Calciphylaxis in pediatric end-stage renal disease

Calciphylaxis is a rare, but life-threatening complication of end-stage renal disease (ESRD) that has been reported mostly in adult patients. The exact etiology is unknown, but the disease is commonly associated with a high calcium-phosphorus product and elevated levels of parathyroid hormone (PTH). We herein review the published reports on calciphylaxis in ESRD patients less than 18 years old and report the case of a patient with severe calciphylaxis who presented with lower extremity pain, muscle tenderness and difficulty in walking. The serum PTH was low, and the calcium-phosphorus product was normal. The diagnosis of calciphylaxis was confirmed by a muscle biopsy. Treatment with low calcium peritoneal dialysate and substitution of calcium-based phosphorus binders with sevelamer (Renagel) was unsuccessful. The patients clinical condition progressed to extensive soft tissue calcification and ulcerating skin lesions. Nine months after the onset of symptoms, the patient died of cardiopulmonary arrest.     

Tuberculosis complicating tertiary hyperparathyroidism in a patient with end-stage renal disease: a case report

A 36-year-old Nigerian woman on thrice-weekly dialysis presented with symptoms and signs of hypercalcaemia. Laboratory findings were consistent with tertiary hyperparathyroidism. Parathyroid hormone levels remained elevated and she underwent elective parathyroidectomy. Intra-operatively all 4 parathyroid glands and local lymph nodes showed necrotising granulomas with occasional acid-fast bacilli, pathognomonic of tuberculosis (TB). Post-operatively she completed a full course of anti-TB therapy and at 9 months she experienced complete resolution in her plasma biochemistry and was essentially symptom-free. This is a rare yet fascinating cause of hypercalcaemia in a dialysis patient and is the first recorded case of tubercular involvement of parathyroid tissue in a case of tertiary hyperparathyroidism. This report demonstrates the coexistence of 2 diseases that simultaneously worsened hypercalcaemia and thus emphasises the importance of the differential diagnosis and of careful histological examination post-operation.     

Hereditary haemorrhagic telangiectasia presented with multiple brain abscesses and renal abscess in a Chinese patient: A case report

Hereditary haemorrhagic telangiectasia (HHT), or Osler–Weber–Rendu syndrome, is an autosomal dominant condition characterized by recurrent epistaxis, multiple telangiectasia and visceral vascular malformations. Brain abscess is a common presentation secondary to pulmonary arteriovenous malformation (AVM) in HHT patients. The prevalence of HHT in the Chinese population is not known, and is not commonly report. Here, we report on a Chinese patient who suffered from HHT and presented with multiple brain abscesses and a renal abscess. Upon further investigation of the patient's medical history and a physical examination, it was found that he had recurrent epistaxis and telangiectasia over his tongue, as well as fingers. Subsequent examination found pulmonary and hepatic AVM. He had a fair recovery after treatment of brain abscesses and pulmonary AVM. We believe HHT is underreported in the Chinese population. Simple history taking and physical examination in patients with a brain abscess can aid diagnosis and prevent complications with prophylactic treatment.     

Calciphylaxis precipitated by ultraviolet light in a patient with end-stage renal disease secondary to systemic lupus erythematosus.

Calciphylaxis is a rare and severe calcification syndrome described mainly in patients with end-stage renal disease (ESRD) undergoing dialysis or with a renal transplant. This life-threatening condition is characterized by the abrupt onset of painful ischemic skin ulcers and necrosis. Secondary local and systemic infection may supervene and, without timely and appropriate interventions, calciphylaxis may be fatal. A precipitant or challenging agent is believed to be necessary to initiate the process. We describe a case of a woman with ESRD receiving continuous ambulatory peritoneal dialysis who developed calciphylaxis in the setting of severe hyperparathyroidism after receiving UV photoradiation therapy. We suggest that the UV light served as the challenging agent in the precipitation of this devastating condition.     

Organ recovery from a donor with end-stage renal disease: a case study

As the field of transplantation enters the new millennium, maximizing organs per donor remains one of the greatest challenges of procurement. This case study outlines nontraditional medical management techniques that facilitated the recovery of 5 transplantable organs from a patient with end-stage renal disease. Strategies utilized in this case included the use of continuous veno-venous hemodialysis and airway pressure release ventilation to maximize the outcomes of the donation. Although the use of these strategies is admittedly limited to hospitals where the resources are available, this case study suggests that utilizing available resources in any clinical setting can make more organs available to people on the waiting list for solid organ transplantation.     

Calciphylaxis: a severe but unrecognized complication in end-stage renal disease patients. A review of 2 cases

Calciphylaxis presents like subcutaneous lesions with livedo reticularis leading to necrotic and painful ulcers, predominantly in the lower limbs and the abdomen. They initially simulate dermohypodermitis. Biology reveals secondary hyperparathyroidism, phosphocalcic metabolism abnormalities and state of hypercoagulability. Histological signs are constant: calcifications in the media of small and sub-cutaneous arteries, intimal hyperplasia and intravascular thrombosis. This complication occurs in 4% of end-stage renal disease patients. Its prognostic is awful with a rate of mortality of 60% due to sepsis. Treatment is based upon the normalization of phosphocalcic rates and local debridement.     

Calciphylaxis causing necrotizing mastitis: a case report

Calciphylaxis is a rare condition most commonly related to ESRD and hyperparathyroidism. We report a case of necrotizing mastitis caused by calciphylaxis following routine breast biopsy for microcalcifications. Early recognition of the potential for this condition should help in clinical management of patients with calciphylaxis.     

Brucella glomerulonephritis resulting in end-stage renal disease: a case report and a brief review of the literature

Brucella glomerulonephritis is a rare condition with only a few reported cases. We review the literature, and describe a 24-year-old female who presented with edema and proteinuria. Blood grew Brucella melitensis. Renal biopsy showed diffuse proliferative glomerulonephritis. The patient progressed to end-stage renal disease despite antibiotic and steroid therapy.     

Recombinant human erythropoietin in a patient with multiple myeloma and end-stage renal disease.

Recombinant human erythropoietin (rHuEpo) is an established, effective treatment for the anemia of chronic renal failure. Recent reports also suggest it may be efficacious in the anemias of drug toxicity, rheumatoid arthritis, and multiple myeloma without renal failure. We describe the positive response to rHuEpo in an end-stage renal disease patient with active multiple myeloma and ongoing chemotherapy. Before rHuEpo therapy, the patient was transfusion dependent, but after rHuEpo was initiated, transfusions were not required. Multiple myeloma with renal failure does not preclude a response to rHuEpo. Further trials of rHuEpo in the treatment of multiple myeloma with and without renal failure are warranted.     

Considerations in spontaneous quadriceps tendon rupture repair in end-stage renal disease patients: A case report

IntroductionSpontaneous quadriceps tendon rupture (SQTR) is a rare injury to the knee extensor mechanism that is usually associated with systemic diseases such as end stage renal diseases (ESRD) and it is more prevalent in the elderly. Due to the underlying pathology, quadriceps tendon rupture warrants special considerations and management in its repair.Case reportWe present two cases of quadriceps tendon rupture in end-stage renal disease (ESRD) patients. The first case; a 57 years old female who had bilateral SQTR and is undergoing hemodialysis. The second patient, a 26 years old male had unilateral quadriceps tendon rupture caused by minimal trauma.The first patient had a trans-osseous repair by direct suturing the quadriceps tendon stump to the proximal pole patella. The second patient was repaired with a modified Bunnel suture and anchor placement on the proximal pole patella. The first case had a re-rupture of the right quadriceps tendon and the second case has recovered with improved outcomes.DiscussionSpontaneous quadriceps tendon rupture is usually underlined by degenerative changes of the tendons. Special care is needed to address the pathologic tendon underlying SQTR. The current surgical literature still lacks the statistical data that shows which surgical approach is most optimal for SQTR in ESRD patients.ConclusionSQTR rupture is generally an injury of brittle tendons caused by underlying diseases. A multidisciplinary and comprehensive approach including a proper surgical approach and postoperative managements are crucial for good functional outcomes of the extensor mechanism.     

Erdheim-Chester disease as cause of end-stage renal failure: a case report and review of the literature

International Urology and Nephrology -     

胰肾联合移植治疗1型糖尿病合并终末期肾病1例报告并文献复习

目的探讨胰肾联合移植治疗糖尿病合并终末期肾病的手术方式及移植效果。方法对1例1型糖尿病合并糖尿病肾病、尿毒症患者施行胰肾联合移植手术,将肾脏移植于左侧髂窝,胰腺移植于右侧髂窝,胰腺移植采用胰腺外分泌肠道引流、内分泌体循环系统回流的术式(肠道-体循环回流术式);术后常规给予免疫抑制、防治感染、支持等治疗。结果受者手术顺利。联合移植后胰、肾均发挥正常功能,血清肌酐逐渐恢复正常水平,血糖趋于稳定,第14日完全停用胰岛素及降糖药物。无严重手术并发症发生,受者健康存活,门诊随访移植胰腺、肾脏功能正常。结论胰肾联合移植是治疗1型糖尿病合并终末期肾病的理想方法 。     

Case report: Pregnancy in hemodialysis-dependent end-stage renal disease: anesthetic considerations

PURPOSE: To describe the obstetrical and anesthetic management of a parturient with end-stage renal disease. CLINICAL FEATURES: A 38-yr-old woman had severe renal impairment due to Wegener's granulomatosis. She was on hemodialysis for 12 years following two failed kidney transplants. She had two unsuccessful pregnancies, two and 12 years previously. The antenatal care of the present pregnancy incorporated a multidisciplinary approach involving obstetrics, nephrology and anesthesiology. Labour was induced at 36 weeks gestation. Bupivacaine 0.0625% with fentanyl 2 microg.mL(-1) was injected through an epidural catheter inserted for labour analgesia. Lidocaine 2% with epinephrine 2.5 microg.mL(-1) was given later for Cesarean delivery. There was no associated maternal or neonatal morbidity. The management focused on minimizing hemodynamic disturbances while providing maximum pain relief. CONCLUSIONS: For a successful outcome in the parturient with end-stage renal disease, a multidisciplinary approach is essential.     

Calciphylaxis in moderate renal insufficiency: changing disease concepts

Calciphylaxis is a rare but frequently fatal complication in patients with end-stage renal disease. Original concepts regarding groups at risk for the disease, predisposing factors, and associated morbidity have changed significantly in the past few years as more cases are reported. We present a patient who developed fatal calciphylaxis in the setting of moderate renal insufficiency to illustrate some of the evolving concepts in this disease process.     

Hepatobiliary cystadenoma combined with multiple liver cysts: report of a case

Hepatobiliary cystadenomas are rare benign tumors with malignant potential. They are almost always solitary lesions accompanied by multilocular cysts in the liver, and are difficult to differentiate from cystadenocarcinoma, despite the diagnostic modalities available. This report describes a case of hepatobiliary cystadenoma with multiple cysts in the left hepatic lobe, diagnosed by magnetic resonance imaging in a 48-year-old woman. Abdominal computed tomography revealed only multiple cystic lesions in the left lobe, but cholangiography via a nasogastric biliary drainage tube combined with percutaneous transhepatic cholangiography showed a stenotic region with fine irregularity in the left lateral posterior segmental bile duct and left lateral anterior segmental bile duct. Hepatobiliary cystadenocarcinoma with multiple liver cysts was suspected. We performed left hepatectomy, and microscopic examination confirmed a diagnosis of hepatic cystadenoma with multiple liver cysts. There was no nuclear atypia or mitosis in the epithelium of the locus, which was constructed of simple columnar-to-cuboidal epithelium with basal nuclei. The patient is well without recurrence more than 4 years after surgery. Received: July 5, 2000 / Accepted: January 9, 2001