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Biomedical subjects

T J Neuhaus

Publications and source records attributed to T J Neuhaus.

34 records · Page 2Linked to original sources
PubMed1997

Heterogeneity of atypical haemolytic uraemic syndromes.

Atypical, non-diarrhoea associated haemolytic uraemic syndrome (D-HUS) is a heterogeneous disorder with a generally poor outcome, although this view has now been questioned. The clinical and laboratory features of 23 children with D-HUS, representing a third of all patients with HUS seen during the last 26 years, were examined. The median age was 4.9 years (range 3 days-13.8 years). Twenty one children (91%) survived the initial phase. All patients except six infants aged < 18 months required dialysis (74%). Hypertension (43%), cardiomyopathy (43%), and cerebral convulsions (48%) were common. Nineteen (83%) children were followed up for a median period of 5.5 years (range 0.5-23.4). Only five (26%) patients, among them four infants, recovered completely. Six (32%) patients had one to 10 recurrences, including two siblings with neonatal onset, and eight (42%) developed end stage renal failure. Five children underwent cadaveric renal transplantation, with recurrence and subsequent graft failure in two. Four children died, resulting in an overall mortality of 26%. Atypical HUS is heterogeneous with regard to epidemiology, pathophysiology, and outcome. Children with a recurrent, familial, or neonatal course have worse outcomes; in contrast, infants not requiring dialysis in the acute phase have a better prognosis.

Acute Disease↗
PubMed1997

Pyelonephritis and vesicoureteral reflux after renal transplantation in young children.

PURPOSE: We assessed morbidity and risk factors of pyelonephritis in children after renal transplantation. MATERIALS AND METHODS: Between 1986 and 1995, 41 children underwent transplantation and all who had documented pyelonephritis were evaluated. RESULTS: Six children who underwent transplantation before age 7 years had 1 to 3 episodes of pyelonephritis with significant renal dysfunction and vesicoureteral reflux into the grafted system. An antireflux reimplantation procedure in 5 children was complicated by temporary functional obstruction in 3. No further infection occurred after correction of vesicoureteral reflux. After a median of 4.5 years post-transplantation all patients have a functioning graft. CONCLUSIONS: After renal transplantation vesicoureteral reflux and young recipient age are major risk factors for pyelonephritis with subsequent graft dysfunction.

Adolescent↗
PubMed1997

Salivary excretion of endogenous proteins in nephrotic syndrome in children.

Size and charge selectivity of capillary permeability in the salivary glands of nephrotic children were investigated by measuring salivary excretion of endogenous plasma proteins of different size and charge. We examined 10 children with steroid-sensitive nephrotic syndrome (SSNS) in relapse and subsequent remission, 11 with steroid-resistant nephrotic syndrome, and 11 healthy children (controls). Albumin [mol. wt. 66 kilodaltons (kDa), isoelectric point (pI) 4.9] was measured by radio-immunoassay, transferrin (mol. wt. 77 kDa, pI 5.9) and immunoglobulins IgG1 (mol. wt. 150 kDa, pI 7-9) and IgG4 (mol. wt. 150 kDa, pI < 6) by enzyme-linked immunoabsorbent assay. In saliva, no significant differences were found between the four groups of children for any of the four proteins. Also, the saliva/plasma ratios of the four proteins were not different among the four groups. From these data, we conclude that in subjects with SSNS in relapse, neither size nor charge selectivity of salivary gland capillary permeability are affected.

Adolescent↗
PubMed1996

[25 years of dialysis and kidney transplantation in children and adolescents].

Between 1970 and 1994 84 children and adolescents with end stage renal failure (ESRF) were started on renal replacement therapy (RRT). Renal transplantation was the main goal from the beginning. The long term results were evaluated with emphasis on survival, development and social integration. RRT was started in nearly half of the children (45%) between the age of 10 and 15 years and in 14% before 5 years. 52 patients were first treated by hemodialysis (HD) and 27 by peritoneal dialysis (PD, since 1979). 5 patients underwent preemptive transplantation. By December 1994, 75 patients had received 99 grafts, i.e. 75 1st, 21 2nd and 3 3rd grafts; 3 kidneys were from living related donors and 8 patients were transplanted elsewhere. 7 patients were still on dialysis and 2 had died before transplantation. Actuarial patient survival (Kaplan-Meier) after start of RRT is 88% at 10 years and 75% at 17-25 years. Actuarial patient survival after first transplantation increased from 91% (1970-1984) at 5 years to 97% (1985-1994). 7 of the first 10 patients transplanted from 1970-1974 are alive, all with functioning grafts (4 with their first graft). 9 patients died after transplantation: 4 of recurrent disease, 2 of viral (CMV, EBV) infections and 1 each of spinalioma, allergic shock and traffic accident. First graft survival was 37% at 10 years. It increased from 53% (1970-1984) to 72% (1985-1994) at 5 years. The main causes of first graft loss (n = 33) were irreversible rejection (21) and recurrent disease (7). All patients aged > 22 years were further evaluated: patients with start of RRT 1970-1979 (group A, n = 18) were compared with those starting RRT from 1980-1987 (B, n = 19). Mean adult height in A was less than in B (163.9 cm vs 168.5 cm in men; 146.3 cm vs 156.5 cm in women). 50% in A vs 32% in B had a disability. Fewer patients in A (39%) than in B (62%) were fully employed. Considerably more patients in A (61%) than in B (37%) lived with their parents or siblings although the mean age in A was 31 years vs 25 years in B. 3 women were married (all in B), 2 of them gave birth to 3 healthy children and the third was pregnant. Long term patient and graft survival, somatic development and social integration have improved over the years due to a variety of factors. A comprehensive approach is necessary in treating children with ESRF.

Adolescent↗
PubMed1996

Renal-hepatic-pancreatic dysplasia: an autosomal recessive disorder with renal and hepatic failure.

UNLABELLED: We report two brothers with renal dysplasia and congenital hepatic fibrosis. One patient died shortly after birth of lung hypoplasia. The second developed end-stage renal failure at 14 months. The hepatic fibrosis progressed to cirrhosis and hepatic failure. Pancreatic function was normal, but increased echogenicity was seen on ultrasound. At age 3 years and 9 months a successful combined liver-kidney transplantation was performed. The features of our patients are compatible with the "renal-hepatic-pancreatic dysplasia" syndrome. CONCLUSION: Renal-hepatic-pancreatic dysplasia is an autosomal recessive disorder with variable expression. Combined liver-kidney transplantation offers a new therapeutic option.

Abnormalities, Multiple↗
PubMed1995

Increased IL-2, IL-4 and interferon-gamma (IFN-gamma) in steroid-sensitive nephrotic syndrome.

We investigated the production of cytokines by peripheral blood mononuclear cells (PBMC) and serum cytokine concentrations in children with steroid-sensitive idiopathic nephrotic syndrome (SSNS). PBMC from patients off treatment were collected during remission and relapse and cultured in medium alone or stimulated with calcium ionophore plus phorbol myristate acetate. Control PBMC were taken from healthy age-matched children. IL-2 was measured by bioassay, IL-4 by immunoradiometric assay, and IL-8 and IFN-gamma by ELISA. After 24 h culture without stimulation, IL-2, IL-4 and IFN-gamma were not detectable in the supernatant in any of the children. After stimulation, the supernatant concentrations of IL-2 (median 172 U/ml at 24 h) and IL-4 (160 pg/ml at 24 h; 210 pg/ml at 72 h) were significantly increased in relapse compared with remission (IL-2 37 U/ml; IL-4 65 pg/ml and 60 pg/ml) and controls (IL-2 69 U/ml; IL-4 40 pg/ml and 40 pg/ml) (P < 0.05). The concentration of IFN-gamma was not significantly increased in relapse compared with remission and controls (600, 325, and 145 U/ml, respectively, at 72 h). IL-8 concentrations were similar in relapse, remission and controls with stimulation (median 32, 40 and 40 ng/ml, respectively) and without (30, 17 and 10 ng/ml). IL-2 was not detectable in serum, but IL-4, IL-8 and IFN-gamma were measurable in about half the patients, both in relapse and remission, though were virtually undetectable in controls. We conclude that relapse of SSNS in children is associated with T lymphocyte activation with release of IL-2, IL-4 and IFN-gamma.

Adolescent↗
PubMed1995

T-lymphocyte activation in steroid-sensitive nephrotic syndrome in childhood.

We undertook a sequential study in 29 children with steroid-sensitive nephrotic syndrome (SSNS) off treatment to seek evidence for T-cell activation in relapse. T-cell subsets and activation markers were analysed using two-colour flow cytometry. Soluble IL2 receptor (sIL2R) was measured in serum and urine by enzyme-linked immunosorbent assay (ELISA). Fifteen children were examined in remission and subsequent relapse (group A) and fourteen remained in remission (group B). In group A the proportion of CD4+ cells expressing the activation marker CD25 (alpha-chain of the IL2 receptor) increased significantly from remission to relapse: CD4+25+ cells rose from 5.6 to 7.0% of total lymphocytes, and from 15.8 to 19.1% of CD4+ lymphocytes (paired t test: P < 0.0005 and < 0.001 respectively). No correlations were found between CD4+25+ cells and plasma albumin or cholesterol concentrations. SIL2R concentration in serum did not change in relapse, but increased significantly in urine from 272 to 592 U/mg creatinine (P < 0.01). No significant difference was found in remission between groups A and B. We conclude that early relapse in SSNS is associated with activation of CD4+ (T-helper) cells which is not secondary due to the nephrotic state itself.

Adolescent↗
PubMed1994

Long-term cyclosporin A treatment of minimal-change nephrotic syndrome of childhood.

We evaluated the efficacy of long-term cyclosporin A (CyA) treatment in the maintenance of remission in 40 children with steroid-dependent minimal-change nephrotic syndrome (MCNS). CyA was given in an initial dose of 5 mg/kg per day, adjusted to maintain a trough whole blood level of 50-150 ng/ml. All the 40 children received CyA for 1 year. In 18 patients, CyA was continued for a further period of at least a year without interruption; 9 patients had a second course of CyA therapy after an interval of at least 1 month. Of the 40 children 29 (72%) had one or more relapses during treatment with CyA, with 16 (40%) relapsing during the 1st year. During the second period of CyA, 10 (56%) of the 18 children treated continuously relapsed, whereas all the 9 children who had an interrupted course of therapy relapsed. CyA was discontinued at one time in 27 patients, all of whom subsequently relapsed, with a median time to relapse of 26 days. Long-term prednisolone in addition to CyA was required to maintain remission in 16 (40%) of the whole group. The results suggest that the long-term use of CyA is able to maintain remission of MCNS, although 40% of the patients also required low-dose alternate-day steroids; patients appeared to fare worse if the CyA course was interrupted; no patient experienced a long-term remission after CyA was stopped.

Adolescent↗
PubMed1994

Alternative treatment to corticosteroids in steroid sensitive idiopathic nephrotic syndrome.

A review was undertaken of the use of alternative immunosuppressive treatment in addition to corticosteroids in a cohort of 429 children with steroid sensitive nephrotic syndrome (SSNS) treated between 1980 and 1994. Two hundred and twenty two children (52%) received at least one course of alternative treatment, 98 (23%) two, and 43 (10%) three. Cyclophosphamide was administered to 196 children (46%); in 181 it was the first course of alternative treatment and in 104 (57%) of those it was also the last ('final course'). Levamisole was given to 56 children (13%) and cyclosporin to 53 (12%). Fifteen children in whom cyclosporin failed were treated with chlorambucil. A few patients received azathioprine or vincristine. Ten children developed secondary steroid resistance, of whom five progressed to chronic renal failure. Acute complications included reversible renal failure, septicaemia, peritonitis, convulsions, and cerebral thrombosis. There were three deaths. It is concluded that half of the referred children with SSNS were deemed to require at least one course of alternative immunosuppressive treatment, and that side effects of the treatment and complications of SSNS are infrequent but occasionally fatal.

Adrenal Cortex Hormones↗
PubMed1992

[New faces, forgotten diseases:border medical examination of asylum seekers' children 1990-1991].

Hitherto there has been no epidemiologic basis for the extent of initial medical examination (IME) of children of refugees. In Switzerland little data is published on the incidence of diseases among refugees; in particular information on their children is scarce. We report the results of IME in these children in the Canton of Zürich from 1 July 1990 to 30 June 1991. The Federal Refugees Office assigned 1487 children to the Canton of Zürich. 920 children (61.9%) were registered, 259 (17.4%) at Zürich Children's Hospital and 661 with local physicians (44.5%). The current IME included a tuberculin skin test only, with additional hepatitis B screening of children from high risk countries. At the Zürich Children's Hospital the IME was extended: every child was examined clinically and a history was taken. The findings in the children examined at the Zürich Children's Hospital were as follows: 171 (66%) were healthy. 5 children (2%) had tuberculosis, 2 (0.8%) vitamin D deficiency rickets, 5 (2%) had iron deficiency anemia, 9 had hepatitis B (all recovered), 25 (9.7%) had various skin diseases and in 51 a variety of diseases of differing clinical significance were diagnosed. The local physicians found a similar incidence of tuberculosis, vitamin D deficiency rickets, iron deficiency anemia and skin diseases.(ABSTRACT TRUNCATED AT 250 WORDS)

Anemia, Hypochromic↗
PubMed1992

Long-term low-dose cyclosporin A in steroid dependent nephrotic syndrome of childhood.

Therapy of steroid-dependent idiopathic nephrotic syndrome is often unsatisfactory. Since 1986 we have treated nine children (six male and three female), aged 3-16 years, with cyclosporin A (CsA) during 2.0-5.2 (median 3.1) years. All had minimal change disease on renal biopsy and had previously received cyclophosphamide. Mean daily dosage of CsA was 4.1 mg/kg (range 2.7-5.8) and mean whole blood trough level was 220 ng/ml (range 141-271). The relapse rate decreased from 3.4/patient year before CsA to 0.55 on CsA. Discontinuation of CsA or reduction below 2 mg/kg daily was always followed by a relapse. The overall relapse rate, including the period with very low-dose CsA, was 0.95/patient year. Four patients required additional low-dose alternate-day prednisone. Repeat renal biopsy showed minimal change disease in eight patients and focal segmental glomerulosclerosis in one; CsA-toxicity was mild in two and moderate in one. The latter was the only patient with slightly reduced glomerular filtration rate. Two boys with delayed puberty spontaneously matured and reached expected final height. We conclude that long-term low-dose CsA is very effective and steroid-sparing. Its use is justified in selected patients, particularly in those with numerous relapses and in male patients before and during puberty, as long as renal function and CsA-toxicity are carefully monitored.

Adolescent↗
PubMed1991

Epoetin alfa in anaemic children or adolescents on regular dialysis.

Eighteen patients aged 5-18 years on regular dialysis had a packed cell volume (PCV) less than 0.27. On treatment with epoetin alfa (EA) PCV increased by 0.05 or more in all patients. Iron supplementation was necessary in 13 patients with a ferritinaemia less than 300 micrograms/l before study. During treatment, plasma potassium increased significantly and more vigorous antihypertensive measures were required in 8 patients, 5 of them being already on antihypertensive drugs before EA. Iliofemoral thrombosis occurred in 1 patient 10 days after renal transplant. The data indicate that EA ameliorates the anaemia of chronic renal disease. The main concerns arising during treatment with EA are hyperkalaemia, arterial hypertension and possibly thrombosis.

Adolescent↗
PubMed1986

Morphology of original and transplanted thymuses in severe combined immunodeficiency.

Twenty-six patients with severe combined immunodeficiency (SCID) were examined. In 20 cases no defect of the biochemical pathways was found; 6 cases showed a deficiency in adenosine deaminase (ADA) activity. In 19 cases histological sections of the thymus were available. In 3 cases, in addition to the original thymuses, transplanted thymic allografts were microscopically examined. The thymus in SCID without abnormality of the ADA pathway showed a uniform dysplastic pattern with only moderate variations related to mode of inheritance and length of survival. The thymus in SCID with ADA deficiency displayed a heterogeneous pattern ranging from almost normal to a completely dysplastic structure, whereas the transplanted thymic allografts presented either a normal or a dysplastic appearance. The morphology of the thymus is not pathognomonic of any given biochemical defect, clinical course, or type of SCID. SCID with apparently normal biochemical pathways probably results from a variety of pathogenetic mechanisms.

Adenosine Deaminase↗