摘要:

We have defined the clinical and laboratory characteristics of a group of patients with HbS-βo+α-thalassemia plus α-thalassemia, by analysis of erythrocyte indices, hemoglobin A2and F levels, globin biosynthesis studies and α-globin gene mapping. Patients with HbS-βo+ α-thalassemia closely resembled individuals with HbS-βo-thalassemia except for balanced globin synthesis ratios and a lower HbF level. The frequency of painful crises, leg ulceration, aseptic necrosis of bone and acute chest syndrome was similar in HbS-βo+ α-thalassemia patients and controls with sickle cell anemia (HbSS), HbSS-α-thalassemia and HbS-βo-thalassemia. These findings are consistent with previous work which failed to show a reduction in the vaso-occlusive severity of sickle cell disease by the coexistence of α-thalassemia.

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

摘要:

Abnormal serum angiotensin converting enzyme (ACE) activity has been reported in various human lung disorders and in laboratory animals with acute lung injuries. To test the value of serum ACE activity as an indicator of lung damage and its assistance in diagnosis or prognosis, 328 serum samples were obtained from 108 hospitalized patients with lung disease and 26 normal subjects. When patients were clinically grouped by disease entity, only the sarcoidosis group showed elevated mean serum ACE. Significantly increased serum ACE was found in 17 patients with various lung diseases (15% of hospitalized patients) 12 of whom also had concomitant liver disease. It is hypothesized that the liver may play a role in the normal metabolism of ACE being released by lung endothelial injury. Significantly low levels were seen in many acute and chronic lung injuries; specifically the groups with chronic obstructive lung disease, lung cancer, acute pneumonia, aspiration pneumonitis, gram-negative sepsis, acute myocardial infarction, and congestive heart failure. Serial measures of ACE in 71 patients with lung injuries showed that significantly decreasing levels over successive days were associated with a very high mortality. A single ACE measurement did not predict the presence or extent of lung injury, or aid in diagnosis or prognosis, but serial levels are of value prognostically. [Am J Med Sci 1984; 288(5):200–207.]

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

摘要:

Von Hippel-Lindau disease (VHLD) is a rare disorder known to occur with pheochromocytoma. We report a case with this association and also with pancreatic apudoma. We believe that this is the fifth reported case of this triad, which represents an overlap in the multiple endocrine neoplasia (MEN) classification. Many recent reports of overlap of MEN syndromes challenge the validity of this classification. New understanding of the embryologic origin of cells of endocrine organs has led to the elucidation of the APUD (amine precursor uptake and decarbozylation)-neuroendocrine system. Integration of the APUD system may be important to understanding the pathogenesis of the MEN-like syndromes as well as their relationship to the neurocutaneous syndromes. Any endocrine tumor or neurocutaneous syndrome should be evaluated to exclude a spectrum of possible associations. [Am J Med Sci 1984; 288(5):212–216.]

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

摘要:

The number of patients achieving long-term survival following neck irradiation for Hodgkin's disease and other malignancies is increasing. Paralleling this increase in survivors is the development of late complications of the therapy itself. Eleven patients have previously been reported who developed Graves' ophthalmopathy 18 months to seven years after receiving neck radiotherapy for non-thyroidal malignancies. The seven patients who had HLA typing were all HLA-B8 negative, despite the reported association of the HLA-B8 antigen with Graves' disease. A patient who is HLA-B8 positive who developed Graves' ophthalmopathy and hyperthyroidism nine years after receiving mantle radiotherapy for Hodgkin's disease is reported. It is recommended that Graves' disease be included among the thyroid diseases that receive consideration during follow-up of patients who have received mantle radiotherapy. [Am J Med Sci 1984; 288(5):217–220.]

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

摘要:

A 32-year-old presented with fulminant, bilateral airspace pneumonia due toCryptococcus neoformanswhile under cytotoxic therapy for advanced Hodgkin's disease. We alert physicians to this rapidly progressive form of cryptococcosis which has been poorly described previously and which may closely mimic bacterial pneumonia. [AM J MED SCI 1984; 288(5):221–222.]

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

摘要:

Mural thrombus is a well-recognized cause of systemic embolization. A case is presented where systemic emboli occurred from a mural thrombus in a patient who had no underlying intrinsic heart disease but who had an apparent hypercoagulable state due to hyperaggregable platelets. This mural thrombus was diagnosed by two dimensional echocardiography in which it was observed that the left ventricular wall motion was normal. [Am J Med Sci 1984; 288(5):223–227.]

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID

摘要:

Dietary factors may have substantial impact on the clinical manifestations and even the progression of chronic renal failure. Proper dietary management can prevent certain uremic complications, decrease azotemia, and may even prevent the loss of residual renal function. Recent studies indicate that dietary protein may accelerate the normal age-related deterioration of renal function in rats. The extensive ablation of functional renal mass in rats leads to hyperemia and hyperfiltration in remnant nephrons. Continued hyperfiltration theoretically results in glomerular damage, proteinuria, and ultimately glomerular sclerosis. Dietary protein restriction reduces the remnant nephron hyperfiltration and reduces the rate of glomerular sclerosis, at least in the rat. The role of dietary protein in the pathogenesis of human nephrosclerosis remains controversial. Though dietary factors may or may not affect the rate of progression of renal insufficiency, there is no doubt that proper dietary management can limit or forestall uremic symptoms and the need for dialysis. Diets containing about 0.5 gm protein/kg body weight/day usually maintain a neutral or slightly positive nitrogen balance, while lesser amounts usually result in malnutrition. When protein intake exceeds 0.5 gm/kg/day azotemia increases dramatically. The use of nitrogen-free keto- or hydroxy-analogues of amino acids promotes positive nitrogen balance while reducing azotemia in patients with near-end-stage renal disease. [Am J Med Sci 1984; 288(5):228–234.]

ISSN:0002-9629    

出版商:OVID    

年代:1984

数据来源: OVID