Abdominal operations after lung transplantation. Indications and outcome

OBJECTIVE: To assess the outcomes of abdominal operations in patients with lung transplants and identify adverse risk factors. DESIGN: Matched cohort study. SETTING: University referral center. PARTICIPANTS: Twelve lung transplant recipients who required abdominal operations (hereafter referred to as case patients) and 12 age-, sex-, and pulmonary diagnosis-matched lung transplant recipients who had not undergone an abdominal procedure (hereafter referred to as control patients). INTERVENTIONS: Elective abdominal operations including laparoscopic cholecystectomies (n = 5), laparoscopic repair of a colovaginal fistula (n = 1), and open colectomy for a benign colovesical fistula (n = 1) and urgent operations including bowel resections (n = 3), subtotal pancreatectomy (n = 1), and hepatorrhaphy for an iatrogenic liver injury (n = 1). MAIN OUTCOME MEASURES: Morbidity and mortality. RESULTS: Abdominal operations were performed in 12 (11%) of the patients undergoing lung transplantation at the university referral center since 1987, with an associated mortality rate of 25%. Morbidity and mortality rates of electively performed procedures were 28% and 14%, respectively. An urgent indication for abdominal procedure was associated with 100% morbidity and 40% mortality. Compared with a matched group of 12 control patients, the long-term survival of the case patients was reduced (18% vs 64% at 4 years). Case patients undergoing an abdominal procedure in the posttransplantation period tended to have a higher prevalence of previous rejection (67% vs 25%), a higher perioperative steroid dosage (53 mg/d vs 36 mg/d), and a significantly lower posttransplantational forced expiratory volume in 1 second (FEV1, 1.23 L vs 1.91 L; P < .05). CONCLUSIONS: Elective abdominal operations are relatively safe in properly prepared lung transplant recipients. However, laparotomy for urgent surgical conditions is associated with increased morbidity and mortality rates caused in part by the magnitude of the abdominal operation and influenced by the status of the lung transplant as manifested by previous rejection episodes, perioperative steroid dosages, and FEV1 values.     

Late outcome and quality of life after complicated heart operations

The coexistence of two cutaneous non-Hodgkin's lymphomas of different lineage is rare. We report a patient with an indolent erythrodermic cutaneous T-cell lymphoma followed by an aggressive B-cell lymphoma. To our best knowledge, this is the first report describing Epstein-Barr virus-associated B-cell lymphoma in a patient with cutaneous T-cell lymphoma. We suggest that the long-standing cutaneous T-cell lymphoma, as well as the long-term chemotherapy, suppressed host immunity and caused reactivation of latent Epstein-Barr virus.     

Long-term outcome after discharge from a stroke rehabilitation unit

The evidence is compelling that stroke units are effective when compared to management of patients on general medical wards. However, the evidence remains equivocal that better outcome is sustained in the longer term. This paper reports an investigation of cognitive and emotional outcome, environmental consequences, social activities, and physical outcome in 57 consecutive one-year survivors of a stroke discharged from a stroke and neurological rehabilitation unit. Satisfaction with inpatient and outpatient services was also investigated. Results were compared with previously reported studies of long-term outcome after stroke. Mean Barthel activities of daily living score at one year or more post-stroke was 16.8. Arm function was impaired in 43% of the participants in the study. Nearly half had cognitive and emotional problems, 19% communication problems, and 25% problems with access both inside and outside their house. All but four of the one-year survivors were less active after their stroke than before. More than 40% were dissatisfied with at least one aspect of inpatient and/or outpatient services. The long-term consequences of stroke in all areas investigated were considerable and in line with previous reports. Some suggestions for reducing these effects are made, including better information for patients about stroke and rehabilitation, improved access to psychology services, detailed assessment prior to hospital discharge of the patient's living environment and effective coordination with social services to improve access to their living environment.     

Long-term outcome after marrow transplantation for severe aplastic anemia

We reviewed the records and reevaluated 212 patients with aplastic anemia transplanted at the Fred Hutchinson Cancer Research Center (FHCRC) between 1970 and 1993 who survived >/=2 years and who have been followed for up to 26 years. Parameters analyzed included hematopoietic function, chronic graft-versus-host disease (GVHD), skin disease, cataracts, lung disease, skeletal problems, posttransplant malignancy, depression, pregnancy/fatherhood, and the return to work or school, as well as patient self-assessment of physical and psychosocial health, social interactions, memory and concentration, and overall severity of symptoms. Survival probabilities at 20 years were 89% for patients without (n = 125) and 69% for patients with chronic GVHD (n = 86) (the status was uncertain in 1 surviving patient). All patients had normal hematopoietic parameters. Skin problems occurred in 14%, cataracts in 12%, lung disease in 24%, and bone and joint problems in 18% of patients. Eleven patients (12%) developed a solid tumor malignancy and 19% of patients experienced depression. Chronic GVHD was the dominant risk factor for late complications. Seventeen patients died at 2.5 to 20.4 years posttransplant; 13 of these had chronic GVHD and related complications. At 2 years, 83% of patients had returned to school or work; the proportion increased to 90% by 20 years. At least half of the patients preserved or regained the ability to become pregnant or father children. Patients rated their quality of life as excellent and symptoms as minimal or mild. In conclusion, marrow transplantation in patients with aplastic anemia established long-term normal hematopoiesis. No new hematologic disorders occurred. The major cause of morbidity and mortality was chronic GVHD. However, the majority of patients who survived beyond 2 years returned to a fully functional life.     

Surgical management of sagittal synostosis: a comparative analysis of strip craniectomy and calvarial vault remodeling

Approximately 25% of patients with Fanconi anemia (FA) have evidence of spontaneously occurring mosaicism as manifest by the presence of two subpopulations of lymphocytes, one of which is hypersensitive to cross-linking agents (e.g. mitomycin C) while the other behaves normally in response to these agents. The molecular basis of this phenotypic reversion has not yet been determined. We have investigated 8 FA patients with evidence of mosaicism. Epstein-Barr virus-immortalized lymphoblastoid cell lines established from these patients exhibited an IC50 for mitomycin C of 25 to > 100 nM compared to a mean of 2 +/- 2 nM for 20 nonmosaic FA patients and 49 +/- 11 nM for 8 healthy controls. In 3 patients who were compound heterozygotes for pathogenic FAC gene mutations the molecular mechanism of the mosaicism was investigated by haplotype analysis. The results indicated that an intragenic mitotic recombination must have occurred leading to a segregation of a wild-type allele in the reverted cells and suggested two patterns of recombination. In 1 patient a single intragenic crossover between the maternally and paternally inherited mutations occurred associated with markers located distally to the FAC gene; in the other 2 patients (sibs) the mechanism appears to have been gene conversion resulting in segregants which have lost one pathogenic mutation. In 6 of the 8 patients the hematological symptoms were relatively mild despite an age range of 9-30 years.     

Long-term follow-up after bowel resection for necrotizing enterocolitis: factors affecting outcome

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common surgical emergency among newborns and is associated with a high morbidity and mortality. This study evaluates the long-term survival of infants requiring surgical intervention for NEC and factors affecting outcome. METHODS: A retrospective review of infants requiring surgery for complications of NEC at a tertiary care, pediatric hospital over a 16-year period was performed. Patients were evaluated for early and late morbidity and mortality, length of intestinal resection, presence of the ileocecal valve (ICV), days of parenteral nutrition (PN), and growth. RESULTS: Two hundred forty-nine patients were included, with an average gestational age of 30 +/- 5 (+/- SD) weeks and birth weight of 1.50 +/- 0.89 kg. The surgical mortality rate was 45%, with survivors (137) being larger (P < .001) and older (P < .001) at time of birth than nonsurvivors. Mortality rates varied inversely with gestational age and birth weight. Surgical survivors had an average of 21 +/- 26 cm of intestinal length resected. The ileocecal valve was preserved in 45% of infants. Growth was similar between infants with or without an ICV. Stratification of length of intestine resected showed that infants with larger resections had greater requirements for parenteral nutrition, but this had no influence on long-term growth at follow-up. CONCLUSIONS: Survivors of NEC are characterized by greater gestational age, greater birth weight, and older postgestational age at surgery. Infants who underwent greater intestinal resections required longer periods of PN. The length of intestine resected or presence of the ileocecal valve had no overall bearing on long-term outcome.     

Long-term neuropsychological outcome and loss of social autonomy after traumatic brain injury

OBJECTIVE: To assess which social activities were still impaired 5 years after a traumatic brain injury (TBI) in adults, and which neuropsychological impairments were associated with this loss of social autonomy. DESIGN: Cross-sectional study of 79 patients selected from the follow-up cohort of an epidemiologic survey of 2,116 TBI patients. SETTING: The present study was of ambulatory patients seen at hospital or at their homes. The inception cohort was from the trauma center of a university hospital and from a general hospital that is representative of level II trauma centers in Aquitaine, France. PATIENTS: Seventy-nine patients selected from a representative sample of 407 patients who were included in the 5-year follow-up study of the initial cohort (convenience sample). MAIN OUTCOME MEASURES: Glasgow Outcome Scale (GOS) and loss of social autonomy as assessed by the European Brain Injury Society's European Head Injury Evaluation Chart; assessment of neurobehavioral impairments by means of the Neurobehavioral Rating Scale-Revised. RESULTS: Up to 16 patients suffered disability for at least one social skill because of cognitive/behavioral reasons. Seven needed full-time supervision. Performing administrative tasks and financial management, writing letters and calculating, driving, planning the week, and using public transport were the most impaired social abilities. Loss of social autonomy was mainly observed in severely injured patients. Univariate analysis showed that mental fatigability, motor slowing, memory difficulties, and disorders of executive function were associated with low scores on the GOS, unemployment, and difficulties in shopping, using public transport, and performing financial management and administrative tasks. CONCLUSION: Persistent impairments of executive functions and speed of psychomotor processing are major factors associated with loss of social autonomy and inability to return to work long after TBI in adults. Improving these impairments in concrete social situations represents a major challenge for cognitive rehabilitation.     

Long-term studies of metopic synostosis: frequency of cognitive impairment and behavioral disturbances

The most evident immunosuppressive effect of cyclosporin A (CsA) on T cells is suppression of interleukin-2 (IL-2) production through the suppression of type-2B serine/threonine-specific phosphatase, calcineurin. To test whether suppression of IL-2 production is a major mechanism of CsA-mediated suppression of allograft rejection, we treated allogeneic skin-grafted mice with CsA and IL-2, and observed that IL-2 did not override the suppressive effect of CsA. Specific cytotoxic T lymphocyte (CTL) activity and natural killer (NK) activity of the spleens were increased by treatment with IL-2, and CsA significantly suppressed the killing activity. We also found that CsA-treatment decreased the expression of lck kinase of T cells and the production of IL-2 in response to concanavalin A (ConA), with minimum effect on IL-4 production. These results suggest that T cell dysfunctions other than decreased production of IL-2 are essential for suppressive effect of CsA on skin allograft rejection.     

A normal sagittal spinal configuration: a desirable clinical outcome

OBJECTIVE: To review our experience of 9 patients who had attempted suicide by swallowing alkaline substances. DESIGN: Retrospective study. SETTING: Two university hospitals, Finland. PATIENTS: During the years 1987-1996. 9 patients who had swallowed more than 100 ml of strong alkali. RESULTS: 2 patients with oesophagorespiratory fistulas of the four with third degree injuries died in hospital, the first died of overwhelming sepsis and the second of multiple organ failure. The other two survived emergency staged resection and subsequent reconstruction. Delay between swallowing the alkali and the initial operation, and development of oesophagorespiratory fistulas were the most important predictors of death. Four patients with second degree injuries underwent oesophageal resection and reconstruction after they had developed oesophageal strictures. Two of these six survivors developed anastomotic strictures after reconstruction, both of which necessitated repeated oesophageal dilatations. One patient with a first degree injury was treated conservatively. CONCLUSIONS: An early and aggressive approach to severe third degree corrosive injuries of the upper gastrointestinal tract is needed to resect all necrotic tissue and to make it possible to do a successful reconstruction later. The development of oesophagorespiratory fistulas requires immediate attention, whereas first and second degree injuries can be treated after the patient has been stabilized.     

Long-term neurodevelopmental outcome after intrauterine transfusion for the treatment of fetal hemolytic disease

OBJECTIVE: The aim of the study was to assess the developmental outcome of neonatal survivors of hemolytic disease of the neonate treated with modern intrauterine transfusion techniques. STUDY DESIGN: In this prospective, observational study, auditory evoked-response tests were performed in the nursery. Neurodevelopmental evaluation with the Gesell Developmental Schedules was performed between 9 and 18 months of corrected age to assess motor skills, language development, comprehension capacity, and social skills. The McCarthy Scales of Children's Abilities were administered between 36 and 62 months. RESULTS: Forty children who survived severe fetal hemolytic disease were followed up until 62 months old. Demographic data included gestational age at first intrauterine transfusion (26.4 +/- 3.7 weeks), median number of intrauterine transfusions (4, range 1-8), lowest fetal hematocrit (20.2% +/- 7.8%), peak fetal bilirubin (7.1 +/- 2.1 mg/dL), incidence of hydrops fetalis (45%), and mean gestational age at delivery (35.6 +/- 2.2 weeks). One case of severe bilateral deafness and 1 case of right spastic hemiplegia were diagnosed. The Gesell Developmental Schedules score was assessed between 9 and 18 months of corrected age in 22 infants. The global developmental quotient was 101.9 +/- 9.5 (mean for normal population is 100). Regression analysis revealed no correlation between the global developmental quotient and gestational age at the first intrauterine transfusion, gestational age at birth, or the severity of the fetal hemolytic disease (fetal hematocrit, fetal bilirubin, presence of hydrops fetalis, total number of intrauterine transfusions, duration of neonatal phototherapy, and number of neonatal exchange transfusions). Eleven of the 40 children were followed up until they were 62 months old, and the McCarthy Scales of Children's Abilities were administered. The mean cognitive index was 107.6 +/- 9.4 (90-109 is considered average). CONCLUSION: Despite severe fetal hemolytic disease, normal developmental outcome can be expected for children treated with intrauterine transfusions.     

Lambdoid synostosis

Recent increases in presentation of occipital deformities have presented the craniofacial team with new challenges in diagnosis and treatment. As presented in this article, they appear to have a functional abnormality of the suture that results in a localized growth deformity. The growth inhibition in severe cases can result in changes in the local bone and brain, along with the entire skull and cranial base. Histologic analysis supports the theory that this is true lambdoid synostosis. Surgical correction is indicated in moderate to severe cases, and the authors have presented their technique and new data on outcomes.     

Long-term outcome of adult-onset minimal-change nephropathy

BACKGROUND: Adult-onset minimal-change nephropathy has been associated with a slower response to corticosteroids and a less benign prognosis when compared to children. However, there are few long-term outcome data reported. METHODS: We have reviewed retrospectively 51 idiopathic adult-onset minimal-change nephropathy patients investigated and treated at a single centre. RESULTS: Male to female ratio was 1:1.4, mean age at diagnosis was 37 years, and average length of follow-up was 14.1 years. Significant comorbidity was identified in 33%. A raised serum creatinine was found in 55% but returned to normal almost invariably upon remission. At presentation, hypertension was found in 47% of patients, microscopic haematuria in 33%, hypercholesterolaemia and hypertriglyceridaemia in 96%, and hyperuricaemia in 42%. Remission (complete or partial) was achieved by 46, 70 and 92% within 4, 8 and 21 weeks respectively, in patients treated with steroids; steroid resistance was encountered in 8%. The time to remission was positively correlated with age (P = 0.002) and initial albumin level (P = 0.005), and negatively correlated to the number of subsequent relapses (P = 0.029); 33% of patients had a spontaneous remission at some time during the disease course. Patients with multiple relapses were treated with cyclophosphamide and 63% of them had remained in remission after 5 years. Hypertension was present in 25% of patients after an average interval of 11 years. At the time of the final follow-up, only three patients had a raised creatinine and all but three patients were in complete remission. CONCLUSIONS: Adult-onset minimal-change nephropathy shares the same good long-term outcome as the childhood counterpart, with sustained remission and preserved renal function.     

Peripheral retinal cryocoagulation. Long-term outcome

Meaning of psychiatric disorders in Alzheimer' disease has been underestimated for a long time. Among these disorders, delusional states represent the most frequent causes of such phenomenon after depressive disorders. It's probably of importance to distinguish delusional and paranoid ideation from the missidentidentification phenomenon. Neuroleptic treatment contribute to improve the quality of life of these delusional patients.     

Tuberculosis after intestinal bypass operations

Two cases of tuberculous infections occurred after intestinal shunt operations for obesity among 161 patients observed for more than one year. One died of generalized tuberculosis. Another 2 patients, who before the operation had had tuberculosis, managed well without signs of postoperative activation of the tuberculous infection seems to be unpredictable.     

Long-term outcome of migration in childhood and adolescence

Self-injurious behavior occurring in persons with severe mental retardation is a clinically significant and poorly understood problem. Multiple neurotransmitter systems have been implicated in the pathogenesis of this behavior, particularly dopaminergic, opioidergic, and serotonergic systems. Pemoline, a central stimulant, administered systemically at high doses reliably produces self-biting behavior in the rat. The systemic bolus of pemoline produces sustained neostriatal levels of pemoline for over 24 h in a continuous infusion paradigm. Studies of the effect of cortical lesions on pemoline-mediated behaviors reveal that cortical damage, as is common in profound mental retardation, lowers the threshold for pemoline-induced self-biting behavior. Data from the corticostriatal slice suggests that sustained exposure to pemoline produces a shift in N-methyl-D-aspartate receptor-mediated responses rendering them more susceptible to dopaminergic enhancement. Thus, dopaminergic and glutamatergic interactions appear to play an important role in the development and expression of self-biting in the pemoline model.