Anesthetic Challenges for a Pediatric Leukemia Patient Undergoing Total Body Irradiation (TBI): A Case Report.

Radiotherapy of the whole body is called total body irradiation (TBI). It is a well-established component of conditioning regimens before stem cell transplantation in juvenile leukemia. The patient was a three-year-old child with a diagnosis of B-cell acute lymphoblastic leukemia and planned for stem cell transplantation. He was given TBI under anesthesia for three consecutive days prior to the bone marrow transplantation under general anesthesia. The important concerns were related to neutropenia/immune suppression, parental consent for repeated anesthesia, nothing per oral guidelines for the TBI treatment, the possibility of high-grade fever, high chance of respiratory tract infections with repeated anesthesia exposure, etc. Proper preparation, teamwork, and collaborative efforts and the child's parents made this treatment possible with intended success.

Slipped Capital Femoral Epiphysis Associated With Vitamin C Deficiency in a 7-year-old Boy.

Scurvy is rare in the present world and is mostly found in children with abnormal dietary habits and physical and mental disabilities. Scurvy can present in various forms, mimicking several common diseases, thus making the diagnosis difficult. Spontaneous epiphyseal separation is known to occur in scurvy, although rarely reported. The usual locations of these epiphyseal separations are distal femur and proximal humerus. Our case is unique in that scurvy in a seemingly normal child resulted in proximal femur epiphyseal separation which was not reported previously. We report a case of a 7-year-old boy presenting with pain and swelling in multiple joints for 6 months and later inability to walk. Pseudoparalytic frog-leg posture, dietary history of selective eating, and typical radiologic features made us consider a diagnosis of scurvy which was confirmed by a low serum vitamin C level. He developed epiphyseal separation of proximal femur and was treated with percutaneous screw fixation. Vitamin C supplementation resulted in prompt improvement clinically and radiologically.

SKINTED: A Rare Complication After Total Knee Arthroplasty.

BACKGROUND: Surgery of the knee, injury to the infrapatellar branch of the saphenous nerve, traumatic eczematous dermatitis is a neuropathic dermatitis specific to total knee arthroplasty (TKA), occurring around the healed surgical scar area. Very few case reports exist in orthopaedic literature regarding this rare skin complication after TKA. We report a series of cases and estimated the incidence of this condition in our institute. METHODS: During the 1-year period from January 2018 to December 2018, patients who have undergone TKA and later presented with skin lesions adjacent to the operated site were identified. Detailed history was taken, and full clinical examination was performed for all the reported cases. RESULTS: A total of 9 lesions in 8 patients were identified out of a total of 203 consecutive TKAs operated during the study period, with an estimated incidence of 4.4%. The mean age was 64 years (range, 58-78 years). The mean time from surgery to diagnosis was 4 months (range, 3-6 months). CONCLUSIONS: This group of dermatitis caused due to surgical transection of the infrapatellar branch of the saphenous nerve during TKA is a rare cutaneous complication, with an estimated incidence of 4.4% from this study. Lesions typically appear lateral to the operative scar within an area of hypoesthesia. Lesions in all patients improved after topical steroid therapy with no recurrences at further follow-up. Arthroplasty surgeons should have awareness of this benign complication, thereby avoiding unwarranted additional workup and alleviating unnecessary psychological stress to the patient.

Pharmacokinetics of mycophenolate mofetil in Omani patients on cyclosporine or tacrolimus.

BACKGROUND: Mycophenolate mofetil (MMF) reduces rejection in organ transplantation and is effective in controlling autoimmune diseases. Determining the area under the receiver operating characteristic curve (AUC) of MMF is not routine despite interindividual variability. The aim of this study was to look into MMF-AUC in Omani patients on cyclosporine (CsA) or tacrolimus (Tac). METHODS: We measured MMF-AUC in 27 stable Omani patients. We used the 4-time-points-limited sampling strategy. RESULTS: Sixteen, 8, and 3 recipients were receiving Tac, CsA, and calcineurin inhibitor (CNI)-free regimens, respectively. The mean MMF dose was 1,685 ± 442 mg/d. All except 3 patients were within or above the target AUC. The AUC was significantly higher in patients receiving Tac versus CsA. All patients on Tac were within or above the target AUC. Seven of the 8 patients with MMF-AUC above target were receiving Tac. Two of the 3 patients with MMF-AUC below target were on CsA. DISCUSSION: These preliminary results confirm the significant interindividual variability of MMF-AUC. None of the recipients on Tac had an AUC below target. Most patients above the target AUC were receiving Tac. Two of the 3 patients with MMF-AUC below target were on CsA and receiving 1 g/d MMF. Patients on Tac are at risk of having higher than the target AUC, exposing them to overimmunosuppression. Recipients on CsA seem to require >1 g/d MMF. CONCLUSIONS: These preliminary results encourage measuring the AUC of MMF. The fixed MMF dose regimen seems to be unreliable to predict the AUC. Preventing under- or overimmunosuppression offsets the burden.

Deceased donor renal transplantation and the disruptive effect of commercial transplants: the experience of Oman.

The Oman Renal Transplantation Program was established in 1988 as a joint venture between Sultan Qaboos University and the Ministry of Health. It began with both living related donor (LRD) and deceased donor (DD) transplants. Over the next nine years, while the LRD programme progressed relatively well, there were only thirteen DD transplants. Two of the DD kidneys were obtained from overseas via an active collaboration with the Euro-transplant organisation, and one DD kidney was obtained from Saudi Arabia within the Gulf Cooperative Council exchange programme. The rest of the DD kidneys were obtained in Oman. The Omani DD programme, although it was a pioneering effort in the Gulf region at the time, was not entirely sustainable. In this paper we focus on the challenges we encountered. Among the major challenges was the absence of resources to establish a dedicated DD programme and particularly the failure to develop a cadre of dedicated transplant coordinators.

Metabolic syndrome performs better than the individual factors in predicting renal graft outcome.

AIM: Individual components of the metabolic syndrome (MS), especially obesity and hypertension, have a deleterious effect on renal graft outcome. Whether MS is better than its individual components in predicting the decline of renal function is unknown. We studied the presence of MS and its individual components at 12 months post-transplantation according to the Adult Treatment Panel III classification and their influence on measured graft function. METHODS: A cohort of 322 patients who underwent transplantation between 1996 and 2003 and who agreed to have their glomerular filtration rate (GFR) measured by urinary clearance of technetium 99m (Tc*-DTPA) (measured GFR [mGFR]) at 3, 12, 48, 60, and 96 months after transplantation were included. The patients were followed up until patient death, graft loss, or December 2009 (mean follow-up: 3 ± 2.8 years). The linear mixed effect model for longitudinal repeated measures was applied. To compare MS versus its components we used the Akaike information Criterion (AIC) to determine the best model according to the Anderson and Burnham method. RESULTS: Univariate and multivariate analyses models using MS were more efficient than those using the individual components, which consisted of waist circumference, low high-density lipoprotein-cholesterol, hypertriglyceridemia, hyperglycemia, and systolic and diastolic blood pressure. The AIC was the lowest with MS models indicating better prediction on graft function than the individual components. CONCLUSION: MS is a better predictor of mGFR decline than its individual components. It is a valid and precious tool to assess outcomes.

Different classifications yield variance in metabolic syndrome prevalence and dynamics in renal transplant recipients.

INTRODUCTION: The metabolic syndrome (MS) is a combination of factors that are associated with increased cardiovascular diseases. High MS prevalence was reported in renal transplant recipients. However, little is known about the longitudinal prevalence and its dynamic properties in this population. We studied the longitudinal prevalence of MS at 3 and 12 months post-transplantation using 3 classifications. PATIENTS AND METHODS: We studied a cohort of 322 patients who underwent transplantation between 1996 and 2003 who had isotopic measurement of transplant glomerular filtration rate and MS assessment at 3 and 12 months after transplantation. Prevalence and change of MS status in terms of acquisition or regression were analyzed based on World Health Organization (WHO), International Diabetic Federation (IDF), and The Adult Treatment Panel-III (ATP-III) classifications. RESULTS: The prevalences at 3 and 12 months were as follows: WHO, 8.4% and 8.1%; IDF, 25.8% and 29.8%; and ATP-III, 34.3% and 36.6%, respectively. Change in MS status was noted in 9.7%, 16.4%, and 20.5% of subjects within WHO, IDF, and the ATP-III classifications, respectively. Prevalence was significantly lower with WHO than IDF and ATP-III. Prevalence was the highest with ATP-III. However, the difference with IDF was significant only at 3 months post-transplantation. Depending on the classification used, 10%-21% of subjects change MS status within the first year of transplantation. CONCLUSION: Longitudinal analysis confirms the high prevalence of MS and also highlights the dynamics of MS. We think both prevalence and dynamics should be accounted for when studying outcomes.

Attitude of the Omani population toward organ transplantation.

UNLABELLED: Transplantation from deceased donors is still scarce in Oman, mainly due to family refusal. We conducted a survey to learn the attitudes of the Omani population regarding transplantation. SUBJECTS AND METHODS: Among 500 individuals who were distributed, a questionnaire 304 responded including 247 (81%) Omani and 57 (19%) foreign residents. There were 213 (70%) male respondents of the 304 subjects, 256 individuals (84%) were between 18 and 50 years of age, and 270 (89%) had at least a high school education. RESULTS: Thirty-eight percent and 32% of Omani individuals had a family member or a friend with kidney disease or a renal transplantation, respectively. Only 42% of respondents knew that renal transplantation is performed in Oman. It was encouraging to note that 65% of Omanis knew that commercial transplantations are against Islamic and international standards. Sixty-four percent of the respondents stated that they would donate a kidney to a relative with renal failure. Nevertheless, only 49% knew that donation after death is permitted by Islam; 42% respondents would accept a kidney from a deceased person. Only 35% would donate a kidney or an organ after death. We concluded that the awareness of the Omani people toward donation after death is low, with a great need for public education and awareness programs, particularly for high school and university students.

Emphysematous pyelonephritis: a case report series of four patients with review of literature.

Emphysematous pyelonephritis (EPN) is an acute necrotizing infection with gas presence in the kidney, perinephric space, and/or urinary collecting system that carries a bad prognosis. Some clinical conditions predispose to this entity, such as diabetes mellitus, urinary tract obstruction, and immune-incompetence. Immediate resuscitation, broad-spectrum antibiotics, percutaneous and surgical drainage, and emergent and delayed nephrectomy are therapeutic options that should be applied in a timely fashion. We report our experience of four patients with EPN. Two of the patients were kidney transplant recipients, one patient had bilateral urolithiasis, and one patient was an elderly patient with debilitated general condition and an abdominal mass that could not be defined. Late transplant nephrectomy was performed in one patient, and three patients were treated conservatively. Three patients died, including the patient who had transplant nephrectomy. One patient who presented with lithiasis showed a remarkable recovery with conservative management. The bacteria involved were E. Coli and a resistant Klebsiella. We conclude that EPN is a life-threatening condition that carries a bad prognosis. Early diagnosis is essential for a positive outcome. Therapeutic measures should be applied immediately after diagnosis. An aggressive approach including nephrectomy may be emergently required.

Acute tubular necrosis associated with non-hemorrhagic Dengue fever: a case report.

Dengue fever (DF) is an arthropod-born viral infection affecting humans. Dengue viruses are transmitted through the bites of the mosquito Aedes aegypti. Acute renal failure (ARF) is reported in patients who are affected mainly with Dengue hemorrhagic fever (DHF), which is a severe presentation of the disease. We report the case of a 24-year-old Omani female with no past history of particular medical problems. She was referred to our hospital for the further management of acute renal failure. She had clinical features of DF without DHF. The kidney biopsy showed features of acute tubular necrosis (ATN). She had a complete recovery after 25 days and required three sessions of hemodialysis. We conclude that DF even without DHF may lead to ATN and ARF. Clinicians should be aware of this etiology. Treatment is supportive and may require dialysis. The prognosis could be favorable.

Femoral vein tunneled catheters as a last resort to vascular access: report of five cases and review of literature.

Vascular access (VA) remains a thorny problem in at least some patients requiring hemodialysis (HD). When options like arterio-venous fistulae, grafts, and thoracic tunneled central catheters are exhausted, the patient survival becomes endangered. The choices left are limited to transplantation, peritoneal dialysis, and translumbar and femoral catheter insertion. The latter may, in many instances, be the only possibility. We report our experience in six patients, in whom all the vascular accesses were exhausted and transplantation or peritoneal dialysis could not be performed. Hence, we opted for femoral tunneled catheters (FTC) as a permanent and sole VA. The mean follow up period was 16 months (8-22 months). The mean age of the patients was 53.1 (35-72) years, the mean time on HD was 3.125 years (2-4.5), and the mean number of vascular accesses was 4.7 (4-7). In five patients, a Permcath (Quinton, Kendall) was inserted, and in one patient, a twin Tessio catheter (MPS, Germany) was inserted. All patients were administered aspirin or clopidogrel to prevent catheter thrombosis. Aseptic nursing procedures and personal hygiene were emphasized. All catheters were functional at 2.5 months. The mean blood flow was 220 mL/min (200-240 mL/min). One patient died at 18 months, with a functional catheter, due massive gastro-intestinal bleed not directly related to the FTC. In one patient, the site of the catheter was changed at 6.5 months due to accidental pulling of the catheter by the patient. One catheter flow became inadequate after nine months, which could be reversed with streptokinase or tissue plasminogen activator. One patient presented excessive bleeding at the time of insertion that was controlled after 25 minutes of compression against the pelvic bone. One patient presented deep vein thrombosis with catheter thrombosis that required reinsertion of the catheter three months later. In one patient, the catheter functioned for 19.5 months after insertion. It is noteworthy that no patient developed catheter-related septicemia. None of the patients developed late hemorrhage. We conclude that tunneled femoral catheter is a viable option in patients with exhausted VA. Strict aseptic nursing technique and personal hygiene are essential. A multi-center study would give a better insight into this type of VA.

Selenium intoxication with selenite broth resulting in acute renal failure and severe gastritis.

Selenium (Se) is an essential trace element in human and animal nutrition. It is also widely utilized in industrial processes. Reports of acute selenium toxicity in humans are rare. We report a case of a 23-year-old female who consumed about 100 mL of liquid selenite broth and presented with severe nausea, vomiting, abdominal pain, hematemesis and acute renal failure (ARF). The serum selenium level was significantly increased. Gastro-duodenoscopy revealed severe corrosive gastritis. Renal biopsy showed features of acute tubular necrosis (ATN), affecting primarily the proximal tubules. The patient was managed with gastric lavage, blood transfusions, infusion of fresh frozen plasma (FFP) and platelet concentrates and hemo-dialysis. The patient was discharged five weeks after admission and her renal functions recovered completely by eight weeks after admission. She continues to be on regular follow-up for any possible sequelae of mucosal corrosive damage. This case highlights a case of selenium intoxication from selenite broth resulting in ARF and corrosive gastritis. The recovery was complete.

Donor gender balance in a living-related kidney transplantation program in Oman.

It has been observed in several Eastern and Western countries that there is a gender imbalance among kidney donors. In the international experience, approximately 65% of live kidney donors have been women. We retrospectively studied the distribution of female and male donors or recipients among living kidney transplantations performed from 1980 to 2005, namely 198 Omani recipients of living-related kidney transplantations. To examine cultural influences, transplantations performed or expatriates were excluded from the study. For the whole period, 98 out of 198 donors (49.5%) were women. The number of female recipients 75 of 198 (38%) versus males were 123 (62%) recipients. We then subdivided the period into three intervals: 1980 to 1990, 1991 to 2000, and 2001 to 2005. The numbers of female donors for these three periods were 29 of 64 (45%), 42 of 89 (47%), and 27 of 45 (60%), respectively. There was a persistent preponderance of male recipients ranging from 58% to 66% during these periods. We concluded that there was no gender imbalance for kidney donors. Nevertheless, there was a male preponderance in the recipient group.

Complete regression of cutaneous B-cell lymphoma in a renal transplant patient after conversion from cyclosporin to sirolimus.

Posttransplant lymphoproliferative disease remains a serious morbidity. Herein we have reported a case of complete regression of a biopsy-proven B-cell lymphoma that occurred in the posttransplant period. A 48-year-old man received a living donor renal transplant for end-stage renal disease due to undetermined etiology. His initial immunosuppression consisted of corticosteroid, mycophenolate mofetil, and cyclosporin. The patient developed severe pneumonia within the first 2 months after transplantation due to Acineotobacter, fungus, and cytomegalovirus infections. He experienced a complete recovery and was discharged for regional follow-up. Four months after discharge, he was referred again because of presence of two nodules on his trunk. A biopsy of the nodules revealed B-cell lymphoma. Cyclosporin was stopped and he was converted to sirolimus. The lesions regressed progressively and completely within 7 weeks. The patient remains well without clinical relapses at 19 months after conversion. Renal functions remained stable. We postulated that the antincoplastic properties of sirolimus may have played an active part in the positive outcome.

Retinal injury as an early manifestation of posttransplant thrombotic microangiopathy: recovery with plasma exchanges and conversion to sirolimus--case report and review of the literature.

Thrombotic microangiopathy (TMA) remains a serious event. We report a case of occurrence of TMA in the immediate postoperative period after a living-related renal transplantation. A distinguished feature of the case was major, early involvement of the retina with marked by decreased visual acuity along with thrombocytopenia and renal functional impairment. The syndrome was reversible with plasma exchange and conversion from tacrolimus to sirolimus. The patient's renal function is excellent at 11 months after transplantation. Decreased visual acuity and cotton wool exudates may be the first manifestation of posttransplant TMA.

Low tacrolimus dose requirements in renal transplant recipients in the omani population: implications for pharmacogenetics?

Clinical observations suggested that the dose requirements for some immunosuppressive drugs might be lower in some Eastern populations. We performed a study of 50 among 162 patients who were converted from a cyclosporine-based immunosuppression regimen to one based on tacrolimus. The reasons for conversion were as follows: acute rejection (n = 23), hirsutism and gingival hypertrophy (n = 12), chronic allograft nephropathy (n = 4), hypertension (n = 3), and other reasons (n = 8). Target levels were 10-15 ng/mL for patients with acute rejection and 5-8 ng/mL for patients after the first year of transplantation. We measured 180 tacrolimus blood levels in 44 patients using the IMx Abbott system of mean weight 64.5 kg and mean daily dose of 4.89 +/- 2.56 mg with mean blood levels of 11.31 +/- 5.22 ng/mL. Thus, to achieve the above-mentioned levels the average dose was 0.076 mg/kg, which is lower than that generally recommended to achieve 5-8 ng/mL levels, namely, 0.15 mg/kg. In this study, we did not address the reasons for these findings. The possibilities include dietary factors influencing the absorption of tacrolimus, and genetic polymorphisms in the Omani population that affect drug levels through several possible pharmacogenetic/genomic mechanisms. We intend to perform such studies in the future.

High incidence of early posttransplantation diabetes mellitus in an eastern population.

The prevalence of diabetes mellitus (DM) in the Gulf region is among the highest in the world. In the general population of Oman, the rate is approximately 11.7% with an additional 6.1% of the population having an abnormal glucose tolerance. This study reviewed the data for 162 adults who received kidney transplants between 2001 and 2004. The immunosuppression regimen was cyclosporine, mycophenolate mofetil (MMF), and steroids. The mean age of the group was 40.2 years. Twenty-two patients (13.6%) had DM prior to transplantation. Within the first 2 months after transplantation, 45 (32%) of the remaining 140 patients required insulin, and 10 (7.1%) required oral agents. A further 16 patients (11.4%) displayed blood glucose levels >11 mmol/L, but required only a special diet for control. The data indicate that 50% of recent adult kidney recipients in Oman receiving cyclosporine develop posttransplantation DM. This major problem in our transplant population requires special attention. Protocols with minimal steroid use and/or steroid withdrawal may be beneficial for the Oman kidney recipient population.

Complete regression of visceral Kaposi's sarcoma after conversion to sirolimus.

The prevalence of Kaposi's sarcoma (KS) is much greater in organ transplant recipients than it is in the general population. Its etiology appears to be related to geographic, genetic, and viral factors. Treatment of transplant-related KS has, until now, consisted mainly of reduction of, or withholding of, immunosuppression, often with deleterious effects on both graft and patient survival. In recent years, the immunosuppressive drug, sirolimus, has been demonstrated as possessing anti-neoplastic properties in both in vitro and animal models. In view of these properties and some preliminary clinical experience, we postulated that sirolimus would be beneficial in our patients who developed transplant-related KS. Here, we report the first case of a patient with both cutaneous and visceral KS who was successfully treated in the Middle East by conversion from a cyclosporine-based to a sirolimus-based immunosuppression regimen. The KS regressed completely within a few months after the conversion. The chronologic events and the extensive documentation, which included repeat computed tomography scans, are very suggestive of a selective anti-neoplastic effect of sirolimus.