Response to: myositis after SARS-CoV-2 vaccination occurs more frequently than assumed and is probably causally related.

We thank Finsterer et al. for the attention paid to our publication; we recognize the validity of the points mentioned in their letter to the editor and will try to answer the observations made.

Idiopathic inflammatory myopathies linked to vaccination against SARS-CoV-2: a systematic review.

The COVID-19 pandemic represents a global health problem, which has been mitigated by the opportune introduction of vaccination programs. Although we already know the benefit that vaccines provide, these are not exempt from adverse events which can be mild to deadly, such as idiopathic inflammatory myopathies, in which a temporal association has not been defined. It is for this reason that we carried out a systematic review of all reported cases of vaccination against COVID-19 and myositis. To identify previously reported cases of idiopathic inflammatory myopathies associated with vaccination against SARS-CoV-2 we registered this protocol on the website of PROSPERO with identification number CRD42022355551. Of the 63 publications identified in MEDLINE and 117 in Scopus, 21 studies were included, reporting 31 cases of patients with vaccination-associated myositis. Most of these cases were women (61.3%); mean age was 52.3 years (range 19-76 years) and mean time of symptom onset post-vaccination was 6.8 days. More than half of the cases were associated with Comirnaty, 11 cases (35.5%) were classified as dermatomyositis, and 9 (29%) as amyopathic dermatomyositis. In 6 (19.3%) patients another probable trigger was identified. Case reports of inflammatory myopathies associated with vaccination have heterogeneous presentations without any specific characteristics: as a consequence, it is not possible to ensure a temporal association between vaccination and the development of inflammatory myopathies. Large epidemiological studies are required to determine the existence of a causal association.

Human foamy virus DNA forms and expression in persistently infected Dami megakaryocytic cells.

We have characterized human foamy virus (HFV) proviral DNA and determined HFV expression in a persistent infection model, the Dami megakaryocytic cell line. Molecular studies were performed on parental persistently infected cells (Dami-P), as well as on derived clones (Dami-Cl). We report that in these nonlytic and non-HFV producer cells, viral DNA was found to be integrated into the cellular genome and that the few free proviral forms detected in Dami-P cells were deleted in their 5' LTR. Our molecular analysis indicates the presence of undeleted 5' LTR forms in the integrated provirus within a proviral population mainly composed of deleted forms. In addition, the deletion in the bel1 trans-activator gene, previously described by Saïb et al., was found to be highly predominant. However, in 5-iodo-2'-deoxyuridine treated Dami-Cl cultures, virus production occurred, providing evidence for the presence of complete viral genome. Analysis of HFV expression in Dami-Cl cells, by Northern blot and immunoprecipitation, shows that the most striking difference between cytolytic and persistent HFV infection was the lack of expression of structural viral proteins, in contrast with Bet protein expression, which is maintained. Our data suggest that the Bet protein could be involved in the maintenance of viral persistency and that the persistently infected Dami system provides a suitable model for clarifying its function.

Anatomic correction of transposition of the great arteries.

Complete anatomic correction of transposition of the great arteries (TGA) and ventricular septal defect (VSD) was performed on 2 infants with high pulmonary arterial resistance. Both patients were operated upon under deep surface-induced hypothermia and limited cardiopulmonary bypass. Direct repair of the anomaly was accomplished by switching the aorta and the pulmonary artery with reattachment of the coronary arteries. The clinical result in the first patient was satisfactory. The postoperative cardiac catheterization and angiogram demonstrated no gradient across either outflow tracts and normal arrangement of the great vessels. The second patient died in the operating room, probably owing to compression or kinking of the left coronary artery.

Tetralogy of Fallot with subarterial ventricular septal defect. Diagnostic and surgical considerations.

Since 1964, 77 patients underwent repair for tetralogy of Fallot with subarterial ventricular septal defect. Median age at operation was 5 years. Cyanosis was commonly mild, and hypoxic episodes were infrequent. Accordingly, only 12 patients (15.58%) needed either palliative or corrective operations before the age of 2 years. Characteristic angiographic and echocardiographic features were observed, which allowed this entity to be differentiated from either classic tetralogy or other types of double-outlet right ventricle. The earliest series of operations (in which no right ventricular outflow patch was used) was followed by a prohibitive mortality (50%). Residual subpulmonary obstruction was the cause of all of these deaths. In a second series of patients, a transannular patch was frequently used (79.59%), with the rationale that closure of the ventricular septal defect would make restrictive the deficient infundibulum of these patients. In a later series, we observed that most of the patients had a nonrestrictive pulmonary anulus (80%); the patch therefore was limited to the ventriculotomy. In the absence of pulmonary annular hypoplasia, the need for patching of the right ventricular outflow tract in this entity has to be confined to preventing the subpulmonary obstruction induced by closure of the ventricular septal defect. A considerable improvement in the results followed our first series (4.3% mortality). Follow-up of the survivors (mean 7 years) has been satisfactory.

Atriopulmonary anastomosis.

Atriopulmonary anastomosis (APA) has been performed in 29 patients, 3 to 22 years of age, since 1971. The diagnoses were tricuspid atresia in 21, single ventricle with low pulmonary vascular resistance in seven, and one case of dextro-transposition of the great arteries with ventricular septal defect and pulmonary stenosis. Four different techniques were used: Technique I (anterior end-to-end APA with a homograft or Dacron tube); Technique II (anterior end-to-end APA with the patient's own pulmonary artery); Technique III (nonvalved anterior anastomosis between the right atrium and the right ventricle); and Technique IV (largest, posterior, nonvalved direct APA between the right atrium and the main pulmonary artery and its right branch). Since the right atrium does not function as a pump, caval valves were never used. The total hospital mortality was 17.2%. Proper patient selection and the development of Technique IV reduced the mortality to 9%. Low end-diastolic ventricular pressure and a nonrestrictive APA are mandatory to obtain a good clinical result without pleural effusion. Twenty-one survivors are in Functional Class I, 17 of them without medication. Twelve of the 24 survivors were recatheterized. The best clinical and hemodynamic results were achieved in patients with low right atrial pressure and low end-diastolic ventricular pressure. The follow-up demonstrated a consistent superiority of the posterior nonvalved APA (Technique IV) in comparison with other techniques described. Therefore, this technique is proposed as the procedure of choice for the performance of an APA, irrespective of the precise diagnosis (tricuspid atresia or single ventricle) and irrespective of the type of great arterial relationship.

Tetralogy of Fallot with subpulmonary ventricular septal defect.

Among a total of 608 patients with tetralogy of Fallot, 35 with tetralogy and subpulmonary ventricular septal defect (VSD) have undergone intracardiac repair at the Children's Hospital, Buenos Aires. The crista supraventricular was absent in 62.9% of these patients; it was hypoplastic in 37.1%. Only 3 patients (8.6%) were severely cyanotic and required operation in infancy. The main and left and right pulmonary arteries were relatively large in all but 1 patient. Nevertheless, patch-graft enlargement across the pulmonary valve ring was necessary in 20 (57.1%). We believe that closure of the subpulmonary VSD increase the apparent severity of the pulmonary stenosis and necessitates patch-graft enlargement in patients in whom the appearance of the right ventricular outflow tract before repair would not have suggested its need.

Does serum rheumatoid factor have an influence on the clinical picture of ankylosing spondylitis?

OBJECTIVE: To describe the influence of serum rheumatoid factor (RF) on the clinical and radiological picture of definite ankylosing spondylitis (AS). METHODS: In a retrospective chart review of 281 AS patients typed for RF, the clinical picture of RF positive patients (Group 1) was compared with RF negative patients (Group 2); mode of onset, disease duration, and treatment were recorded. All patients were examined to determine their clinical status; the blood cell count. HLA-B27, serum IgG, IgM, IgA, and erythrocyte sedimentation rate (ESR) were determined, and radiological studies of the entire spine, pelvis and affected peripheral joints were carried out. In patients from Group 1 the HLA-DR was also determined. RESULTS: Fifteen of 281 patients (8 men, 7 women) with AS were RF+ (1:64 to 1:1024) (5.3%) and 11 were HLA-B27+. Seven patients in Group 1 had spine involvement and chronic arthritis of the knees. Four out of these 7 were tested for DR, and none was positive; in 6, AS and rheumatoid arthritis (RA) coexisted, 2 were DR1 and 2 were DR4 (test not carried out in 2). In two others we found spinal involvement only, and one of them had both DR1 and DR4. The onset of AS was similar in both groups. Group 1 was characterized by a chronic disease of moderate intensity with chronic arthritis of the metacarpophalangeal and proximal interphalangeal joints (p = 0.0008 and p = 0.04, respectively), no valvulopathy (p = 0.04) and fewer uveitis sequelae (p = 0.007) than Group 2. The ESR (p = 0.01), IgG (p = 0.008) and IgM (p = 0.0001) were higher in Group 1 than in Group 2. CONCLUSIONS: The presence of RF in AS is associated with a chronic disease of moderate intensity with chronic peripheral arthritis and fewer extra-articular manifestations. The presence of RF, not always associated with HLA-DR, seems to affect the course of AS and does not necessarily indicate an association with RA.

Intestinal colonization of the infant mouse model by attenuated and virulent Vibrio cholerae strains.

BACKGROUND: Intestinal colonization of humans with virulent Vibrio cholerae stimulates substantial, lasting immunity against reinfection. The purpose of this study was to evaluate the colonizing capability of various Vibrio cholerae strains which are promising candidates to oral vaccine. METHODS: Infant mouse model modification was used. In order to standardize the method, several parameters were tested, such as culture medium and optimal time of incubation and appropriate number of cells to be inoculated. The following were tested: Vibrio cholerae strain 81, 413, and 251A, which were obtained at the Molecular Biology Department of the National Center for Scientific Research, Havana, Cuba. Their virulence cassettes which code for the main virulence factors were deleted. RESULTS: Good variance coefficient (VC) was obtained in repeated experiments. The colonizing properties of attenuated Vibrio cholerae strains evaluated by this method correlated well with those observed for parental strains. CONCLUSIONS: Genetically attenuated Vibrio cholera strains have the same intestinal colonization level as their parental strains in the infant mouse model; thus, genetic manipulation does not affect genes that encode for the synthesis of colonization factors.

IFAP syndrome "plus" seizures, mental retardation, and callosal hypoplasia.

Ichthyosis follicularis, congenital alopecia, and photophobia are typical features of a rare X-linked recessive disorder termed ichthyosis follicularis with atrichia and photophobia syndrome. A 3-year-old male with these findings and severe growth failure, mental retardation, generalized seizures, vascularizing keratitis, nail anomalies, inguinal hernia, and a normal chromosome constitution is presented. Two maternal male relatives were affected by the same condition. Magnetic resonance imaging revealed corpus callosum hypoplasia not described at present. Syndromes with alopecia, seizures, and mental retardation are analyzed on the basis of genetic and clinical results.

Atrichia, ichthyosis, follicular hyperkeratosis, chronic candidiasis, keratitis, seizures, mental retardation and inguinal hernia: a severe manifestation of IFAP syndrome?

A boy with congenital atrichia, ichthyosis follicular, keratitis, cutaneous infections and a huge inguinal hernia, but without deafness is reported. We believe it represents a new case of a rare X-linked recessive syndrome known as ichthyosis follicularis, alopecia, photophobia syndrome (IFAP). The differential diagnosis from keratitis ichthyosis deafness is discussed. The cutaneous infections seen in our case suggest the possibility of considering a genetic link between these syndromes.

Two-dimensional echo Doppler findings in juvenile and adult onset ankylosing spondylitis with long-term disease.

The authors' objective was to determine by 2-dimensional echo Doppler (2DECHO) the cardiac abnormalities in juvenile onset ankylosing spondylitis (JOAS) and adult onset ankylosing spondylitis (AOAS) in male patients with long-term disease. Twenty patients with JOAS, 31 with AOAS, and 20 healthy controls of the same age and gender without cardiopulmonary symptoms were studied. Using 2DECHO, the heart dimensions were determined according to American Society of Echocardiography guidelines. The left ventricle ejection fraction (LVEF) was calculated by Teichholz's formula. Cardiomyopathy was established when 2DECHO had diminished LVEF. Statistics used were the Student t and Fisher test, chi2, and ANOVA. Ninety percent of JOAS and 51% of AOAS patients were B27+ (p=0.005). The disease duration was 19.3 +/- 8.8 years in JOAS and 14.8 +/- 12.8 years in AOAS (p=NS). Age at the time of the study was 30.7 +/- 9.9 years in JOAS vs 40.3 +/- 12.7 in AOAS (p=0.003), and vs 40.2 +/- 17 years in controls (p=NS). There was a higher frequency of cardiomyopathy in AOAS (32.2%) than in JOAS (25%) and the controls (0%) (p=0.01). Patients with JOAS had a higher mitral valve gradient (25%) than AOAS patients (19%, p=NS) and controls (0%, p=0.04). Abnormal aortic ring reflectance was shown in 19% of AOAS vs 0% abnormalities in JOAS and controls (p=0.01). The aortic root diameter was increased in 58% of AOAS, 30% of JOAS, and 0% of controls (p=0.001). The frequency of 2DECHO abnormalities was increased in cardiopulmonary asymptomatic spondylitis patients. Despite the high frequency of B27+, JOAS had a lower frequency of aortic abnormalities than AOAS. Mitral valve gradient was found in JOAS and in AOAS that could contribute to a decreased ejection fraction and to left ventricular dysfunction.

[Interventricular communications with aortic insufficiency surgical considerations]. / Les communications interventriculaires avec insuffisance aortique. Considérations chirurgicales

The authors report a series of 11 patients with ventricular septal defect associated with aortic incompetence who underwent surgery between 1963 and March 1976. Separate consideration is given to the operations performed before and after 1972. 5 patients were operated on during the first period, with only mediocre results. At this time, the technique of valvuloplasty did not appear to be the right one. During the second period, 6 patients were operated on. In five of them the technique of Plauth, Frater, Spencer and Trusler was used. All these patients have a satisfactory result. The last of the series had an abnormally low commissure, and the adjacent valves were protuberant, and thickened, so that a valvular replacement was carried out. The operation of choice for a ventricular septal defect associated with aortic incompetence is valvuloplasty with closure of the defect. The following criteria are necessary indications for this type of surgery: --the aortic incompetence must be secondary to the prolapse of a valve; --the aortic valve must have three cusps. An early operation makes the valvuloplasty easier, and avoids the problems of damage to the left ventricle from a persistant and progressive aortic incompetence.

Experimental and theoretical vibrational study of methylene bis(thiocyanate), CH2(SCN)2. A comparison with thiocyanogen, (SCN)2.

The infrared and Raman spectra of methylene bis(thiocyanate), CH2(SCN)2, were obtained. The observed bands were assigned to the different normal modes of vibration using the results of a DFT calculation of the molecular vibrational properties. These results and the experimental data were used to define a Scaled Quantum Mechanics force field for the molecule. A similar treatment was applied to the thiocyanogen molecule, (SCN)2, for which the experimental frequencies were already reported in the literature. The sets of internal force constants for both molecules show very similar values.

A study on parasites in Mexican rheumatic disease patients.

Blastocystis hominis is a common human parasite with infection rates up to 50% in developing countries, and giardiasis is the commonest intestinal one in Mexico. No doubt, various parasites as Giardia lamblia and Entamoeba histolytica can cause rheumatic diseases. This study coproparasitoscopic analysis evaluated the cysts by B. hominis, G. lamblia, E. hartmani, E. coli and E. histolytica in Mexican rheumatic disease patients. Also, ELISA was used to detect E. histolytica, Ascaris lumbricoides, Toxocara canis, and Trichinella spiralis in Mexican patients with rheumatoid arthritis (RA) and ankylosing spondylitis (AS). Thirty-six patients (24 with AS and 12 with RA) and 77 healthy control individuals were enrolled in this study. The frequencies of protozoan cysts were comparable in rheumatic disease patients (AS and RA) and healthy control donors (33 and 25 vs. 26%, respectively; p > 0.05). The frequency of antibodies to T. canis was significantly higher in AS patients than in healthy control donors (16 vs. 2.6%, respectively; p = 0.027), whereas no differences were observed for the prevalence of antibodies for the other parasites (E. histolytica, A. lumbricoides and T. spiralis) (p > 0.05). This information indicates the need to intensify educational efforts for the prevention of parasite infections associated with AS disease that cannot be controlled only by drugs.