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Biomedical subjects

N E Moghal

Publications and source records attributed to N E Moghal.

18 recordsLinked to original sources

A prospective, randomized, multicenter trial of tacrolimus-based therapy with or without basiliximab in pediatric renal transplantation.

In a 6-month, multicenter, randomized, controlled, open-label, parallel-group trial, we investigated the efficacy and safety of adding basiliximab to a standard tacrolimus-based regimen in pediatric renal transplant recipients. Patients < 18 years received tacrolimus/azathioprine/steroids (TAS, n = 93) or tacrolimus/azathioprine/steroids/basiliximab (TAS + B, n = 99). Target tacrolimus levels were 10-20 ng/mL between days 0-21 and 5-15 ng/mL thereafter. Steroid dosing was identical in both groups. Basiliximab was administered at 10 mg (patients < 40 kg) or 20 mg (patients > or = 40 kg) within 4 h of reperfusion; the same dose was repeated on day 4. Biopsy-proven acute rejection rates were 20.4% (TAS) and 19.2% (TAS + B); steroid-resistant acute rejection rates were 3.2% and 3.0%, respectively. Patient survival was 100%; graft survival rates were 95% in both arms. The nature and incidence of adverse events were similar in both arms except toxic nephropathy and abdominal pain, which were significantly higher in the TAS + B arm (14.1% vs. 4.3%; p = 0.03 and 11.1% vs. 2.2%; p = 0.02; respectively). Median serum creatinine concentrations at 6 months were 86 micromol/L in the TAS and 91 micromol/L in the TAS + B arm; glomerular filtration rate was 79.4 and 77.6 (mL/min/1.73 m2), respectively. Adding basiliximab to a tacrolimus-based regimen is safe in pediatric patients, but does not improve clinical efficacy.

Adolescent↗

Selective renal embolisation for renovascular hypertension?

An 11 year old girl developed hypertensive encephalopathy and renal failure from reflux nephropathy. Resection of her shrunken left kidney did not control her hypertension. Two selective arterial embolisations of the scarred right lower pole produced only transient benefit, but a heminephrectomy gave good control. Embolisation may delay definitive treatment.

Child↗

Multicystic dysplastic kidney and Kallmann's syndrome: a new association?

BACKGROUND: Kallmann's syndrome is characterized by anosmia and hypogonadotrophic hypogonadism. Radiographic studies of teenagers and older subjects with the X-linked form of the syndrome have shown that up to 40% have an absent kidney unilaterally. Although this has been attributed to renal "agenesis", a condition in which the kidney fails to form, little is known about the appearance of the developing urinary tract either pre- or post-natally in individuals with Kallmann's syndrome. METHODS: We describe two brothers who had features of Kallmann's syndrome, most probably of the X-linked variety, who both had a major urinary-tract malformation detected before birth. RESULTS: The brothers were found to have unilateral multicystic dysplastic kidneys on routine antenatal ultrasound scanning and both underwent surgical nephrectomy of these organs post-natally. Immunohistochemical studies on the younger sibling revealed hyperproliferative dysplastic kidney tubules which overexpressed PAX2, a potentially oncogenic transcription factor, and BCL2, a cell-survival factor, surrounded by metaplastic, alpha smooth-muscle actin-positive stroma: similar patterns have been observed in patients with non-syndromic multicystic dysplastic kidneys. CONCLUSIONS: Our results describe a new type of urinary-tract malformation associated with Kallmann's syndrome. However, since multicystic kidneys tend to involute, only when more Kallmann's syndrome patients are screened in utero or in early childhood using structural renal scans, will it be possible to establish whether multicystic kidney disease is a bona-fide part of the syndrome.

Child, Preschool↗

Hypertension in Henoch-Schönlein purpura with minimal urinary findings.

Though hypertension is a common feature of Henoch: Schönlein purpura (HSP) acute nephritis, it is seen only rarely and transiently if renal function is normal and abnormal urinary findings are minimal. We report a 3.4 year old girl who had the typical arthritis and rash of HSP, but also had significant hypertension with only minimal urinary findings. Imaging investigations revealed one normal kidney, and one that was almost destroyed from previous reflux nephropathy; her hypertension resolved after unilateral nephrectomy. Other causes of hypertension should be excluded in children with HSP and minimal urinary findings.

Child, Preschool↗

Renal function after pediatric cardiac transplantation: the effect of early cyclosporin dosage.

BACKGROUND: There is little data on renal function in pediatric heart transplant recipients. Early rejection is a major concern and most units run high cyclosporin A (CyA) levels during the 2 to 3 months after transplantation. We sought to document long-term renal function after transplantation and to assess influence of early CyA levels. METHODS: We reviewed all of our pediatric transplants between June 1985 and August 1998 who survived longer than 6 months (n = 54). Glomerular filtration rate (GFR) was estimated at 1, 2, 4, and 8 years posttransplantation using the Schwartz formula: GFR (mL/min/1.73m(2)) = [Ht(cm)/creatinine(micromol/L)] x X We also analyzed whether change in renal function correlated with trough CyA levels. RESULTS: Median age at transplant was 4 years and median follow-up was 5 years. Survival rates were 87% at 1 year and 80% at 5 years. Mean GFR pretransplant was 79 +/- 19 mL/min/1.73 m(2), reflecting prerenal impairment. One year later, mean GFR was 72 mL/min/1.73 m(2); after 2 years it was 65 mL/min/1.73 m(2), after 4 years (n = 35) it was 60 mL/min/1.73 m(2), and after 8 years (n = 14) it was 57 mL/min/1.73 m(2). CyA levels during the first 2 months correlated with the change in GFR during the first year (r(2) = 0.21). CONCLUSIONS: This study demonstrates for the first time that decline in renal function after heart transplantation correlates with early CyA exposure; this dysfunction persists even when CyA doses are subsequently reduced.

Child↗

Coexistence of thin membrane and alport nephropathies in families with haematuria.

The finding of familial haematuria without a history of deafness or renal impairment is often assumed to indicate a benign prognosis. However, we describe three families in whom Alport and thin basement membrane nephropathy were separately identified within the same pedigree. Our findings illustrate the importance of fully investigating families with haematuria, even if thin basement nephropathy has been diagnosed in one member.

Basement Membrane↗

The impact of renal replacement therapy on toddler time.

The impact of renal replacement therapy on the day-to-day activity of 5 toddlers is presented. For those on hemodialysis, 50% of the waking hours involved activity related to management of the renal failure; the remainder was spent feeding or in normal (play) activity. Peritoneal dialysis demanded only 10% of waking hours for direct renal activity. The data are potentially useful in counseling families when discussing the impact of renal failure and its management on the toddler.

Activities of Daily Living↗

Treatment of neutropenia in a renal transplant recipient with granulocyte colony-stimulating factor.

We report the use of granulocyte colony-stimulating factor (G-CSF) in a renal transplant recipient in whom neutropenia developed following a presumed viral infection. G-CSF was successful in producing a rise in neutrophil count which coincided with a resolution of fever; there was no adverse effect on renal function. This is the first use of G-CSF in a paediatric renal transplant recipient, and its use should be considered for the immunosuppressed child with persistent neutropenia.

Granulocyte Colony-Stimulating Factor↗

The late histologic findings in diarrhea-associated hemolytic uremic syndrome.

Kidney biopsies were undertaken for persisting proteinuria 3.3 to 7 years (mean 5.4 years) from the onset of diarrhea-associated hemolytic uremic syndrome (D + HUS) in 5 boys and 2 girls (age at presentation mean 3.2 years, range 1.0 to 9.7 years). At 1 year the mean early morning urine protein/creatinine ratio was 100 mg/mmol, and the mean glomerular filtration rate was 65 mL/min/1.73 m2. At 5 years the mean early morning urine protein/creatinine ratio was 81 mg/mmol, and the mean glomerular filtration rate was 73 mL/min/1.73 m2. The biopsy specimens were compared with those of 7 age- and sex-matched children who were investigated for isolated persistent microscopic hematuria but in whom no abnormality was detected. Global glomerulosclerosis was noted in 6 patients with D + HUS, and 2 of these had segmental sclerosing lesions. Tubular atrophy and interstitial scarring were seen in all but 1 patients. The glomeruli in the D + HUS group were significantly larger than in the control group (P < .01). These findings are typically found in kidneys with reduced nephron numbers and are compatible with changes of hyperperfusion and hyperfiltration in surviving nephrons. Long-term follow-up of patients with D + HUS and proteinuria is advisable.

Biopsy↗

A review of acute renal failure in children: incidence, etiology and outcome.

We reviewed our experience of children with acute renal failure. St James's University Hospital, Leeds, UK is a tertiary referral center that serves a relatively stable regional population (former Yorkshire region). It is a mixed rural and urban population providing a unique profile of the nature of the cases and workload experienced. The data is expressed as a function of age and compared against a previous era of paediatric nephrology and current adult incidence data. Over an 8-year period (1984-1991) 227 children were referred for dialysis management of acute renal failure. The yearly incidence was 0.8 per 100,000 total population. Acute renal failure in the child population was almost a fifth of the adult incidence. Age-related incidence however shows the highest incidence in the neonate/infant population and is comparable to adult data. The intensive care unit was needed for nearly half the children. For all ages hemolytic uremic syndrome was the commonest cause (45%). Surgery for congenital heart disease was predominant (63%) in the neonate group. The overall mortality was 25%. Primary renal disease accounts for only 7% of the etiologies and was the source for the majority that went on to require chronic renal replacement therapy. Acute renal failure is nearly always a secondary event in the face of other organ failure and the majority of the mortality arises from surgery for congenital heart disease. If the underlying condition is treatable, the prognosis for recovery from acute renal failure with appropriate supportive care is excellent.

Acute Kidney Injury↗

Care in the use of ibuprofen as an antipyretic in children.

Ibuprofen is being widely used as an antipyretic in children. Recent studies indicate that it is as efficacious and with no significant difference in side-effects when compared to paracetamol. We describe three cases that illustrate that renal complications can occur when ibuprofen is prescribed in the presence of intravascular volume depletion and/or pre-existing renal problems. We discuss the mode of action of ibuprofen and recommend that its use as an antiypretic in children should be avoided in actual or potential intravascular volume contraction and in cases with pre-existing renal problems.

Analgesics, Non-Narcotic↗

The prevalence and treatment of end-stage renal disease in an Asian child population.

BACKGROUND: There are significant differences in the incidence and aetiology of end-stage renal disease (ESRD) between the Asian and white adult population in the UK. The aim of this study was to determine if similar differences occurred in the paediatric ESRD population. METHODS: A retrospective study of children with ESRD presenting between 1980 and 1995 in the population served by the Birmingham Children's Hospital. RESULTS: Asian children comprised 7.4% of the total child population (0-15 years). ESRD developed in 165 children (138 white, 27 Asian). The prevalence of ESRD for whites was 15 per 10(5) white child population and for Asians, 40 per 10(5) Asian child population. A genetic aetiology was noted in 26 (19%) whites and 12 (44%) Asians (P < 0.001). Of the 147 renal transplants, 22 (15%) were to Asian recipients. The distribution of blood groups in the two populations reflected the pattern in the respective general populations as a whole. There was no significant difference in time to transplantation for the two groups (whites, mean 6 months, 95% confidence interval 6-11 months; Asians, mean 7 months, 95% CI 4-12 months). Asian patients had significantly more mismatches (> or = 3 or > or = 4) compared to white patients. CONCLUSIONS: Asian children had a higher prevalence of ESRD, with genetic disease predominating. Differences in ethnicity or blood group did not influence time to transplantation in those that received a transplant although Asians had more mismatches.

Child↗

A study of sexual abuse in an Asian community.

Sexual abuse in Asian ethnic minority populations in the UK has not previously been investigated. There remain professionals as well as lay people who deny its existence on the basis of assumptions about culture and religion. This retrospective study highlights the existence and difference in the incidence, pattern of presentation, and management in cases of Asian sexual abuse compared with that reported on the indigenous population.

Adolescent↗

Neonatal aortic arch thrombosis.

An infant presented shortly after birth with signs suggestive of aortic coarctation. Echocardiography revealed an extensive aortic arch thrombus, not amenable to surgery. Thrombolytic agents reduced thrombus size, enabling survival, but failed to prevent neurological damage secondary to cerebral embolisation.

Aorta, Thoracic↗

Intraventricular haemorrhage after aspiration of ventricular reservoirs.

A previously unrecognised complication of aspirating ventricular reservoirs is described. Four infants developed fresh bleeding into the cerebrospinal fluid after reservoir taps; ultrasound confirmed intraventricular blood clot in one case. The technique for aspirating the reservoir may have an important bearing on the incidence of this complication.

Cerebral Hemorrhage↗