (eGFR) using a modified Modification of Diet in Renal Disease Study method. The total kidney volume (TKV) has been measured in 86 patients at one center since 2006.

Results

As age increased, eGFR declined significantly (P?Conclusion The declining rate of eGFR was relatively constant and did not correlate with age or eGFR after adolescence. eGFR was already low in young adult patients with hypertension. As age increased after adolescence, eGFR declined and TKV increased similarly between normal and high blood pressure groups. eGFR starts to decline in patients with normal eGFR, suggesting that the decline starts earlier than previously thought.