Long-term effects of fundoplication in children with chronic airway diseases.

BACKGROUND: Association between chronic airway diseases (CAD) and gastroesophageal reflux disease (GERD) is well described, but causality has not yet been conclusively established. This study evaluates the therapeutic significance of laparoscopic Thal fundoplication in children with CAD and diagnosed GERD. METHODS: We performed a retrospective analysis of 182 neurologically nonimpaired children, all with medically refractory CAD and GERD undergoing laparoscopic Thal fundoplication. The clinical response, ability to wean oral and inhaled medication and satisfaction with postoperative results were evaluated. RESULTS: Main symptoms disappeared completely in 68.7% of patients and were markedly improved in a further 22% of patients following surgery. Complete discontinuation of medication was achieved in 70.1-96.4% of cases and reduced in a further 1.8-23.5%. One intraoperative complication occurred (gastric perforation), however no conversion to laparotomy was necessary. Postoperative Dumping Syndrome occurred in 1% of cases and was managed dietetically. Prolonged postoperative dysphagia occurred in 4.3% of patients, but disappeared within 8 weeks in all but one case. CONCLUSIONS: Our study suggests that Thal fundoplication in neurologically nonimpaired children with CAD and documented GERD is effective and safe. Children unresponsive to preoperative medical management showed significant improvement in airway symptoms together with a marked reduction in the need for medication. We conclude that laparoscopic Thal fundoplication represents a significant treatment worthy of consideration in this group of patients.

Elevated plasma concentration of neuropeptide Y in adolescents with primary hypertension.

Neuropeptide Y (NPY) has been recently characterised as a circulating vasoconstrictor peptide which is co-stored with noradrenaline (NA) in sympathetic neurons. We measured NPY by radioimmunoassay and NA by HPLC in plasma of ten healthy volunteers (23-27 years of age) during bicycle ergometry and found a rapid increase of both NPY and NA during exercise. NPY rose from 1.3 +/- 0.5 to 9.6 +/- 7.8 pmol/l and NA from 1.3 +/- 0.3 to 10.8 +/- 5.6 mnol/l (mean +/- SD). Following maximal exercise NA disappeared more rapidly from plasma than NPY. Compared with these healthy volunteers, plasma NPY was found to be elevated in 23 children and adolescents aged 9-18 years with borderline primary hypertension (NPY 3.1 +/- 1.7 pmol/l, P < 0.01). Basal NPY was also elevated when compared with 21 age-matched pediatric controls (P < 0.05). The bicycle ergometry protocol performed in 23 patients separated ten adolescents with normal basal and exercise blood pressure from 13 with high BP also during ergometry. In the latter group, NPY rose to 11.9 +/- 7.3 pmol/l and NA to 12.3 +/- 8.6 nmol/l during exercise. Treatment of the hypertensive patients with the beta-adrenergic blocker atenolol (50 mg per day) lowered basal and exercise BP. Heart rate fell during atenolol treatment from 92 +/- 19 to 72 +/- 15 beats/min. Treatment did not alter plasma concentrations under basal conditions and during exercise (NPY from 2.8 +/- 2.1 to 11.7 +/- 5.3 pmol/l and NA from 2.0 +/- 0.8 to 15.6 +/- 14.1 nmol/l).(ABSTRACT TRUNCATED AT 250 WORDS)

24-hour blood pressure monitoring in children and adolescents after recovery from hemolytic uremic syndrome.

24-hour blood pressure monitoring is a valuable method for the diagnosis of arterial hypertension as well as for assessment of the diurnal rhythm of the arterial blood pressure (BP). The nocturnal decrease of blood pressure ("dipping") may be attenuated or abolished in children with advanced renal failure and glomerular diseases. Arterial hypertension is a longlasting problem in children who had recovered from hemolytic uremic syndrome (HUS). We therefore performed BP monitoring in 11 children and adolescents (age 1.3 to 18.8 years, 6 males, 5 females) after HUS using a portable oscillometric device (SpaceLabs 90207). Six of the subjects had a normal renal function (group A). The other 5 patients had impaired renal function with a glomerular filtration rate <60 ml/min/1.73 m2 (group B). Nocturnal dipping was calculated as nocturnal mean blood pressure minus diurnal mean blood pressure given in per cent of diurnal mean blood pressure. Two of the patients in group A had diurnal mean BP above the 95th percentile of the German collaboration study, but none of the group was hypertensive during the night, and nocturnal dipping was 13.6% (9.7-15.5%, median and range) for systolic BP and 23.7% (15.5-29.9%) for diastolic BP which is very similar to healthy children. All of the patients had a normal diurnal BP rhythm. From patients of group B, 4 had elevated diurnal mean BP and also 4 were hypertensive during the night. Nocturnal dipping was 1.4% (0.7-4.1%) for systolic and 6.8% (0-10.7%) for diastolic BP which is clearly attenuated compared to group A. We therefore conclude that arterial hypertension is more common in patients after HUS if they have impaired renal function, and diurnal rhythm of arterial blood pressure is attenuated in these patients. However, nocturnal dipping of blood pressure is not disturbed in children after HUS without renal insufficiency, even if they were hypertensive.

Bent subcutaneous peritoneal catheter shape for regular dialysis treatment in children.

A permanent bowing of the subcutaneous part of the Tenckhoff-type catheter (bent neck--Quinton, and swan neck--Accurate Surgical Instruments) enables the catheter to turn from an upward direction of the subcutaneous tunnel to a downward direction by a smooth 160 degrees-180 degrees bend creating a downward skin exit. We have used this catheter shape in combination with a coiled intra-abdominal edge. Two sizes are available for children. We use 2 cuffs and glue them ourselves according to the body size. In this study we compare the durability of the traditional subcutaneously straight catheter in 8 children aged 0.1-12.6 years (Group A) with the bent shaped catheter in 8 children aged 3.7-15.8 years (Group B). Median duration of function was 10.5 (2-34) and 8 (3-36) months, respectively. Frequency of complications was equal in both groups: peritonitis episodes 0.69/year in Group A and 0.53/year in Group B; tunnel infection 0.16 vs 0.11/year; skin exit infection 0.54 vs 0.53/year; noninfectious complications 0.16 vs 0.32/year; mean number of catheters used was 1.0 vs 1.1/treatment year. Treatment had to be terminated in some patients: kidney transplantation 5, kidney recovery 1, severe peritonitis 1. The bent subcutaneous catheter shape did not show any medical or technical disadvantage compared with the straight type, but the downward directed catheter skin exit can be covered invisibly under bikini or bermuda shorts which means aesthetic and social advantage. Whether the downward drainage of secretes and cell detritus influences the rate of tunnel infection positively cannot be answered to date.

[Pediatric ureteral ureteral outlet obstruction and obstructive megaureter: observation or operation?]. / Kindliche Ureterabgangsstenose und obstruktiver Megaureter: Observation oder Operation?

Stenosis of the ureteropelvic junction and obstructive megaureter are still a diagnostic and therapeutic problems. So far, there is no reliable prognostic factor to predict the outcome of a primary dilated upper urinary tract under the "wait and see" strategy and to decide which child must be operated upon and which should not. In practice, basic diagnostic evaluation with sonography, possibly i.v.-pyelography and voiding cystography is accompanied by quantifying isotope-based procedures such as DMSA-uptake or diuretic renography. The Whitaker test has become less important. Based on the investigation by Koff et al., it seems to be possible to follow the "wait and see" procedure in more than 85% of the children without any loss of renal function of the dilated kidney.

Differential diagnoses for nocturnal enuresis.

The symptom of nocturnal enuresis can be a feature of many conditions, including renal, neurological and organic disease states. It is important to differentiate primary nocturnal enuresis from secondary enuresis and from daytime incontinence with a nocturnal component. It is recommended that the routine investigation of enuretic children should comprise four components: structured interview, physical examination, urinalysis and ultrasound investigation. Additional intensified diagnostic and invasive procedures should be reserved for patients with suspected neurological disorders or urological dysfunction.

[Urinary tract infections in childhood. Old and new aspects]. / Harnwegsinfektionen im Kindesalter. Altes und Neues.

Urinary tract infections are one of the most frequent infections during childhood. About 5% of all girls and 0.5% of all boys are suffering from at least one urinary tract infection until the end of schooltime. While the boys predominate in the first year of life with decreasing incidence of infection later on the girls remain predisposed up to an age of twelve. Each pediatrician faces every day the problem of a quick diagnosis and an appropriate therapy of urinary tract infections. While symptomless bacteriuria and cystourethritis do not destroy the renal parenchyma recurrent pyelonephritis may cause irreversible parenchyma scars up to a dialysis demanding renal failure. Besides the well-known mechanical risk factors (i.e. obstruction, reflux) functional risk factors like impaired bladder function alone as well as combined with mechanical ones are gaining in significance. Another factor predisponing to recurrent urinary tract infections is a local immunologic impairment of the urinary tract (i.e. P1-blood-group antigen, decreased urothelial function, increased urothelial colonisation). Age-appropriate and individual diagnosis and treatment are necessary first to avoid late damages and second to protect the children against unnecessary diagnostic and therapeutical managements.

[Differential enuresis nocturna diagnosis]. / Differentialdiagnose der Enuresis nocturna.

The symptom of enuresis can be a feature of many conditions, including renal, neurological and organic disease states. It is important to differentiate primary nocturnal enuresis from secondary enuresis and from daytime incontinence with a nocturnal component. It is recommended that the routine investigation of enuretic children should comprise four components: structured interview, physical examination, urine analysis and ultrasound investigation. Additional intensified diagnostic and invasive procedures should be reserved for patients with suspected neurological disorders or urological dysfunction.

[IgA nephritis: a frequent form of glomerulonephritis in childhood]. / IGA-Nephritis: eine häufige Form der Glomerulonephritis im Kindesalter.

Since the introduction of immunohistological techniques in the evaluation of renal biopsies, IGA-nephropathy as first described by Berger in 1968 is diagnosed frequently. The diagnosis of IGA-nephropathy is based upon the demonstration of mesangial IGA deposits. IGA-nephropathy has to be differentiate from post-streptococcal glomerulonephritis, Schönlein-Henoch-nephritis and Lupus-nephritis. With increasing observation periods the prognosis has not been confirmed to be benign, as previously assumed. Every physician interested in paediatrics should be aware of this kind of nephritis, because early renal biopsy is essential for the diagnosis and the estimation of the degree of histological alteration. Furthermore renal biopsy is important for the decision for an aggressive immunosuppressive therapy and therefore for the prognosis.

[Acute reversible kidney failure in IgA nephritis]. / Akutes reversibles Nierenversagen bei IgA-Nephritis.

Four patients with acute renal failure during an acute course of IGA-nephritis are described. Percutaneous renal biopsies showed only minor glomerular lesions in all patients. Immunosuppressive therapy was not necessary. In all patients acute renal failure was completely reversible. The four patient showed further episodes of macrohematuria without acute renal failure. All patients had normal renal function, normotensive blood pressure and microhematuria during interval. In three of the patients we found erythrocytes casts and a mild protein excretion. IGA-nephritis is one of the most common types of glomerulonephritis in adolescents and adults. If acute renal failure occurs during a course of IGA-nephritis with macroscopic haematuria a percutaneous renal biopsy has to be performed. Only in case of severe crescents formation (greater than 75%) immunosuppressive therapy is necessary. Glomerular lesions are responsible for long time prognosis.

[Prophylaxis of recurrent urinary tract infections in children. Results of an open, controlled and randomized study about the efficacy and tolerance of cefixime compared to nitrofurantoin]. / Reinfektionsprophylaxe rezidivierender Harnwegsinfektionen im Kindesalter - Ergebnisse einer offenen, kontrollierten und randomisierten Studie zur Wirksamkeit und Verträglichkeit von Cefixim im Vergleich zu Nitrofurantoin.

UNLABELLED: Urinary tract infections are quite frequent in children. Urinary tract obstruction combined with recurrent urinary tract infections increase the risk for renal impairment. Therefore prophylaxis of reinfection is an important nephroprotective procedure. The aim of this open, controlled, randomised pilot study was to compare the efficacy and tolerance of a low dose prophylaxis with Cefixime versus Nitrofurantoin. 60 girls aged 1 to 11 years with at least 2 urinary tract infections within the preceding year were included in the study. The minimum duration of therapy was 6 months and was extended to 12 months for most of the children. The number of recurrent infections was the main criteria for efficacy evaluation, whereas adverse events were analysed to evaluate tolerance. Statistical significant differences between the two treatment groups, regarding recurrence rates could not be demonstrated. Tolerance was comparable in both groups. The influence on gut flora of cefixime given as a low dose regimen over a long period of time corresponds with already published results and was not correlated with a higher number of gastrointestinal side effects. CONCLUSION: Low-dose Cefixime (2 mg/kg bodyweight) is effective and well tolerated in the prophylaxis of recurrent urinary tract infections. Efficacy and tolerance of cefixime were comparable to the results obtained with nitrofurantoin. Due to the small number of patients this study was only a pilot study. Low-dose cefixime, however, could become an alternative to standard regimens in the prophylaxis of recurrent urinary tract infections. This should be investigated in further studies.

Validity of G1-cells in the differentiation between glomerular and non-glomerular haematuria in children.

Urine samples from 100 children and adolescents with micro- or macrohaematuria were investigated using phase contrast microscopy to establish the percentage of G1-cells that could differentiate glomerular from non-glomerular haematuria. The G1-cell is a special form of dysmorphic erythrocyte which seems to be specific for glomerular haematuria. Glomerular haematuria, defined by clinical criteria from biopsy, physical examination, standard laboratory evaluation and family history, was observed in 51 patients (group 1). Non-glomerular haematuria was found in 49 patients (group 2). The latter group had urinary tract infections, urolithiasis, hypercalciuria or haematuria caused by urological operation or diagnostic procedure. The percentage of dysmorphic erythrocytes differed significantly between the two groups studied (42 +/- 3% in group 1 vs. 6 +/- 1% in group 2, mean +/- SEM, P < 0.01); there was also a significant difference in G1-cells (19.4 +/- 1.7% in group 1 vs. 0.6 +/- 0.2% in group 2, mean +/- SEM, P < 0.01). When glomerular haematuria was defined on the basis of > or = 30% dysmorphic erythrocytes by phase contrast microscopy, sensitivity, specificity and efficiency were 71%, 100% and 85%, respectively. When glomerular haematuria was defined on the basis of > or = 5% G1-cells, sensitivity, specificity and efficiency were 100%, 100% and 100%, respectively. The differentiation of glomerular and non-glomerular haematuria in children by determination of G1-cells appears to be more sensitive and efficient than the determination of the percentage of dysmorphic erythrocytes by phase contrast microscopy.

Influence of steroid medication on bone mineral density in children with nephrotic syndrome.

Bone mineral density (BMD) was studied in 26 children with idiopathic nephrotic syndrome and in age- and sex-matched healthy controls. BMD was selectively measured in trabecular (TBD), cortical (CBD) and total bone (BD) using peripheral quantitative computed tomography. Patients showed a decrease in BD, CBD and TBD. BD and CBD were inversely correlated with the cumulative dose of steroid treatment. Of the 26 patients with high cumulative doses of steroid, 16 were also treated with cyclophosphamide. In this group BD and CBD were decreased significantly compared with the children with a low cumulative steroid dose only. Compared with controls for each subgroup, significant decreases in BD, CBD and TBD were found in the group with high cumulative doses of steroids only. The higher cumulative steroid dose and the initial steroid toxicity which made cytotoxic therapy necessary, rather than cyclophosphamide itself, may be responsible for these findings.

Differentiation of glomerular and non-glomerular hematuria in children by measurement of mean corpuscular volume of urinary red cells using a semi-automated cell counter.

Urine samples from 110 children and adolescents with micro- or macrohematuria were compared using phase-contrast microscopy and a semi-automated cell counter to differentiate glomerular from non-glomerular hematuria. Glomerular hematuria, defined by clinical criteria from biopsy and standard chemical evaluation, was observed in 73 patients (group 1): non-glomerular hematuria was found in 37 patients (group 2). The latter group underwent urological operation and had normal urine before operation. Mean corpuscular erythrocyte volume (MCVU) and percent of dysmorphic erythrocytes were determinated. To exclude the influence of mean erythrocyte volume of blood erythrocytes (MCVB), MCVB was determined and additionally the quotient of MCVU/MCVB was calculated (MCVUB). The percentage of dysmorphic erythrocytes differed significantly between the two groups ((75 +/- 13% in group 1 versus 38 +/- 27% in group 2 (mean +/- SD); p < 0.01), MCVU (34.0 +/- 11.1 fl in group 1 versus 55.5 +/- 16.3 fl in group 2; p < 0.01) and MCVUB (0.41 +/- 0.14 in group 1 versus 0.67 +/- 0.20 in group 2; p < 0.01). When glomerular hematuria was defined on the basis of more than 80% dysmorphic erythrocytes, the sensitivity of phase-contrast microscopy was 0.52, specificity versus 0.96 and efficiency 0.64. When glomerular hematuria was defined as < 50 fl MCVU, sensitivity was 0.92, specificity 0.57 and efficiency 0.80 and as < 0.06 MCVUB, sensitivity was 0.89, specificity 0.62 and efficiency 0.80. The correlation coefficient between MCVU and dysmorphic erythrocytes was -0.71 (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Sudden death in cystinosis.

We report on an 8-year-old boy with infantile cystinosis. The patient died in hospital for unknown reason after cardiopulmonary arrest although resuscitation was started immediately. As opposed to other cases [1-3], neither laboratory investigations nor autopsy in out patient gave an explanation for the sudden death.

[Prenatal ultrasound diagnosis of the kidneys and efferent urinary tract. Possibilities, applications and dangers]. / Die antenatale Ultraschalldiagnostik der Nieren und der ableitenden Harnwege. Möglichkeiten, Nutzen und Gefahren.

Congenital abnormalities of the urinary tract can be detected as early as during the 15th-18th week of gestation. Nevertheless, most abnormalities are detected between the 28th-30th week of gestation. Antenatal fetal abnormalities vary between physiological dilatation to bilateral hydronephrosis and polycystic kidney disease. Abnormalities in antenatal fetal ultrasonography are found in 0.5% of all pregnancies, 30-50% of these abnormalities are related to the urinary tract. Though most of the abnormalities are diagnosed late in pregnancy, irreversible damage of the kidneys has already taken place by that time and the fetus will not profit from antenatal therapy. On the other hand, a fetus with late onset of urinary tract obstruction may profit from antenatal intervention or early labor. Repeated ultrasonography is crucial for initiating further invasive diagnostic procedures, antenatal therapy and for planing the time of labor. There is a great risk of overdiagnosing and inadequate therapy because too little is known about physiological development of the fetal urinary tract in ultrasonography and so physiological and pathological development cannot be differentiated safely.

[Prenatal ultrasound diagnosis in congenital abnormalities of the kidneys and efferent urinary tract from the pediatric nephrology viewpoint]. / Antenatale Ultraschalldiagnostik bei angeborenen Fehlbildungen der Nieren und des ableitenden Harntraktes aus pädiatrisch-nephrologischer Sicht.

Antenatal foetal abnormalities are found in 0.5% of all pregnancies. 30-50% of these abnormalities are related to the urinary tract. Although congenital abnormalities of the urinary tract can be detected as early as in the 15th-18th weeks of gestation, most abnormalities are detected between the 28th-30th weeks of gestation. At this time irreversible damage of the kidneys has taken place and the foetus will not benefit from antenatal therapy. On the other hand, a foetus with late onset of obstruction will benefit from antenatal intervention. Repeated ultrasonography is crucial for initiating further invasive diagnostic procedures, antenatal therapy and for planning the time of labour. There is a great risk of overdiagnosis and inadequate therapy because 25% of all antenatally diagnosed dilatations of the urinary tract could not be confirmed postnatally.

Primary obstructed megaureter in neonates. Treatment by temporary uretero-cutaneostomy.

During the last 10 years we have treated 24 newborn and young infants (27 ureters) in whom the diagnosis of primary obstructed megaureter was established by diuresis-enhanced radionuclide renography and/or by antegrade pressure flow studies (Whitaker test). Temporary uretero-cutaneostomy was performed immediately after diagnosis. After 1 year a second modified Whitaker test showed normal passage of the contrast medium into the bladder in 23 ureters. In only 4 ureters did obstruction persist and uretero-cystoneostomy was performed. Ureterocystoneostomy was also performed on 8 ureters without obstruction but with persistent vesicoureteric reflux, ectopic ureter or diverticula. In 11 patients a temporary uretero-cutaneostomy was closed without corrective surgery at the vesicoureteric junction. Temporary uretero-cutaneostomy is a safe and effective therapy in primary obstructed megaureter in newborns. Spontaneous relief of obstruction appears to be possible in primary obstructed megaureter.