Topical application of isosorbide dinitrate in patients with persistent constipation after pull-through surgery for Hirschsprung's disease.

AIMS: The aim of this study was to evaluate the anal manometric changes and the clinical effects after topical application of isosorbide dinitrate (ISDN) in patients with persistent constipation after pull-through surgery for Hirschsprung's disease (HD). METHODS: We studied 3 children (2 males and 1 female), aged 2, 3 and 5 years respectively, who had undergone the Soave-Boley surgical procedure for HD and who suffered from persistent constipation after operation. We performed a pre- and postoperative anorectal manometry study and we applied ISDN paste (1 mg/kg two times daily) in the anal region for three weeks. All patients were followed-up and re-evaluated at 1, 3, and 6 months. RESULTS: All patients showed an improvement of symptoms, with an average of 4 spontaneous evacuations per week. Prior to the topical treatment, the medium pressure was 115.6 mmHg (range 102 - 130 mmHg), the maximum pressure was 160 mmHg (range 145 - 175 mmHg), and the medium length of the high pressure zone was 1.8 cm (range 1.5 - 2.0 cm). At the 6 month follow-up, the medium pressure was 57.3 mmHg (range 52 - 61 mmHg, a decrease of 54.4 %), the maximum pressure was 98 mmHg (range 88 - 107 mmHg; a decrease of 38.7 %), and the medium length of the high pressure zone was 1.6 cm (range 1.4 - 1.8 cm; a decrease of 11.1 %). CONCLUSIONS: Topical treatment with ISDN is a valid therapeutic alternative to an anal myotomy in patients with persistent constipation after pull-through surgery for HD. However, a greater number of cases and a longer follow-up are necessary to confirm the validity of our experience.     

Increased external tibial torsion in Osgood-Schlatter disease

We studied the relationship between Osgood-Schlatter disease and torsional abnormalities of the lower limb in 21 boys with this condition and 20 age- and sex-matched controls. 3 groups of knees (20 control knees, 21 symptomatic and 21 asymptomatic or less symptomatic knees) were subjected to clinical, radiographic and CT evaluation. We found no statistically significant differences between patients and controls, as regards femoral anteversion, patellar congruence angle, patellar tilt angle and anterior tibial tuberosity-trochlear groove distance, but the condylomalleolar angle and tibial torsion angle were greater in patients. We found no differences between symptomatic and asymptomatic or less symptomatic knees in any of the parameters. All the symptomatic knees were on the side preferentially involved in jumping and sprinting. This increase in external tibial torsion may play a role as a predisposing mechanical factor in the onset of Osgood-Schlatter disease in male athletes.     

Tumor-associated glycoprotein-72 serum levels complement carcinoembryonic antigen levels in monitoring patients with gastrointestinal carcinoma. A longitudinal study

Eighty-two patients diagnosed with gastrointestinal (GI) adenocarcinoma were evaluated before and for 26 months after primary tumor resection for the presence of two serum tumor markers: tumor-associated glycoprotein-72 (TAG-72) and carcinoembryonic antigen (CEA). Elevated TAG-72 and CEA serum levels were found preoperatively in 32 (39%) and 34 (41.5%) of the 82 patients, respectively. The percentage of patients with elevated serum levels of either TAG-72 or CEA was 56.1% (46 of 82). Twelve (15%) patients who had normal CEA serum levels had elevated TAG-72 serum levels, and conversely, serum from 14 (17%) patients who were TAG-72 negative were CEA positive. Forty-five of the 82 patients were diagnosed with advanced disease (i.e., Stages C and D for colorectal, Stages III and IV for stomach), and 29 (64.4%) and 26 (57.8%) of those patients had elevated serum levels of TAG-72 or CEA, respectively. Elevated levels of either TAG-72 or CEA, however, were found in sera of 82.2% of patients with advanced GI cancer, which is an increase of 24.4% over the use of CEA antigen alone as a marker of disease. The measurement of both TAG-72 and CEA may improve the diagnosis of patients with GI malignant disease due to the apparent complementary association which exists between these tumor markers. Serum TAG-72 and CEA levels were monitored in 31 patients for varying lengths of time after resection of the carcinoma; 11 patients developed recurrent disease. Sera from nine of 11 (81.8%) of these patients had elevated TAG-72 levels and six of 11 (54.5%) had elevated CEA levels. Tumor marker elevations were observed either before (35 to 166 days) or at the time of diagnosis of recurrence. The elevation of one or both markers correlated with the clinical status in ten of 11 (90.9%) patients with recurrence. In addition, 20 patients who were clinically free of disease after more than 700 days' follow-up had normal serum levels of both TAG-72 and CEA. These findings suggest that the combined use of serum TAG-72 and CEA measurements may improve detection of recurrence in patients with GI cancer and may be useful in the postsurgical management of GI adenocarcinoma patients.     

The role of IFN-alpha as homeostatic agent in the inflammatory response: a balance between danger and response?

Interferon-alpha (IFN-alpha) is thought of by most immunologists as a fundamental component of the antiviral innate immune response, with other, accessory properties. Several lines of evidence point to a fundamental reappraisal of this conceptual framework because it may neglect other crucial functions of this cytokine under both health and disease conditions. Among these, a regulatory role in the inflammatory response is probably of paramount importance, as shown by in vivo and in vitro studies on humans, pets, and farm and laboratory animals. This role would not conflict with the main functions of IFN-alpha in the innate immune system and would complement these in line with major, evolution-based needs of the host. A hinge role of IFN-alpha between innate and adaptive immunity was recognized in the past on the basis of compelling evidence. This concept should now be widened; not only does IFN-alpha act to trigger, amplify, and sustain the different phases of the immune response, but it could also promote a substantial balance between danger and inflammatory response when an infectious challenge is either declining or completely over.     

Transposition of the great arteries and isolated origin of the sinus node artery

We report the cases of 2 newborns who underwent at 7 days of age an arterial switch operation for transposition of the great arteries with a rare coronary anomaly: the left and right coronary arteries originated with a single ostium from sinus 1 and the sinus node artery had an isolated origin from sinus 2. The sinus node artery was reimplanted into the new aorta in both patients. Both babies were discharged in sinus rhythm. Preserving the vascularization of the sinus node may avoid the occurrence of postoperative atrial rhythm disturbances.     

Rest-injected thallium-201 redistribution and resting technetium-99m methoxyisobutylisonitrile uptake in coronary artery disease: relation to the severity of coronary artery stenosis

To compare rest-injected thallium-201 (Tl) redistribution and resting technetium-99m methoxyisobutylisonitrile (^99mTc-MIBI) myocardial uptake in chronic coronary artery disease (CAD), 15 patients with angiographically proven CAD and left ventricular (LV) dysfunction (ejection fraction 34%±9%) were studied. All patients underwent rest-redistribution Tl and resting ^99mTc-MIBI cardiac imaging. Gated ^99mTc-MIBI images were also acquired to assess regional LV wall motion (WM). Myocardial segments (n=225) were divided into three groups on the basis of the degree of coronary artery stenosis: group 1 (total occlusion, n=82), group 2 (50%–99% of stenosis, n=84) and group 3 (<50% of stenosis, n=59). WM was significantly worse in groups 1 and 2 compared to group 3 (P<0.001), but no difference was observed between groups 1 and 2. TI and ^99mTc-MIBI uptake were significantly lower in groups 1 and 2 compared to group 3 (P < 0.001), and in group 1 compared to group 2 (P<0.001). When TI and ^99mTc-MIBI uptake were directly compared, TI uptake was higher than ^99mTc-MIBI uptake in group 1 (P<0.001), while no significant difference was observed in groups 2 and 3. Thus, both rest-injected TI redistribution and resting ^99mTc-MIBI uptake reflected the severity of coronary artery stenosis in CAD. However, in myocardial segments with total coronary occlusion T1 uptake was significantly higher than ^99mTc-MIBI uptake. Our data suggest that rest-injected Tl redistribution cardiac imaging may identify, more accurately than resting ^99mTc-MIBI imaging, the presence of viable myocardium in chronic CAD, particularly when the coronary blood flow is severely impaired.     

Peripheral nerve involvement in Churg-Strauss syndrome

Summary Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common history of a prodromal phase of allergic diseases (bronchial asthma and rhinitis) followed by a vasculitic phase with mononeuritis multiplex, purpura and arthritis, associated with hypereosinophilia of more than 1500 cells/mm³. All responded well to steroid treatment. Sural nerve biopsy revealed true vasculitis in two of these cases and a mild perivascular inflammatory infiltration in the other. On the basis of their characteristic clinical pattern, we think that our cases best fit the diagnosis of Churg-Strauss syndrome even though the typical histological features were not found in the sural nerves examined.     

“Occult” hydrocephalus in children

The authors describe 32 children between 2 and 15 years of age who had hydrocephalus that was only clinically manifest late in life. The clinical picture of these children did not suggest an obvious increase in intracranial pressure; instead, the presenting signs were rather nonspecific and included macrocrania, mild psychomotor retardation, unsteady gait, increased muscle tone and deep tendon reflexes in the lower limbs, impaired ocular movement, epilepsy, and endocrine dysfunction. Their histories suggest the possible causes of the ventricular dilation in about one third of the cases were: perinatal hemorrhage, leptomeningitis, neurofibromatosis, and untreated aneurysm of the great vein of Galen. In 20 patients, however, no positive anamnestic findings were reported. CT scan revealed triventricular dilation in more than half of the cases; tetraventricular dilation was present in 6 patients, and biventricular dilation in the remaining subjects. All children underwent CSF shunting, which resulted in complete recovery in all but 2 cases. The most frequently recorded surgical complication was postoperative subdural effusion (7 subjects), which required surgical treatment in only 2 cases.Presented at the 15th Annual Scientific Meeting of the International Society for Pediatric Neurosurgery, New York, 1987