Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

Early embryonic loss associated with immune activation in cattle and sheep

Increased peripheral NK cells in aborting women are considered as a predictor of immunological miscarriages. Here, we investigated the diagnostic value of the NK count at the time of abortion. Peripheral NK cells were counted by flow cytometry in 44 women undergoing therapeutic termination of first trimester missed pregnancy (A = 24) or elective abortion (B = 20). Histology of the extracted material was performed and immunological lesions (villitis, intervillositis, vascular thrombosis, increased fibrinoid necrosis) were recorded. Twenty first trimester pregnant women (C1) and 20 nonpregnant women (C2) served as controls. In 20 women of groups A and B (A1 = 10, B2 = 10) count was repeated after 5 days. At abortion time, the NK percentage and number did not differ between groups A and B (18.18%, 360 versus 15.61%, 374), but there was a statistically significant difference between A and C1 (18.18% versus 13.6%, P < 0.03). When histology negative (11) and positive (8) cases were excluded from groups A and B, respectively, NKs were slightly higher in group A (18.18–19.76%) and significantly lower in B (15.61–13.07%, P < 0.05). At the time of second count, aborters' NKs decreased (A1 = 16.64 versus 13.6 and 10.6 in groups C1 and C2 –P = 0.01, respectively). In group B, NK percentage was equal to that of nonpregnant women (10.94 versus 10.6). At the time of abortions, NKs increase only in cases of immune‐mediated miscarriages. Their – in time – count may contribute to the diagnosis of immmunological abortions.     

Detecting pediatric autoimmune neuropsychiatric disorders associated with streptococcus in children with obsessive-compulsive disorder and tics.

BACKGROUND: A subgroup of children with obsessive-compulsive and tic disorders are proposed to have an infectious trigger. The purpose of this study was to investigate the relationship between group A streptococcal titers and symptom fluctuations in children with a clinical course resembling that described for pediatric autoimmune neuropsychiatric disorders associated with streptococcus. METHODS: Twenty-five children with obsessive-compulsive disorder and/or tic disorder were evaluated for neuropsychiatric severity and group A streptococcal antibody titers (streptolysin O, deoxyribonuclease B, and carbohydrate A) at 6-week intervals for > or = six consecutive evaluations (total visits=277). RESULTS: Children with large symptom fluctuations (n=15) were compared with children without dramatic fluctuations (n=10). Co-movements of obsessive-compulsive/tic severity and group A streptococcal antibodies were assessed. In subjects with large symptom changes, positive correlations were found between streptococcal titers and obsessive-compulsive severity rating changes (p=.0130). These subjects were also more likely to have elevated group A streptococcal titers during the majority of observations (p=.001). Tic symptom exacerbations occurred more often in the fall/winter months than spring/summer months (p=.03). CONCLUSIONS: Patients with marked obsessive-compulsive/tic symptom changes may be characterized by streptococcal titer elevations and exhibit evidence of seasonal tic exacerbations.     

The relation between the plasma lipoprotein pattern and the waist/hip ratio in non-diabetic individuals

Relations between lipoprotein indices and the waist/hip ratio (WHR) were examined in normoglycemic subjects (124 women and 63 men). Among women, an atherogenic lipoprotein pattern was strongly associated with WHR. The relation was greater than that of the lipoprotein pattern with either the body mass index or the subscapular/triceps ratio, and was independent of these adiposity measures. The association between low-density-lipoprotein cholesterol (LDL-cholesterol) and WHR was much stronger in thinner women than in those more obese (r = 0.64 vs r = 0.22, p = 0.001 for difference). Men had a more atherogenic lipoprotein pattern than women; however, with allowances for WHR the lipoprotein patterns were similar. These findings suggest that: (1) WHR is more strongly related to plasma lipoproteins than other adiposity measures; (2) there is effect modification by the extent of adiposity for the relation between LDL-cholesterol and WHR; and (3) the adipose distribution may be important in explaining lipoprotein differences between men and women.     

Pancreatic islet cell tumors

Tumors arising from the pancreatic islet cells represent a heterogeneous group of lesions. Some tumors present with well-characterized syndromes, while others appear to be nonfunctioning. Eighty-four patients with pancreatic islet cell tumors operated on at the Cleveland Clinic during a 35-year period were reviewed. The tumor types include 21 nonfunctioning tumors, 41 insulinomas, 16 gastrinomas, two vasoactive intestinal polypeptide (VIP)-omas, two carcinoids, and two probable cases of pancreatic parathyrinoma. Eleven patients had multiple endocrine neoplasia type I syndrome. Preoperative localization was possible in 63% of patients in whom it was attempted. Complete mobilization of the head and distal pancreas with bimanual palpation of the entire gland is critical for intraoperative tumor localization. Distal pancreatectomy is favored for tumors in the body and tail. In the head of the pancreas, small, benign lesions require enucleation, and large or malignant lesions necessitate a Whipple procedure. The operative morbidity rate was 24%, and the mortality rate was 3.6%. The 10-year survival rate was 54.7% for nonfunctioning lesions, 68.4% for gastrinomas, and 92.4% for insulinomas. At this time surgery represents the only way to cure these lesions.     

Cinefluoroscopic evaluation of periprosthetic cardiac valve regurgitation

The results of cinefluoroscopic evaluation in 509 patients in whom there was no evidence of prosthetic mitral or aortic valve regurgitation were compared with those in 41 patients who had perivalvular aortic or mitral regurgitation. Rotational motion of the base ring of each prosthesis (base-ring tilt) was measured in at least two views. A base-ring tilt of 7 degrees or more for aortic prostheses or 11 degrees or more for mitral prostheses was associated with an increased incidence of significant perivalvular regurgitation. Likewise, in patients who had multiple studies, a change between studies in base-ring tilt of 4 degrees or more for aortic prostheses or 5 degrees or more for mitral prostheses was associated with significant perivalvular regurgitation. These data suggest that the presence of either an abnormal base-ring tilt or an abnormal increase in base-ring tilt is strong, supportive evidence of partial prosthetic valve dehiscence.     

Experimental models of hypersensitivity pneumonitis.

Experimental models of hypersensitivity pneumonitis are important tools for the study of the pathogenesis of this disease. In this paper we review the characteristics of the main animal models developed until now. The HP models in rats seem to be particularly appropriate for studying pigeon fancier's disease and the HP induced by chemicals, as well as for studying mediators of acute lesions induced by immunocomplexes. However, the HP models developed in rats are of less value in the evaluation of other aspects of the pathogenesis of this clinical entity in humans. The murine models of HP offer several advantages: the ease and simplicity of intranasal administration, the ability to produce acute and subacute pulmonary lesions similar to those found in humans, the possibility of reproducing lesions similar to those of nonaffected exposed subjects and the possibility of pharmacologically modulating the process. Their disadvantages lie in the different pulmonary lymphocyte response and the difficulty in reproducing a model of chronic fibrosis. The HP models in rabbits are extraordinarily useful for evaluating the immunological mechanisms through which subjects repeatedly exposed to the antigen do not develop clinical manifestations. However, the rabbit has several immunological differences when compared to humans, and the effect of some immunomodulators in this animal is different. The models of HP in guinea-pigs have as advantages the ease in handling the animals, the possibility of pharmacological manipulation, and the ability to induce an acute phase that is very similar to that observed in humans. The drawback, however, is the low lymphocyte response and the striking eosinophilic reaction that contrast with the bronchoalveolar data found in HP in humans. In conclusion, there is no ideal model to reproduce all the findings observed in humans, suggesting that the experimental animal and the method of developing HP should be selected on the basis of concrete research aims.     

Heterotopic heart transplantation: a radiographic review

Heterotopic heart transplantation can be performed in the presence of high pulmonary vascular resistance. The authors call attention to a rare, but potentially life-saving procedure.     

Evaluation of API Coryne system for identifying coryneform bacteria.

AIM--To identify rapidly and accurately coryneform bacteria, using a commercial strip system. METHODS--Ninety eight strains of Corynebacterium species and 62 additional strains belonging to genera Erysipelothrix, Oerskovia, Rhodococcus, Actinomyces, Archanobacterium, Gardnerella and Listeria were studied. Bacteria were identified using conventional biochemical tests and a commercial system (API-Coryne, BioMèrieux, France). Fresh rabbit serum was added to fermentation tubes for Gardnerella vaginalis isolates. RESULTS--One hundred and five out of the 160 (65.7%) organisms studied were correctly and completely identified by the API Coryne system. Thirty five (21.8%) more were correctly identified with additional tests. Seventeen (10.6%) organisms were not identified by the system and three (1.9%) were misidentified. CONCLUSIONS--The system was a good alternative for identification of coryneform organisms. When occasionally performed with some additional tests, this method permits reliable and rapid identification of coryneform organisms compared with conventional methods.