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.     

Narrowband UVB therapy for vitiligo: does the repigmentation last?

BACKGROUND: Since 1997, a number of trials have shown promising results in treating generalized vitiligo with narrowband ultraviolet B (UVB) both in adults and children. However, there is little knowledge concerning the duration and permanency of the treatment-induced repigmentation. OBJECTIVE: Our main objective was to perform a follow-up trial of successfully treated patients receiving narrowband UVB for generalized vitiligo. METHODS: We have investigated to what degree the treatment-induced repigmentation remains stable for up to 2 years post-treatment. We performed an initial open trial including 31 patients with generalized vitiligo. They received narrowband UVB thrice weekly for up to 12 months. Patients experiencing > 75% repigmentation were defined responders and were included in the follow-up trial. Responders were followed every 6 months for up to 2 years after cessation of treatment. We observed the pigmentation status and registered any changes indicating loss of pigmentation and relapse. RESULTS: Eleven of the 31 treated patients were included in the follow-up trial. Six patients had relapse and five patients had stable response 24 months after cessation of treatment. Four out of six relapses were within 6 months post-treatment. CONCLUSION: In our study population of 31 patients with generalized vitiligo, five patients (16%) experienced > 75% stable repigmentation 2 years after cessation of a treatment programme of up to 1 years narrowband UVB therapy.     

Ongoing pregnancies after intracytoplasmic injection using cryopreserved testicular spermatozoa

We report two clinical pregnancies occurring after intracytoplasmic sperm injection (ICSI) using cryopreserved spermatozoa obtained from testicular biopsy, made in two different infertility situations in our clinic. The first patient showed a secretory azoospermia associated with elevated serum follicular stimulating hormone (FSH) level and spermiogenesis maturation arrest. The second patient was affected by azoospermia resulting from bilateral epididymal obstruction. Spermatozoa present in the wet preparation of testicular biopsy made on the day of scrotal exploration were cryopreserved within the testicular tissue for both men. Intracytoplasmic injections were performed at a later date, using spermatozoa prepared from frozen-thawed tissues. In each case, three embryos were obtained and transferred in utero. The transfers resulted in a twin pregnancy for the first case, and in a singleton pregnancy for the second. Living foetuses were seen in the ultrasound scan at the 7th week and both pregnancies are proceeding to date beyond 30 weeks without complications.      

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.