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     

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.     

Genitourinary complications of sickle cell disease

PURPOSE: In the last half century the molecular biology, pathophysiology and natural history of sickle cell disease have been well defined. Sickle cell disease causes microvascular occlusion, which is manifested in most organ systems. The genitourinary tract is most commonly affected by hematuria, urinary tract infection and priapism but other more serious sequelae have been identified. MATERIALS AND METHODS: We performed a computerized MEDLINE search from 1965 to the present and a bibliographic review of cross references. These references were analyzed for meaningful findings and case reports. RESULTS: The diagnosis and management of sickle cell disease have advanced rapidly with a significant increase in the life expectancy of affected patients and recognition of a greater number of genitourinary complications. Renal function may be mildly altered or lost completely. Patients with sickle cell disease are at increased risk for urinary tract infection. Priapism is a painful complication of sickle cell disease that is poorly understood and challenging to treat and prevent. Testicular infarction has also been noted. Furthermore, renal medullary carcinoma, a highly lethal tumor, develops almost exclusively in young patients with sickle cell trait. CONCLUSIONS: Heightened awareness of the genitourinary complications of sickle cell disease may prevent end stage disease, including renal failure and impotence. New forms of therapy for sickle cell disease, such as hydroxyurea, may prevent these complications in the future.     

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.     

Urinary endothelin-1 as a marker of renal damage in sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) affects the kidney by acute mechanisms as well as by insidious renal medullary/papillary necrosis, resulting in tubular defects, which increase the risk of dehydration and subsequent sickle crisis. Hypoxia has been reported to stimulate endothelin-1 (ET-1) synthesis by endothelial cells and also in the renal tubule. METHODS: This case-control study measured ET-1 in urine as a marker of its renal synthesis in asymptomatic SCD patients. Baseline plasma and urinary ET-1 levels were measured and followed during a water deprivation study and a subsequent administration of desmopressin. RESULTS: Urine and plasma levels of ET-1 were elevated in patients with SCD, compared with carefully matched African-French and African controls, and urine ET-1 excretion was associated with a marked urine-concentrating defect. Moreover, urinary ET-1 output was correlated with microalbuminuria in SCD patients. CONCLUSIONS: ET-1 is known to antagonize the tubular effects of vasopressin and to promote renal scarring; increased renal production of ET-1 could produce nephrogenic diabetes insipidus and dehydration in SCD patients through a combination of fibrosis and functional resistance to vasopressin. This study provides a rationale for trials with endothelin receptor antagonists in sickle cell disease nephropathy.     

Elective cholecystectomy for the patient with sickle cell disease and asymptomatic cholelithiasis

Experience with elective cholecystectomy in ten patients with sickle cell disease and one patient with sickle-thalassemia from 1977-1984 was reviewed. In contrast to an earlier review from our institution, the current series had a low morbidity. This improvement is attributed to careful perioperative care, especially preoperative transfusion, hydration, and oxygenation. Because of the increasing longevity of sickle cell patients, because of the incidence of eventual significant complications of even asymptomatic cholelithiasis, and in order to simplify medical management by eliminating the diagnostic confusion between acute cholecystitis and sickle cell hepatobiliary crisis, the authors believe that elective cholecystectomy is to be recommended for the sickle cell patient with asymptomatic gall stones.     

Laparoscopic cholecystectomy in adult patients with sickle cell disease

Cholecystectomy is a common surgical procedure performed in patients with sickle cell disease (SCD). Postoperative complications, including acute painful vaso-occlusive crisis and acute chest syndrome, have been described frequently after either traditional or laparoscopic cholecystectomy (LC). It's still not clear if preoperative blood transfusion, hyperhydration, intraoperative body temperature conservation may reduce complications rate. The Authors reviewed the charts of seven patients with SCD operated on LC for symptomatic gallbladder lithiasis and describe their perioperative management. In 3 patients preoperative endoscopic removal of stones was achieved. Five patients with HB lower than 9 g/dl and/or HbS higher than 40% were transfused preoperatively and all the patients were hyperhydrated. Intraoperative monitoring was achieved for early recognition of ventilation to perfusion mismatch and acid-base balance or temperature modifications. The Authors reported only one case of postoperative lower extremities pain. This study suggests that LC is a safe procedure in SCD if appropriate monitoring and perioperative management are achieved.     

Sickle cell disease and the kidney

Sickle cell disease (SCD) affects the kidney by acute mechanisms, as a form of the sickle crisis, and insidiously with renal medullary/papillary necrosis, with resulting tubular defects. Glomerular hyperperfusion and hypertrophy results in a chronic sickle cell nephropathy that results in a significant morbidity in the progression to end-stage kidney disease. Kidney transplantation offers a major advantage to survival, and should be coupled with efforts toward prevention of recurrent disease.     

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.     

Assessment of perioperative transfusion therapy and complications in sickle cell disease patients undergoing surgery

BACKGROUND: Perioperative blood transfusion is usually given to sickle cell disease patients to reduce or prevent perioperative morbidity. Assessment of such a practice was the subject of our study. METHODS: A retrospective one year survey of sickle cell disease patients undergoing surgery at Salmaniya Medical Complex, Bahrain was conducted. The medical records were reviewed to characterize the surgical procedure, transfusion management and perioperative complications. RESULTS: 85 sickle cell disease patients who underwent surgery were studied. Preoperatively, 21.2% had exchange transfusion (ETX), 24.7% had simple transfusions (STX) and 54.1% had no transfusion (NTX). 14.1% of all patients had postoperative complications, and 50% of those, had complications from the laparoscopic cholecystectomy group. The incidence of sickle cell crisis postoperatively was 22.2% in ETX group, 9.5% in STX group and 4.34% in the NTX group. The incidence of acute chest syndrome postoperatively was found to be 5.55% in the ETX group, 4.76% in the STX group and 4.34% in the NTX group. No intraoperative complications were recorded in all groups. All patients who had postoperative complications had a preoperative HBSS > 40%. CONCLUSION: Exchange transfusion does not prevent perioperative complications of sickle cell disease patients. HBSS > 40% carries a higher risk of postoperative complications.     

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.     

Anaesthesia for patients with sickle cell disease (and other haemoglobinopathies)

Sickle cell disease is a common inherited disorder of haemoglobin associated with significant morbidity and mortality. It arises from a mutation in chromosome 11, where valine is substituted for glutamic acid, producing an abnormal β globin chain. Under adverse conditions these chains undergo polymerization, leading to deformation of red cells and subsequent sickling crises.Individuals with sickle cell disease have multisystem problems including anaemia, respiratory disease, cardiomegaly, cerebrovascular accident, painful bone disease and renal impairment. Sickle crises may be precipitated by hypoxia, venous stasis, hypothermia, acidosis and dehydration. The mainstay of perioperative care is the active avoidance of these triggers. Should a crisis occur, management is largely symptomatic.     

Liver transplantation in sickle cell anemia: a case of acute sickle cell intrahepatic cholestasis and a case of sclerosing cholangitis

Very few cases of liver transplantation in patients with sickle cell disease have been reported in peer-reviewed literature. We reviewed the medical records of two patients with sickle cell disease that received liver transplantation at our institution. The first patient was a 27-year-old female who presented with encephalopathy and cholestatic jaundice with a Hemoglobin S (HbS) level of 69.6%. She was diagnosed with acute sickle cell intrahepatic cholestasis. The second patient was a 26-year-old female with sclerosing cholangitis who presented with encephalopathy, bleeding, and cholestatic jaundice. Her HbS level was normal. Both patients underwent liver transplantation successfully but died in the postoperative period from multiorgan failure. We report a rare case of liver transplantation for acute sickle cell intrahepatic cholestasis and a novel case of transplantation in a patient with sickle cell disease and sclerosing cholangitis. Liver transplantation did not lead to a successful outcome in either case.     

Management of priapism in a child with sickle cell anemia; successful outcome using epidural analgesia

PURPOSE: To describe the successful management of priapism secondary to sickle cell anemia in a child using neuraxial analgesia provided via an epidural catheter. CLINICAL FEATURES: A seven-year-old male presented with chest crisis and priapism which, following hemoglobin electrophoresis led to a new diagnosis of sickle cell anemia. Epidural management was attempted as an alternative to surgery after failure of more conventional medical and surgical methods to treat the priapism. The patient's clinical condition improved with this intervention and together with further conservative therapy resulted in complete resolution of the priapism. CONCLUSIONS: Priapism is a well described complication of sickle cell anemia that is painful and difficult to manage. Surgical intervention is the last therapeutic resort and often results in significant long-term morbidity. This case highlights how select cases of priapism can be successfully managed with epidural neuraxial blockade which not only provides superior analgesia for the often painful conservative treatments, but may also per se impart a direct and salutary therapeutic benefit.     

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.     

Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis

BACKGROUND: Sickle cell intrahepatic cholestasis is a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. METHODS: Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken to maintain hemoglobin S <20% to prevent sickle cell crisis. RESULTS: Although the allograft functioned well initially, the patient developed veno-occlusive disease and required repeat transplantation at 5 months after transplant. Histologic examination of the explant revealed occlusion of the terminal hepatic venules due to fibrosis and packed red cells. Repeat transplant was complicated by thrombosis of the intrahepatic portion of the hepatic artery, and sepsis. The patient died of sepsis after a third transplant. CONCLUSION: Liver transplantation for sickle cell disease involving the liver may carry a high risk of graft loss due to vascular problems. Repeat transplantation may not be feasible if disease recurs.     

Bile ptyalism associated with chest trauma and sickle cell crisis simulating bronchobiliary fistula

Bile ptyalism, the elaboration of copious amounts of sputum virtually indistinguishable from bile, is nearly always indicative of the presence of a bronchobiliary fistula, a lesion usually produced by trauma and requiring surgical correction. This report deals with 4 patients, in 3 of whom the appearance of bilious sputum was known to have been preceded by chest trauma. None of these patients had a bronchobiliary fistula. However, all 4 had sickle cell disease and were suffering a hemolytic crisis complicated by pulmonary consolidation at the time. Although it is a rare complication, the fact that biliary ptyalism not due to fistula may occur during crisis in patients with sickle cell disease is important knowledge in the management of victims of this disorder.     

Bone and joint manifestations of sickle cell anaemia

We investigated 57 patients with sickle cell anaemia (HbSS) and bone and joint changes. Osteonecrosis simulating a wide range of conditions was a common radiological feature, and osteomyelitis occurred in 61% of cases. Salmonella species were the commonest causative organisms, occurring in 71% of patients with osteomyelitis, although salmonella septic arthritis occurred in only two. The distinction between vaso-occlusive bone crisis and acute osteomyelitis was often difficult since the classical clinical and radiological features and laboratory findings also occurred in bone infarction, a common feature of the disease.     

Perioperative considerations for patients with sickle cell disease: a narrative review

Purpose

Approximately 200,000 individuals worldwide are born annually with sickle cell disease (SCD). Regions with the highest rates of SCD include Africa, the Mediterranean, and Asia, where its prevalence is estimated to be 2-6% of the population. An estimated 70,000-100,000 people in the United States have SCD. Due to enhanced newborn screening, a better understanding of this disease, and more aggressive therapy, many sickle cell patients survive into their adult years and present more frequently for surgery.

Source

The authors identified relevant medical literature by searching PubMed, MEDLINE®, EMBASE?, Scopus?, Web of Science, and Google Scholar databases for English language publications appearing from 1972-September 2016. Case reports, abstracts, review articles, and original research articles were reviewed—with particular focus on the pathophysiology and medical management of SCD and any anesthesia-related issues.

Principal findings

Perioperative physicians should be familiar with the triggers of a sickle cell crisis and vaso-occlusive disease. Sickle cell disease affects various organ systems, including the central nervous, cardiovascular, pulmonary, genitourinary, and musculoskeletal systems. Preoperative assessment should focus on end-organ dysfunction. Controversy continues regarding if and when sickle cell patients should receive transfusions and which anesthetic technique (regional or general) confers any benefits. Timely, appropriate, and sufficient analgesia is critical, especially when patients experience a vaso-occlusive crisis, acute chest syndrome, or acute postoperative pain.

Conclusion

Effective management of SCD patients in the perioperative setting requires familiarity with the epidemiology, pathophysiology, clinical manifestations, and treatment of SCD.

     

Laparoscopic cholecystectomy in sickle cell disease

STUDY AIM: Sickle cell affection is a public health problem in Africa. The aims of this prospective study were to evaluate the early results of laparoscopic cholecystectomy in sickle cell patients in Senegal. METHOD: From January 1998 to June 2002 all the sickle cell patients undergoing a laparoscopic cholecystectomy were included. Intra- and post-operative protocol (blood transfusion if Hb < 9 g/dl, rehydration, oxygenotherapy) was standardized. RESULTS: Forty-two patients with sickle cell of types SS-33 and AS9 were operated upon by same surgeon. One case of conversion due to an effraction of biliary junction was reported. One homozygote patient died post-operatively because of peritonitis. Two acute thoracic syndromes, three vaso-occlusive crisis, and two cases of wound infection constituted the post-operative morbidity. No case of complication was noted in those who underwent pre-operative transfusion. CONCLUSION: Laparoscopic cholecystectomy can be carried out in sickle cell patients with biliary lithiasis provided that general anaesthetic rules are respected.