T. Linné

Urine interleukin-6 and interleukin-8 in children with acute pyelonephritis, in relation to DMSA scintigraphy in the acute phase and at 1-year follow-up

The relationship between urine interleukin-6 (IL-6) and interleukin-8 (IL-8)/creatinine quotients and99mTc-dimercaptosuccinic acid (DMSA) scintigraphy, performed within 10 days of acute first-time pyelonephritis and after 1 year, was studied in 41 children. The urine IL-6 and IL-8/creatinine quotients were also related to the urineN-acetyl-β-D-glucosaminidase (NAG) and albumin/creatinine quotients. Presence of DMSA uptake defects, reflecting local inflammation, in children in the acute phase of pyelonephritis, were associated with elevated urine IL-6/creatinine quotients (median 27 pg/μmol); in children without DMSA changes there was no increase in quotients (median non-detectable) (P<0.05). Persistent DMSA changes at the 1-year follow-up, probably reflecting renal scarring, were only seen in children with increased urine IL-6/creatinine quotients in the acute phase (P<0.01). No correlation was found between urine IL-8 and DMSA uptake defects. Vesicoureteral reflux (VUR) at 6–8 weeks did not correlate with the urine cytokine levels in the acute phase. The urine excretion of NAG and albumin, reflecting renal dysfunction, was associated with values of both urine IL-6 and IL-8/creatinine quotients, but not with DMSA defects or VUR. Thus, the initial urine IL-6/creatinine quotients might be used as an indicator of risk for persistent renal damage in acute pyelonephritis.

COURSE OF RENAL FUNCTION IN IgA GLOMERULONEPHRITIS IN CHILDREN AND ADOLESCENTS

ABSTRACT. The pathophysiology of IgA GN was investigated in different stages of the disease. Seventeen patients who were between 3.5 and 16.5 years of age at the onset were included in the study. Clearance studies were performed repeatedly in 6 patients (in 5 of them over a period extending from the onset to 5‐9.5 years) and only once in 9 patients (10‐23 years after the onset). Two patients (one with uremia) were only evaluated clinically. CIn, CPAH and UNaV were studied during hydropenia (HP) and 3% isotonic saline volume expansion (VE). Shortly after the onset CIn, CPAH and UNaV were depressed. Renal function was essentially normal 1 and 2 years after the onset in spite of signs of active disease. A supernormal GFR was found in 7 patients after they had had the condition between 5 and 17 years. After a duration of IgA GN for >9 years 3 of 12 patients had developed hypertension and uremia and 2 had hypertension or labile BP. Three of 10 patients had a normal GFR and BP, but had increased natriuresis during VE. Only 2 of 10 patients were normotensive and had normal renal function. Disturbancies in the renal function are thus frequent in all stages of IgA GN and the changes seem to be related to the duration of the disease. Exaggerated natriuresis may indicate progressive disease.

Renal involvement in the Laurence-Moon-Biedl syndrome. Functional and radiological studies.

ABSTRACT. The renal abnormality of the Laurence‐Moon‐Biedl syndrome (LMBS) was investigated in six patients. The glomerular filtration rate (CIn) and the effective renal plasma flow (CPAH) were evaluated by standard clearance techniques and the single injection method, and the maximal concentration capacity was estimated by the ddAVP test. The kidney surface area and length were related to the body surface area, as well as the lumbar vertebrae L1‐L3, and the relationship with the GFR was studied. All six patients showed renal abnormalities: Five had small kidneys with reduced GFR and concentrating ability, and one had hyperaminoaciduria (but normal kidney size). Two patients developed terminal renal failure (one of them was successfully transplanted), illustrating the progressive character of the renal lesion. Three of the patients had had recurrent urinary tract infections, but the radiological changes of the kidneys were of the same character as in the others (symmetrical and irregular parenchymal reduction, and blunting and clubbing of the calyces). Since renal abnormalities, with considerable risk for progression to terminal renal failure, are common in the LMBS, regular urine cultures and blood pressure measurements are probably of great importance.

Incidence of microalbuminuria in children with pyelonephritic scarring

Abstract. There is experimental evidence that loss of renal parenchyma results in hyperfiltration in the remnant glomeruli followed by development of glomerulosclerosis. Microalbuminuria, i.e., a urinary albumin excretion rate of 20 – 200 μg/min, is considered to be an early predictor of diabetic glomerulosclerosis. Hypothetically, increased urinary albumin excretion in patients with pyelonephritic scarring may also indicate glomerulosclerosis, with risk for future deterioration of renal function. This study was performed to determine the incidence of increased albumin excretion in children with mild to moderate pyelonephritic scarring, and to relate the information to glomerular filtration rate (GFR; clearance of inulin) and effective renal plasma flow (clearance of para-aminohippuric acid), as well as to the degree of scarring. The functional investigations were performed under water diuresis. Fifty-seven children, aged 1.7 – 17.9 years, with pyelonephritic renal scarring were included in the study. Nine young healthy adults were used as controls. The GFR was significantly lower in the children with pyelonephritic scarring than in the controls (median 93 ml/min per 1.73 m2, range 48 – 133 vs. 111 ml/min per 1.73 m2, range 89 – 121, P<0.05), and the urine albumin excretion was significantly higher (median 20 μg/min per 100 ml GFR, range 0.8 – 170 vs. 9.2 μg/min per 100 ml GFR, range 3.3 – 21, P<0.05). An inverse correlation was found between urine albumin excretion and GFR. Increased urine albumin excretion was found in 70% of the children with a GFR below 90 ml/min per 1.73 m2 compared with 41% of the children with a GFR above this level. Increased urine albumin excretion (>20 μg/min per 100 ml GFR) was found in 51% of the children with pyelonephritic scarring, while only 14% had increased age-adjusted serum creatinine concentrations. The high incidence of microalbuminuria in children with pyelonephritic scarring indicates long-term follow-up until the ultimate outcome has been better defined.

Functional parameters and99mtechnetium-dimercaptosuccinic acid scan in acute pyelonephritis

The diagnostic value of99mtechnetium-dimercaptosuccinic acid (DMSA) scintigraphy, ultrasonography and renal functional parameters [urineN-acetyl-β-d-glucosaminidase (NAG)/creatinine and urine albumin/creatinine quotients] in acute pyelonephritis (APN) were studied in 39 children (28 girls, 11 boys, median age 9 months, range 2 weeks to 9.4 years, 28 patients <1 year, 11 patients >1 year) with first-time urinary tract infection. Ultrasonography of the urinary tract was performed on admission and together with DMSA scintigraphy (<10 days from admission). Urine NAG/creatinine and urine albumin/creatinine quotients were measured daily and after 6–8 weeks. Ultrasonography revealed abnormalities in 12 of 39 (31%) patients [11/32 patients (34%) with positive DMSA scintigraphy], while DMSA uptake defects were present in 32 of 39 (82%) patients [21/28<1 year (75%), 11/11 >1 year (100%),P=0.08]. Urine NAG/creatinine and urine albumin/creatinine quotients were significantly higher in children <1 year with APN, as well as in non-renal fever controls, than in older children. However, in both age groups the urine NAG/creatinine and urine albumin/creatinine quotients were significantly higher in APN than in non-renal fever. The urine NAG and albumin excretion decreased rapidly after the initiation of antimicrobial therapy and had normalized at 6–8 weeks. The size and grade of the DMSA uptake defect (DMSA score) did not correlate with duration of disease at admission, maximum C-reactive protein or maximum fever. The urine NAG/creatinine quotient in the children <1 year showed, however, a significant correlation with the DMSA score (r=0.58,P<0.05), while no correlation was found in the older children. We conclude that DMSA scintigraphy is a sensitive method to confirm the clinical diagnosis of APN, although a substantial number of infants appear to have normal scans. Early determination of the urine NAG/creatinine and albumin/creatinine quotients may further improve the diagnostics in the infant.

Familial Fanconi syndrome with malabsorption and galactose intolerance, normal kinase and transferase activity. A report on two siblings.

ABSTRACT. Aperia, A., Bcrgqvist, G., Linné, T. and Zetterström, R. (Department of Paediatrics, Karolinska Institute, St. Görans Childrens Hospital, Stockholm, Sweden). Familial Fanconi syndrome with malabsorption and galactose intolerance, normal kinase and transferase activity. A report on two siblings. Acta Paediatr Scand, 70:527,.–Two siblings of Turkish‐Assyrian extraction, whose parents were first cousins, had poor appetite, slow weight gain and retarded psychomotor development. When given milk the galactose concentration in blood increased. An oral galactose load showed a markedly reduced capacity to metabolize galactose. Fanconi syndrome was present as in classical galactosemia. A galactose‐free diet reduced the aminoaciduria but did not normalize the renal tubular function nor the childrens general condition. Galactokinase and galactose‐1‐phosphate uri‐dyltransferase activities in red blood cells were normal. The physical appearance of the children (sparse subcutaneous fat, thin extremities and distended abdomen) and the results of vitamin A and xylose absorption tests, were in accordance with a malabsorption condition. Glucose, however, seemed to be absorbed normally from the gut. There was no evidence of significant primary liver disease. Since the condition did not normalize with a galactose‐free diet, an enzyme defect of galactose metabolism is unlikely. Instead, a more general transport defect with autosomal recessive inheritance is proposed.

EFFECT OF METABOLIC CONTROL AND DURATION ON EXERCISE - INDUCED ALBUMINURIA IN DIABETIC TEEN-AGERS

ABSTRACT. Nineteen type I diabetic teen‐agers without clinical signs of nephropathy with a duration of diabetes varying from 3 to 16.8 years were examined by a standardized exercise test for analysis of urinary excretion of albumin and β2‐microglobulin. The patients were studied both in poor and improved (but not perfect), metabolic control as defined by HbA1 and blood glucose profiles, and the values were compared to those of 14 age‐matched healthy controls. The controls showed no increase in albumin excretion rate during excercise as was found in diabetic patients. The albumin excretion rate during exercise was significantly correlated (p<0.05) to systolic blood pressure in the diabetic patients. Blood pressure in the diabetic patients was, however, similar to that of controls both at rest and during exercise. Urinary β2‐microglobulin did not change during exercise. The urinary albumin excretion during exercise decreased significantly with improved metabolic control in diabetic patients, but the albumin excretion rate was not correlated with either blood or urinary glucose or diuresis during the exercise test. When metabolic control was unproved there was a significant correlation between the increase in albumin excretion rate during exercise and the duration of diabetes, indicating that part of the exercise‐induced albumin excretion might reflect irreversible morphological changes in the diabetic kidney. This test might therefore have a predictive value for diabetic nephropathy if performed during strict metabolic control.

Partial Remission of Nephrotic Syndrome during Whooping Cough

The pathogenesis of the nephrotic syndrome of childhood is not fully understood, although there is evidence which suggests an underlying immunological disturbance (1, 2). Steroid treatment induces remission in most patients and treatment with cytostatics may give prolonged remission. During relapse, an increased number of suppressor T-cells have been found. Measles infection, which gives a T-cell lymphocytopenia, but no change in the ratio between T-helper and T-suppressorkytotoxic cells (3) , may also induce remission of the nephrotic syndrome (4). We here report a boy who had a partial remission of a nephrotic syndrome while he had whooping cough. A proportional increase in both T and B lymphocytes has been shown to occur during this disease (5).

A prospective psychological and cytogenetic study of three girls with mosaic mongolism.

Abstract. Three girls with mosaic mongolism (46, XX/47, XX, +21) were followed cytogenetically and psychologically from the time of diagnosis at 8, 17 and 32 months of age, respectively, to the age of 13–15.5 years. All showed muscular hypotonia and hyperflexible joints at the time of diagnosis, but otherwise the physical characteristics of Downs syndrome were weakly expressed. The percentage of trisomic cells in the peripheral blood decreased with time, but were still higher in lymphocytes than in skin fibroblasts at the last investigation. Developmental milestones were delayed in all cases, and the developmental and intelligence quotients were decreased. Mental retardation was only slight in one of the cases (I.Q. =65 at the age of 14 years 8 months). The intelligence quotients showed declining trends with time. Social ability and school results tended to be better than could be expected from the test results.