Urothelial cell carcinoma of the bladder in pediatric patients: a systematic review and data analysis of the world literature.

BACKGROUND: Urothelial cell carcinoma (UCC) of the bladder is exceedingly rare in pediatric patients. Limited data are available to guide management in this population. METHODS: The authors systematically searched MEDLINE, Cochrane Library, and Google Scholar (through February 2019) for case reports and series to summarize data regarding presentation, evaluation, management, and follow-up for patients ≤ 18 years diagnosed with UCC of the bladder. Patient-level data were abstracted, and adjusted logistic regression was used to identify factors associated with a combined outcome of recurrence or death. RESULTS: One hundred two articles describing 243 patients from 26 countries met criteria. Average age was 12.5 years, 32.6% were female, 15.3% had medical comorbidities, and 13.2% had known risk factors for bladder cancer. Initial management was transurethral resection in 95.5% of patients, whereas 6.2% required secondary intervention. Tumor stage was TaN0M0 in 86.4% and low grade in 93.4%. Recurrence and death occurred in 8.6% and 3.7%, respectively. Mean time to recurrence or death was 8.6 months (standard deviation [SD] 7.6) for 10.7%. Mean disease free follow-up without recurrence or death was 56.9 months (SD 54.2) for 89.3%. Patients with comorbidities, risk factors, or family history (odds ratio [OR]: 2.4, 95% confidence interval [CI]: 1.02-5.6); ≥TaN0M0 disease (OR: 6.2, 95% CI: 2.5-15.6); and larger tumors at diagnosis (OR: 1.7, 95% CI: 1.2-2.4) had significantly greater adjusted odds of recurrence or death after initial treatment. CONCLUSION: Based on pooled results, disease recurrence or death occurred in 10.7% of pediatric patients and within 9 months for most and within 32 months for all patients. This may suggest that low-grade and stage UCC of the bladder in pediatric patients can be systematically monitored for at least 3 years. However, prospective evaluation of this clinical strategy is warranted.

Enterotoxigenic Escherichia coli in children with acute diarrhoea and controls in Teresina/PI, Brazil: distribution of enterotoxin and colonization factor genes.

AIMS: To investigate the distribution of the genes that encode enterotoxins and the colonization factors (CF) types as well as the antibiotic susceptibility profile of enterotoxigenic Escherichia coli (ETEC) isolated from children from the Brazilian Northeast. METHODS AND RESULTS: We conducted a 3·5-year prospective study that involved 250 children with and 150 without diarrhoea, aged 1-60 months, from low-income families in Teresina/Brazilian Northeast. All samples were assayed for E. coli, enterotoxin and CF genes and antimicrobial susceptibility by microbiological methods and PCR. ETEC strains were isolated from 9·2% children with and 4·0% without diarrhoea. Infection was more common in children aged 6-24 months in rainy months. elt⁺ /CFA/IV⁺ and elt⁺ /CS14⁺ were the most frequent genotypes. Susceptibility to nalidixic acid, ciprofloxacin and gentamicin and resistance to ampicillin, cephalothin and sulfamethoxazole-trimethoprim were common. CONCLUSIONS: elt ⁺isolates and ETEC strains harbouring genes encoding CFA/IV and CS/14 were the most common ETEC found in Brazilian Northeast. SIGNIFICANCE AND IMPACT OF THE STUDY: Our data, the first generated for north-eastern Brazilian children, may be important for the development of an effective vaccine and for facilitation of an empirical choice of antibiotic treatment or prophylaxis for traveller's diarrhoea in the area studied.

Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis

The objective of the present study was to determine the possible prognostic factors which may explain the difference in the survival of patients with cystic fibrosis (CF) with and without meconium ileus. Over a period of 20 years, 127 patients with CF, whose diagnosis was confirmed by typical clinical characteristics and altered sweat chloride levels, were studied retrospectively. The patients were divided into two groups: group 1 consisted of patients who presented CF and meconium ileus (N = 9), and group 2 consisted of patients with CF without meconium ileus (N = 118). The characteristics studied were based on data obtained upon admission of the patients using a specific protocol. Demographic, clinical, nutritional and laboratory data were obtained. The genotype was determined in 106 patients by PCR. Survival was analyzed using the Kaplan-Meier method. The median follow-up period was 44 months. A statistically significant difference was observed between the groups studied regarding the following variables: age at diagnosis and weight and height z scores. The presence of meconium ileus was associated with an earlier diagnosis; these patients had greater deficits in height and weight at the time of diagnosis and at the end of the study. The estimated probability of survival for patients with CF without meconium ileus was 62 ± 14 percent and for those with meconium ileus 32 ± 18 percent. Patients with CF and meconium ileus presented a poor nutritional status at diagnosis and a lower survival rate compared to the general CF population

Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis.

The objective of the present study was to determine the possible prognostic factors which may explain the difference in the survival of patients with cystic fibrosis (CF) with and without meconium ileus. Over a period of 20 years, 127 patients with CF, whose diagnosis was confirmed by typical clinical characteristics and altered sweat chloride levels, were studied retrospectively. The patients were divided into two groups: group 1 consisted of patients who presented CF and meconium ileus (N = 9), and group 2 consisted of patients with CF without meconium ileus (N = 118). The characteristics studied were based on data obtained upon admission of the patients using a specific protocol. Demographic, clinical, nutritional and laboratory data were obtained. The genotype was determined in 106 patients by PCR. Survival was analyzed using the Kaplan-Meier method. The median follow-up period was 44 months. A statistically significant difference was observed between the groups studied regarding the following variables: age at diagnosis and weight and height z scores. The presence of meconium ileus was associated with an earlier diagnosis; these patients had greater deficits in height and weight at the time of diagnosis and at the end of the study. The estimated probability of survival for patients with CF without meconium ileus was 62 +/- 14% and for those with meconium ileus 32 +/- 18%. Patients with CF and meconium ileus presented a poor nutritional status at diagnosis and a lower survival rate compared to the general CF population.

Serum antigliadin antibody levels as a screening criterion before jejunal biopsy indication for celiac disease in a developing country

The objective of the present study was to determine the efficacy of detection of antigliadin immunoglobulins G and A (IgG and IgA) for the diagnosis of celiac disease in a developing country, since other enteropathies might alter the levels of these antibodies. Three groups were studied: 22 patients with celiac disease (mean age: 30.6 months), 61 patients with other enteropathies (mean age: 43.3 months), and 46 patients without enteropathies (mean age: 96.9 months). Antigliadin IgG and IgA ELISA showed sensitivity of 90.9 and 95.5 percent, respectively. With the hypothetical values of prevalence ranging from 1:500 to 1:2000 liveborns, the positive predictive value varied from 8.5 to 2.3 percent for IgG and from 4.8 to 1.1 percent for IgA. Considering the patients without enteropathies, specificity was 97.8 and 95.7 percent for IgG and IgA, respectively. In patients with other enteropathies, specificity was 82.0 and 84.1 percent, respectively. When patients with and without other enteropathies were considered as a whole, specificity was 88.8 and 91.6 percent, respectively. The specificity of positive IgG or IgA was 93.5 percent in children without enteropathies and 78.7 percent in the presence of other enteropathies. The negative predictive value for hypothetical prevalences varying from 1:500 to 1:2000 liveborns was 99.9 percent. Thus, even in developing countries where the prevalence of non-celiac enteropathies is high, the determination of serum antigliadin antibody levels is a useful screening test prior to the jejunal biopsy in the investigation of intestinal malabsorption

Serum antigliadin antibody levels as a screening criterion before jejunal biopsy indication for celiac disease in a developing country.

The objective of the present study was to determine the efficacy of detection of antigliadin immunoglobulins G and A (IgG and IgA) for the diagnosis of celiac disease in a developing country, since other enteropathies might alter the levels of these antibodies. Three groups were studied: 22 patients with celiac disease (mean age: 30.6 months), 61 patients with other enteropathies (mean age: 43.3 months), and 46 patients without enteropathies (mean age: 96.9 months). Antigliadin IgG and IgA ELISA showed sensitivity of 90.9 and 95.5%, respectively. With the hypothetical values of prevalence ranging from 1:500 to 1:2000 liveborns, the positive predictive value varied from 8.5 to 2.3% for IgG and from 4.8 to 1.1% for IgA. Considering the patients without enteropathies, specificity was 97.8 and 95.7% for IgG and IgA, respectively. In patients with other enteropathies, specificity was 82.0 and 84.1%, respectively. When patients with and without other enteropathies were considered as a whole, specificity was 88.8 and 91.6%, respectively. The specificity of positive IgG or IgA was 93.5% in children without enteropathies and 78.7% in the presence of other enteropathies. The negative predictive value for hypothetical prevalences varying from 1:500 to 1:2000 liveborns was 99.9%. Thus, even in developing countries where the prevalence of non-celiac enteropathies is high, the determination of serum antigliadin antibody levels is a useful screening test prior to the jejunal biopsy in the investigation of intestinal malabsorption.

Dietary fiber intake, stool frequency and colonic transit time in chronic functional constipation in children

The objective of the present study was to evaluate associations between fiber intake, colonic transit time and stool frequency. Thirty-eight patients aged 4 to 14 years were submitted to alimentary evaluation and to measurement of colonic transit time. The median fiber intake of the total sample was age + 10.3 g/day. Only 18.4 percent of the subjects presented a daily dietary fiber intake below the levels recommended by the American Health Foundation. In this group, the median left colonic transit time was shorter than in the group with higher dietary fiber intake (11 vs 17 h, P = 0.067). The correlation between stool frequency and colonic transit time was negative and weak for left colon (r = -0.3, P = 0.04), and negative and moderate for rectosigmoid and total colon (r = -0.5, P<0.001 and r = -0.5, P<0.001, respectively). The stool frequency was lower in the group with slow transit time (0.8 vs 2.3 per week, P = 0.014). In conclusion, most patients with chronic functional constipation had adequate dietary fiber intake. The negative correlation between stool frequency and colonic transit time increased progressively from proximal segments to distal segments of the colon. Patients with normal and prolonged colonic transit time differ in terms of stool frequency

Dietary fiber intake, stool frequency and colonic transit time in chronic functional constipation in children.

The objective of the present study was to evaluate associations between fiber intake, colonic transit time and stool frequency. Thirty-eight patients aged 4 to 14 years were submitted to alimentary evaluation and to measurement of colonic transit time. The median fiber intake of the total sample was age + 10.3 g/day. Only 18.4% of the subjects presented a daily dietary fiber intake below the levels recommended by the American Health Foundation. In this group, the median left colonic transit time was shorter than in the group with higher dietary fiber intake (11 vs 17 h, P = 0.067). The correlation between stool frequency and colonic transit time was negative and weak for left colon (r = -0.3, P = 0.04), and negative and moderate for rectosigmoid and total colon (r = -0.5, P<0.001 and r = -0.5, P<0.001, respectively). The stool frequency was lower in the group with slow transit time (0.8 vs 2.3 per week, P = 0.014). In conclusion, most patients with chronic functional constipation had adequate dietary fiber intake. The negative correlation between stool frequency and colonic transit time increased progressively from proximal segments to distal segments of the colon. Patients with normal and prolonged colonic transit time differ in terms of stool frequency.

Influence of oral inoculation with plasmid-free human Escherichia coli on the frequency of diarrhea during the first year of life in human newborns.

BACKGROUND: This study was carried out to determine whether early inoculation of the plasmid-free human Escherichia coli into human newborns would reduce the frequency of acute diarrhea during a 1-year period. The plasmid-free E. coli strain isolated from the fecal microbiota of a healthy adult was nontoxigenic in vivo and in vitro and sensitive to all usual antibiotics. METHODS: In the experimental group, 51 healthy newborns were inoculated orally with 106 viable cells of the bacteria within 2 hours after birth. In the control group, the same number of newborns received the heat-killed bacteria. The clinical trial was double blind, and the newborns were randomly assigned to the experimental and control groups. RESULTS: Six months and 1 year after bacterial inoculation, infants in the experimental group showed a higher mean body weight (7.59 +/- 1.15 kg and 9.88 +/- 1.31 kg, respectively; P < 0.05) when compared with the control group (7.03 +/- 1.09 kg and 8.92 +/- 1.38 kg, respectively). At the end of the clinical trial, 48% (23/48) of the infants in the experimental group had shown at least one diarrhea episode during the 1-year period, as opposed to 71% (34/48) in the control group. These values were significantly different (P = 0.037), showing a 32.3% protective effect of inoculation. CONCLUSIONS: The present study shows that protection against diarrhea was obtained by oral inoculation with a single dose of plasmid-free human E. coli soon after birth.

Quadro clínico e nutricional de pacientes com fibrose cística: 20 anos de seguimento no HC-UFMG / Clinical and nutritional aspects of a Center of Cystic Fibrosis-HC-UFMG: 20 years of follow-up

OBJETIVOS: A fibrose cística (FC) é a doença genética letal, de herança autossômica recessiva, mais comum entre pacientes de cor branca. O presente estudo foi realizado com o objetivo de identificar o quadro clínico e nutricional à admissäo dos pacientes no Centro de Tratamento de FC do HC-UFMG e avaliar a sobrevida a longo prazo. PACIENTES E MÉTODOS: Em um período de 20 anos, 127 pacientes portadores de FC foram acompanhados longitudinalmente e submetidos a protocolo previamente estabelecido, após confirmaçäo do diagnóstico pelo teste do suo...

Pressure pain threshold in children with recurrent abdominal pain.

BACKGROUND: Experimental studies on humans have shown that recurrent pain is associated with altered pain perception. We measured the pressure pain threshold in regions of the body surface in a group of children who had recurrent abdominal pain and in a group of children with chronic or recurrent disease but with no pain. METHODS: Each group consisted of 45 boys and 55 girls ranging in age from 5 to 15.8 years. The regions of the body were the trapezius, deltoid, and supraspinous muscles, nine areas on the abdominal wall, and the median part of the tibias. Using an algometer, pressure was applied through a rubber disc with a surface area of 1 cm2 at a rate of 0.5 kg/cm2/s. The pressure values recorded with the algometer when the children communicated that they started to feel pain were considered as pressure pain thresholds. RESULTS: The pain thresholds were reduced in all regions investigated in children with recurrent pain. The median thresholds for all regions of the patients with and without pain were 1.60 and 2.2 kg/cm2, respectively. The diseases of children with pain did not influence the pain thresholds. CONCLUSION: There was an association between recurrent abdominal pain and a lower pressure pain threshold, with no influence of the type of disease, and there was a central nervous system alteration in the perception of pain in these patients.

[Clinical and nutritional aspects in patients with cystic fibrosis: 20 years of follow-up in the Clinical Hospital-Federal University of Minas Gerais (HC-UFMG)]. / Quadro clínico e nutricional de pacientes com fibrose cística: 20 anos de seguimento no HC-UFMG.

BACKGROUND: Cystic fibrosis (CF) is the most common severe autosomal recessive disease in caucasian population. The life expectancy of patients with CF has improved dramatically during the past three decades. A better approach to these patients depends on a better knowledge of clinical course and prognosis of CF. The purpose of the present study was to identify aspects clinical and nutritional on admission at Center of CF (HC-UFMG). MATERIAL AND METHODS: Over a period of 20 years 127 patients with CF were admitted, submitted to a systematic protocol and prospectively followed. Data associated with demographic conditions, clinical presentation, nutritional and laboratory findings on admission were studied. The median follow-up was 44 months. The genotype was performed by PCR method. The survival analysis was carried out by Kaplan-Meier method. RESULTS: The median age at diagnosis was 33 months. Sixty-one per cent of patients at diagnosis had chronic pulmonary and gastrointestinal symptoms. Seventeen patients (16%) were homozygous for DF508 mutation and 30 (28%) were heterozygous. There was a standard prevalence of malnutrition of 63% at diagnosis and of 45% at the end of follow up. Twenty patients (15,7%) died during follow up. The estimated probability of survival after one year from diagnosis was 96% and after five years was 80%. CONCLUSIONS: CF diagnosis has been later in our country and the survival is shorter than in developed countries. Management of cystic fibrosis in pediatric specializing centers results in a better nutritional state in spite of clinical progression of the disease.

[Chronic intestinal pseudo-obstruction in children - report of seven cases] / Pseudo-obstrução intestinal crônica na infância - relato de sete casos.

OBJECTIVE: The purpose of this report is to describe the clinical characteristics and histological abnormalities on full-thickness intestinal biopsy in seven children from the Gastroenterology (GI) outpatient clinic. METHODS: A retrospective study of all patients who attended the GI outpatient clinic in the last ten years. The medical records of seven patients were reviewed, and all the relevant clinical data recorded. Diagnosis was based on clinical, laboratorial, and histological findings. CONCLUSION: Although this is a rare syndrome, the differential diagnosis shall be performed whenever the patient presents signs and symptoms of obstruction along the digestive tract with no association of any mechanical cause. Studies shall be encouraged regarding the precocious and accurate diagnosis, using less invasive techniques and specific therapeutics (both clinical and surgical), in order to improve life quality and extended survival of affected patients.

[Up-to-date clinical and experimental basis for the use of probiotics] / Bases experimentais e clínicas atuais para o emprego dos probióticos.

OBJECTIVE: To evaluate the potential of probiotics or biotherapeutic agents for the prevention and/or treatment of selected intestinal infections. METHODS: Medline database was searched for all relevant articles between 1990 and February 1998. Bibliographies of articles were also used. All animal experiments and placebo-controlled human studies were reviewed in order to provide information on the mechanisms of action, potential efficacy, or adverse effects of these biotherapeutic agents. RESULTS: In the first part of this review, the different mechanisms of action that are effective in the treatment of diarrhea were discussed, and they were well demonstrated in laboratory animals. The most important are: enzymatic induction of disaccharidase activity, trophic effects on the intestinal mucosa, action in blocking bacterial toxins, and also induction of the immunologic response. Therapeutic effects of probiotics in humans, mainly in the gastrointestinal tract, were reported in the second part. Placebo-controlled studies have shown that biotherapeutic agents have been used successfully in the treatment of acute diarrhea in infants, traveler s diarrhea, antibiotic-associated diarrhea, with or without Clostridium difficile-associated enterocolitis (pseudomembranous colitis), and in immunosuppression-associated diarrhea, including AIDS. Lactobacillus, Bifidobacterium and Saccharomyces boulardii were the most important biotherapeutic agents to be considered. CONCLUSIONS: Currently, there is evidence that the administration of selected microorganisms is benefic in the prevention and treatment of certain intestinal infections. According to the literature, Saccharomyces boulardii is the most important probiotic. Possible future indications were discussed, such as the probable synergic effect of many probiotics due to their different and complementary mechanisms of action. The importance of new experimental and clinical studies for the better understanding of actions and the use of probiotics in other clinical situations was emphasized.

[Gastroesophageal reflux] / Refluxo gastroesofágico.

OBJECTIVE: To discuss clinical, diagnostic and therapeutic aspects of gastroesophageal reflux. METHOD: We accomplished a literature review of the last 30 years, by means of Lilacs and Medline databases. RESULTS: The gastroesophageal reflux is one of the most frequent causes of medical appointments with pediatric gastroenterologists. It represents a benign condition, characterized by regurgitations that can be resolved with general measures. Medical management with prokinetics and antacid agents controls clinical manifestations and prevents complications. Fundoplication is reserved to a minority of cases. COMMENTS: Some aspects of the clinical treatment have to be emphasized. Thickened/Solid diet and erect posture must be always recommended. Cisapride, the most commonly employed prokinetic agent, may prolong ventricular repolarization. Other prokinetic agents should be used in children. Bronchospasm or clinical manifestations of esophagitis indicate the use of antacid drugs.

[Pain threshold and age in childhood and adolescence] / Limiar de dor e idade na infância e adolescência.

OBJECTIVE: The few investigations about pressure pain threshold in corporeal surface areas and children age showed no correlation between these parameters in all areas. In this research the existence of correlation between age and pressure pain threshold in seventeen areas of the corporeal surface of children was investigated. METHODS: A descriptive research was made with one hundred children from the Clinical Hospital of the Federal University of Minas Gerais. The selection of patients was made by directed search. There were forty five boys and fifty five girls with ages varying between 5.3 and 15.8 years old, and a mean (-/+ standard deviation) of 9.6 (-/+ 2.7) years old. The seventeen corporeal areas studied were regions of the trapezium, deltoid and supraspinal muscles, nine areas of the abdomen and the median part of the tibias. The pressure of 0.5 kg/cm(2)/s was made using a mechanical algometer. The pressure values that were recorded in the equipment when the children started to complain about the pain, by verbal communication, were considered as pressure pain thresholds. RESULTS: Positive correlations with significant statistic values were found between pressure pain threshold and age in every area investigated: trapezium, r = 0.36; deltoid, r = 0.34; supraspinal, r=0.42; hypochondrium, r=0.41; epigastrium, r=0.41; flank, r = 0.48; periumbilical, r = 0,40; iliac fossa, r = 0.49; hypogastrium, r = 0.45 and tibia, r = 0,29. CONCLUSIONS: Since correlations between age and pressure pain threshold were found in all areas studied, the age of a patient must be taken into consideration during clinical investigations and pain evaluations in children and adolescents. The younger the patient, the lower the pain thresholds.

[Glycogenosis type I] / Glicogenose tipo I.

OBJECTIVE: To o present up-to-date knowledge about Glycogen storage disease type I (GSD-type I) - a disease caused by the deposit of glycogen resulting from the deficiency of the enzyme glucose-6- phosphatase - and to provide the pediatricians with the necessary information for a precocious diagnosis and an adequate conduct for those cases where this metabolic disturbance is present. METHODS: Through Medline, the most significant articles published during the last 20 years were selected from national and international journals of medicine, with special attention to dietary treatment of glycogen storage disease type I. RESULTS: The metabolism of glycogen and the metabolic consequences of glycogen storage disease type I were discussed, especially hypoglycemia, the principal metabolic disturbance of the disease. The clinical and laboratory findings are described together with the histopathology. The use of uncooked cornstarch and enteral carbohydrate infusion are the means used for the maintenance of normoglycemia. The control of hyperuricemia, hyperlipidemia and platelet disorders are other aspects of the treatment as well as the prevention of infections and the use of G-CSF for glycogen storage type Ib. Hepatic transplant and its principal indications are commented on. Hepatic adenomae, which always have the potential of malignant transformation, are the results of incomplete treatment. CONCLUSIONS: Although it occurs rarely, glycogen storage type I is an important cause of volumous hepatomegaly which is associated with hypoglycemia among the infants. The dietary treatment of this illness has significantly altered the clinical course and has improved the prognosis. Therefore it is indispensable that the general pediatrician should be familiar with the diagnosis of this clinical state so as to act rigorously in favor of the dietary control.