Antenatal oligohydramnios of renal origin: postnatal therapeutic and prognostic challenges.

Urinary tract anomalies (UTA) including polycystic kidney disease nowadays can be detected antenatally by ultrasound. The concomitant presence of oligohydramnios has been regarded as a severe risk factor for renal dysfunction and pulmonary hypoplasia, although clinical data after birth are scarce. We report the postnatal course and long-term follow-up of 10 infants with oligohydramnios due to congenital UTA from two pediatric nephrology centers. The underlying final diagnoses were autosomal-recessive polycystic kidney disease (ARPKD, n = 2), familial tubular dysgenesis (n = 2) and bilateral renal hypoplasia (n = 6) including 3 children with posterior urethral valves. Two children died in the neonatal period while 8 children are currently alive at a median age of 2.5 (range 1.1-10) years. In the postnatal period, respiratory failure necessitating mechanical ventilation occurred in 7 infants (including the 2 non-survivors). All surviving children had chronic renal failure, which could be managed conservatively in 6 children (median GFR 45 (range 15-53) ml/min/1.73 m2) while 2 reached end-stage renal disease; one undergoing preemptive kidney transplantation and one peritoneal dialysis. Seven of 8 children reached normal developmental milestones. In conclusion, the presence of antenatal oligohydramnios in infants with UTA does not always carry a poor prognosis. The high incidence of perinatal complications, the complexity of underlying causes and the prevalence of postnatal chronic renal dysfunction calls for a multidisciplinary approach in the management of these children.

Nosocomial outbreak of vancomycin-resistant Enterococcus faecium at a German university pediatric hospital.

Nosocomial Infections caused by vancomycin-resistant enterococci (VRE) are an emerging threat to critically ill patients. At the University Hospital Eppendorf, VRE were isolated from 38 patients between August 1993 and April 1997, of whom 32 were hospitalized at the Department of Pediatrics. Pulsed-field gel electrophoresis revealed that 26 Enterococcus faecium isolates from patients of the Department of Pediatrics were identical or closely related, and that isolates from three additional patients of the same department were possibly related. All of these isolates were of vanA genotype. They were resistant to glycopeptides, ampicillin, ciprofloxacin, clindamycin, and erythromycin. Most isolates displayed high-level resistance to gentamicin, but all remained susceptible to quinupristin/dalfopristin. Implementation of stringent hand disinfection and environmental disinfection policies, as well as measures for patient isolation contained this first outbreak of VRE at a German Children's hospital, which emphasizes the importance of hygienic measures for the control of nosocomial spread of these organisms.

Intensive care management after pediatric liver transplantation: a single-center experience.

A retrospective study was conducted to determine the significance of intensive care management on outcome after liver transplantation (LTx) in children. Of 195 transplants performed in 162 children, factors affecting morbidity and mortality were documented during the post-operative intensive care unit (ICU) stay. To assess the gain in experience of ICU management, we compared mean ventilation time and stay in the ICU as well as mortality, incidence of surgical complications, infections, and rejection episodes, during three different time-periods (October 1991-August 1994, September 1994-July 1996, and August 1996-February 1998). The time spent by patients in the ICU (9.7 days vs. 7.9 days vs. 4.7 days, p < 0.001) and time on ventilation (5.2 days vs. 3.1 days vs. 1.2 days, p < 0.001) were significantly reduced over the duration of the study. The overall mortality was 18.0% (n = 30) and 76.7% (n = 23) of these deaths occurred during the early post-operative period in the ICU. The incidence of severe surgical complications decreased significantly over time, and the application of intra-operative Doppler ultrasound since 1994 led to detection of 27 correctable vascular complications. The overall incidence of acute cellular rejection episodes in our center was 64.1%: 43.5% of the infectious episodes occurred in the ICU (bacterial 70.2%, viral 12.3%, and fungal 17.5%). The main side-effect from immunosuppressive drugs was arterial hypertension in 29% of the patients. We conclude that our efforts to improve intensive care management and monitoring were the key elements in reducing morbidity and mortality after pediatric LTx.

[Treatment of hemophagocytic lymphohistiocytosis, HLH, with bone marrow transplantation]. / Behandlung der Hämophagozytischen Lymphohistiozytose, HLH, mit Knochenmarktransplantation.

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of infancy and young childhood. The clinical presentation includes recurrent unexplained fever with hepatosplenomegaly. Cytopenia, hypofibrinogenemia and/or hypertriglyceridemia and hemophagocytosis in bone marrow, spleen and lymphnode confirm the diagnosis. Hemophagocytosis may not be present at the beginning. In these cases, diagnosis is facilitated by a positive family history, a relapsing course of the disease, the frequent involvement of the central nervous system and positive findings on immunological work-up. Treatment by chemotherapy and immunosuppressants can achieve sustained remissions in most patients and reinduction of remission after relapse is possible. Most children however, eventually die from progressive disease. At present, allogeneic bone marrow transplantation is the only curative therapeutic option. Between August 1992 and May 1997 eleven consecutive patients with HLH received bone marrow from unrelated (n = 7) or matched sibling donors (n = 4). The conditioning regimen consisted of busulfan, VP-16 and cyclophosphamide. Patients engrafted after a median time of 16 days (13-43). Only one patient developed grade III acute GVHD, another patient, grade II acute GVHD. Although regimen-related toxicity was extensive, all patients have survived without signs of HLH after a median follow up of 20 months (8-63). One patient suffers from chronic GVHD, three patients reveal psychomotoric retardation and one patient has severe impairment with spastic tetraparesis, amaurosis and seizures. Our experience shows that HLH can be successfully treated by allogeneic BMT from unrelated donors.

Diagnosis of congenital absence of internal carotid artery by power angio sonography.

This case report describes aplasia of the internal carotid artery (ICA) in a preterm infant. The collateral circulation could be mapped with power angio mode (PAM) and was confirmed by conventional angiography. In the literature, there is no case of ICA aplasia diagnosed at this early age. PAM is a method for imaging infantile cerebral vessels as reliably as angiography.

Hyperkalemia: therapeutic options in acute and chronic renal failure.

Hyperkalemia is a life threatening emergency and warrants immediate treatment because of its deleterious cardiac consequences. Initial measures in mild cases include restriction and binding of dietary potassium, correction of metabolic acidosis and increasing urinary excretion by furosemide. In moderate and severe hyperkalemia infusion of glucose with insulin has been regarded as the standard medical treatment so far. However, recently also the beta 2 stimulatory drug salbutamol has been shown to be an effective agent to treat hyperkalemia by inducing a shift of potassium into the intracellular compartment. We treated 15 pediatric patients of different age groups (mean age 5.16, range 0.1-16 years) suffering from acute hyperkalemia (mean level 6.6, range 5.9-7.7 mmol/l) by means of a single infusion of salbutamol (5 micrograms/kg over 15 minutes). Serum potassium concentrations decreased significantly to 5.74 +/- 0.53 after 30 minutes and furthermore to 5.19 +/- 0.48 and to 4.92 +/- 0.53 mmol/l after 60 and 120 minutes, respectively (p < 0.001 at all stages compared to pre-treatment). Since no side effects occurred besides a slight increase of heart rate in 3 patients, we conclude that short-term intravenous salbutamol infusion is an effective, rapid, safe and predictable way to treat children of any age suffering from acute hyperkalemia and therefore has become the first line treatment in our center.

Effective treatment of acute hyperkalaemia in childhood by short-term infusion of salbutamol.

UNLABELLED: Hyperkalaemia is a life-threatening emergency and infusion of glucose with insulin has so far been regarded as the standard treatment of choice. Recently the beta-2 stimulatory drug salbutamol has been shown to be an effective agent to treat hyperkalaemia by inducing a shift of potassium into the intracellular compartment. We treated 15 children aged 0.1-14 (mean 5.2) years suffering from acute hyperkalaemia (mean level 6.6 +/- 0.54, range 5.9-7.7 mmol/l) with a single infusion of salbutamol (5 micrograms/kg over 15 min). Serum potassium concentrations decreased significantly within 30 min to levels of 5.74 +/- 0.53 and 4.92 +/- 0.53 mmol/l after 120 min (P < 0.001, respectively). No side-effects occurred other than a light increase in heart rate in 3 patients. CONCLUSION: A single intravenous infusion of salbutamol at a dose of 5 micrograms/kg is a highly effective treatment for hyperkalaemia with minimal clinical side-effects. The effect lasts for at least 120 min and may reverse hyperkalaemia in some patients without further interventions so that salbutamol seems justified as the first choice treatment for this condition in childhood.