Síndrome compartimental agudo / Acute compartmental syndrome

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Diagnosis of Mycobacterium avium complex in granulomatous lymphadenitis in slaughtered domestic pigs.

The aim of this study was to compare two diagnostic methods for the detection of Mycobacterium avium complex (MAC) infection in lymph nodes with granulomatous lymphadenitis from slaughtered domestic pigs. Fifty affected lymph nodes were collected from 50 pigs and examined microscopically and by polymerase chain reaction (PCR). Microscopically, granulomatous lesions were observed in 92% of the samples, consisting mostly of central necrosis (78%) with dystrophic calcification (46%) and associated with inflammatory infiltration by epithelioid giant cells, lymphocytes, neutrophils (92%), eosinophils (60%) and Langhans-type cells (70%). In 64% of the lesions, a capsule of connective tissue was found. Acid-fast bacilli were observed in all cases. PCR detected DNA from Mycobacterium spp. in 82% (41/50) of the lymph nodes. MAC was confirmed in 58% (24/41) and M. avium avium/silvaticum subspecies in 39% (16/41). The results of this study suggest that combined histopathology and PCR of lymph nodes are useful in the diagnosis of granulomatous lymphadenitis in slaughtered pigs.

Reversão de bloqueio cardíaco fetal após terapêutica com dexametasona: caso clínico / Reverted fetal heart atrioventricular block after treatment with dexametasone: a clinical case

Los autores presentan un caso clínico de bloqueo cardíaco fetal, diagnosticado a las 20 semanas de embarazo, asociado a la presencia de anticuerpos antinucleares maternos, que revertió tras tratamiento con dexametasona

The authors present a case report of a fetal heart atrioventricular block, diagnosed at 20 weeks of gestation, associated with maternal antinuclear antibodies, that reverted after a short course of dexamethasone treatment

Enfermedad celíaca, fibrosis quística y miocardiopatía dilatada / Celiac disease, cystic fibrosis and dilated cardiomyopathy

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Cardiovascular manifestations in Marfan syndrome.

INTRODUCTION: Cardiovascular complications are the major cause of morbidity and mortality in Marfan syndrome (MS), a common connective tissue disorder. Currently it is considered that the prognosis and morphologic characteristics in infantile Marfan syndrome may be quite different from those reported in older patients. The objective of this study was to analyze the cardiovascular manifestations and evolution of the patients with Marfan syndrome followed at our pediatric cardiac unit. METHODS: The authors reviewed the clinical files of all the patients that fulfilled the diagnostic criteria for MS according to De Paepe et al. (1996). We analyzed the following parameters: gender, age at referral, race, family history, clinical examination, diagnostic exams, therapy and evolution. The patients were divided into two groups according to the age at diagnosis: infants (group 1) and older patients (group 2). RESULTS: Group 1 included 3 infants, two boys and one girl, sporadic cases, presenting congestive heart failure. The major cardiac diagnoses were aortic dilatation (1/3) and mitral valve prolapse with severe mitral regurgitation (2/3). Congenital heart disease was associated in two cases (patent ductus arteriosus and atrial septal defect). Two needed cardiac surgery at an early age and one was recently proposed for surgery. There were no deaths. Group 2 included 20 patients, 14 boys and 6 girls, first seen at a mean age of 8 years. Ten had a positive family history and none presented cardiac symptoms. The major cardiac manifestations were mitral valve prolapse (18/20) and aortic dilatation (17/20). There was no significant progression of the cardiac lesions, except for one case, during the 12 years of follow-up. CONCLUSION: Infantile MS presented high morbidity; mitral regurgitation was severe in two cases. All patients presented heart failure, two needing early operations. In classic MS evolution was favorable, and the commonest cardiac lesions were mitral valve prolapse and aortic dilatation. We emphasize the need for beta-blockers to prevent progression of aortic dilatation. The decision for surgery rests upon the severity of valve regurgitation and the rate of progression of aortic dilatation. It is important to inform patients and family about physical exercise, prevention of endocarditis, risks associated with pregnancy and genetic counseling.

[Rheumatic fever--a review of cases]. / Febre remática--revisão casuística.

OBJECTIVE: To analyse clinical presentation of rheumatic fever (RF), with special emphasis on cardiac involvement, electrocardiographic and echocardiographic findings and the outcome of the cases referred to Maria Pia Children's Hospital from January 1990 to September 1999. METHODS: We retrospectively analyzed the clinical files of all cases referred to pediatric cardiology clinics with the suspicion of acute RF (Group 1) or with rheumatic valvular disease and heart failure (Group 2). In group 1 we studied the following: age and sex distribution, year of diagnosis, presence of Jones criteria treatment and outcome. In group 2 we analysed provenance, age of initial onset of RF, age of cardiology referral, treatment and outcome. RESULTS: Thirteen cases were identified, 8 in groups 1 and 5 in group 2. Group 1 included 3 girls and 2 boys, mean age of 10 years. The diagnosis of RF was based in the presence of 2 major and 1 minor manifestation (4/8), 1 major and 2 minor manifestations (1/8) and chorea in 3 cases associated with clinical carditis in one and subclinical carditis in another. Colour Doppler echocardiography showed pathological mitral regurgitation jet in 6 cases, associated with aortic regurgitation in 2 and dilatation of left ventricle in 3. All were treated with penicillin associated with anti-inflammatory drugs in 5 and haloperidol in 3. Group 2 included 3 girls and 2 boys, mean age 9.56 years. Four were from African countries (Angola and Guinea), and one came from the north of Portugal. The elapsed time between the initial acute attack and cardiology referral varied from 5 months to 3 years. All presented severe mitral insufficiency associated with aortic and/or tricuspid valve lesions, and heart failure. All five underwent valve surgery. The secondary prophylaxis was recommended in every patient. There was a recurrence in a child who had interrupted chemoprophylaxis. The patients from African countries were lost for follow-up. CONCLUSIONS: RF still remains a problem in present times, with serious cardiac sequela in African countries. Colour Doppler echocardiography is a valuable tool for the detection of pathological valvular regurgitation and subclinical carditis if strict criteria are used. The need for appropriate treatment of streptococcal pharyngitis and secondary prophylaxis is emphasized.

Divided left atrium associated with supravalvar mitral ring.

Reported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.

[Case report of tuberous sclerosis and congenital heart disease]. / Esclerose tuberosa e cardiopatia congénita caso clínico.

The cardiac manifestation usually associated with Tuberous Sclerosis is rhabdomyoma. The authors present a clinical case of Tuberous Sclerosis with the particular coexistence of congenital heart disease (mitral anomaly and pulmonary stenosis) and a single intracardiac rhabdomyoma that appeared at the age of four years.

[Cardiac involvement in metabolic diseases]. / Envolvimento cardíaco nas doenças metabólicas.

OBJECTIVE: To evaluate cardiac involvement in children with metabolic disease in the out patient clinic of the Pediatric Cardiology Unit of Maria Pia Children's Hospital and their follow-up. MATERIAL AND METHODS: Twenty-nine medical records belonging to out patients with metabolic disease in consultation at our unit were reviewed. The following data from each record was analyzed: sex, metabolic disease diagnosis, age and motive for referral to a pediatric cardiology unit, cardiology diagnosis, therapy and evolution. RESULTS: Seventeen patients were boys and 12 girls. The average age of referral was 7.2 years (SD 4.8). The motives for referral were: screening for heart disease, 16; heart murmur, 7; congestive heart failure, 3; heart murmur and fatigue, 2; poor weight gain, 1. The following metabolic diagnoses were made: lysosomal diseases, 21; mitochondrial citopathies, 5; disorder of beta-oxidation of fatty acids, 2; carbohydrate deficient glycoprotein syndrome (CDG syndrome), 1. The cardiologic evaluation was normal in ten patients (4 with lysosomal disease, 4 with mitochondrial citopathy, one disorder of beta-oxidation of fatty acids, the CDG syndrome). Mitral and aortic valve lesions predominated in lysosomal diseases (12/21); myocardial involvement alone was present in two patients, and both myocardial and valvular lesions were present in three. Dilated cardiomyopathy was the presented manifestation in two patients-one with mitochondrial citopathy and one with a disorder of beta-oxidation of fatty acids. Three patients died and 26 remain out-patients. One patient was submitted to valve surgery. The average duration of follow-up was 21 months (SD 24). COMMENTS: Lysosomal diseases were the most representative in our patients, as described in the literature. Heart valve disease was the most frequent alteration. Indication for heart valve surgery is dependent on systemic involvement of the primary disease. All children with a metabolic disease with eventual heart involvement should be evaluated periodically by a cardiology unit. On the other hand, it is mandatory to screen a cardiomyopathy of unknown cause for a metabolic disease. The authors draw attention to the importance of infectious endocarditis prophylaxis in this group of patients.

[Transient hypertrophic neonatal myocardiopathy after acute fetal distress]. / Miocardiopatia hipertrófica neonatal transitória após sofrimento fetal agudo.

Hypertrophic cardiomyopathy is rare in neonates. The causes include metabolic disease, maternal diabetes, dexamethasone therapy in premature newborns for bronchopulmonary dysplasia and idiopathic hypertrophic cardiomyopathy. The authors present a case report regarding a neonate with transient hypertrophic cardiomyopathy, after acute fetal distress. Signs of congestive cardiac failure appeared at day 1. Echocardiography showed signs of mild pulmonary hypertension and normal ventricular wall thickness. Echocardiography on day 2 showed hypertrophy of myocardial walls affecting first the right ventricular anterior wall and the interventricular septum, followed by the left ventricular posterior wall. With therapy, clinical improvement was noted on day 4. The ventricular wall thickness was almost normal at three weeks. The authors alert physicians to the importance of echocardiographic examination of these neonates.

Cardiac consequences of renal transplantation changes in left ventricular morphology.

OBJECTIVE: The aim of this study was to characterize changes in left ventricular morphology and function by conventional echocardiography in a pediatric population after renal transplantation (RT). MATERIAL AND METHODS: This study includes 21 children, 11 female and 10 male, aged 11.8 +/-2.6 years at the time of renal transplantation. Eleven were on regular hemodialysis treatment and two on continuous peritoneal dialysis for 31.8 +/- 31.6 months; eight were transplanted without prior dialysis. Eight children were hypertensive before RT and nine (seven de novo) after RT. The patients were analysed in a prospective follow-up study with echocardiography immediately before and 30.2 +/- 15 months after successful renal transplantation. We measured the following echocardiographic parameters: Left ventricular end diastolic (LVED) and end systolic diameter (LVES), left atrial dimension (LAD), septal thickness (IVST), posterior wall thickness (PWT), shortening fraction (SF) and left ventricular index mass (LVIM). The paired Student's test, Fisher exact test and Pearson's correlation were used for statistical analysis. We analyzed the echocardiographic changes in the total group and separately in the subgroups with and without prior hemodialysis, as well as the influence of the duration of dialysis, anemia and hypertension. RESULTS: 1. After renal transplantation we found a decrease in the LVED LVES, LAD, IVST and PWT measurements (p < 0.05). There was a negligible increase in SF. The LVIM decreased from 139 g/m2 to 104 g/m2, representing a 21% reduction of the initial value, but this reduction was not of statistically significant. 2. The findings were similar in the hemodialysis group-LVIM decreased from 167.6 g/m2 to 94.9 g/m2, representing a 35% reduction in the initial value. 3. In the non dialysis group LVED, LVES, LAD and SVT decreased and LVIM showed a minor increase. There was a negligible increase in SF. 4. We found an increased LVIM in children with prior hemodialysis before RT, especially if dialysis lasted for more than two years. 5. Amelioration of hematocrit correlated with reduction of LVIM. 6. Regression of LV hypertrophy after RT was not dependent on blood pressure levels. CONCLUSIONS: Renal transplantation resulted in a tendency towards normalization of the echocardiographic parameters analysed: Contractility, as assessed by shortening fraction, was normal in this population. Although cardiac hypertrophy and dilation is reversible after RT, it would seem that children may benefit from an earlier transplantation.

[Initial assessment of heart murmurs in children: role of complementary diagnostic tests]. / Avaliação inicial do sopro cardíaco na criança: papel dos exames complementares de diagnóstico.

UNLABELLED: Heart murmur, most of them innocent, are the most common reason for referrals to a pediatric cardiologist. OBJECTIVE: The objective of this study was to analyse the utility of the electrocardiogram (ECG) and echocardiography in the initial assessment of heart murmurs in an asymptomatic pediatric population. MATERIAL AND METHODS: For each of 170 consecutive children who were seen for first time evaluation of a heart murmur, the clinical diagnosis was registered and the patients were classified as having innocent murmur (IM), pathologic murmur (PM), or possible pathologic murmur (PPM). The results of ECG and echocardiogram two-dimensional/Doppler were registered and changes in diagnosis were recorded and compared with the original impressions. RESULTS: After ECG no diagnosis was changed. After two-dimensional/Doppler echocardiogram the clinical diagnosis of innocent murmur in 118 children was changed to pathologic in one (one trivial pulmonary stenosis); pathologic murmurs in 43 children was changed to innocent in two; and possible pathologic in nine children was changed to innocent in six and possible pathologic in three. The clinical examinations had a sensitivity of 97.6%, specificity of 93.6%, positive predictive value of 95.3%, and negative predictive value of 99.1%. CONCLUSIONS: Clinical assessment by a Pediatric Cardiologist is sufficient to distinguish pathologic from innocent heart murmurs. Echocardiography, although diagnostic when heart disease is suspected, is unnecessary in a pediatric patient with clinically diagnosed innocent murmur.

[Cleft sternum in an infant]. / Fenda esternal em lactente.

The authors report the clinical case of a female black infant, one month 22 days old, with sternal malformation (complete sternal cleft). She didn't show other associated malformations. Early surgical repair was done with success. The relevant nomenclature of sternal defects is discussed.

[Vascular rings: surgical treatment]. / Anéis vasculares: tratamento cirúrgico.

From 1982 to 1992, nine patients underwent correction of vascular rings. The diagnosis was strongly suggested by symptoms of tracheoesophageal obstruction and was confirmed by barium esophagogram and angiography. Endoscopic studies were considered unnecessary and potentially harmful. Seven patients had double aortic arch, one patient right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum and one patient anomalous origin of the left pulmonary artery. The anomaly was approached through a left thoracotomy in every patient. There were no operative or late deaths. When patients are symptomatic, vascular rings should be promptly repaired. The surgical risk is minimal, and the long term results are excellent.

[Inguinal hernioplasty by a modified Shouldice technic. Our experience with 350 treated cases]. / Ernioplastica inguinale secondo la tecnica di Shouldice modificata. La nostra esperienza su 350 casi trattati.

A statistical analysis of 340 patients operated between 1986 and 1991 for inguinal hernia using the Shouldice procedure are reported by the authors. The majority of patients (60%) treated was between 45 and 65 years old. Two-hundred-eighty-one (82%) were controlled with a mean follow-up of 30 months (range 1-6 years) and was executed with physical examination and a medical questionnaire looking for recurring factors of risk. Hundred-twenty-eight patients (45% of controls) were at the high risk, nevertheless hernia recurrences were not noticed. Postoperative course was uncomplicated in 93% of patients. Early or late loco-regional complications had an incidence of 7%. Local infections and edema were reported in 10 of 22 patients. Acute urinary retention, cephalalgia and hypotension correlated with an anesthesiologic method had an incidence of 5% (17 cases). There were no deaths was pulmonary embolisms. A review of literature was performed and compared with our work. From experience in our Division, Shouldice's hospital technique had a high reliability compared to methods utilized in the previous ten years (Bassini, McNealy, Postemskj, Mugnai-Ferrari), in terms of recurrences.

[Endomyocardial fibrosis. Presentation of a clinical case]. / Fibrose endomiocárdica. Apresentação de um caso clínico.

The authors report a case of right ventricle endomyocardial fibrosis in a 6-year-old boy. Particular attention is paid to the discussion of the new methods for diagnosing and treating these conditions, namely echocardiography, endomyocardial biopsy and surgery.