Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature.

In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schönlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features.

Amlodipine once-daily in systemic hypertension.

UNLABELLED: The calcium channel blocker nifedipine is widely used in children with systemic hypertension: however, because of the short duration of action, three to four daily doses of the standard preparation are required. Amlodipine once-daily, a calcium channel blocker structurally related to nifedipine with an excellent bioavailability and a long elimination half-time, has been shown to reduce blood pressure in adults. No information is available on the use of amlodipine in childhood. The effects of amlodipine once-daily (5 to 10 mg) were therefore assessed in 28 paediatric patients with hypertension. Amlodipine was withdrawn in five patients who experienced oedema and flushing. In the remaining 23 patients blood pressure was significantly reduced 3 weeks after amlodipine (on average by 7/5 mm Hg) and further decreased at 12 weeks (by 21/12 mm Hg). Heart rate and body weight were unchanged. In eight patients concomitantly treated with cyclosporine, the blood level of this agent was stable throughout the study, thus not requiring any dose adjustment. CONCLUSION: The study illustrates the antihypertensive properties of amlodipine once-daily in paediatric hypertension. Amlodipine appears particularly indicated in patients concomitantly treated with cyclosporine.

Sedation with intravenous benzodiazepine and ketamine for renal biopsies.

"Lytic cocktail" is the customary sedation before percutaneous renal biopsy. Owing to deficient sedation and respiratory depression, this approach is no longer advised. The intravenous combination of a benzodiazepine plus ketamine, which has been found to be effective and safe in several conditions, was therefore used for 60 pediatric biopsies. In addition to pulse oximetry, a physician trained in airway management monitored the patient. The quality of sedation was acceptable (n = 7) or optimal (n = 53) in all cases. None of the patients required ventilation or intubation. A mild oxygen desaturation lasting 4 min or less was detected in 6 patients. None of the patients experienced major change in respiratory rate or blood pressure. The administration of ketamine and, in some cases, the biopsy puncture were associated with a mildly increased heart rate. In conclusion, a benzodiazepine plus ketamine provides effective deep sedation for renal biopsy in childhood.

[Urinary tract infections in pediatric practice--which new aspects must be considered?]. / Harnweginfektionen in der pädiatrischen Praxis--welche neuen Aspekte müssen berücksichtigt werden?

The prevalence of urinary tract infections varies markedly with sex and age. They are caused mainly by colonic bacteria. Acute cystitis should be promptly treated either with cotrimoxazol or coamoxyclav to prevent its possible progression to pyelonephritis. In acute febrile infections suggestive of pyelonephritis, the initial use of broad-spectrum antibiotics capable to reaching significant tissue levels is advised, mostly amoxycillin with an aminoglycoside. A variety of functional voiding disorders including bladder instability, detrusor-sphincter dyscoordination, postponing syndrome or lazy bladder syndrome have been identified since the early 1970s, each with its own characteristics and clinical importance. Functional voiding disorders are nowadays a major cause of recurrent urinary tract infections in children. A careful investigation to confirm or exclude functional voiding disorders is warranted in children with recurrent urinary tract infections. Management will vary according to the type and severity of the dysfunction present.

Conditions currently associated with erythema nodosum in Swiss children.

UNLABELLED: A review was made of the 36 paediatric patients in whom the diagnosis of erythema nodosum had been established between 1977 and 1996 at the Department of Paediatrics, University of Bern, Switzerland. Infectious diseases were associated with erythema nodosum in 20 (including 10 streptococcal infections) and non-infectious inflammatory diseases in 8 patients. None of the 36 patients had tuberculosis or had been exposed to sulphonamides, phenytoin or hormonal contraceptives. There were eight patients in whom either the associated disease was not diagnosed, or there was no other disease. CONCLUSION: Most cases of erythema nodosum are nowadays caused by non-mycobacterial infectious diseases or by non-infectious inflammatory diseases.

[How is hemolytic-uremic syndrome in childhood acquired in Switzerland?]. / Wie wird das hämolytisch-urämische Syndrom des Kindesalters in der Schweiz erworben?

Intestinal infections with shigatoxin-producing Escherichia coli or Shigella dysenteriae type I play a major role in the pathogenesis of the hemolytic-uremic syndrome in childhood. Escherichia coli has been repeatedly detected in the intestines of healthy cattle. Twenty-seven children with hemolytic-uremic syndrome were treated at our hospital between June 1990 and March 1997. Factors indicating a possible previous contact with bovine intestinal content were found in 18 out of the 27 patients: parents stockbreeders (n = 7), recent visit to a cowshed or contact with cowdung or manure (n = 5), residence in a rural cattle-breeding area (n = 5), or consumption of raw milk (n = 1). The factors mentioned were found in 5 out of 27 control patients (p < 0.01). Two children experienced hemolytic-uremic syndrome after a stay respectively in Egypt and Tunisia. Our results indicate an important source for acquisition of hemolytic-uremic syndrome in childhood. Observing simple hygienic rules such as washing of hands and pasteurization of milk is likely to have a positive influence on the incidence of this illness. There are also grounds to consider adding the hemolytic-uremic syndrome to the list of travel-related diseases.

Extracellular magnesium depletion in pediatric patients with insulin-dependent diabetes mellitus.

It is unclear whether insulin-dependent diabetes mellitus is a state of magnesium depletion. This is a relevant question, since magnesium deficiency has been implicated in the pathogenesis of diseases that develop to an increased extent into diabetes mellitus. Total plasma magnesium was not statistically different in 76 pediatric patients with insulin-dependent diabetes mellitus (0.77 [0.73-0.81] mmol/l; median and interquartile range), 59 healthy adults (0.80 [0.77-0.83] mmol/l) and 19 healthy children (0.80 [0.78-0.83] mmol/l). In contrast, plasma ionized magnesium, the most interesting form with respect to physiological and biological properties, was significantly lower in diabetic patients (0.50 [0.48-0.53] mmol/l) when compared with healthy adults (0.53 [0.50-0.56] mmol/l; p < 0.01) and healthy children (0.54 [0.51-0.56] mmol/l; p < 0.02). Our report confirms recent findings of reduced circulating ionized magnesium but normal circulating total magnesium in adults with non-insulin-dependent diabetes mellitus.

Acute metabolic alkalosis in cystic fibrosis: prospective study and review of the literature.

Between January 1993 and April 1994, 5 patients with cystic fibrosis, aged 4-9 months, were admitted to the Department of Pediatrics, University of Berne, Switzerland, with acute, severe metabolic alkalosis (sodium < 133 mmol/l, plasma potassium < 3.5, chloride < 85, bicarbonate > 35.0 mmol/l, blood pH > 7.43). 87 cases of acute metabolic alkalosis complicating cystic fibrosis reported in the literature between 1951 and 1995 were also reviewed. Our cases and those described in the literature demonstrate that acute metabolic alkalosis occurs in patients aged 2 years or less. Anorexia, vomiting, respiratory exacerbation, fever, and body weight loss often precede metabolic alkalosis. Furthermore, metabolic alkalosis is a common initial presentation of cystic fibrosis, suggesting that this diagnosis should be considered in the context of unexplained metabolic alkalosis.

Evidence for fluid volume depletion in hyponatraemic patients with bacterial meningitis.

Since the mechanisms underlying hyponatraemia in meningitis are poorly understood, we retrospectively reviewed the records of 187 paediatric patients with bacterial meningitis treated at the Department of Pediatrics, University of Bern, Switzerland, between 1982 and 1994. The degree of dehydration calculated from naked weight on admission and at 5 days was consistently (by 2.8 x 10(-2) and significantly more pronounced in 30 hyponatraemic (plasma sodium 130 mmol l-1 or less) than in 157 normonatraemic patients (plasma sodium 131 mmol l-1 or more). Furthermore, a tendency towards reduced sodium excretion was noted in hyponatraemic patients. The results suggest that in bacterial meningitis hyponatraemia is mostly induced by clinically latent fluid volume depletion.

Plasma ionized magnesium during acute hyperventilation in humans.

1. Respiratory alkalosis accompanies the clinical syndrome of tetany, precipitates cardiac arrhythmias and predisposes to coronary vasoconstriction. Magnesium plays a critical role in the maintenance of membrane function, and magnesium depletion is often associated with cardiac arrhythmias or vasoconstriction. 2. As technology for detecting circulating ionized magnesium (the most interesting form with respect to physiological and biological properties) is now available in the form of new magnesium-selective electrodes, the effect of respiratory alkalosis induced by voluntary overbreathing for 30 min on circulating ionized magnesium was studied in eight healthy subjects. 3. The total plasma magnesium concentration was not modified by hyperventilation. On the contrary, hyperventilation was associated with a significant reduction in the ionized magnesium concentration of 0.05 (0.02-0.15) mmol/l (median and range) and in the free magnesium fraction of 0.06 (0.01-0.19). During hyperventilation the relative intravascular magnesium mass, calculated from changes in total plasma magnesium concentration and haematocrit, decreased significantly. 4. It is concluded that acute overbreathing reduces the circulating ionized magnesium concentration and the intravascular magnesium mass. It is therefore conceivable that extracellular magnesium deficiency is at least a subsidiary cause of the syndrome of tetany and the cardiac complications that are precipitated by hyperventilation.

[Schoenlein-Henoch purpura: course in 139 children]. / Purpura Schönlein-Henoch: Verlauf bei 139 Kindern.

We evaluated the records of 139 pediatric patients with Schönlein-Henoch purpura (aged 0.4 to 15.1 years, median 5.4 years), referred from 1974 to 1993 to the University Children's Hospital, Berne. An acute febrile illness preceded Henoch-Schönlein purpura in 83 (60%) out of the 139 children. The purpuric papules were distributed over the lower extremities and the buttocks in 68 patients (49%), and more extensively in the remaining 71 (51%). Joint tenderness or swelling was observed in 110 patients (79%). Abdominal involvement occurred in 92 patients (66%): abdominal pain (n = 63), melena or hematemesis (n = 23), and intussusception (n = 6). The following rather rare features were observed: scrotal swelling (n = 11), neurologic involvement (n = 3), and stenosing ureteritis (n = 1). A remission lasting at least 4 weeks occurred within 4 weeks in 84, within 5-8 weeks in 19, and within 9-53 weeks in 18 out of 121 patients. A relapse was observed in 10 subjects. Renal involvement occurred in 60 patients. Severe renal involvement, defined as proteinuria exceeding 40 mg/[m2 X h], occurred in 18 of the patients with renal involvement. Progredient renal failure developed in one male. Children with renal involvement tended to be older (6.6 versus 4.1 years) and to have more prolonged extrarenal manifestations (6 versus 3 weeks). It is concluded that in children with Schönlein-Henoch purpura extrarenal involvement often lasts more than one month. Its outcome is almost always favourable. Children with heavy proteinuria tend in some cases to develop renal failure. For these patients, new treatment regimens aimed at preventing renal failure warrant evaluation in prospective controlled studies.

[Precision of glomerular filtration estimation by height/creatinine ratio in the child]. / Précision de l'estimation de la filtration glomérulaire par les formules taille/créatinine chez l'enfant.

Formulae for estimating glomerular filtration rate with the use of body size, circulating creatinine, and an empirically derived constant are widely used. The aim of the present study was to determine whether these formulae enable accurate prediction of glomerular filtration measured from the labeled ethylenediaminetetraacetic acid in 843 pediatric patients. Bland-Altman analysis was performed on the measured glomerular filtration rate and that estimated using the constant developed in our laboratory. Age and sex of the 843 patients failed to consistently influence the empirically derived constant. Measured and estimated glomerular filtration rate were on the average identical. However, the 95% limits of agreement of the measured glomerular filtration rate with that estimated were wide (measured over estimated glomerular filtration from 0.53 to 2.04). Use of height to creatinine formulae to estimate glomerular filtration is therefore frequently insufficiently accurate.

Urinary chloride excretion distinguishes between renal and extrarenal metabolic alkalosis.

UNLABELLED: The aetiology of normotensive hypokalaemic metabolic alkalosis is sometimes not obtainable from the history. Observations in adults indicate that the urinary chloride excretion is low in metabolic alkalosis of extrarenal origin. The chloride/creatinine ratio in random urines was therefore compared in 283 healthy children and in eight paediatric patients with metabolic alkalosis. The urinary chloride/creatinine ratio was reduced in four patients with metabolic alkalosis of extrarenal origin and within reference values or above in four patients with metabolic alkalosis of renal origin. CONCLUSION: The study confirms that urinary chloride/creatinine ratio discriminates between extrarenal and renal forms of metabolic alkalosis.

[Management of children and adolescents with kidney transplantation]. / Betreuung von nierentransplantierten Kindern und Adoleszenten.

Successful renal transplantation is widely accepted as the treatment of choice for end-stage renal failure in infants, children and adolescents. The major issues currently requiring consideration when contemplating renal transplantation in the mentioned patients are: primary renal disease, psychosocial status, life-related versus cadaver donor allograft, optimal immunosuppressive regimen including cyclosporine, and maximization of growth and pubertal development. In transplanted adolescents, noncompliance is now a major problem, ranking only second to rejection as a cause of graft loss.

Histological features of glomerular immaturity in infants and small children with normal or altered tubular function.

In children with renal tubular disorders the existence of retarded histological features of glomerular maturation has been suggested by our group. However, no valuable information is available on the frequency of histopathologically immature glomeruli in the normal kidney. For this reason we established a simple, semiquantitative definition of postnatal glomerular development: immature glomeruli (with at least half of the circumference of capillary loops densely lined with cuboidal epithelial cells), intermediate glomeruli (circumference of capillary loops lined with at least five adjoining cuboidal cells), and mature glomeruli. This definition was applied in a set of 71 normal kidneys from ages birth to 5 years. The relative frequency of the mentioned stages of glomerular maturation was strongly age dependent. In comparing the patients data with the normal findings it was possible to separate patients with the finding of disproportionately high fractions of immature glomeruli, provided the right age at biopsy is chosen. It is therefore concluded that the previous suspicion of late glomerular maturation may be validated in at least in some bioptic specimens.