When the earth trembles in the Americas: the experience of Haiti and Chile 2010.

The response of the nephrological community to the Haiti and Chile earthquakes which occurred in the first months of 2010 is described. In Haiti, renal support was organized by the Renal Disaster Relief Task Force (RDRTF) of the International Society of Nephrology (ISN) in close collaboration with Médecins Sans Frontières (MSF), and covered both patients with acute kidney injury (AKI) and patients with chronic kidney disease (CKD). The majority of AKI patients (19/27) suffered from crush syndrome and recovered their kidney function. The remaining 8 patients with AKI showed acute-to-chronic renal failure with very low recovery rates. The intervention of the RDRTF-ISN involved 25 volunteers of 9 nationalities, lasted exactly 2 months, and was characterized by major organizational difficulties and problems to create awareness among other rescue teams regarding the availability of dialysis possibilities. Part of the Haitian patients with AKI reached the Dominican Republic (DR) and received their therapy there. The nephrological community in the DR was able to cope with this extra patient load. In both Haiti and the DR, dialysis treatment was able to be prevented in at least 40 patients by screening and adequate fluid administration. Since laboratory facilities were destroyed in Port-au-Prince and were thus lacking during the first weeks of the intervention, the use from the very beginning on of a point-of-care device (i-STAT®) was very efficient for the detection of aberrant kidney function and electrolyte parameters. In Chile, nephrological problems were essentially related to difficulties delivering dialysis treatment to CKD patients, due to the damage to several units. This necessitated the reallocation of patients and the adaptation of their schedules. The problems could be handled by the local nephrologists. These observations illustrate that local and international preparedness might be life-saving if renal problems occur in earthquake circumstances.

Comparison of nutritional status in hemodialysis patients with and without failed renal allografts.

BACKGROUND: The survival of patients returning to hemodialysis (HD) following kidney transplant failure is unfavorable. However, the factors responsible for this poor outcome are largely unknown; chronic inflammation due to failed allograft and malnutrition may contribute to morbidity and mortality. We aimed to compare the nutritional status and its relation with inflammation in patients on HD with and without previous kidney transplantation. METHODS: Forty-three patients with failed renal allografts (27 males; mean age 36±9 yr) and 40 never transplanted HD patients (24 males; mean age 39±9 yr) were included in the study. Body weight, triceps (TSF), biceps (BSF), subscapular (SSSF), and suprailiac skinfold thicknesses (SISF); mid-arm, mid-arm muscle, hip and waist circumferences; as well as body mass indices (BMIs) were determined as anthropometric parameters. Moreover, biochemical markers of nutritional status, including serum cholesterol and albumin as well as high-sensitive C-reactive protein (hs-CRP), as a marker of inflammation, were measured. Associations among these variables were analyzed. RESULTS: There were no significant differences considering age, gender or duration of renal replacement therapy between the two groups. The TSF (p<0.0001), BSF (p=0.005), SSSF (p=0.001), SISF (p<0.0001) skinfold thicknesses; mid-arm (p=0.003) and mid-arm muscle circumferences (p=0.037) and BMIs (p=0.001) of the patients with failed renal allografts were significantly lower than those of the never transplanted HD patients. Waist circumference was significantly lower as well (p=0.028). Patients with failed transplants were characterized by lower serum albumin (p<0.0001) and higher hs-CRP levels (p=0.001) as compared with never transplanted HD patients. CONCLUSIONS: This study confirms the concept that retained failed allografts may induce chronic inflammation in chronic HD patients which may result in a worse nutritional status.

Panel Reactive Antibody Responses Against Influenza Vaccination in Kidney Transplant Recipients.

INTRODUCTION: Seasonal influenza is an important cause of morbidity and mortality in the post-transplant period; therefore, the influenza vaccination has been recommended for all kidney transplant recipients before the influenza season. However, at least theoretically, the introduction of antigens via vaccines may trigger rejection attacks by causing an antibody response. In this study, we examined the development of de novo panel reactive antibody (PRA) development against the influenza vaccine in kidney transplant recipients. MATERIALS AND METHODS: Overall, 41 kidney transplant recipients who received the influenza vaccination and 50 kidney transplant recipients (study group) who refused to receive the influenza vaccination (control group) were enrolled in the study. Following basal biochemistry examination, the inactivated trivalent influenza vaccine was administered intramuscularly. Panel reactive antibodies were screened in all patients before and after vaccination on days 30 and 180. The primary outcome variable was development of de novo panel reactive antibodies. RESULTS: One patient in the study group developed de novo class I and II PRA at 6 months after vaccination (P > .05), while no antibody development was noted in the control group. Graft dysfunction or biopsy-confirmed rejection was not observed during the follow-up period in both groups. CONCLUSION: The influenza vaccination is generally effective and safe in solid organ transplant recipients. The vaccination procedure has the potential to trigger antibody development and occurrence of rejection. Therefore, vaccinated kidney transplant recipients should be monitored more carefully with regard to PRA; if the graft deteriorates, a rapid transplant biopsy should be performed.

Relevance of Flow Cytometric Auto-Crossmatch to the Post-transplant Course of Kidney Transplant Recipients.

INTRODUCTION: The crossmatch test is essential prior to kidney transplantation (tx) to confirm compatibility between the donor and the recipient. However, its results can be misleading due to "undetectable antibodies" in the recipient's serum. To establish if undetectable autoantibodies are responsible for a positive result, an auto-crossmatch test can be performed. In this study, we aim to determine the long-term prognostic value of auto-flow cytometric auto-crossmatch (FCXM) test on kidney survival in kidney tx recipients. MATERIALS AND METHODS: The primary outcome variable was reduced renal function. Secondary endpoints were incidence of biopsy-confirmed chronic antibody-mediated rejection (CAMR) and recurrent glomerulonephritis (GN). RESULTS: There were no differences regarding initial serum creatinine levels between the study and control groups (P = .441). Patients who had positive auto-B FCXM had a significantly reduced renal function compared with the control group (P = .016). Four patients developed biopsy-confirmed CAMR in the study group and 1 patient in the control group (P = .047). Five patients had biopsy-confirmed recurrent GN in the GN study group, and only 1 patient had recurrent GN in the GN control group (P = .026). DISCUSSION: Kidney transplant recipients with positive auto-FCXM test had significantly reduced renal function and a higher incidence of recurrent GN and CAMR compared with the control group. The findings of this study suggest a potential role of auto-antibody causing positive auto-FCXM test result, meanwhile increasing the risk of CAMR, recurrent GN, and new-onset diabetes after tx.

A Novel Biomarker for Post-Transplant Recurrent IgA Nephropathy.

BACKGROUND: The serum levels of galactose-deficient immunoglobulin (Ig)A1 (Gd-IgA1) represent the most promising candidate biomarker for IgA nephropathy (IgAN). The aim of this study was to evaluate the serum levels of Gd-IgA1 as a novel noninvasive biomarker for post-transplant IgAN recurrence. METHODS: Serum Gd-IgA1 levels of 18 patients with recurrent IgAN were compared with control renal transplant recipients (n = 23) with non-recurrent IgAN and control non-transplant IgAN patients (n = 44) and healthy relatives (n = 11). Serum Gd-IgA1 levels of patients were measured with the use of KM55 enzyme-linked immunosorbent assay (ELISA). The effects of serum Gd-IgA1 concentrations on IgAN recurrence, post-transplant events, and graft survival were evaluated. RESULTS: All recurrent IgAN patients presented with renal dysfunction (mean serum creatinine, 1.62 ± 0.39 mg/dL) and detectable proteinuria at the time of diagnosis. Serum Gd-IgA1 levels of recurrent IgAN patients (8735 ± 10854 ng/mL [log10: 3.71 ± 0.45]) were significantly higher than those of non-recurrent IgAN patients (4790 ± 6089 ng/µL [log10: 3.31 ± 0.64]) (P = .027). Serum Gd-IgA1 levels of non-transplant IgAN patients were significantly higher (8791 ± 8700 ng/µL [log10: 3.79 ± 0.36]) than those of non-recurrent IgAN patients (4790 ± 6089 ng/µL [log10: 3.31 ± 0.64]) and healthy relatives (2615 ± 1611 ng/µL [log10: 3.34 ± 0.27]) (P < .001 and P = .021, respectively). Receiver-operating characteristic curve analysis revealed that the area under the curve for recurrence of IgAN was 0.69 (0.53-0.85) for serum Gd-IgA1 (P = .038). Biopsy-confirmed allograft rejection rates were similar in the recurrent IgAN group [3 (17%)] compared with the non-recurrent IgAN [6 (26%)] group (P = .47). Graft failure rate was not also significantly different in the recurrent IgAN group [4 (22.2%)] compared with the non-recurrent IgAN group [2 (8.7%)] (P = .224). CONCLUSIONS: This novel lectin-independent Gd-IgA1 ELISA that can detect serum Gd-IgA1 in patients with recurrent IgAN can be used as a biomarker for diagnosis and activity assessment of post-transplant recurrent IgAN.

Utilization of elderly donors in living related kidney transplantation.

Kidney transplantation has become the treatment of choice for end-stage renal disease. However, its application is limited due to inadequate organ supply, mainly because many dialysis patients do not have suitable living donors. The increasing discrepancy between organ supply and demand has forced many transplant centers to consider using organs procured from marginal donors. The aim of this study was to investigate whether utilization of kidneys from living related elderly donors is safe for the recipients in the long term. We analyzed the clinical results of 296 consecutive recipients of living related renal transplants, among whom 44 recipients received kidneys from donors over 60 years of age. By the end of 12 months, the mean serum creatinine level of the recipients who were transplanted from the older donors was higher (1.55 +/- 0.45 mg/dL) than that from other donors (1.21 +/- 0.3 mg/dL), but the difference was not significant (P = .08). In the long term (60 months), the graft function was similar (1.88 +/- 0.55 vs 1.52 +/- 0.38) for both groups. The similarity in outcomes of ideal versus older donors as shown less in the present series has encouraged us to utilize elderly living donors. We concluded that transplantations performed from the elderly donors yielded similar results to those of conventional donors. The long waiting list for transplantation, the treatment of choice for end-stage renal disease, should encourage us to be more flexible about donor selection.

Health-related quality of life after kidney transplantation in comparison intermittent hemodialysis, peritoneal dialysis, and normal controls.

The purpose of this study was to compare the quality of life (QOL) in renal transplantation patients. QOL is one of the important indicators of the effects of medical treatment. In this cross-sectional study, QOL was analyzed in 302 renal transplant recipients compared with 64 hemodialysis (HD) patients, 207 (PD) peritoneal dialysis patients, and 278 normal controls (NC) matched as closely as possible to the grafted patients regarding age, gender, education, and occupation. All groups were asked to estimate their subjective QOL by responding to sociodemographic data, Turkish adapted instruments of the Nottingham Health profile (NHP), and the Short-form 36 (SF-36). Transplant recipients were significantly younger than the HD and PD patients (P < .0001). There was no statistically significant differences between normal controls and transplant patients ages. Among the three renal replacement methods, QOL in transplants was clearly better than that in HD or PD patients (P < .0001). The QOL measured by the NHP and SF-36 scale showed that the normal population was statistically significantly better than the transplant recipients (P < .0001). Transplant renal replacement therapy provides a better QOL compared with other replacement methods.

Isolated hepatic tuberculous abscess in a renal transplant recipient.

Tuberculous liver abscess is rare worldwide. We report a 26-year-old renal transplant recipient who presented with fever, fatigue, and weight loss. Ultrasound (US) of the abdomen showed a cystic mass of 7x6 cm in the subcapsular region of right liver lobe. US-guided percutaneous drainage was performed and 100 mL of yellow-colored pus was aspirated. The patient was empirically started on ampicillin sulbactam treatment. Despite this treatment, the symptoms persisted. Subsequent control abdominal US showed the persistence of a cystic mass of 7x6 cm with thin septation in the subcapsular region near the right liver lobe, which were subsequently diagnosed as a focal hepatic tuberculous abscess by positive culture in Löwenstein-Jensen medium. He was concomitantly started on systemic antituberculous therapy. A tuberculous liver abscess must be considered in the differential diagnosis. Percutaneous drainage along with systemic antituberculous chemotherapy must be considered as an alternative to surgery for the management. A greater awareness of this clinical entity is required for successful treatment.

Demographic analysis and outcome features in a transplant outpatient clinic.

AIM: This retrospective report analyzed the demographic features of kidney transplant outpatients grafted in Turkey and in different regions of the world. METHODS: The patients were divided into 4 groups: Every cohort was subgrouped as either receiving a cadaveric (CT) or a live donor transplant (LT) performed in our institute (ICT, n = 147, ILT, n = 304), other institutions in Turkey (TCT, n = 16, TLT, n = 86), or in a foreign country (FCT, n = 66, FLT, n = 154). RESULTS: Among the patients with functional grafts, FLT transplantations, of which the vast majority were paid transplantations, constituted the second largest group (n = 105) following ILT (n = 214). In the ILT group, 5- and 10-year graft survival rates were 76% and 50%, respectively, and patient survival rates were 88% and 78%, respectively. Considering the FLT group, 5- and 10-year graft survival figures were 70% and 42%, respectively, while patient survival rates in these periods were 81% and 69%, respectively. Patients with paid donor transplantations were characterized by a high risk of unconventional infectious complications in the early period, while midterm patient and graft survival was somewhat better than expected considering the high rate of complications in the early period. CONCLUSIONS: Paid organ transplantation should be discouraged. Organ donation should be stimulated by every means to avoid potentially fatal unconventional infections after transplantation from paid donors. If patients receive a paid transplant, however, they should be closely followed for these complications, since, if they survive the early period the midterm and long-term outcomes are quite reasonable.

Renal transplantation in a patient with chronic granulomatous disease: case report.

Chronic granulomatous disease (CGD) is a genetic disease caused by structural mutations in the enzyme NADPH oxidase that results in severe immunodeficiency. End-stage renal disease occurs in this patient population and is attributed to various factors, including infections, amyloidosis, and nephrotoxic anti-infective agents. In this report, we present our experience in transplantation for a patient with CGD complicated by isolated hepatic tuberculosis abscess. The course of the case demonstrates the absolute requirements for a multidisciplinary and compulsive approach before, during, and after transplantation. This case report also highlights the unexpectedly benign effects of immunosuppressive therapy in this patient population.

Pericarditis following renal transplantation.

We analyzed data on renal allograft recipients over a 27-year period in order to investigate the frequency, etiology, and outcome of pericarditis developing during the first two months following renal transplantation. Of the 1497 patients receiving renal transplants between 1963 and 1990, 34 patients developed 36 episodes of pericarditis and/or pericardial effusions, for an overall incidence of 2.4%. Pericarditis was attributed to uremia in 14 episodes, cytomegalovirus infection in three, both uremia and CMV infection in four, nonspecific bacterial infection in three, and tuberculosis and minoxidil therapy in one episode each. No etiologic diagnosis could be established in 10 episodes. No statistically significant differences were found between pericarditis and case-matched control patients considering demographic features, the number of immediately functioning grafts, the duration of posttransplant acute renal failure, the number of supportive dialysis days, pre- and postoperative CMV status of the patients, and pretransplant BUN and serum creatinine levels. There were more uremic-related complications (pulmonary edema, gastrointestinal bleeding, central nervous system symptoms) in the pericarditis group. Five allografts in the pericarditis group never functioned, versus only one in the control group. Three patients with pericarditis developed pericardial tamponade. Early diagnosis, close follow-up, and in the case of cardiac tamponade early invasive treatment, should improve the prognosis of this potentially life-threatening complication.

Increased QT dispersion in hemodialysis patients improve after renal transplantation: a prospective-controlled study.

Increased QT dispersion (QTd), predicting patients with risk of malignant arrhythmia, have recently been reported in hemodialysis patients (HDp). In this prospective study, we aimed to investigate changes in QTd and signal averaged-ECG (SAECG) in HDp after transplantation. Twenty-seven HDp (M/F:18/9, mean age 30+/-8 years) and 24 controls (M/F:14/10, mean age 33+/-6 years) were included. All QT parameters (QTmax, Qtmin, and QTd) were increased in HDp. QTmax and QTd started to decrease at the first month after transplantation. Percentage change in QTd at the third month was significantly correlated with percentage change in LV mass index (r=0.45, P=0.04), serum calcium (r=-0.47, P=0.02) and intact parathyroid hormone (r=0.68, P=0.01). In multivariate regression analysis, only percent chance in LV mass index was retained as significant. As for analysis of SAECG, 4 of the 23 (17%) HDp has abnormal late potentials which disappeared after transplantation. HDp with LV hypertrophy had higher filtered-QRS duration compared to patients without hypertrophy (110+/-12 vs. 97+/-11 msec, P=0.01). It was concluded that increased QTd and presence of late potentials improved early after renal transplantation. These changes were mainly associated with the regression of the LV mass.

Posttransplant malaria.

The frequency and clinical characteristics of plasmodium infection were reported in 420 renal transplant recipients who were followed in the Transplantation Unit and Out-Patient Clinic of the Medical School of Istanbul. Plasmodium infection was diagnosed in eleven (9 male, 2 female) of the 420 patients (2.6%). Ten of the patients were transplanted in India, and one in our institution. The mean duration between the transplantation and the diagnosis of malaria was 21.7 + 44.4 days in patients who were transplanted in India. All of the patients were taking triple immunosuppressive drugs (CsA, AZA, PRED). Plasmodium falciparum was diagnosed in 6 patients, P vivax in 1 patient and P malariae in 1 patient. Also mixed infection with P falciparum and P malariae was diagnosed in 3 patients. After definite diagnosis, the patients were hospitalized. Chloroquine phosphate plus primaquine phosphate was administered for P vivax infection, whereas chloroquine phosphate alone was given for P falciparum and P malariae infection as a first line antimalarial therapy. As a result of therapy, infection improved clinically and the plasmodia disappeared rapidly from the thick blood film in 10 of the patients. Severe hemolysis and acute renal failure developed in one patient, who improved after hemodialysis therapy and exchange transfusions. It was concluded that malaria is quite a frequent infection of transplant recipients who get their allografts from donors living in high-risk areas, and all transplant recipients having this kind of transplantations should be suspected and examined for malaria. This may help to diagnose and treat the complication in the early period, thus resulting in an improved prognosis for this potentially life-threatening complication of the posttransplant period.

Comparison of the effects of enalapril and losartan on posttransplantation erythrocytosis in renal transplant recipients: prospective randomized study.

BACKGROUND: The aim of this prospective randomized study was to compare the safety and efficacy of enalapril (E) and losartan (L) in the treatment of posttransplantation erythrocytosis and the effect of the ACE genotype on response to therapy. METHODS: Twenty-seven (24 male and 3 female, mean age 34+/-8 years) renal transplant recipients with erythrocytosis were treated either with E (15 patients) (10 mg/day) or L (12 patients) (50 mg/day) for 8 weeks. RESULTS: The hemoglobin levels were significantly decreased in the L (17.1+/-0.7 to 15.9+/-1.3 g/dl, P=0.01) and E groups (17.4+/-1.1 to 14.9+/-2.2 g/dl, P=0.001). Among the responders who discontinued treatment, there was a trend for longer time to relapse in the L group (7.38+/-3.75 months; 95% confidence interval: 0.03-14.7) compared with the E group (2.75+/-0.70 (95% confidence interval: 1.37-4.13) (P=0.11). Decrease in hemoglobin was more prominent with E compared with L (-3.26+/-0.65 vs. -1.70+/-0.39 g/dl, P=0.05). Decrease in hemoglobin levels between DD and non-DD genotype groups was similar (-2.0+/-1.5 vs. -1.7+/-2.3 g/dl, P=0.69). CONCLUSIONS: Enalapril caused a greater decrease but faster relapse in hemoglobin levels compared with losartan in patients with posttransplantation erythrocytosis. The DD type polymorphism had no effect on response.

The characteristics of infections in crush syndrome.

OBJECTIVE: To analyze the results of clinical and bacteriological examinations of crush syndrome patients admitted to our institute after the Marmara earthquake. METHODS: Data were collected retrospectively from patients' files. Forty patients are included. Their mean age was 31.5 +/- 13.5 years and 18 were male. Their mean time under the rubble was 13.1 +/- 14.4 h. Fasciotomies were performed on 41 extremities of 30 patients. One hundred and twelve (mean 2.9 +/- 1.9 samples/patient) bacteriological samples were collected from wounds (51), blood (23), urine (25) and catheters (13). RESULTS: Microbial growth was detected in 67 samples from 38 (95%) patients. Non-fermenting Gram-negative bacilli, Gram-positive cocci, Enterobacteriaciace and yeast-like fungi were isolated in 67%, 17%, 12% and 4% of the samples, respectively. Acinetobacter (36%) and Pseudomonas aeruginosa (21%), the major bacterial isolates from wound infections, were resistant to carbapenems and sensitive to quinolones. As the hospitalization period increased, other infections supervened. Gram-positive cocci and non-fermenting Gram-negative bacilli were detected in six blood and seven catheter samples and methicillin-resistant Staphylococcus aureus was the major isolate. Nine (22%) of the patients died due to sepsis despite all supportive therapies. CONCLUSIONS: Infections are still major factors in crush syndrome-related deaths.

No relation of tuberculin reactivity with quantitative analysis of peripheral blood lymphocyte subsets in haemodialysis patients.

Anergic response to tuberculin (PPD) is well known in haemodialysis patients (HDp). This cross-sectional controlled study was conducted to analyse the correlation of PPD response with demographical features, nutritional parameters and the distribution of peripheral blood lymphocyte (PBL) subtypes. In this study 29 HDp (17 men, 12 women; mean age 30.9 +/- 9.5 years) and 13 controls (eight men, five women; mean age 29.2 +/- 6.4 years) were included. The mean time spent on dialysis was 20.5 +/- 17.4 months. The mean PPD response was lower in HDp than controls (7.5 +/- 8 mm vs. 15 +/- 4 mm, P = 0.001). Fourteen patients (48%) were PPD (-) (eight men, six women; mean age 34.1 +/- 11.1 years) and 15 were PPD (+) (normergic) (nine men, six women; mean age 26.8 +/- 3.4 years). No difference was observed between PPD (-) and (+) groups for age, sex and time spent on dialysis. As nutritional parameters, body mass index, serum albumin, creatinine and cholesterol levels were measured and no differences were found between controls and the PPD (-) and (+) groups. Absolute lymphocyte counts were lower in HDP compared to controls (1290 +/- 296 vs. 1570 +/- 307 cells ml-1; P = 0.01). PBL subtype percentages and absolute counts (CD3, CD4, CD8, CD4/CD8, HLADR+CD3+, CD16+56+, CD19) were also similar between PPD(-) and (+) HDp. It was concluded that PPD response cannot be predicted by the distribution of PBL subtypes. The most probable cause of this observation is regulation of PPD reactivity by in situ immune cells whose composition is not reflected in the distribution of PBL.

Cardiac and intestinal amyloidosis in a renal transplant recipient with familial Mediterranean fever.

In Turkey, familial Mediterranean fever (FMF) is an important cause of nephrotic syndrome and endstage renal disease due to renal deposition of AA type amyloid. We report a case of living-related donor renal transplant recipient with FMF and renal AA type amyloidosis, who died of progressive heart failure due to cardiac involvement. The patient also had intractable diarrhea caused by biopsy-proven intestinal amyloidosis. The patient was on 1 mg/day colchicine. Although he was attack-free throughout the post-transplant period, intestinal and clinically significant cardiac amyloidosis, which implied the presence of sustained inflammation and continuing amyloid deposition, appeared three years after renal transplantation. Cardiac deposition of AA amyloid may cause clinically significant heart disease, leading to cardiovascular mortality after renal transplantation for end-stage renal disease in FMF patients.

Successful therapy combined with surgery for severe post-transplant nocardiosis.

We report a case of a 35-year-old man with nocardiosis infection involving soft tissue and the central nervous system who had received a cadaveric donor kidney. The patient was admitted with fever, malaise and right shoulder pain. Soft tissue abscess was seen on ultrasound examination. It was presumed due to gram (+) microorganisms, so 4 g day (IV) ampicillin/sulbactam was started empirically once the abscess was drained. Nocardia asteroides was found in the pus specimen. On the second day in hospital, severe headache, ataxia and signs of meningeal irritation appeared. The cranial CT showed two intracranial abscesses in the frontal lobe and cerebellum. We assumed Nocardia asteroides was the infective agent for the cerebral abscesses, so antibiotic therapy was switched to trimethoprim-sulphamethox-asole (3x160/800 mg/d). Nausea and vomiting occurred on the fifth day of therapy, improving after drainage from the frontal abscess. However, these complaints recurred five days later. CT showed cerebellar abscess had become bigger. The patient's complaints improved after the second surgical drainage. N. asteroides was again grown in the aspiration fluids of both cerebral abscesses. Complete regression of the abscesses was seen in the CT after two months. Co-trimoxazole was continued for six months then withdrawn. Graft dysfunction was not observed. Early medical and surgical interventions may be life-saving in this potentially lethal disease.