1/36. patients with morbid obesity don't get life-saving bone marrow transplants.When the patient, a registered nurse, was surgically treated for morbid obesity she initially lost 54.5 kg. Approximately 2 years after gastric bypass, she received a diagnosis of chronic myelogenous leukemia and subsequently underwent a successful allogenic bone marrow transplant (BMT). When her surgical history was taken at the transplant facility, the significant weight loss and gastric bypass were discussed. She was informed that at 140 kg, she would not have been eligible nor considered a candidate for transplant. A search of the literature and a survey of other facilities confirmed this view as typical. The reasons cited were that the chemotherapy dosage required for the morbidly obese weight level would cause fatal organ damage as opposed to organ-sparing dosages, which would not eradicate all leukemic cancer cells. An additional general view was that the morbidly obese could not survive the rigors of the transplant preoperative regimen. This patient had an uneventful recovery and remains disease-free today, 3 years after BMT and 5 years after gastric bypass, with a sustained total weight loss of 73 kg.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/36. Adverse psychologic reactions to ileal bypass surgery.Of 33 patients who underwent ileal bypass surgery for morbid obesity and were followed up with psychiatric interviews and consultation postsurgery, five appear to have had adverse psychologic sequelae related to the procedure. The emotional problems of these five patients were in part related to or precipitated by their drastic weight loss after ileal bypass. In most cases, the patients generally had depressive symptoms and, in dynamic terms, were dependent individuals with lifelong problems in object relations. The coping styles demonstrated, while not rigorously classified as psychiatric illness, appeared to predispose them for certain difficulties even when weight had been lost. Ileal bypass surgery apparently is not psychologically innocuous as previously thought, and psychiatric follow-up of patients is indicated.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
3/36. A case of huge ovarian cyst of 21-year-old young woman.Huge ovarian tumors are rarely seen in modern surgical practice. As health care education and access to hospitals have improved over the past 30 years, the number of these reports have become almost negligible. However, these huge ovarian tumors still present many challenge, even life-threatening risks due to severe cardiovascular, pulmonary, and circulatory problems, including technical difficulties of surgery, massive hemorrhage, and postoperative complications. A knowledge of the deranged physiology and its management may avert these complications. We present the case of a 21-year-old woman with huge ovarian tumor. The total weight of the tumor was 136 pounds (62 kg). She was treated surgically with good results.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
4/36. Chronic single needle predilutional haemofiltration in a pre-school child.peritoneal dialysis is the preferred dialysis mode for children with end stage renal disease (1). It avoids problems with vascular access and enables near normal life style. Haemodialysis is the only mode of treatment for a child waiting for renal transplantation when peritoneal dialysis is not possible. Haemofiltration, as a mode of renal replacement therapy, was introduced in 1967 by Lee Henderson (2).- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
5/36. Balloon angioplasty of critical coarctation in a 970-gram premature infant.We describe a 970-gram infant born prematurely at 28 weeks gestation. On the third day of life, she presented in heart failure with Shone's syndrome and severe coarctation of the aorta. Despite initiation of intravenous prostaglandins, she developed progressive severe biventricular dysfunction and acidosis. Balloon angioplasty of the coarctation was successfully performed on the fifth day of life, with a dramatic improvement in ventricular function. At seven weeks of age, after improvement of ventricular function, she underwent surgical resection of the coarctation and arch advancement. At six months of age she is thriving and developing normally.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
6/36. Normal statural growth in 2 infants on chronic peritoneal dialysis: anecdotal or whole management-related.AIMS: Growth retardation is usual in children on chronic peritoneal dialysis (CPD). Despite attention to many contributing factors (nutrition, dialysis dose, hemoglobin level, adynamic bone disease, hyperparathyroidism or rickets, growth hormone resistance, etc.), normal growth is rarely obtained in infants on CPD. MATERIALS AND methods: We had the chance to observe normal growth over a 1 year period in 2 consecutively treated infants on CPD. Louise (renal hypodysplasia) required CPD at the age of 1 month: creatinine 430 micromol/l; oliguric, creatinine clearance lower than 5 ml/min/1.73 m2. Nutrition was achieved orally with human milk during the first 6 months of life. Tidal peritoneal dialysis allowed a high dialysis dose Kt/V urea 3.8/week and Kcreatinine 105 l/week/1.73 m2. Hemoglobin was maintained over 13 g/dl and low levels of vitamin d analogue were prescribed to avoid adynamic bone disease. At the age of 1 year her height was 75 cm. i.e. in the normal range for age. Madeline (renal hypodysplasia) commenced on CPD at the age of 6 weeks and managed similarly. Her height at 1 year of age was 74 cm. RESULTS: In our 20 years of experience with children on dialysis, these 2 cases of normal statural growth for age at 1 year warrant discussion. As well as nutritional support, the new and recent therapeutic options in our team were: firstly, to avoid high doses of activated vitamin d to control PTH, as high doses are able to induce both a risk of adynamic bone disease and a direct bone cartilage toxicity: secondly, to maintain normal hemoglobin level; and thirdly, to deliver a high dialysis dose (urea, creatinine clearance) based on an individually adapted prescription. CONCLUSION: We feel this management approach is necessary to achieve optimal statural growth in children on chronic peritoneal dialysis. But this management concept only based on clinical anecdotal observations needs further evaluation before its use in clinical guidelines.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
7/36. Hypertrophic obstructive cardiomyopathy in a neonate with the carbohydrate-deficient glycoprotein syndrome.The carbohydrate-deficient glycoprotein (CDG) syndrome in its most severe form (neonatal olivopontocerebellar atrophy) is a life-threatening multisystem disease. We report a neonate who was referred for cardiological assessment because of respiratory distress, a murmur and episodes of desaturation. After initial spontaneous improvement he presented at 9 weeks with evidence of a severe hypertrophic obstructive cardiomyopathy (HOCM). The diagnosis of CDG syndrome was suggested by the characteristic dysmorphic features, hypotonia, visual inattention and severe failure to thrive; it was confirmed by electrophoresis of serum transferrin. HOCM can be a feature of the CDG syndrome, in addition to the (previously reported) pericardial effusions.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
8/36. Hereditary progressive dystonia with diurnal fluctuation (Segawa's syndrome)--an unusual case.A young girl with hereditary progressive dystonia with diurnal fluctuation or Segawa's syndrome, completely handicapped and confined to a wheelchair between the age of 5 and 9, revealed an unusually slow response to levodopa. The ability to walk returned only after 12 to 14 months of treatment. Apart from peculiarities of behaviour due to longstanding immobility and ensuing parental overprotection, there were no psychological or mental abnormalities. Other organic diseases were ruled out. A series of regular follow-ups over the course of 7 years was performed. While residual and irregular day-to-day variation of functional capacities almost disappeared after conversion to a preparation with a decarboxylase inhibitor, some mild neurological abnormalities and a slight choreic hyperkinesia persisted with doses providing functional results. The patient today leads an almost normal teenage life and has performed well in school.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
9/36. The long-term management of obesity with continuing pharmacotherapy.OBJECTIVE: Long-term, possibly lifetime, use of medications for the management of obesity may be thought to be similar to the use of pharmacotherapy for other chronic diseases such as hypertension or diabetes. Because there have been no systematic studies of this extended use, the experience of eight patients who have used obesity medications in a sustaining manner was studied. research methods AND PROCEDURES: The clinical characteristics of eight adult patients, each of whom has experience with long-term (more than 10 years) use of medications for weight loss and weight maintenance, were studied. RESULTS: The clinical experience of these eight patients was analyzed. Each chose to sustain the use of weight management medications for more than 10 years because of perceived benefit, comfort, and the absence of significant side effects. There has been no evidence of the development of tolerance, addiction, or misuse and no adverse events related to the medication. The beneficial effects of the medication have not diminished with time. DISCUSSION: The clinical characteristics of eight patients, each of whom has used obesity pharmacotherapy for more than 10 years, are described. The experience of these eight individuals cannot be generalized to the entire population of overweight or obese patients. It does suggest, however, that some patients respond successfully to this form of therapy and that they will derive value from it for the management of this disease. Efforts should be made to identify these patients, and consideration should be given to the use of chronic medications for the continuing management of obesity.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
10/36. Management of methylmalonic acidaemia by combined liver-kidney transplantation.Methylmalonic acidaemia (MMA) is a rare autosomal recessive inborn error of metabolism that typically presents in infancy with recurrent episodes of metabolic acidosis, developmental delay and failure to thrive. The disease course is complicated by the development of chronic tubulointerstitial nephritis progressing to end-stage renal disease in adolescence. We describe two adolescents with cobalamin-nonresponsive MMA (mut0) who developed polyuria, chronic tubulointerstitial nephritis, dystonia but normal synthetic liver function. Both patients received combined liver-kidney transplantation (CLKT), preceded by a single pretransplant haemodialysis for clearance of methylmalonic acid. Post CLKT there was 95-97% reduction in serum and urine methylmalonic acid, leading to significant liberalization of dietary protein intake and a consequent increase in body mass index, muscle strength and energy. In addition, renal function normalized and clinical neurological status stabilized. We propose that CLKT be considered as a therapeutic option early in the course of cobalamin-nonresponsive MMA. Progressive tubulointerstitial nephritis with disabling polyuria is a confounder in patient management even in the absence of end-stage renal disease. Successful CLKT restores methylmalonyl-coa mutase enzyme levels in the liver and kidney, improves clearance of methylmalonic acid with resultant dietary protein liberalization, and offers excellent graft and patient outcomes with improvement in quality of life.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
| | Next -> |