Seasonal variation of blood pressure in children.

Seasonal blood pressure (BP) variation is mostly found between the summer and winter months. Guidelines for diagnosis and treatment of hypertension in children have not considered this variation until recently. This review aims to present an overview of seasonal BP variation in childhood along with potential underlying pathophysiological mechanisms and long-term implications as well as conclusions for future studies. In pediatric cohorts, seven studies investigated seasonal changes in BP. These changes amount to 3.4-5.9 mmHg (or 0.5-1.5 mmHg per - 1 °C difference in environmental temperature) in systolic BP with a peak in fall or winter. Potential mechanisms and mediators of seasonal BP variation include sympathetic activation of the nervous system with an increase of urinary and plasma norepinephrine levels in the winter season. Additionally, the physical activity among children and adolescents was inversely correlated with BP levels. Temperature sensitivity of BP and pediatric BP levels predict future systolic BP and target-organ damage. Therefore, cardiovascular events may even be long-term complications of seasonal BP variation in pediatric hypertensive patients. Overall, these data strongly suggest an important effect of ambient temperature on BP in children. Additional studies in pediatric cohorts are needed to define how best to incorporate such variation into clinical practice.

Blood pressure tracking in children and adolescents.

BACKGROUND: High blood pressure is a major risk factor for cardiovascular disease. Blood pressure tracking could help to identify individuals with potential hypertension. Therefore, we have asked whether or not tracking was of predictive value for the development of hypertension in early life. METHODS: Blood pressure was routinely measured in 13,261 children and adolescents in outpatient clinics as well as during hospitalization. In one analysis, 568 individuals with elevated and normotensive blood pressure values were evaluated after 2, 4, and 6 years of follow-up. In a second analysis, 2,157 individuals with normotensive blood pressure were examined in a paired t test. RESULTS: The follow-up analysis showed a significant tracking effect. However, the Pearson correlation coefficients of the systolic and diastolic blood pressure standard deviation scores (SDS) decreased over time. Upon the follow-up after 6 years, 35.6 % of the children and adolescents with elevated blood pressure values remained in the elevated range group. Of the children within the normotensive blood pressure range, 80.4 % remained normotensive after 6 years. Children with normotensive blood pressure showed a stronger tracking than those who had had one hypertensive blood pressure reading. Children with higher body mass index (BMI) at follow-up changed blood pressure SDS track from initially normal to higher blood pressure values. CONCLUSIONS: Blood pressure tracking in children and adolescents is moderate. We conclude that the predictive power of a single hypertensive blood pressure measurement during a single visit is rather small, and thus repetitive measurements across several consecutive visits are necessary.

Influence of seasonal variation on blood pressure measurements in children, adolescents and young adults.

BACKGROUND: Seasonal fluctuations in outdoor temperature have been shown to affect blood pressure in adults. The aim of our study was to determine whether blood pressure measurements in children and adolescents in Central Europe undergo seasonal variation or are influenced by outdoor temperature. METHODS: The blood pressure of 6,714 subjects (3,497 boys, 3,237 girls) aged 3 to 21 (median age 10.6) years was routinely measured. The study cohort comprised both healthy and sick children and adolescents visiting outpatient clinics and during hospitalisation. RESULTS: Cross-sectional analysis showed a significant seasonal variation in blood pressure measurements. The mean increase of systolic/diastolic blood pressure was 4.45/2.42 mmHg during the winter. A significant correlation between average outdoor temperature and systolic blood pressure was found (ρ = -0.074 p < 0.001). However, the effect was only detectable at an average temperature below 0 °C/32 °F and above 10 °C/50 °F. For each 1 °C increase in average outdoor temperature, the systolic blood pressure fell by 0.12 mmHg. CONCLUSIONS: Blood pressure measurements in children and adolescents, even in a temperate climate, are influenced by temperature and subject to seasonal variation. Considering seasonal variations in blood pressure could be of clinical interest.

Citric acid as the last therapeutic approach in an acute life-threatening metabolic decompensation of propionic acidaemia.

The tricarboxylic acid (TCA) cycle represents the key enzymatic steps in cellular energy metabolism. Once the TCA cycle is impaired in case of inherited metabolic disorders, life-threatening episodes of metabolic decompensation and severe organ failure can arise. We present the case of a 6 ½-year-old girl with propionic acidaemia during an episode of acute life-threatening metabolic decompensation and severe lactic acidosis. Citric acid given as an oral formulation showed the potential to sustain the TCA cycle flux. This therapeutic approach may become a treatment option in a situation of acute metabolic crisis, possibly preventing severe disturbance of energy metabolism.

Fulminant and fatal course of acute lymphoblastic leukemia due to lactic acidosis and suspected abdominal compartment syndrome.

Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy and its prognosis has considerably improved over the past 2 decades due to new therapeutic approaches. In some cases, however, it can develop very rapidly and cause possibly fatal complications. We report on the case of an 11-year-old boy with ALL, who rapidly developed severe lactic acidosis and abdominal compartment syndrome. He died of multiorgan failure only 5 days after diagnosis of ALL had been established. Autopsy revealed systemic leukemic infiltrations. We suppose that the mass of tumor cells induced a cascade of metabolic and endocrine reactions, which not only triggered the rapid progression of the disease but were also accountable for the lack of response to treatment. The pathophysiology of abdominal compartment syndrome as a rare and in our case ultimately fatal complication of ALL is described.

Combined partial trisomy 11q and partial monosomy 10p in a 19-year-old female patient: phenotypic and genotypic findings.

Constitutional partial trisomy 11q in man mostly occurs in combination with partial trisomy 22 due to a balanced parental translocation t(11;22). Occasionally a chromosome other than 22 is involved in the parental translocation with chromosome 11, resulting in partial monosomy for the other participating chromosome. We report of a patient with partial trisomy 11q and partial monosomy 10p [46,XX,der(10)t(10;11)(p15;q22)] due to a paternal balanced translocation [46,XY,t(10;11)(p15;q22)]. Array CGH showed heterozygosity for a deletion of ∼3.46 Mb at 10p15.3p15.2 and gain of ∼32.21 Mb at 11q22.2q25. The patient, a 19-year-old woman, has a multiple congenital anomaly syndrome with severe developmental and growth delay, muscular hypotonia, iris coloboma, abnormal external ears, widely spaced nipples, atrial septum defect, clubfoot, and arthrogryposis multiplex congenita. Despite multiple health problems and numerous hospitalizations due to massive seizures, pulmonary insufficiency and recurrent infections the patient reached adulthood. The clinical features in our patient are compared to other cases reported in the literature of either partial monosomy 10p or partial trisomy 11q. To the best of our knowledge, this is the first report of the combination of partial trisomy 11q and partial monosomy 10p. Comparing the molecular karyotype and the phenotype of our patient to other patients, the clinical features of our patient are more likely due to partial trisomy 11q than to partial monosomy 10p.

Acute cerebral infarction and extra pontine myelinolysis in children with new onset type 1 diabetes mellitus.

Neurological complications of diabetic ketoacidosis (DKA) are still associated with significant mortality and morbidity. We report on two children who suffered from acute cerebral infarction (CI) and extra pontine myelinolysis (EPM) at onset of type 1 diabetes. Initially, clinical management had not been performed according to generally accepted guidelines. Putative risk factors that may have predisposed for the development of acute cerebrovascular complications are discussed. Not only cerebral edema (CE) but also other severe neurological complications such as CI should be suspected when neurological deterioration occurs during DKA. We conclude that not only an exceeded rehydration therapy but also a rapidly reduced serum osmolality due to an unbalanced rapid blood sugar decrease and serum sodium increase may have lead to the neurological disease. We propose that a reserved and well-defined rehydration strategy in the first 6 (-12) h of therapy is crucial for recovery and can reduce neurological complications of patients with DKA.

Increased arterial stiffness in children and adolescents with type 1 diabetes: no association between arterial stiffness and serum levels of adiponectin.

OBJECTIVE: Type 1 diabetes is associated with an increased risk of atherosclerosis. Adiponectin serum levels correlate inversely with cardiovascular disease in adults. The aim of this study was to examine associations between arterial stiffness indices and serum adiponectin concentrations in children and adolescents with type 1 diabetes and to study the impact of metabolic control. SUBJECTS AND METHODS: We evaluated arterial stiffness, distensibility, and compliance in 93 children and adolescents with type 1 diabetes and correlated the data with clinical parameters and HbA1c levels. The control group comprised 85 matched healthy children. Serum levels of adiponectin in children with diabetes were measured by enzyme-linked immunoassay and correlated with arterial stiffness indices. RESULTS: Arterial stiffness was significantly increased in children and adolescents with type 1 diabetes (aged 13.0 +/- 3.8 yr) compared with matched healthy children (p = 0.03). Arterial stiffness was elevated in males with type 1 diabetes compared with females (p = 0.023). Arterial distensibility was significantly lower in children with diabetes compared with healthy controls (p = 0.025). Arterial stiffness, distensibility, and compliance did not correlate with diabetes duration, level of HbA1c, or serum cholesterol. Adiponectin concentrations in children and adolescents with diabetes were significantly elevated compared with normal values based on gender, age, and body mass index. We found no significant associations between arterial stiffness indices and adiponectin levels in children with type 1 diabetes. CONCLUSIONS: Children and adolescents with type 1 diabetes had increased arterial stiffness and reduced arterial distensibility and arterial compliance. However, no associations between arterial functional alterations and adiponectin concentrations were seen.

Exercise capacity in children with bronchopulmonary dysplasia at school age.

OBJECTIVE: To assess the exercise capacity, exercise habits, and lung function of preterm born children with bronchopulmonary dysplasia (BPD) compared to term born controls at school age. METHODS: Cardiopulmonary exercise test (CPET) by cycle ergometer and pulmonary function test were performed in children with BPD (n = 42) and compared with a term born control group (n = 42). Daily activity, participation in sports and respiratory symptoms were assessed by questionnaire. RESULTS: Children with BPD versus controls had significantly lower values for oxygen consumption (V̇O2 [mL/min] 1442 ± 417 vs. 1766 ± 541), minute ventilation (VE [L/min] 48 ± 14.92 vs. 60 ± 18.33), and workload (W [watt] 96.1 ± 16.7 vs. 110.6 ± 17.2) at peak exercise and a lower anaerobic threshold (VO2 AT [mL/min] 1183 ± 345 vs. 1382 ± 398). When corrected for weight, only for the workload (2.7 ± 0.5 vs. 3.1 ± 0.5, p = 0.0013) did significant differences persist. The forced expiratory volume in 1 s and forced expiratory flow between 25 and 75% of expired forced vital capacity were significantly reduced in the BPD group (p < 0.0001). Children with BPD have a higher risk of reporting difficulties in physical activity (OR 2.5) and of suffering from wheezing or shortness of breath while exercising (OR 2.5). CONCLUSION: Compared to term born controls, children with BPD at school age show airflow obstruction, a lower workload in CPET, and more respiratory symptoms related to physical activity. The comparable oxygen consumption based on weight suggests a functionally normal alveolar compartment.

Incompatible intravenous drug combinations and respective physician and nurse knowledge: a study in routine paediatric intensive care.

OBJECTIVES: To identify incompatible intravenous drug combinations in routine paediatric intensive care and evaluate physician and nurse knowledge. METHODS: In a university paediatric intensive care unit, intravenous drug incompatibilities were analysed using a database and physician and nurse knowledge of incompatibilities was assessed using a questionnaire. RESULTS: We analysed 665 prescriptions in 87 patients. Incompatible drug administration was identified in 9 (10%) of the 87 patients with a median of 3 different incompatibilities per patient (Q25/Q75: 1/3). We found 26 incompatible combinations. The most frequently involved drugs were cefotaxime, pantoprazole and vancomycin. A median of 10 of the 15 drug combinations were correctly assessed as compatible or incompatible (Q25/Q75: 8/11). Pantoprazole had a low number (20%) of correct answers. CONCLUSIONS: One in 10 patients in paediatric intensive care was affected by drug incompatibility, with knowledge deficits seen in a third of assessed combinations. This indicates quality improvement strategies should be urgently implemented by pharmacists.

Successful liver transplantation in a child with Caroli's disease.

CD is a rare autosomal recessive disease, characterized by multifocal cystic dilatation of intrahepatic bile ducts. The course of the disease is characterized by intrahepatic cholelithiasis, recurrent episodes of cholangitis, because of cholelithiasis, hepatic abscesses often ending in death caused by uncontrolled infection. Other conditions such as choledochal cyst and renal cystic disease are frequently associated, and patients have a higher risk for the development of cholangiocarcinoma. Endoscopic drainage of the bile duct is palliative and ineffective. OLT appears to be the treatment of choice. In monolobar cases partial liver resection has been shown to be a curative therapeutic option. We report on the course of disease in a Turkish girl who was diagnosed with CD in the neonatal period. At the age of 8.2 yr, she received OLT and is in good health 57 months post-transplantation.

Invasive pneumococcal diseases in children and adolescents--a single centre experience.

BACKGROUND: S. pneumoniae is a major cause of meningitis, pneumonia and sepsis in children. In 2006 universal pneumococcal vaccination was recommended in Germany for all children up to their second birthday. We have compared the prevalence and outcome of IPD at a single hospital before and after the introduction of vaccination. FINDINGS: 55 cases of IPD were identified over an 11 year period. Almost half of the patients were younger than 2 years of age. Most of the children were affected by pneumonia. The second highest incidence seen was for meningitis and sepsis. 17 patients exhibited additional complications. Significant pre-existing and predisposing disorders, such as IRAK 4 defect, ALPS or SLE were identified in 4 patients. Complete recovery was seen in 78% of affected children; 11% had a fatal outcome and 11% suffered from long term complications. Only 31% overall had been vaccinated. The most common serotype was 14. Serotypes not covered by any of the current vaccines were also found. Antibiotic treatment commenced with cephalosporins in over 90%. CONCLUSION: Frequency of IPD in our hospital did not decrease after initiation of the pneumococcal vaccination. This might be due to vaccinations not being administered satisfactorily as well as to poor education about the need of the vaccination. Pre-existing diseases must be monitored and treated accordingly and rare deficiencies taken into account when IPD takes a foudroyant course. In addition, antibiotic stewardship has been initiated at this hospital centre as a consequence of the high cephalosporin use detected in this study.

[Retrospective analysis of 44 childhood drowning accidents]. / Retrospektive Analyse von 44 Ertrinkungsunfällen von Kindern und Jugendlichen.

BACKGROUND: Worldwide, drowning is the second leading cause of unintentional death and the leading cause of cardiovascular failure for children [1-3]. The number of near-drownings, where the incident is survived for at least 24 hours, is assumed to be four times as high [5]. In the years 1994 until 2008 there were 44 cases of drowning treated at the children's department of the University of Leipzig. This number shows that even in a medical centre drowning incidents are only occasional incidents. Therefore it is important to know the sequelae and handlings to be able to react in case of an emergency. PATIENTS: A total of 44 children suffering a drowning accident within the last 48 hours who were treated during the period of 01.01.1994 through 30.06.2008 at the Children's Centre at the University of Leipzig. METHODS: A retrospective analysis using a structured questionnaire was done. Social demographic data, accident progress, clinical results and progress as well as outcome of the cases were investigated. RESULTS: During the analysed period in the median three children were treated each year after drowning incidents. Clustering in the summer and winter months and on the weekends was recognizable. The median age was 3.33 years and the group of high risk were children aged 1-3 years, especially boys. Sixty percent of the children came from stable social backgrounds. Half of the children suffered from drowning in created swimming pools or ponds, the rest in natural waters, public pools and sources of water in the household. The median submersion lasted 2 minutes. Correlation of submersions below 1 minute with a good, and submersions above 10 minutes with a negative outcome was shown. A Glasgow Coma Scale (GCS) of 3 points (n = 15) and pupils without light reaction (n = 14) were associated with a lethal outcome or residual neurological deficits. Looking at the laboratory values, correlation between severe acidotic pH-values with a very low base excess, high blood sugar as well as high lactate values and a poor outcome is revealed. Six patients died within the first 24 hours, 6 more over the course suffering organ failure or brain death. Five children retained neurological damages. Twenty-seven children could be released from the clinic healthily. CONCLUSION: The risk of suffering a drowning incident is highest for boys aged 1-3 years, playing in the yard by themselves. Prognosis is depending on multiple factors and especially the circumstances of the accident. High influence is seen in the time of submersion and the need for cardiopulmonary resuscitation. Clinics and laboratory values at the time of hospital admittance may hint to the outcome. Basic life support at the scene of the accident has the highest impact on the outcome. Training of parents and supervisors in prevention and first aid after drowning incidents can avoid accidents.

Combination of spit fistula advancement and external traction for primary repair of long-gap esophageal atresia.

Primary repair of long-gap esophageal atresia with almost complete absence of thoracic esophagus was usually believed to be impossible. Thus, esophageal replacement with colon or gastric interposition seemed inevitable. Esophageal lengthening techniques could be an alternative approach. Herewith we describe for the first time the successful combination of the stepwise subcutaneous advancement of the upper esophageal segment (Kimura's technique) with transthoracic traction on the lower esophageal segment (Foker's technique). This combined lengthening technique leads to the primary repair of a long-gap esophageal atresia.

Central lactic acidosis, hyperventilation, and respiratory alkalosis: leading clinical features in a 3-year-old boy with malignant meningeal melanoma.

Meningeal tumors are extremely rare in children and are diagnostically as well as therapeutically challenging. Among the least common types of malignancies in childhood is malignant melanoma, counting for less than 1% of pediatric tumors. Due to the rarity and the wide spectrum of appearance, initial clinical features may be misleading. A 3-year-old boy was referred to our hospital with symptoms of hyperventilation, dyspnoea, tachycardia, respiratory alkalosis, inarticulate speech, and fatigue. Measurement of pH in cerebrospinal fluid (CSF) yielded central lactic acidosis despite alkalosis in peripheral blood. Diagnostic imaging procedures as well as histology and immunohistochemistry revealed the diagnosis of a malignant meningeal melanoma. We hypothesize that central lactate production of the tumor nests might have induced central acidification, thus inducing hyperventilation by stimulation of central chemoreceptors. This case is a model example of the key role of central pH as an inducer/suppressor of ventilation in humans and illustrates the critical importance of central pH for regulating both ventilation and acid-base homeostasis. Thus, pH of CSF should be measured whenever a malignant brain tumor is suspected.

Successful therapy of paediatric ethylene glycol poisoning: a case report and annual survey by a regional poison centre.

UNLABELLED: Severe poisoning with ethylene glycol, often used as antifreeze, is a rare, life-threatening event. Neurological symptoms are accompanied by metabolic acidosis with elevated anion gap and osmotic gap. We report on a 7-year-old boy suffering from severe ethylene glycol intoxication. Early diagnosis based on typical clinical signs and rapid initiation of specific therapy with ethanol resulted in complete and rapid recovery without haemodialysis becoming necessary. While one laboratory initially had not been able to detect ethylene glycol in the patient's blood, an ethylene glycol serum level of 3900 mg/L was measured by a second laboratory, the highest value ever reported in the scientific literature for paediatric cases. Ethylene glycol poisoning is verified by quantitative glycol analysis in serum, but only few laboratories are able to perform this investigation in emergency cases. Therefore, in the beginning diagnosis has to be based on patient's history and clinical signs. Every substantial suspicion of ethylene glycol poisoning has to be treated with an antidote (fomepizole or ethanol) immediately. CONCLUSION: Ethylene glycol poisoning is rare in the paediatric age group in our series of glycol poisonings. One should keep in mind, that early diagnosis and treatment due to clinical signs is important and haemodialysis usually is not necessary.

Unusual presentation of congenital disorder of glycosylation type 1a: congenital persistent thrombocytopenia, hypertrophic cardiomyopathy and hydrops-like aspect due to marked peripheral oedema.

UNLABELLED: Of the congenital disorder of glycosylation (CDG) syndromes, type 1a is the most common. CDG 1a is a multisystem disorder with a wide clinical spectrum. We report on a term newborn with a severe and fatal clinical course of CDG 1a syndrome. Skin fibroblasts showed a reduced activity of phosphomannomutase 2 (PMM2) and mutation analysis revealed a compound heterozygous PMM2gene mutation (F119L/F157S). Presenting features at birth were hypertrophic non-obstructive cardiomyopathy, "orange-peel" skin, inverted nipples and a hydrops-like aspect due to marked peripheral oedema. Suspected hydrops fetalis was not confirmed due to lack of ascites and pleural effusions. Striking clinical problems were therapy-resistant arterial hypertension, recurrent pericardial and pleural effusions and feeding difficulties with failure to thrive. Persistent congenital thrombocytopenia and hyperferritinaemia in the absence of infection were noted. Bone marrow cytology revealed a macrophage activation of unknown aetiology. CONCLUSION: Congenital thrombocytopenia, unspecific macrophage activation and a hydrops-like aspect without a real hydrops fetalis broaden the already wide phenotypic spectrum of congenital disorder of glycosylation syndrome type 1a.

Tachydyspnea in an infant chimpanzee.

Reports on intensive care and invasive treatments of primates are scarce. Generally, there is little knowledge and experience in regard to resuscitation, cardiac support and ventilation support especially in small infants of primate species. We therefore report on our experience with respect to the successful treatment of a former small-for-date chimpanzee infant with severe cardiorespiratory distress due to pneumonia inflicted by an unknown infective agent. Treatment was primarily with analgosedation, oxygen application and dobutamine infusions. Cooperation of neonatologists and veterinarians is recommended for treatment of young primates.