Maria Cristina de Andrade

Bacterial Sepsis in Brazilian Children: A Trend Analysis from 1992 to 2006

BACKGROUND The objective of this study was to determine the epidemiology of hospitalized pediatric sepsis in Brazil (1992-2006) and to compare mortality caused by sepsis to that caused by other major childhood diseases. METHODS AND FINDINGS We performed a retrospective descriptive study of hospital admissions using a government database of all hospital affiliated with the Brazilian health system. We studied all hospitalizations in children from 28 days through 19 years with diagnosis of bacterial sepsis defined by the criteria of the International Classification of Diseases (ICD), (Appendix S1). Based on the data studied from 1992 through 2006, the pediatric hospital mortality rate was 1.23% and there were 556,073 pediatric admissions with bacterial sepsis with a mean mortality rate of 19.9%. There was a case reduction of 67% over 1992-2006 (p<0.001); however, the mortality rate remained unchanged (from 1992-1996, 20.5%; and from 2002-2006, 19.7%). Sepsis-hospital mortality rate was substantially higher than pneumonia (0.5%), HIV (3.3%), diarrhea (0.3%), undernutrition (2.3%), malaria (0.2%) and measles (0.7%). The human development index (HDI) and mortality rates (MR) by region were: North region 0.76 and 21.7%; Northeast region 0.72 and 27.1%; Central-West 0.81 and 23.5%; South region 0.83 and 12.2% and Southeast region 0.82 and 14.8%, respectively. CONCLUSIONS We concluded that sepsis remains an important health problem in children in Brazil. The institution of universal primary care programs has been associated with substantially reduced sepsis incidence and therefore deaths; however, hospital mortality rates in children with sepsis remain unchanged. Implementation of additional health initiatives to reduce sepsis mortality in hospitalized patients could have great impact on childhood mortality rates in Brazil.

Bone mineral density and bone histomorphometry in children on long-term dialysis.

Bone mineral density (BMD) at the lumbar vertebrae (L1–L4) was assessed by dual-energy X-ray absorptiometry (DXA) in 20 children with chronic kidney disease (CKD) on dialysis, and its results were compared with bone biopsy and biochemical parameters. Biopsy specimens provided evidence of hyperparathyroid bone disease in eight cases (40%), and low bone turnover in 12 (60%). For BMD, expressed as Z-scores relative to normal, median Z-scores were −1.05 (range −2.36 to 1.06) for hyperparathyroid patients and −1.05 (range −4.40 to −0.03) for low bone turnover patients, with no statistical differences between groups (P = 0.512). In relation to BMD, of the whole sample, five (25%) had a Z-score under −2.0. When it was corrected for height, BMD was in the normal range. Additionally, there were no significant differences in single samples of serum calcium, alkaline phosphatase, phosphorus and intact parathyroid hormone (PTH) between groups with high or low bone turnover. Assessment of nutritional status, through height/age, showed that ten patients had Z-scores below −2.0 (median −2.12, range −7.13 to 0.73). In conclusion, renal osteodystrophy (ROD) seems to have a high prevalence among CKD pediatric patients, although only approximately a quarter of them developed changes in BMD. In children with CKD, measurements of bone mineral density may not be used for classification of various forms of ROD.

Hemolytic Uremic Syndrome in Pediatric Intensive Care Units in São Paulo, Brazil

The hemolytic uremic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli (STEC) is one of the most frequent causes of pediatric acute renal failure. The aim of this study was to report the clinic and microbiologic features associated with 13 post-diarrheal HUS cases identified in pediatric intensive care units in the city of São Paulo, Brazil, from January 2001 to August 2005. Epidemiologic, clinic, and laboratorial information, along with fecal and serum samples, were collected for identifying the genetic sequences of Stx and for studying antibodies directed against LPS O26, O111 and O157. STEC was isolated from three patients, and serotypes O26:H11, O157:H7 and O165:H- were identified. In nine patients, high levels of IgM against LPS O111 (n=2) and O157 (n=7) were detected. Dialysis was required in 76.9% of the patients; arterial hypertension was present in 61.5%, neurological complications were observed in 30.7%, and only one patient died. During a 5–year follow-up period, one patient developed chronic kidney disease. The combined use of microbiologic and serologic techniques provided evidence of STEC infection in 92.3% of the HUS cases studied, and the importance of O157 STEC as agents of HUS in São Paulo has not been previously highlighted.The hemolytic uremic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli (STEC) is one of the most frequent causes of pediatric acute renal failure. The aim of this study was to report the clinic and microbiologic features associated with 13 post-diarrheal HUS cases identified in pediatric intensive care units in the city of Sao Paulo, Brazil, from January 2001 to August 2005. Epidemiologic, clinic, and laboratorial information, along with fecal and serum samples, were collected for identifying the genetic sequences of Stx and for studying antibodies directed against LPS O26, O111 and O157. STEC was isolated from three patients, and serotypes O26:H11, O157:H7 and O165:H - were identified. In nine patients, high levels of IgM against LPS O111 (n=2) and O157 (n=7) were detected. Dialysis was required in 76.9% of the patients; arterial hypertension was present in 61.5%, neurological complications were observed in 30.7%, and only one patient died. During a 5-year follow-up period, one patient developed chronic kidney disease. The combined use of microbiologic and serologic techniques provided evidence of STEC infection in 92.3% of the HUS cases studied, and the importance of O157 STEC as agents of HUS in Sao Paulo has not been previously highlighted.

CEREBRAL SINOVENOUS THROMBOSIS IN A NEPHROTIC CHILD

Nephrotic syndrome in infancy and childhood is known to be associated with a hypercoagulable state and thromboembolic complications, but cerebral sinovenous thrombosis (CST) is a very rare and serious one, with only a few isolated reports in the literature. A case is presented of a 9-year-old boy with nephrotic syndrome that acutely developed signs and symptoms of intracranial hypertension syndrome. CST was diagnosed on cranial CT and MRI and he gradually recovered after treatment with anticoagulants. The diagnosis of CST should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. The discussion of this case, coupled with a review of the literature, emphasizes that early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. This report also illustrates the difficulties that may be encountered in managing such a patient.

Síndrome de Lowe: relato de cinco casos

INTRODUCTION Lowe Syndrome, or Oculocerebrorenal Dystrophy (OCRL), has a recessive inheritance linked to X chromosome. It presents cataracts and glaucoma, delay in neuropsychomotor development, cognitive deficits, and renal Fanconi syndrome. OBJECTIVE Describe five patients with OCRL, attended at Tubulopathy outpatient clinic. METHOD We performed a retrospective assessment of 5 male patient clinical charts of OCRL patients. RESULTS Mean age at first consultation was 76.5 and mean follow up interval was 30.5 months (8-53 months). Symptoms and clinical signs included cataracts and nystagmus. Neuropsychomotor development and weight and height deficits were present in whole cases, as well as polyuria, polydipsia, and intestinal constipation, metabolic acidosis, phosphaturia, bicarbonaturia, proteinuria, hypercalciuria, hyperuricosuria. Nephrocalcinosis was identified in one, renal lithiasis in three, and reduced kidney size in two patients. We found pathological fractures and rachitism in two, bone rarefaction and delay of bone age in all of the patients. One patient presented a reduction in the rhythm of glomerular filtration. Therapeutically, all patients received alkali, phosphorus and vitamin D reposition in addition to a dietary orientation adequate to their needs. CONCLUSION This study emphasizes the importance of early diagnosis and medico-nutritional followup, to avoid complications related to metabolic disturbances.

Avaliação nutricional de crianças com doença renal crônica

OBJECTIVE:Malnutrition is a frequent complication among children with renal diseases. Short stature is the main clinical sign. The aim of this study is to analyze the nutritional status of children with renal disease using anthropometry. METHODS: This cross sectional study enrolled 21 (43%) boys and 28 (57%) girls with age ranging from 5.3 to 19.5 years. They were divided in three groups based on their creatinine clearance (mL/min/1.73m2): Group 1, >37 (n=19); Group 2, between 15 and 37 (n=9) and Group 3, <15 (n=21). Weight and height were obtained in order to calculate the following indexes: Weight/age (W/A), height/age (H/A) and body mass index (BMI); then, Z scores were obtained. Malnutrition was defined as Z scores below -2. ANOVA test was used to compare groups. RESULTS: There were no differences among the groups for anthropometric data. 19 patients (38.8%) presented short-stature and 22 (44.8%) low-weight. Z scores were similar among groups relative to W/A, H/A and BMI values. W/A Z score values were: Group 1: -1.9±1.8; Group 2: -2.6±3.1 and Group 3: -2.5±1.4 (p=0.47). H/A Z scores values were: Group 1: -1.5±1.2; Group 2: -2.3±1.8 and Group 3: -2.1±1.1 (p=0.18). The calculated BMI Z scores were: Group 1: -1.2±1.4; Group 2: -1.7±3.9 and Group 3: -1.6±1.3 (p=0.82). 19 children presented short stature and 22 presented low weight. There were no differences between the studied groups. CONCLUSIONS: The sample presented high prevalence of malnutrition. Even considering the disease stage, there were no nutritional differences between the studied groups.

RAQUITISMO HIPOFOSFATÊMICO: RELATO DE CASO

ABSTRACT Objective: Early diagnosis and immediate treatment of hypophosphatemic rickets is of utmost importance as it may prevent subsequent sequelae. This report aims at warning pediatricians to consider the presence of the disease. Case description: Description of the metabolic profile, creatinine clearance, nutritional status, weight and body structure of a patient who presented the clinical-laboratorial characteristics of hypophosphatemic rickets and was followed in an outpatient clinic for tubulopathies over the period of 12 months. The patient had been bedridden for some time, was dependent on mechanical ventilation and presented an altered metabolic bone condition. Treatment was phosphate (initial: 65 mg/kg/day and final: 24,2 mg/kg/day), calcium (initial: 127 mg/kg/day, final: 48,4 mg/kg/day) and calcitriol (initial: 0.06 mcg/kg/day, final: 0.03 mcg/kg/day). The patient improved, evolving into spontaneous breathing and walking unaided. Laboratory results: calcium (mg/dL) initial 7.1, final 10.1; phosphate (mg/dL) initial 1.7 final 3.2; magnesium (mg/dL) initial 1.5 final 2.1, parathyroid hormone (pg/l) initial 85.8, final 52.7, alkaline phosphatase (UI/l) initial 12660, final 938; there was also improvement in weight/structural development (Z score: H/A initial: -6.05, final -3.64; W/A: initial -2.92, final -1.57) with presence of transitory gallstones. Creatinine clearance (mL/min/1.73m2bsa) was constant. The medication improved his laboratory results and nutritional status, but the patient did not return for two years for follow-up and, during this period, his condition has noticeably deteriorated. Comments: Early diagnosis and follow-up are essential in dealing with this pathology.

Evaluation of carotid intima-media thickness and factors associated with cardiovascular disease in children and adolescents with chronic kidney disease

OBJECTIVE To assess the carotid intima-media thickness and factors associated with cardiovascular disease in children and adolescents with chronic kidney disease. MATERIAL AND METHODS Observational, cross-sectional study carried out at the Universidade Federal de São Paulo (chronic kidney disease outpatient clinics) with 55 patients (60% males) with a median age of 11.9 years (I25-I75: 9.2-14.8 years). Of the 55 patients, 43 were on conservative treatment and 12 were on dialysis. Serum laboratory parameters (creatinine, uric acid, C-reactive protein, total cholesterol and fractions, and triglycerides), nutritional status (z-score of body mass index, z-score of height/age), body fat (fat percentage and waist circumference), and blood pressure levels were evaluated. The carotid intima-media thickness measure was evaluated by a single ultrasonographer and compared with percentiles established according to gender and height. Data collection was performed between May 2015 and March 2016. RESULTS Of the children and adolescents with chronic kidney disease, 74.5% (95% CI: 61.0; 85.3) showed an increase (>P95) in carotid intima-media thickness. In patients with stages I and II hypertension, 90.9% had increased carotid intima-media thickness. Nutritional status, body fat and laboratory tests were not associated with increased carotid intima-media thickness. After multivariate adjustment, only puberty (PR=1.30, p=0.037) and stages I and II arterial hypertension (PR=1.42, p=0.011) were independently associated with carotid intima-media thickness alterations. CONCLUSION The prevalence of increased carotid thickness was high in children and adolescents with chronic kidney disease. Puberty and arterial hypertension were independently associated with increased carotid intima-media thickness.

Síndrome de Bartter: avaliação do desenvolvimento estatural e perfil metabólico

OBJETIVO: El sindrome de Bartter (SB) es una enfermedad rara, pero una de las mas frecuentes condiciones congenitas perdedoras de cloro. Este trabajo tiene por objetivo relatar la evolucion de diez pacientes con SB. METODOS: Estudio observacional, descriptivo, obtenido mediante analisis de prontuarios medicos. Relata el perfil metabolico, la depuracion de creatinina, el estado nutricio-nal y ponderoestatural de los diez pacientes atendidos en el ambulatorio de Tubulopatias de Universidade Federal de Sao Paulo con caracteristicas clinico-laboratoriales de SB, seguidos por un periodo mediano de 43 meses (3-76 meses). Durante el seguimiento se practico protocolo de tratamiento que consistio en la administracion de suplemento de potasio (100%), magnesio (60%), anti-inflamatorios no hormonales (90%), inhibidores de enzima convertidora de angiotensina (40%) y espironolactona (50%). Se consideraron criterios de exclusion la presencia de alteraciones sericas y urinarias no compatibles con SB. El analisis estadistico consto de la comparacion de datos de la primera y la ultima consulta, utilizandose la prueba de Wilcoxon. RESULTADOS: Se observo mejora numerica de los valores absolutos de los items evaluados y del desarrollo ponderoestatural con la terapeutica utilizada, pero solamente la calemia [mediana inicial 3,05mEq/L y final 3,25mEq/L (p=0,01)] y el escore Z de peso/edad [mediana inicial -2,47 y final 1,35 (p=0,02)] presentaron mejora significante. De los 10 pacien-tes estudiados, dos presentaban reduccion de la depuracion de creatinina con enfermedad renal cronica etapa 2 y en el final del seguimiento (ambos habian iniciado el seguimiento con depuracion renal comprometida). CONCLUSIONES: Los datos enfatizan la necesidad de la ins-titucion terapeutica precoz para mejorar los niveles sericos de los electrolitos y el estado nutricional, sin comprometer la depuracion de creatinina.

Bartter's syndrome: evaluation of statural growth and metabolic profile

OBJETIVO: El sindrome de Bartter (SB) es una enfermedad rara, pero una de las mas frecuentes condiciones congenitas perdedoras de cloro. Este trabajo tiene por objetivo relatar la evolucion de diez pacientes con SB. METODOS: Estudio observacional, descriptivo, obtenido mediante analisis de prontuarios medicos. Relata el perfil metabolico, la depuracion de creatinina, el estado nutricio-nal y ponderoestatural de los diez pacientes atendidos en el ambulatorio de Tubulopatias de Universidade Federal de Sao Paulo con caracteristicas clinico-laboratoriales de SB, seguidos por un periodo mediano de 43 meses (3-76 meses). Durante el seguimiento se practico protocolo de tratamiento que consistio en la administracion de suplemento de potasio (100%), magnesio (60%), anti-inflamatorios no hormonales (90%), inhibidores de enzima convertidora de angiotensina (40%) y espironolactona (50%). Se consideraron criterios de exclusion la presencia de alteraciones sericas y urinarias no compatibles con SB. El analisis estadistico consto de la comparacion de datos de la primera y la ultima consulta, utilizandose la prueba de Wilcoxon. RESULTADOS: Se observo mejora numerica de los valores absolutos de los items evaluados y del desarrollo ponderoestatural con la terapeutica utilizada, pero solamente la calemia [mediana inicial 3,05mEq/L y final 3,25mEq/L (p=0,01)] y el escore Z de peso/edad [mediana inicial -2,47 y final 1,35 (p=0,02)] presentaron mejora significante. De los 10 pacien-tes estudiados, dos presentaban reduccion de la depuracion de creatinina con enfermedad renal cronica etapa 2 y en el final del seguimiento (ambos habian iniciado el seguimiento con depuracion renal comprometida). CONCLUSIONES: Los datos enfatizan la necesidad de la ins-titucion terapeutica precoz para mejorar los niveles sericos de los electrolitos y el estado nutricional, sin comprometer la depuracion de creatinina.