[A case of sepsis by Neisseria meningitidis beginning with pneumonia during a trip abroad].

A 62-year-male presented a high fever and a dry cough during a trip to Australia. He was admitted to a hospital as soon as be returned to Japan. The next day after returning to Japan, he was transferred to our hospital with septic shock and loss of consciousness. Neisseria meningitidis was cultured from his blood. N. meningitidis is rare in Japan. However its seems common, in some foreign countries. With these findings, it can be postulated that N. meningitidis might be one of the etiological agents of the imported infectious disease.

CT findings of descending necrotising mediastinitis via the carotid space ('Lincoln Highway').

A 3-year-old girl with fever and neck swelling showed widening of the superior mediastinum on chest radiographs. Contrast-enhanced CT of the neck revealed ill-defined low-attenuation areas with a thick, enhanced rim adjacent to the hypertrophied palatine tonsil. The abscess extended inferiorly within the carotid sheath between the carotid artery and the internal jugular vein into the anterior mediastinum. The carotid space is considered an important conduit of descending necrotising mediastinitis and is called the 'Lincoln Highway' as previously suggested.

Negative C-reactive protein in children with bacterial infection.

PURPOSE: To evaluate the practical value of initial C-reactive protein (CRP) in the diagnosis of bacterial infection in children. METHODS: The subjects comprised 11 children, six boys and five girls, aged 3 months through to 3 years (median age 16 months), whose initial CRP levels were < 1.0 mg/dL despite bacterial infection. C-reactive protein was quantitated at the first medical examination by nephelometry. RESULTS: The diagnosis was urinary tract infection (n = 4), bacterial meningitis (n = 2), sepsis (n = 2), pneumonia (n = 2) and arthritis of the hip joint (n = 1). The CRP levels were significantly elevated during the course of infection, ranging from 7.6 to 28.5 mg/dL. The bacterial etiology was non-specific. Eight patients were examined within 12 h of onset, three exhibited negative CRP values despite the duration of the insult over 12 h. Six patients were tentatively diagnosed as having a bacterial infection, but the other five were not. Each patient was treated, leading to a favorable outcome without any serious complications. CONCLUSIONS: Low levels of CRP do not rule out the possibility of bacterial infection in children. The initial value of CRP may be negative, even in patients with severe bacterial infection or even after 12 h from onset. The data suggest that pediatricians should consistently be aware of the possibility of bacterial infection even if the initial CRP test result is negative and that serial CRP measurements appear to be practical.

Flow and volume dependence of respiratory mechanics in anesthetized children.

With the use of constant flow, end-inspiratory airway occlusion, respiratory system resistance (Rrs) can be partitioned into a flow resistive component (Rint) and an additional component (deltaR), reflecting viscoelasticity and time constant inequality. Similarly, respiratory system elastance (Edyn) can be partitioned into static elastance (Est) and elastance due to viscoelasticity and time constant inequality (deltaE). We measured Rrs and Edyn and their subdivisions (Rint and deltaR, Est and deltaE, respectively) and studied their flow and volume dependence in eight otherwise healthy children (median age 3.6 y; range 1.9-5.2 y) undergoing general anesthesia for oral rehabilitation. With a constant inspiratory flow (VI) of approximately 15 mL/s/kg and tidal volume of 12 mL/kg, the mean values of Rrs, Rint, and deltaR were: 0.20, 0.11, and 0.10 cmH2O/mL/s.kg. Under the same conditions, the mean Est and deltaE were: 1.04 and 0.12 cmH2O/mL/kg. With increasing VI and under constant VT, deltaR decreased (p < 0.001) progressively. Rint also decreased paradoxically (p < 0.001). Hence, Rrs decreased (p < 0.001) with increasing VI. Est decreased (p < 0.001) with increasing VI, whereas delta E increased (p < 0.005). With increasing VT and under constant VI, Rint decreased (p < 0.001) and deltaR tended to increase (p = 0.058); Rrs did not change. With increasing VT under constant VI, both Est and deltaE decreased (p < 0.001 and p = 0.001, respectively). Thus, in contrast to the findings in adults, Rint and Est decreased in children with increasing flow and under constant tidal volume, probably reflecting decreased functional residual capacity in anesthetized children, compared with adults. The flow and volume dependence of deltaR and deltaE were similar to those in adults, whereas Rrs did not necessarily follow the direction of changes of deltaR.

Another observation of microphthalmia in an XX male: microphthalmia with linear skin defects syndrome without linear skin lesions.

A case of microphthalmia with Xp microdeletion is reported. The patient was a boy who showed bilateral microphthalmia with corneal opacities, hypospadias without evidence of hypogonadism, and a conduction disturbance of the heart (Wenckebach conduction). No skin lesion was discerned. High-resolution chromosome analysis revealed the karyotype of 46,X,del(X)(p22). The phenotype was considered to be microphthalmia with linear skin defects (MLS) syndrome without skin lesions. Polymerase chain reaction and fluorescence in-situ hybridization analyses revealed that the chromosome aberration resulted from an X;Y translocation: the presence of pseudoautosomal boundary Y and the sex-determining region of Y was confirmed, while Xp deletion involving the region distal to DXS1129 was ascertained. Thus the chromosome designation using the ISCN 1995 nomenclature is 46,X,der(X),t(X;Y)(p22.13;q11.2). Despite the absence of skin lesions, the Xp deletion of our patient corresponded to those of previously reported typical cases of MLS syndrome. Our observation further supports the current hypothesis that the phenotypic variation of MLS syndrome represents tissue-different X inactivation rather than different genetic effects of two contiguous genes.

Assessment of the severity of coronary artery disease by thallium-201 washout rate after dipyridamole infusion--a coronary hemodynamic and metabolic study.

This study examined the relationship between the myocardial washout rate (WR) of thallium-201 (201Tl) in dipyridamole scintigraphy and both coronary flow reserve (CFR) and myocardial lactate extraction rate (LER) after dipyridamole infusion in 31 patients with coronary artery disease (CAD) without myocardial infarction and 16 control patients. Patients with CAD demonstrated significantly lower WR (21 +/- 17 vs 43 +/- 10%, p < 0.001), lower CFR (128 +/- 82 vs 242 +/- 89%, p < 0.001) and lower LER (-2 +/- 20 vs 10 +/- 10%, p < 0.05) than did the control patients. WR was significantly correlated with CFR (r = 0.50, p < 0.001) and LER (r = 0.41, p < 0.01) in all of the patients. CAD patients with dipyridamole-induced chest pain demonstrated significantly lower WR (14 +/- 20 vs 27 +/- 12%, p < 0.05), lower CFR (97 +/- 71 vs 162 +/- 82%, p < 0.05) and lower LER (-13 +/- 21 vs 11 +/- 9%, p < 0.001) than did CAD patients without chest pain. CAD patients with dipyridamole-induced ST depression demonstrated significantly lower WR (14 +/- 20 vs 29 +/- 8%, p < 0.05), lower CFR (105 +/- 79 vs 170 +/- 73%, p < 0.05) and lower LER (-8 +/- 21 vs 11 +/- 10%, p < 0.01) than did CAD patients without ST depression. These results suggest that the myocardial washout rate of 201Tl after dipyridamole infusion reflects the severity of coronary artery disease as assessed by coronary hemodynamics, myocardial metabolism, symptoms and electrocardiography.

Hemodynamic effects and metabolic fate of inhaled nitric oxide in hypoxic piglets.

We describe the hemodynamic effects and metabolic fate of inhaled NO gas in 12 anesthetized piglets. Pulmonary and systemic hemodynamic responses to incremental [NO] (5-80 ppm) were tested during ventilation with high- [0.30 inspired O2 fraction (FIO2)] and low-O2 (0.10 FIO2) mixtures. In six animals, inhalation of 40 ppm NO was maintained over 6 h to test effects of prolonged exposure (0.30 FIO2). In the other six animals, pulmonary hypertension was induced by hypoxic ventilation (0.10 FIO2) and responses to NO were tested. Inhaled low [NO] partially reversed pulmonary hypertension induced by alveolar hypoxia; mean pulmonary arterial pressure decreased from 31.4 +/- 2.3 mmHg during hypoxia to 18.2 +/- 1.2 mmHg during 5 ppm NO. Mean pulmonary arterial pressure at 0.10 FIO2 did not fall further at higher [NO] (10-40 ppm) and never reached control levels. Pulmonary vascular resistance increased with institution of hypoxic ventilation and fell with subsequent administration of NO, ultimately reaching control levels. Inhaled NO did not affect systemic vascular resistance. Plasma levels of NO2- + NO3- and methemoglobin (MetHb) levels increased with increasing [NO]. Over 6 h of NO administration during high-O2 ventilation, MetHb equilibrated at subtoxic levels while NO2- + NO3- increased. Nitrosylhemoglobin, analyzed by electron paramagnetic resonance spectrophotometry was not detected in blood at any time. At the relatively low concentrations (5-80 ppm) that are effective in relieving experimental pulmonary hypertension induced by alveolar hypoxia, inhaled NO gas causes accumulation of NO2- + NO3- in plasma and a small increase in MetHb but no detectable nitrosylhemoglobin.

[Severe theophylline toxicity in a pregnant asthmatic patient].

A 32 year old pregnant woman who had taken large doses of theophylline for asthmatic attack was brought to our emergency medical center with convulsion and respiratory disturbance. Serum theophylline concentration was 52 micrograms.dl-1 on admission. We intubated the patient to control convulsion and respiration and performed cesarean section. Serum theophylline concentration of the newborn was almost at the same level as that of her mother. Baby was placed on ventilator for several days and discharged without trouble. We must consider the pregnancy as a major risk factor of theophylline toxicity.

Effects of exercise and pacing loads on myocardial amino acid balance in patients with normal and stenotic coronary arteries, with special reference to branched chain amino acids.

Arterial and coronary sinus differences (A-S) of alanine, glutamate, isoleucine, leucine, valine and phenylalanine were measured in 7 control subjects and 12 patients with coronary artery disease (CAD) at rest and during exercise, and in 8 controls and 21 CAD patients at rest and during pacing. Lactate, great cardiac vein flow and oxygen were also measured. However, none of these parameters distinguished CAD from controls. Changes in alanine and glutamate during each load were, for the most part, consistent with previous studies, i.e., a greater release of alanine and uptake of glutamate was observed in the ischemic group. A-S of isoleucine, leucine and valine showed significant positive correlation to that of alanine (r = 0.59, r = 0.89, r = 0.77, respectively, during exercise, and r = 0.57, r = 0.65, r = 0.72, respectively, during pacing). A-S of isoleucine, leucine and valine showed significant positive correlations to each arterial concentration during exercise (r = 0.54, r = 0.62, r = 0.63, respectively), but not during pacing. Although none of the uptakes of the branched chain amino acids (BCAA) were significant, the mean A-S of each BCAA was positive at rest in both controls and CAD, and declined during each load. A-S of leucine was significantly smaller in CAD than in controls during exercise (0.7 +/- 7.0 vs 6.8 +/- 4.1 mumol/l, p < 0.05) and those of leucine and valine were significantly smaller in CAD patients with ischemic electrocardiographic change than in those without electrocardiographic change during pacing (0.1 +/- 5.9 vs 6.1 +/- 5.5, p < 0.05; -3.1 +/- 10.1 vs 9.9 +/- 6.8 mumol/l, p < 0.01, respectively). These results suggest that BCAA, especially leucine and valine, tend to be taken up by human myocardium physiologically and show characteristic changes under myocardial stress similar to those seen with alanine and glutamate.

[Effect of L-carnitine in patients with ischemic heart disease].

To assess the protective effects of L-carnitine (LC) infusion on ischemic heart disease, 30 patients who had angina and ischemic ECG changes during exercise were evaluated by bicycle ergometry. They were categorized in LC and non-treatment (NT) groups. There were no significant differences in age and sex between the 2 groups. Before exercise, 15 patients (9 males and 6 females) received 60 mg/kg LC and the results including hemodynamics, coronary circulation, and cardiac metabolism at rest and during exercise were compared with those of the NT group studied in the same protocol (50 watts x to cycle, 15 min). At the end of 30 min LC drip infusion, the arterial carnitine content (LC (a)) reached 1,980 +/- 257.3 microM and then was maintained at 1,212.7 +/- 136.2 microM during exercise. There was no correlation of LC (a) with the coronary arterio-venous difference nor with myocardial uptake of LC. Although there was no significant difference in coronary blood flow (CBF: mliters/100 g/min) between the LC and NT groups at rest (LC: 92.1 +/- 29.0 vs NT: 88.0 +/- 26.5), CBF during exercise increased significantly in the LC group compared with the NT group (LC: 230.4 +/- 113.8 vs NT: 139.1 +/- 52.7; p < 0.05). In the NT group, there was no significant change in coronary arterio-venous oxygen difference ((a-cs) O2: vol %) during exercise, but in the LC group (a-cs) O2 increased significantly from 10.2 +/- 1.3 to 11.5 +/- 1.9 (p < 0.01). Furthermore, although there was no significant difference in myocardial oxygen consumption (MVO2: mliters/100 g/min) at rest between the 2 groups (LC: 9.30 +/- 2.96 vs NT: 9.71 +/- 3.09), it increased significantly in the LC group compared with the NT group during exercise (LC: 25.11 +/- 9.98 vs NT: 15.55 +/- 6.09). MVO2/LVWI (LVWI = left ventricular work index) and MVO2MT (MT = myocardial tension) did not significantly differ at rest between the 2 groups. However, these 2 indices decreased significantly during exercise (p < 0.05) in the NT group, and remained unchanged in the LC group, showing a significant difference between the 2 groups (both p < 0.05). In myocardial energy substrates, the myocardial uptake ((a-cs) x CBF) of free fatty acid (FFA: muEq/100 g/min) increased significantly in the LC group compared with that of the NT group (LC: 10.16 +/- 13.26-->31.88 +/- 27.58* vs NT: 16.02 +/- 27.92-->18.11 +/- 31.00;* = p < 0.05, LC vs NT).

[Coronary reserve of the hypertensive heart: relationship to left ventricular hypertrophy].

We investigated the relationship between electrocardiographic left ventricular hypertrophy with ST-changes, and coronary circulation and myocardial metabolism in 25 patients with essential hypertension (systolic blood pressure greater than or equal to 160 mmHg, diastolic blood pressure greater than or equal to 95 mmHg). No patients had abnormal coronary arteriograms. They were categorized in two groups: non-hypertrophy group; cases with no hypertrophy on echocardiograms (interventricular septum and posterior wall thickness less than or equal to 11 mm), and hypertrophy group; cases with hypertrophy (wall thickness greater than or equal to 12 mm). Supine bicycle ergometry (50 watts, 50 rpm, 15 min) was performed during coronary sinus catheterization and electrocardiography. For cardiac hemodynamics, the coronary circulation and myocardial metabolism were observed at rest and during exercise. There was no significant difference in unit coronary blood flow (coronary blood flow per 100 gm) before exercise between the two groups. Unit coronary blood flow was significantly greater in the non-hypertrophy group after exercise. However, total coronary blood flow (coronary blood flow per 100 gm x left ventricular mass) showed no significant difference between the two groups. Coronary vascular resistance per 100 gm was higher in the hypertrophy group during exercise, though there was no significant difference at rest. Myocardial oxygen extraction O2 (A-C) was not different between the two groups at rest and during exercise, but unit myocardial oxygen consumption (MVO2) during exercise was higher in the non-hypertrophy group than in the hypertrophy group. The lactate extraction ratio tended to decrease in the hypertrophy group during exercise, and tended to increase in the non-hypertrophy group. Myocardial potassium flux tended to increase in the hypertrophy group during exercise.(ABSTRACT TRUNCATED AT 250 WORDS)

[Left ventricular rupture following the procedure of percutaneous transluminal coronary angioplasty: a case report].

A 50-year-old man with 90% concentric stenosis at the proximal portion of the left anterior descending artery was tried to be treated with percutaneous transluminal coronary angioplasty (PTCA). The guide wire did not pass through the stenotic portion, and the staining out of coronary artery was recognized. Four hours after the procedure, he fell to shock status with a sign of electro-mechanical dissociation. An emergent operation confirmed the left ventricular rupture. Repair of the tear and saphenous vein bypass to the left anterior descending artery were performed rapidly, however, he was not weaned from cardiopulmonary bypass. Close communication between cardiologist and surgeon, and immediate surgical back up are essential for saving these patients from severe ischemic events following PTCA failure.

[Coronary circulation, myocardial metabolism and cardiac catecholamine flux in patients with syndrome X].

To clarify the pathogenesis of syndrome X (Sx), hemodynamics, coronary circulation, myocardial lactate metabolism and cardiac catecholamine flux were studied before and during bicycle ergometry in 25 patients (pts) and these data were compared with those in 15 with effort angina (EA) and in eight controls. The results were as follows: 1. The cardiac index during exercise decreased in EA pts probably due to their coronary lesions, while those in the controls and Sx were well preserved. 2. An increase of coronary blood flow during exercise, both in Sx and EA pts, was significantly impaired and the myocardial extraction ratio of lactic acid was significantly lower compared with those in the controls. The cardiac norepinephrine release during exercise was markedly higher in Sx, compared with that in controls and in EA. These results suggested that in Sx, excessive sympathoadrenergic stimulation may play an important role in restricting coronary reserve and in accelerating myocardial anaerobic metabolism.

Tracheal agenesis with bronchoesophageal fistulas--a case report.

Although tracheal agenesis is presently considered to be a rare and fatal anomaly, an increasing number of institutions have been reporting such cases. Reported herein, is a clinical and necropsy report of a premature infant whose tracheal agenesis was associated with broncho-esophageal fistulas and an imperforate anus. Accidental esophageal intubation enabled a perineal anoplasty to be performed under general anesthesia and allowed him to survive for 14 days.