Ketorolac-induced irreversible renal failure in sickle cell disease: a case report

 Nonsteroidal anti-inflammatory drugs are often used in the management of those with acute pain secondary to sickle cell disease due to potent analgesic effects along with a lack of addictive potential, respiratory depression, and central nervous system effects, as may occur with narcotics. Caution should be observed in the use of nonsteroidal anti-inflammatory drugs in patients with compromised renal function. We present a case of a 17-year-old sickle cell disease patient with an acute painful episode and normal renal function indices who subsequently developed irreversible renal failure and a perirenal hematoma following the administration of ketorolac, despite adequate hydration. Due to its inhibitory effect on prostaglandin-mediated vasodilation, we advise caution in the use of ketorolac for the pain management of sickle cell painful episodes. We recommend following the administration guidelines for ketorolac for renal-compromised patients in those with painful episodes of sickle cell disease, and if used in this patient population, renal function must be very closely monitored. Received: 24 March 1998 / Revised: 5 June 1998 / Accepted: 10 June 1998     

Guideline on the peri-operative management of patients with sickle cell disease

Sickle cell disease is a multisystem disease characterised by chronic haemolytic anaemia, painful vaso-occlusive crises and acute and chronic end-organ damage. It is one of the most common serious inherited single gene conditions worldwide and has a major impact on the health of affected individuals. Peri-operative complications are higher in patients with sickle cell disease compared with the general population and may be sickle or non-sickle-related. Complications may be reduced by meticulous peri-operative care and transfusion, but unnecessary transfusion should be avoided, particularly to reduce the risk of allo-immunisation. Planned surgery and anaesthesia for patients with sickle cell disease should ideally be undertaken in centres with experience in caring for these patients. In an emergency, advice should be sought from specialists with experience in sickle cell disease through the haemoglobinopathy network arrangements. Emerging data suggest that patients with sickle cell disease are at increased risk of COVID-19 infection but may have a relatively mild clinical course. Outcomes are determined by pre-existing comorbidities, as for the general population.     

Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary.

BACKGROUND: The health risks associated with sickle cell trait are minimal in this sizable sector of the world's population, and many of these patients have no information about their sickle cell status. Splenic syndrome at high altitude is well known to be associated with sickle cell trait, and unless this complication is kept in mind these patients may be subjected to unnecessary surgery when they present with altitude-induced acute abdomen. METHODS: Four patients were admitted to the surgical ward with a similar complaint of acute severe left upper abdominal pain after arrival to the mountainous resort city of Abha, Saudi Arabia. All were subjected to splenectomy because of lack of suspicion regarding sickle cell status. RESULTS: Histologic examination of the spleen showed all patients had sickle cells in the red pulp. On further assessment all were found to have sickle cell trait with splenic infarction. In a similar study of 6 patients with known sickle cell disease who had comparable problems when they travelled to the Colorado mountains, all made an uncomplicated recovery with conservative management. CONCLUSIONS: In ethnically vulnerable patients with splenic syndrome, sickle cell trait should be ruled out before considering splenectomy. These patients could respond well to supportive management, and splenectomy would be avoided.     

Surgical management of osteonecrosis of the femoral head in patients with sickle cell disease

Sickle cell disease is a known risk factor for osteonecrosis of the hip. Necrosis within the femoral head may cause severe pain, functional limitations, and compromise quality of life in this patient population. Early stages of avascular necrosis of the hip may be managed surgically with core decompression with or without autologous bone grafting. Total hip arthroplasty is the mainstay of treatment of advanced stages of the disease in patients who have intractable pain and are medically fit to undergo the procedure. The management of hip pathology in sickle cell disease presents numerous medical and surgical challenges, and the careful perioperative management of patients is mandatory. Although there is an increased risk of medical and surgical complications in patients with sickle cell disease, total hip arthroplasty can provide substantial relief of pain and improvement of function in the appropriately selected patient.     

Slipped capital femoral epiphysis in a child with sickle cell disease

Slipped capital femoral epiphysis in a child with sickle cell disease has not been reported previously. The diagnostic challenges, role of imaging techniques, and the medical treatment of this patient are discussed. The presentation of acute hip or leg pain in a child with sickle cell disease should alert the treating physician to the possibility of a vaso-occlusive crisis as the likely source of the child's pain. The goal of the current case report is to emphasize the need to maintain a high index of suspicion for other potential causes of hip, thigh, or knee pain such as slipped capital femoral epiphysis in an adolescent. Preoperative and postoperative care for a child with sickle cell disease needs to be modified to minimize the risks of vaso-occlusive complications.     

Repeated testicular infarction in a patient with sickle cell disease: a possible mechanism for testicular failure

We report a case of repeated testicular infarction in a 39-year-old man with sickle cell disease. The patient presented with a 2-week history of testicular pain and was found clinically and sonographically to have a testicular mass, suspicious for a testicular tumor. The pathologic examination of the orchiectomy specimen revealed multiple infarcts, showing temporal variation ranging from acute (several days old) to recent (2 to 3 weeks old) to remote. This is the fifth case of segmental testicular infarction reported in patients with sickle cell disease/trait. We propose repeated testicular infarction as a probable mechanism of testicular failure and impaired fertility in patients with sickle cell disease.     

Osteomyelitis of the spine due to Salmonella infection — conservative treatment with quinolone: a case report

Although osteomyelitis due to Salmonella infection is known to be associated with sickle cell anemia, various hemoglobinopathies and immune suppressive states, it may also occur in normal hosts. A 16-year-old Chinese boy without sickle cell disease or any other condition that would compromise the immune system had osteomyelitis of the lumbar spine caused by Salmonella enteritidis. The condition was treated conservatively with ciprofloxacin (quinolone group). This may be the first reported case in which a patient with spinal osteomyelitis due to Salmonella infection, who was otherwise healthy, was successfully treated nonoperatively with quinolone.     

The role of laparoscopic cholecystectomy in the management of acute cholecystitis in patients with sickle cell disease

BACKGROUND: As emergency surgery in sickle cell disease patients is associated with high morbidity, the aim of the study was to assess the safety of laparoscopic cholecystectomy in the acute state for these patients. METHODS: Over a 5-year period, April 1994 till December 1998, 35 sickle cell patients with acute cholecystitis had laparoscopic cholecystectomy within the first 5 days of presentation. A retrospective study of these was undertaken. RESULTS: Thirty-five patients were diagnosed as having acute cholecystitis with sickle cell disease. There were 26 female and 9 male patients; 5 patients needed preoperative and 1 patient needed postoperative endoscopic retrograde cholangiopancreatography. Twenty-seven patients needed simple transfusion and 8 needed partial exchange; conversion was necessary in two cases (5.7%). The mean hospital stay was 5.3 days and the complication rate was 17.5%. CONCLUSIONS: Because of the lack of significant complications, we believe that laparoscopic cholecystectomy for acute cholecystitis is safe and recommended in experienced hands with adequate preoperative preparation for patients with sickle cell disease.     

Value of elective cholecystectomy in children with homozygote sickle cell anemia. Apropos of 3 recent cases

Homozygous SS sickle cell anemia affects 0.25% of the population of the West Indies. Gallstones are frequently found in this blood disease and are the cause of recurrent abdominal pain, cholecystitis and dangerous infectious complications in these patients. These complications are difficult to distinguish from very frequent episodes of vaso-occlusive abdominal pain. Three cases in childhood sickle cell disease are reported. The authors believe that elective cholecystectomy is to be recommended (emergency cholecystectomy is associated with a high morbidity) as the children operated were improved by surgery, with resolution of abdominal pain.     

Urologic manifestations of hematologic disease sickle cell,leukemia, and thromboembolic disease

Advances in medicine are allowing patients with hematologic disease to live longer and healthier lives than ever before. As these patients age, however, manifestations of their disease processes may develop as complications in other organ systems. We discussed the major genitourinary complications of sickle cell anemia, leukemia, and thromboembolic disease. These range from the benign inability to concentrate urine that is seen in sickle cell disease to renal infarction that results from nephrotic syndrome. Our ability to treat and prevent these complications will improve as our understanding of these disease processes and their pathophysiology grows. Additionally, it is important for urologists to understand the underlying pathophysiology of hematologic disease to best serve the patients. For example, it may be the urologist who makes the diagnosis of ovarian vein thrombosis in a pregnant woman with right lower quadrant pain and fever. This diagnosis, with the proper treatment of antibiotics and anticoagulation, could prevent the potential development of septic thrombophlebitis. Urologists will increasingly be called upon to deal with the manifestations of these complex diseases as these patients are living longer. It is our duty to educate ourselves about these disease processes so that we can make the best clinical decisions for our patients.     

Acute splenic sequestration crisis in sickle cell disease: early detection and treatment.

Acute splenic sequestration crisis (ASSC) in children with various forms of sickle cell disease can result in life-threatening circulatory collapse due to the loss of circulating blood volume. Over a 6-year period we have treated 12 patients ranging in age from 5 1/2 months to 7 years presenting with acute sequestration crisis. Eleven had homozygous sickle cell disease and the other had sickle-thalassemia. One patient died of acute circulatory collapse. Eight patients underwent splenectomy after a major episode of sequestration with no serious infectious complications up to 5 years following splenectomy. Three patients with minor episodes have been followed with no recurrences. To foster early detection of this potentially lethal complication of sickle cell disease, an educational program in our Comprehensive Sickle Cell Center instructs the parents to examine the spleen and bring their child in for evaluation if the spleen enlarges. A newly developed videotape describes the common symptoms of ASSC and illustrates the technique of palpating the spleen. With early detection of sickle cell disease by neonatal screening and the educational program, the morbidity and mortality from this complication of sickle cell disease can be reduced.     

Segmentaler Hodeninfarkt bei Sichelzellenanämie

Vascular occlusions are the clinical indicators of sickle cell disease and in urology they can lead to papillary necrosis, renal infarction or priapism. Segmental testicular infarction in patients with sickle cell disease is a rare event and only a few cases have been reported. We present a 25-year-old man with right testicular pain increasing over 3 days and sickle cell disease. Ultrasound of the right scrotum presented an inhomogeneous, mainly hypoechegenic mass with a hyperechogenic margin and no sign of blood flow. A partial orchiectomy was performed with total enucleation of the lesion, which was histologically diagnosed as benign hemorrhagic necrotic testicular tissue.     

Two cases of hematuria with hemoglobin C trait

Patients with sickle cell disease commonly experience painless hematuria. Hematuria may be found in patients with sickle cell trait, sickle cell anemia, and sickle cell hemoglobin C disease, but it is believed to be uncommon in patients with other hemoglobinopathies, such as hemoglobin C disease and hemoglobin C trait. We report two cases of children with hemoglobin C trait who presented with persistent painless hematuria. Because it is possible that hematuria in a patient with hemoglobin C trait is purely coincidental, all patients with a hemoglobinopathy and hematuria should undergo a complete evaluation so as not to overlook other causes of hematuria.     

Perioperative management in children with sickle cell disease undergoing laparoscopic surgery.

OBJECTIVE: The aim of this study was to evaluate our experience with laparoscopic surgery in children with sickle cell disease. METHODS: A retrospective chart review was performed to analyze the indication for surgery, perioperative management, surgical technique, complications, duration of hospitalization, and outcome. One pediatric surgeon performed all procedures. RESULTS: Thirteen children underwent laparoscopic surgery for the following indications: symptomatic cholelithiasis/cholecystitis in 9; recurrent splenic sequestration in 3; and hypersplenism/symptomatic cholelithiasis in 1. The 7 boys and 6 girls had a median age of 7.8 years. Patients undergoing splenectomy only were younger than those undergoing cholecystectomy (median age, 3.6 years versus 11.5 years, respectively). Four children underwent endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy because of common bile duct dilatation and stones. Twelve patients received packed red blood cell transfusions prior to surgery. The median operative time was 150 minutes, and the median hospitalization was 3 days. Four patients suffered postoperative complications (2 with acute chest syndrome, 1 with recurrent abdominal pain, and 1 with priapism). The patient with abdominal pain was found to have a retained stone in the common bile duct, which was retrieved via endoscopic retrograde cholangiopancreatography and sphincterotomy. All complications resolved with medical management. CONCLUSIONS: Laparoscopic surgery is safe in children with sickle cell disease. Meticulous attention to perioperative management, transfusion guidelines, and pulmonary care may decrease the incidence of acute chest syndrome.     

带血管蒂髂骨瓣移位治疗青少年镰状细胞病股骨头缺血性坏死

目的探讨带血管蒂髂骨瓣移位治疗青少年镰状细胞病股骨头缺血性坏死的临床疗效。方法1998年～2001年在尼日尔的马腊迪省医院，治疗患者12例14髋，其中男5例，女7例；单侧10例10髋，双侧2例4髋；年龄11～22岁。均为FicatⅢ、Ⅳ期患者。Harris评分平均75分。采用带血管蒂髂骨瓣移位12例12髋，有2例双髋患者，仅作一侧手术。结果术后患者均获随访24～30个月，平均27．4个月。髋部疼痛消失，无感染等并发症，Harris评分平均90分，X线片示病变无进展，无重复手术，近期疗效满意。结论对于青少年FicatⅢ、Ⅳ期的镰状细胞病股骨头缺血性坏死患者，采用带血管蒂髂骨瓣移位术治疗，能解除疼痛，恢复髋关节功能，延缓人工全髋关节置换的时间。     

Ulcerative colitis in patient with sickle cell disease: A conspiracy of nature that turns surgeon’s decision into a challenge

Introduction

Concurrent presentation of ulcerative colitis and sickle cell disease is a very rare incident. We report the fifth case to be reported worldwide.

Case Report

A 21-year-old woman with coincidental ulcerative colitis and sickle cell disease presented complaining of an acute abdominal pain, rendering the differential diagnosis as a very challenging task. Finally, she underwent surgery involving a long-term and multistep procedure.

Discussion

Abdominal pain could be ascribed to ulcerative colitis or a sickle cell crisis. Surgical intervention is reserved for acute complications of both diseases. If surgery is chosen as the treatment modality in a surgical emergency, a multistep and extended procedural plan must be considered to ensure the optimal outcome of the patient.

Conclusion

The rarity of this coincidental abdominal pain renders differential diagnosis a very challenging task for the attending physician. If surgical intervention is inevitable, both the patient and surgeon must understand the long-term and multistep plan to ensure an optimal outcome.     

High Flow Priapism Associated with Sickle Cell Disease

Priapism associated with sickle cell disease is classically described as a low flow state. We report 2 cases of high flow priapism associated with sickle cell disease. High flow priapism has previously been reported almost exclusively in patients with traumatic rupture of the cavernous artery. Neither of our patients had historical or radiographic findings consistent with injury to the penile vasculature. One patient was treated unsuccessfully with intracorporeal injection of methylene blue and 1 underwent successfully bilateral pudendal artery embolization. The pathophysiological mechanism(s) responsible for the production of high flow priapism in patients with sickle cell disease is not known.     

Salmonella osteomyelitis of multiple ribs and thoracic vertebra with large psoas muscle abscesses

Backgroud contextSalmonella vertebral osteomyelitis is a well-known complication of sickle cell disease. However, it has been infrequently reported in immunologically normal people or diabetic patients.PurposeTo report a case of Salmonella vertebral osteomyelitis in a diabetic patient with multiple rib infections, large bilateral psoas muscle abscesses, and pleural effusion.Study design/settingCase report.MethodsA case of Salmonella vertebral osteomyelitis is reported in a 42-year-old man with diabetes. The patient had multiple rib and T12 vertebral infections with pleural effusion and large bilateral psoas muscle abscesses. The adjacent discs were intact.ResultsDiagnosis was made by computed tomography–guided psoas muscle abscess drainage and aspirate culture. Appropriate antibiotic treatment resulted in a favorable outcome. The patient had mild back pain with a 38° residual kyphosis at his thoracolumbar junction after treatment.ConclusionsVertebral osteomyelitis caused by Salmonella is uncommon in diabetic patients. Salmonella can cause multiple bone infections and large abscesses of psoas muscles in patients without sickle cell disease. Correct diagnosis can be confirmed by blood, biopsy, or abscess culture. Appropriate antibiotic treatment was effective.     

Renal abnormalities in sickle cell disease

Renal abnormalities in sickle cell disease. Sickle cell nephropathy is indicated by sickled erythrocytes, with the consequent effects of decreased medullary blood flow, ischemia, microinfarct and papillary necrosis. Impaired urinary concentrating ability, renal acidification, hematuria, and potassium secretion are also found. There may be a causal relationship between an increase in nitric oxide synthesis and experimental sickle cell nephropathy, and some studies have indicated that the progression of sickle cell nephropathy is hemodynamically mediated. Although there are many studies showing that proteinuria, nephrotic syndrome, chronic progressive renal failure, and acute renal failure syndromes are the outcome of this disease, the pathogenic mechanism(s) and potential therapies remain to be elucidated. Survival of patients with sickle cell nephropathy who progress to end-stage renal disease (ESRD) is equal to non-diabetic ESRD patients, and graft survival rates are also similar for those who undergo renal transplantation. This article presents a historical review of the glomerular and tubular disorders associated with sickle cell nephropathy, and reviews therapeutic indications to slow its progression. Further research is needed.     

Shoulder arthroplasty in sickle cell patients with humeral head avascular necrosis

The purpose of this study was to evaluate the results of shoulder arthroplasty for the treatment of avascular necrosis in patients with sickle cell disease. Medical records, radiographs, operative reports, and outcome scores of 8 adult patients with sickle cell disease were evaluated. The mean follow-up was 51 months (range, 2-10 years). Seven patients had a hemiarthroplasty, and one had a total shoulder arthroplasty. One patient had an intraoperative rotator cuff tear. Two had sickle cell crises in the immediate postoperative period. In one patient, stiffness developed that required arthroscopic capsular release 22 months after her arthroplasty. Another patient with a hemiarthroplasty underwent revision to a total shoulder arthroplasty 5 years after the index procedure. The mean American Shoulder and Elbow Surgeons score improved by 31.9 points. However, only 2 patients reported improvement in pain as assessed with a visual analog scale. Although shoulder arthroplasty provides improvements in range of motion and function in the majority of patients, pain relief is less predictable.