Fatal hyperammonemia in a patient with systemic lupus erythematosus

We treated a 31-year-old woman with systemic lupus erythematosus, renal failure with nephrotic syndrome, and a long-standing seizure disorder, who developed severe hyperammonemia with a fatal outcome. Blood chemistry examination did not indicate liver disease, and amino acid concentrations did not suggest a defect in the urea cycle. Discontinuation of anticonvulsant treatment with valproic acid (VPA) failed to bring about improvement. We speculated that hyperammonemia in this case was induced by VPA, and the existence of other underlying factors, including the administration of aspirin and cimetidine, hypoalbuminemia, and renal failure might elevate the concentration of the serum free fraction of VPA.     

Fenoldopam mesylate versus sodium nitroprusside in the acute management of severe systemic hypertension

Thirty-three patients with severe systemic hypertension defined as a diastolic blood pressure (DBP) > or = 120 mm Hg were randomized in a single-blind fashion to be treated with either intravenous fenoldopam mesylate (FNP) or sodium nitroprusside (NTP). Fenoldopam mesylate and NTP infusion rates began at 0.1 microgram/kg/minute and 0.5 microgram/kg/minute, respectively and were titrated to achieve a goal DBP of between 95 and 110 mm Hg; or a reduction of at least 40 mm Hg if the baseline DBP was > 150 mm Hg. Fenoldopam mesylate (n = 15) reduced blood pressure from 217/145 +/- 6/5 to 187/112 +/- 6/3 mm Hg (P < .001) at an average infusion rate of 0.5 +/- 0.1 microgram/kg/minute. The average time to achieve goal DBP with FNP was 1.5 +/- 1.4 hours. Nitroprusside (n = 18) reduced blood pressure from 210/136 +/- 5/2 to 172/103 +/- 6/2 mm Hg (P < .001) at an average infusion rate of 1.2 +/- .24 micrograms/kg/minute. Nitroprusside response time averaged 2 +/- 2.5 hours. There was no significant difference between the magnitude of effect seen with either FNP or NTP; nor was there any difference observed in the adverse effect rates of the two agents. Fenoldopam mesylate and NTP demonstrate similar overall efficacy in the treatment of severe systemic hypertension.     

Secondary pulmonary hypertension in a patient with severe chronic restrictive pulmonary insufficiency--normalized during treatment with non-invasive intranasal ventilation and oxygen

The present case report describes a young woman with severe restrictive lung failure, who developed pulmonary hypertension. She was treated successfully with nocturnal noninvasive nasal ventilation and oxygen, which removed symptoms and signs of cardiac incompensation. The improvement has persisted for five years.     

Cytomegalovirus-induced interstitial pneumonitis in a patient with systemic lupus erythematosus

BACKGROUND: Antibodies of the Knops system have been referred to as nonneutralizable because they cannot be inhibited with serum, saliva, or urine. Because the Knops system antigens have been located on complement receptor 1 (CR1), the question of whether the antibodies could be neutralized with soluble CR1 (sCR1) produced by recombinant DNA techniques was studied. STUDY DESIGN AND METHODS: First, radiolabeled immunoprecipitation techniques were used to test sCR1 for the expression of the high-incidence Knops system antigens. Then, a total of 45 antibodies were neutralized with sCR1, including the following: one each of anti-Cr(a), -Dr(a), -Do(b), -Hy, -Ge, -Jr(a), -Sc1, -Jk(a), -Cs(a), and -Kp(b); two each of anti-Lu(b), -Yt(a), and -JMH; three each of anti-McC(a), -Rg, and -Sl(a); and four each of anti-Ch, -Kn(a), -Yk(a), -Kn/McC. In addition, two examples of anti-Kn(a) + K, one example of anti-Sl(a) + K + Fy(a), and one example of anti-Yk(a) + E were tested. The sCR1 was added to each test serum and 6-percent albumin was added to the control; this was followed by neutralization incubation for 5 minutes at 25 degrees C. The antibody samples were then tested by a low-ionic-strength solution, anti-human globulin technique. RESULTS: The sCR1 expressed Kn(a), McC(a), Sl,a and Yk(a). All Knops system antibodies (n = 22) were neutralized by the sCR1, but none of the other 23 alloantibodies decreased in reactivity. The samples containing antibodies of two specificities showed inhibition of the Knops system antibody but not of the second antibody. CONCLUSION: This neutralization method, in which recombinant protein is used, provides an expedient and definitive method of identifying Knops system antibodies.     

Chronic systemic aspergillosis in a patient with acute myeloid leukemia

Systemic aspergillosis is a well-recognized complication of chemotherapy-induced neutropenia. In this report a patient with acute myeloid leukemia is described in whom a chronic aspergillosis with systemic involvement developed after recovery from neutropenia following intensive chemotherapy and allogeneic bone marrow transplantation. The clinical features of a chronic course of systemic aspergillosis suggest a distinct clinical entity comparable to chronic systemic candidiasis.     

Development of systemic lupus erythematosus in a male patient after 14 years on hemodialysis

Graves' disease (GD), myasthenia gravis (MG), and polymyositis (PM) are organ-specific autoimmune diseases. The association between GD and MG is well known, and an association between MG and PM has been reported. However, only one patient with all three diseases has been reported. We describe a patient with GD and PM developing 6 and 29 years, respectively after the onset of MG. This case suggests that the expression of autoimmunity occurred over a prolonged period of time and these organ-specific autoimmune diseases may be induced by a similar mechanism.     

Microcirculation in systemic hypertension

In 40 patients with idiopathic systemic hypertension, skin blood flow was evaluated with laser-Doppler flowmetry, transcutaneous measurements of partial pressure of oxygen (PO2) and partial pressure of carbon dioxide (PCO2), and determination of capillary permeability before and after treatment with nifedipine (10 mg tid for four weeks). Also 35 normal subjects matched for age and sex distribution were studied. Before treatment, microcirculatory studies showed a significant decrease in skin flow and venoarteriolar response in hypertensive patients in comparison with normal subjects. Moreover, PO2, PCO2, and capillary permeability were significantly lower in hypertensives. All these microcirculatory parameters significantly increased after nifedipine treatment while both systolic and diastolic pressures decreased. In conclusion, laser-Doppler flowmetry used with other microcirculatory techniques was able to discriminate between normal subjects and hypertensive patients, and it was able to show the improvement in the microcirculation after nifedipine treatment.     

Resuscitation of the injured patient with polymerized stroma-free hemoglobin does not produce systemic or pulmonary hypertension

BACKGROUND: Hemoglobin-based blood substitutes appear poised to deliver the promise of a universally compatible, disease-free alternative to banked blood. However, vasoconstriction following administration of tetrameric hemoglobins has been problematic, likely because of nitric oxide binding. Polymerized hemoglobin is effectively excluded from the abluminal space because of its size, and is thus less likely to perturb vasorelaxation. We therefore hypothesized that hemodynamic responses would be no different in injured patients receiving polymerized hemoglobin versus banked blood. METHODS: Injured patients requiring urgent transfusion were randomized to receive either polymerized hemoglobin or banked blood. Systemic arterial pressure, pulmonary arterial pressure, cardiac index, pulmonary capillary wedge pressure, systemic vascular resistance, and pulmonary vascular resistance were measured serially. RESULTS: There was no difference in any of the measured hemodynamic parameters between patients resuscitated with polymerized hemoglobin versus blood. CONCLUSIONS: Polymerized hemoglobin given in large doses to injured patients lacks the vasoconstrictive effects reported in the use of other hemoglobin-based blood substitutes. This supports the continued investigation of polymerized hemoglobin in injured patients requiring urgent transfusion.     

Glomerulomegaly and proteinuria in a patient with idiopathic pulmonary hypertension

In an attempt to identify genes involved in neuroblastoma, we scanned neuroblastoma tumour DNAs for homozygous deletions by representational difference analysis (RDA). The RDA produced several difference products, nine of which represented hemizygous deletions located on chromosome 1 or 3. In order to detect deletions, a genomewide loss of heterozygosity (LOH) screening with polymorphic markers was performed. Allelic losses on a number of different chromosomes were detected, mainly in favourable neuroblastomas (stage 1, 2 and 4S). The most frequently deleted region, apart from 1p, was chromosomal region 3p. A more detailed study was made in this region, which showed that 9 out of 58 (16%) tested neuroblastoma tumours showed allelic loss in the same region on chromosome 3p, i.e. 3pter-14.2. Thus, both RDA and LOH studies showed chromosome region 3p as being frequently involved in deletions and/or rearrangements in neuroblastoma tumours. Therefore, it is possible that one or more of the 3p genes implicated in the development of other cancers also play a role in neuroblastoma development and/or progression.     

Mitral valve repair in a patient with severe porcelain aorta

We repaired the mitral valve in a patient with severe porcelain aorta. Significant mitral regurgitation developed in a 66-year-old woman with heavy calcification throughout the whole aorta. At operation, cardiopulmonary bypass was properly established by combined axillary and femoral arterial cannulations for sufficient systemic flow. Likewise, the combination of a superior mitral approach and profound hypothermic fibrillatory arrest in conjunction with low-flow cardiopulmonary bypass allowed us to repair the mitral valve successfully.