[Cerebral ischemic infarction in newborn infants. Diagnosis using pulsed and color Doppler imaging]. / Infarctus ischémique cérébral du nouveau-né. Diagnostic par doppler pulsé et couleur.

In newborn infant, cerebral infarction is often difficult to distinguish from cerebral hemorrhage, both pathologies being moreover frequently associated. We report a left anterior stroke in a fullterm newborn who had seizures on the third day of life. Ultrasound scanning showed a hyperechogenic zone in the territory of the left anterior cerebral artery. Pulsed and color Doppler imaging showed a decreased blood flow velocity in comparison to the opposite side, thus enabling the diagnosis.

[Nutritional evaluation of children with respiratory failure (RF): anthropometric evaluation upon admission to the pediatric intensive care units]. / Evaluación nutricional de niños con insuficiencia respiratoria (IR): antropometría al ingreso en cuidados intensivos pediátricos.

OBJECTIVE: Protein-energy malnutrition (PEM) constitutes an important complication in children submitted to intensive care treatment for respiratory failure (RF). This complication is not usually evaluated in assessment protocols. Nutrition assessment in a Pediatric Intensive Care Unit (PICU) was performed in children with respiratory failure. PATIENTS AND METHODS: A prospective study involving 65 children (1-158 months of age) with RF at admission to the PICU was carried out. The patients studied were divided into four groups according to their etiology: Group I (n = 29) included those with acute bronchial pathology; Group II (n = 17) those with pneumonia: Group III (n = 11) those in the post-operative period following cardiac surgery; and Group IV (n = 8) those patients with multiple-organ dysfunction. All patients in Groups I, II, and III had chronic diseases. Anthropometrical and nutritional evaluation included weight, height and body mass index (w/h2). RESULTS: The weight for age was low (59%). Height deficit was observed in a significantly lower percentage of the patients (13.1%). The body mass index showed values in relationship to the 50th percentile that varied between 83.4 +/- 23.1% and 97.1 +/- 4.7%. In 68.9% of the patients the criteria of global malnutrition were met according to the classification of McLaren and Read. CONCLUSIONS: The data confirm that PEM is frequent in patients with RF in the PICU and emphasize the usefulness of anthropometrical evaluation as an important, simple and non-invasive method of nutritional evaluation. Early and intensive nutritional intervention can reduce or prevent the vicious circle of malnutrition in RF patients hospitalized in the PICU.

Gastric inflammatory myofibroblastic proliferation in children.

Gastric inflammatory myofibroblastic proliferation (IMP) is an extremely rare entity in children, which to our knowledge has only been mentioned in case reports. We describe the ninth pediatric case and review the literature concerning the etiology, clinical and laboratory features, pathology, treatment, and outcome. There has been a predominance in preschool females. Abdominal pain, upper gastrointestinal hemorrhage, and an abdominal mass, either isolated or associated, have been the main clinical features. Iron-deficiency anemia has been a constant finding. Lesions are elevated and involve the full thickness of the gastric wall, usually with ulceration of the luminal surface; extragastric extension suggesting malignancy is frequent. Diagnosis is made by histology after surgical excision. There was no mortality directly related to gastric IMP, and only one case recurred after surgical excision. The pathogenesis is controversial, but the finding of Helicobacter pylori in our case may indicate an inflammatory origin. Awareness of this benign lesion and its mimicry of malignancy is important so that inappropriately aggressive therapy can be avoided.

Genetic and environmental factors regulating blood pressure in childhood: prospective study from 0 to 3 years.

OBJECTIVES: Blood pressure (BP) regulation depends on the interaction between multiple environmental and genetic factors. Of these, BP sensitivity to dietary sodium intake has been one that has been investigated in adults but not in children. The aim of the present study was to investigate, prospectively, the BP profile in relation to different genetic and hormonal factors, in the first 3 years of life. POPULATION AND METHODS: Thirty-nine children born at term following normal pregnancies, with uncomplicated neonatal periods, were randomly selected to take part in the study. BP, weight and length were evaluated every 3 months from birth to 3 years. At the age of 12 months, haptoglobin phenotypes and plasma active renin concentration were determined as well as random urine evaluation of aldosterone, cAMP, dopamine and digoxin-like immunoreactive substances (DLIS). Family history of cardio-vascular diseases was also recorded. RESULTS: Systolic BP (SBP) demonstrated a gradual increase until the age of 6 months with little variation up to 36 months. Tracking of SBP values was also observed from the first year as infants with high values (above the 75 percentile) maintained this tendency up to, at least, the age of 36 months. The comparison between SBP and diastolic BP (DBP) according haptoglobin phenotypes demonstrated that SBP was systematically higher in allele 1, with apparently an increasing tendency with age, although the differences did not have statistical significance. The comparative study between haptoglobin phenotypes, with correction for the covariates fractional excretion of sodium and potassium, showed that allele 1 carriers had significantly lower plasma renin and urine aldosterone and cAMP concentrations than allele 2, but dopamine excretion was found to be higher in allele 1 than in allele 2. There were no differences among variables relating to family history of cardiovascular disease. CONCLUSIONS: There was an early tracking process of BP values from the first 6 months of life which persists through, at least, to the age of 36 months. Differences in sodium handling between haptoglobin 1 and 2 phenotypes were already present in early childhood, although no significant repercussion in BP values could be demonstrated in the 3-year duration of this study.

[Acute pericarditis in childhood. The 9-year experience of a tertiary referral center]. / Pericardite aguda na criança. Experiencia de 9 anos de um centro de referencia terciário.

Sixteen children, aged 7 months to 12 years, with acute pericarditis, admitted between 1985 and 1993 to a tertiary referral centre were analyzed retrospectively for their presentation, etiology, work-up, management and prognosis. It was found that most of the presenting signs were not specific and were often related to associated diseases such as respiratory tract infections. In 50% of the cases a cause was not found, the others had viral infections (12.5%), tuberculosis (12.5%), Haemophilus influenzae infection (6.25%), Toxocara canis infection (6.25%) and collagen diseases (12.5%). In eight cases non-steroidal anti-inflammatory drugs associated with steroids were given, 7 patients received non-steroidal anti-inflammatory drugs and 1 steroids. The mean follow-up time was 3 years (1 to 5). Six patients had one or more relapses. Five of the 6 patients with relapses were in the group which received steroids. The two patients with tuberculosis underwent pericardiectomy. One child died due to complex heart disease and the remaining 15 were cured. It was concluded that in pericarditis an extensive work-up may not reveal the major etiologies and that long term prognosis is good.

Dyssegmental dysplasia: a case report of a Rolland-Desbuquois type.

Two different forms of dyssegmental dysplasia, mild and severe, can be distinguished on clinical, radiographic and chondro-osseous morphologic grounds. The milder Rolland-Desbuquois type is frequently characterised by survival beyond the newborn period. The lethal Silverman-Handmaker type has distinctive and more severe findings. In this paper, a rare case of the Rolland-Desbuquois type is presented. The case is unusual because although it was the milder form, the child died on the 10th day of life.

[The noise in neonatal intensive care units]. / Le bruit dans une unité de soins intensifs néonatals.

BACKGROUND: Noise constitutes a significant problem in Neonatal Intensive Care Units (NICU) because of its potential deleterious effects on the newborn patients and staff. The aim of this study was to evaluate the degree of noise in the NICU of Hospital S João, Oporto, in order to identify its origin and possibility of reducing it. MATERIAL AND METHODS: Measurements of noise were made for 24 hours, between 13 and 14 June 1994, in five different locations inside the NICU, using a slow time constant sonometer. RESULTS: The surrounding noise level changed between 61 and 67 dB (A), with spikes over 100 dB (A). CONCLUSIONS: Our results were similar to those previously reported in the literature. The following measures to reduce the level of noise were taken: 1) reduction of alarm sounds to minimal safe level; 2) replacement of doors; 3) instructions to visitors and staff.

[Sepsis in the very low-birth-weight newborn infant]. / Sépsis no recém-nascido de muito baixo peso.

Sepsis is a common pathology during the neonatal period. Low-birth weight is the most important isolated risk factor. With the purpose of determining the prevalence of nosocomial infection in newborns of very low birth weight (VLBW), finding the microbial flora and the mortality of these patients, the authors retrospectively analysed the clinical processes of the hospitalized VLBW in the Neonatal Intensive Care Unit (NICU) in S. João Hospital in 1993 and 1994. Of the 1091 newborns admitted to the hospital in these two years, 112 were VLBW (10%). Of these, 48 had sepsis-31 (28%) confirmed sepsis and 17 (15%) probable sepsis. The agents most commonly found were Staphylococcus epidermidis (44%) and Klebsiella pneumoniae (21%). Thirteen patients (27%) died, with infection as the cause of death in nine (19%). Our results confirm the importance of nosocomial neonatal infection, which should involve the strict observance of the asepsis norms and a proper policy of antibiotics.

[Pattern of intrauterine growth and Doppler flowmetry]. / Padrões de crescimento intra-uterino e fluxometria Doppler.

The aim of this study was to evaluate the accuracy of ultrasound biometric and hemodynamic fetal parameters for the prenatal diagnosis of intrauterine retardation in a sample of 438 pregnant women who had their first visit before the 16th week of gestation at the outpatient clinic of the Department of Obstetrics and Gynecology, S. Joào Hospital. The results are presented for 427 mother-infant pairs corresponding to singleton pregnancies uncomplicated by congenital malformations. The prevalence of SGA was 4.9%, 86.2% for AGA and 8.9% for LGA. A relatively low sensitivity and high specificity were calculated for the ultrasound parameters evaluated, as observed in previous studies performed in other populations. Mean values for the differences of umbilical vessels resistance index measured at 28-32 and 35-37 gestation weeks were significantly lower in SGA newborns, probably reflecting a sustained compromise of vascular compliance (umbilical/placental). The observed lack of agreement between the pre and the postnatal diagnosis of SGA emphasises the need to define local reference birthweight curves or the careful adoption of European standards obtained in populations identical to our.

Gastric duplication presenting rectal bleeding. A clinical case supporting McLedtchie's theory.

This is a case of a 5-month-old girl with massive rectal bleeding caused by a gastric duplication. Cyst heterotopic pancreas and pancreatic duplication, an association not previously reported were present. The peculiar clinical presentation was due to cyst perforation that had subsequently eroded the transverse colon. Coexistent gastric containing heterotopic pancreas and pancreatic duplications were probably produced as traction diverticular by an embryonic entoectodermal adhesions.

[Myocarditis in early childhood. Two years' experience at the pediatric cardiology unit at the S. João Hospital]. / Miocardites na primeria infância. Experiência de dois anos da unidade de cardiologia pediátrica do Hospital de S. João.

OBJECTIVE: The authors studied 14 children with myocarditis in order to correlate clinical and laboratorial data with evolution. DESIGN: Retrospective study of all cases with myocarditis admitted during a 2 year period. SETTING: Unidade de Cardiologia Pediátrica do HSJ. PATIENTS: 14 patients, aged 7 to 34 months, with the diagnosis of myocarditis on the ground of biopsy or necropsy findings. The clinical data analysed were: medical history, physical examinations, hematological and biochemical studies, chest X ray, electro and echocardiogram, virus and immunological studies and cardiac catheterization. RESULTS: All the patients had congestive heart failure and 10 had a history of a previous viral infection. Those who survived the first week were submitted to hematological and biochemical studies, chest X Ray, electrocardiogram, echocardiogram, virological and immunological studies and cardiac catheterization. All patients were treated with captopril and furosemide, 11 received dobutamine and 6 an immunosuppressive regime. Six patients died in the first week and one, nine months after diagnosis. One patient had an unfavourable course and the other six improved clinically. CONCLUSIONS: The authors were not able to find any correlation between laboratorial data and the disease outcome. Age less than one year and an abrupt onset were associated with 100% mortality. Immunosuppression was associated with improvement in 66% of the patients.

A juvenile variant of glycogenosis IV (Andersen disease).

An unusual patient with Andersen disease (glycogenosis type IV) is presented, with only relatively mild clinical symptoms at the age of 8 years. The patient has a profound deficiency of glycogen-branching enzyme.