Cerebral venous thrombosis in the nephrotic syndrome.

A 4-year-old boy with idiopathic steroid responsive nephritic syndrome developed bilateral sixth-nerve palsy and lethargy secondary to cerebral sinus thrombosis. Treatment with heparin, fresh frozen plasma as source of antithrombin III and vitamin K inhibitors may have prevented further sequels. However, anti-coagulation, as assessed by partial thromboplastin and prothrombin time, was difficult to achieve. Despite these problems the child made a complete neurological recovery.

Osteopetrosis in children.

Over a 10-year period, 28 Arab children with autosomal recessive osteopetrosis were seen in two hospitals in Riyadh, Saudi Arabia. Eighteen (64%) had osteopetrosis associated with metabolic acidosis probably due to a renal tubular defect; nine (32%) had a malignant infantile form of osteopetrosis and one had a mild form with delayed onset. Parental consanguinity was 56% and 40% among patients with and without acidosis respectively. Somatic and psychomotor retardation and recurrent bone fractures were common in both groups. Dental caries, cerebral calcification and optic atrophy were more frequent in patients with acidosis, while anaemia, hepatosplenomegaly and deafness were more common in patients without acidosis. To guarantee optimal rehabilitation, children with this progressive disease require an early multiteam approach.

The impact of renal biopsy in the clinical management of childhood renal disease.

This study was carried out to determine whether renal biopsy altered patient management or expected prognosis among children with renal disease. A retrospective analysis of case records of 106 children with renal disease having adequate renal biopsies was made. The diagnosis was altered in 47.2% and therapeutic approach was changed in 33% of the patients after obtaining the renal biopsy findings. Also, the estimate of prognosis was altered in 31.1% of the cases. These results were greater for patients with steroid resistant and congenital nephrotic syndrome, acute renal failure, asymptomatic urinary abnormalities and mixed nephritic-nephrotic syndrome. Subsequent management and prognosis was least affected by the biopsy results in patients with steroid sensitive nephrotic syndrome and chronic renal failure.

Acrorenal syndrome associated with visual defect.

The clinical biochemical, radiological, and histological data of a 5-year-old boy with severe limb deformities and renal failure due to oligomeganephronia and renal hypoplasia are reported. This patient represents another example of acrorenal syndrome. This boy has a severe visual defect due to pigmentory retinopathy, which has not been reported previously.

Coagulopathy of childhood nephrotic syndrome--a reappraisal of the role of natural anticoagulants and fibrinolysis.

In an attempt to characterise further the coagulopathy of childhood nephrotic syndrome, this study concentrates on simultaneous measurements of the natural anticoagulants [antithrombin III (ATIII), proteins C and S] and the fibrinolytic factors, tissue plasminogen activator (tPA) and plasminogen activator inhibitor (PAI). The study groups consisted of 41 children (ages ranging from 2 to 14 years; median 7.1) in the relapse of nephrosis and 48 children (ages ranging from 3 to 14 years; median 7.6) in remission. The results obtained were compared with normal values obtained in healthy age- and sex-matched controls (n = 103). During relapse, there was a marked increase in the plasma level of fibrinogen, protein C, and protein S and reduced plasma ATIII level; tPA level was similar to control but PAI level exhibited a significant reduction. During remission, the protein C level either remained elevated or increased further, but some decreased. Protein S and plasma ATIII level normalised. The fibrinolytic activator tPA dropped slightly but the PAI level remained significantly below control levels. We conclude that in the relapse of childhood nephrosis, despite the existence of a significant prothrombotic tendency as featured by hyperfibrinogenaemia and markedly reduced ATIII level, the simultaneous elevation of the natural anticoagulant, protein C level and enhanced fibrinolysis that persist until the remission phase, seem to be major preventive mechanisms guarding nephrotic children against thromboembolic phenomena.

Normal levels of the natural anticoagulants (proteins C & S and antithrombin III) and the fibrinolytic factors (tPA and PAI) in Arab children.

The diagnosis of many hemostatic defects in infancy and childhood depends on the establishment of normal levels of various hemostatic factors. In this study, measurements of the natural anticoagulants (proteins C, S, and antithrombin III), as well as the fibrinolytic factors (tPA and PAI) were undertaken in healthy neonates (cord blood; n = 56), as well as in healthy children, up to 12 years of age (n = 103). The results were compared to normal adult values obtained from blood donors (n = 49). Neonatal values were found to be 50% of those obtained in adults and their mean concentrations were as follows: ATIII antigen = 48.4%, ATIII activity = 61.6%, protein C antigen = 47.7%, protein C activity = 57.2%, total protein S = 41.8% and tPA = 1.9 ng/mL. PAI level (25.7 ng/mL) was similar to adult values. In the first three years of life, almost all the hemostatic factors, other than PAI, gained adults levels. The diminished concentrations of the natural anticoagulants, in addition to the hypofibrinolysis in neonates, shifts the hemostatic balance towards fibrin formation and safeguards effective hemostasis. The values obtained in this study may serve as local reference values.

Renal sonographic patterns in Bartter's syndrome.

The renal sonographic findings in ten cases of Bartter s syndrome investigated at the King Khalid University Hospital, Riyadh, Saudi Arabia are described. There were various sonographic abnormalities other than those of hyperechoic pyramids as previously described. These were diffuse increased renal echogenicity and hyperechoic echogenicity in the kidneys with the exception of the pyramids. This condition can be suspected early if nephrocalcinosis is present in a child with a history of polyhydramnios and premature delivery.

Childhood renal diseases in Saudi Arabia. A clinicopathological study of 167 cases.

Between April 1982 and September 1994, 167 renal biopsies were performed in 167 children at King Khalid University Hospital, Riyadh, Saudi Arabia. The data were analysed to show a correlation between clinical presentation and histological findings. Nephrotic syndrome was the most common indication for renal biopsy, accounting for 77% of all cases. Of these, 23.3% showed minimal change lesions, 24% showed mesangial proliferative glomerulonephritis and 24% showed focal segmental glomerulosclerosis. We noted a higher incidence of congenital nephrotic syndrome and Alport's syndrome as compared with the West. On the other hand, IgA nephropathy was less common (3%), and there was a complete absence of type II membranoproliferative glomerulonephritis.

Acute viral hepatitis, glucose-6-phosphate dehydrogenase deficiency and prolonged acute renal failure: a case report.

A 10-year-old boy with glucose-6-phosphate dehydrogenase deficiency developed acute renal failure during the icteric phase of non-fulminant hepatitis A infection. He needed peritoneal dialysis for 54 days. Acute tubular necrosis was confirmed by percutaneous renal biopsy. He had complete recovery of his renal function when he was discharged.

The aetiological and clinical pattern of childhood urolithiasis in Saudi Arabia.

The clinical and aetiological pattern in 85 stone-forming children presenting to an integrated nephrourological service in Riyadh is reviewed. All patients were below the age of 15 years, the male to female ratio being 2:1. Only 2 children presented with bladder calculi. The remaining all had upper tract stones and, in 12 cases, these were bilateral. Of 34 calculi recovered for analysis, one-third was predominantly calcium oxalate and a further third was composed of uric acid or urate. Four patients had cysteine stones and the remaining 7 presented mixed calcium stones, 6 (17.6%) being struvite and infection-related. Of the 85 patients 55 were treated successfully with extracorporeal shock wave lithotripsy, 16 underwent surgery and 7 had their stones removed by endourological procedures. In the remaining 7 children, stones dissolved or were passed spontaneously during medical therapy. Nine children (10.6%) showed a primary metabolic defect leading to their stone formation, 10 (11.8%) had a predisposing anatomical anomaly and 15 (17.6%) presented with urinary tract infection. Of the remaining 51 patients (60%) with idiopathic disease, 6 showed hypercalciuria on investigation and 2 children may have formed their stones due to prolonged recumbency.

Platelet Aggregometry - Dose-related Responses to Arachidonic Acid in Childhood Nephrotic Syndrome.

A recent unexpected finding of inhibited platelet aggregation in response to a single (1.64 mmol/l) dose of arachidonic acid (AA), during the relapse of childhood nephrosis, prompted us to assess aggregation in response to multiple doses of AA: 1.64,0.82,0.41,0.20 mmol/l, in two groups of children, in the relapse (n = 34) or remission (n=41) phase of nephrotic syndrome. During relapse: the highest dose of AA (1.64 mmol/l) evoked reversible and inhibited aggregation in 91% of patients. However, at the lower doses there were enhanced responses as measured by both maximum aggregation (%) and slopes of the aggregation curves. In contrast, during remission, irreversible aggregation was obtained at the highest AA dose, while at the lowest two doses (0.41 and 0.20 mmol/l), no aggregation responses were obtained in 4 (9%) and 7 (17%) patients respectively; in those who responded there was a long lag phase. Healthy controls (n = 21) exhibited their highest responses to 1.64 and 0.82 mmol/l AA and at the lowest AA doses (0.41 and 0.20 mmol/l), a total absence of responses was noted in 40% and 71% of samples respectively. We conclude that during relapse platelet sensitivity, as shown by irreversible aggregation in response to multiple AA doses, shifts towards the lower doses, when compared with healthy controls; while during remission responses fall in-between the relapse and control groups, indicating the maintenance of platelet sensitivity during this phase of nephrosis.

Growth hormone and IGF1 profile in short children with osteopetrosis.

Osteopetrosis is commonly associated with short stature. To identify the cause, 8 children with osteopetrosis and short stature were studied. One showed evidence of renal tubular acidosis, none showed evidence of anaemia, and all were clinically and biochemically euthyroid. Growth hormone profile was assessed using night sampling (8 patients), growth hormone provocative testing using insulin-induced hypoglycaemia (6 patients), and L-dopa-propranolol (8 patients). The mean nocturnal growth hormone values taken at 60 and 90 min after onset of sleep and at 4 a.m. were 5.5, 12.8, and 11.5 mu/L respectively. The peak stimulated growth hormone mean values with the insulin-induced hypoglycaemia (glucose 1.6-3.0 mmol/L, mean = 2.2) was 14 mu/L (range 7.3-24.5 mu/L) and with the L-dopa-propranolol was 25.6 mu/L (range 12.3-49 mu/L). IGF1 levels taken at 0 and 120 min of insulin-induced hypoglycaemia (0 min for L-dopa-propranolol), and at 120 min of L-dopa-propranolol, showed normal values for age. We conclude that: (1) growth hormone profiles in these children are normal; (2) tissue unresponsiveness to growth hormone and/or IGF1 is not likely to be the cause of short stature in children with osteopetrosis; and (3) osteopetrosis per se is not an indication for assessment of growth hormone status.

The management of fungal obstructive uropathy in neonates and infants.

Obstructive uropathy caused by upper urinary tract fungal ball formation is an uncommon but well recognized clinical entity. The clinical course and management of an infant with unilateral fungal ball obstruction is described. Ultrasound and Tc-diaminotetraethylpentacetic acid (DTPA) renal scan contributed significantly to the diagnosis and management of this patient. Complete resolution of the obstruction was achieved by treatment with intravenous amphotericin B and oral 5-fluorocytosine. The clinical course and management of 35 patients described in the literature indicate that prematurity, use of broad spectrum antibiotics, prolonged hospital stay and the use of intravascular catheters are predisposing factors. The mortality rate is 34%. Young age, small size, the presence of candidaemia and withholding antifungal therapy are poor prognostic factors. A rational plan of treatment, extrapolated from the literature, is presented which may help to reduce the mortality rate in this condition.

Ultrasound detection rate of childhood urolithiasis.

A retrospective study of 42 children with 45 proven urinary tract stones who had had intravenous urography (IVU), ultrasound examination and a plain abdominal X-ray (KUB) was undertaken in order to evaluate the prediction rate of each method. Ultrasound was more sensitive than KUB (91% vs 80%) in detecting stones. All renal stones were detected by ultrasound. KUB was superior to ultrasound in detecting ureteral stones (66.6% vs 55.5%). All stones were detected by the combination of ultrasound and KUB. Two renal radiolucent stones were missed by IVU. The results of this study suggest that the combination of ultrasound examination and KUB is more sensitive in detecting stones than IVU and is more convenient and less invasive.

Persistent insulin-dependent diabetes mellitus in hemolytic uremic syndrome.

A 1.5-year-old Saudi girl with hemolytic uremic syndrome is described. She developed hyperglycemia in the acute stage which required insulin therapy. After a short remission, she developed permanent insulin-dependent diabetes mellitus. Review of the literature of the occurrence and the pathophysiology of this phenomenon is presented.

Septicaemia in febrile neutropenic children with cancer in Saudi Arabia.

The pattern of sepsis among 56 children admitted for various forms of cancer to the King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia during a 6-year period, was retrospectively reviewed. A total of 148 febrile neutropenic episodes occurred and 55 of these, in 40 patients, were associated with positive blood cultures. The isolates were Gram-positive bacteria in 54% of instances, Gram-negative bacteria in 39.4% and Candida in 6.6% and polymicrobial sepsis occurred in five patients. Profound neutropenia (neutrophil counts less than 0.1 x 10(9) l-1) significantly predisposed to Gram-negative sepsis (P less than 0.02), which was responsible for about one-third of deaths in this series. Central venous catheters were present prior to 49% of the septicaemic episodes, but were not significantly associated with either increased Gram-negative or Gram-positive bacterial sepsis. However, all four cases of candidaemia occurred in patients with a central venous catheter in situ, and it is recommended that early empirical treatment for candida be considered in all febrile neutropenic cancer patients with central venous catheters.

Henoch-Schönlein syndrome in Saudi Arabia.

A retrospective study was carried out on 40 children from Saudi Arabia with Henoch-Schönlein syndrome to delineate its clinical pattern. More than 50 per cent of the cases occurred in winter. There was no apparent causal relationship with B-haemolytic streptococcal infection. All patients had skin rash. Fifty-eight per cent had gastrointestinal manifestations, 58 per cent had joint manifestations and 38 per cent had renal manifestations. The main clinical features of Henoch-Schönlein syndrome in Saudi children are comparable to those reported elsewhere.

Postrenal-biopsy hematoma in infants and children: evaluation by ultrasonography.

Postbiopsy hematoma was assessed using ultrasonography in 106 infants and children (age 1 month to 15 years) who underwent a total of 122 percutaneous renal biopsies. Postbiopsy hematoma occurred in 22 (21%) of 105 successful biopsies. However, only 2 of these were symptomatic. Renal failure, mild and moderate hypertension and inexperience of the operator were not found to increase the incidence of postbiopsy hematoma in infants and children.

Sonographic findings in infantile idiopathic nephrotic syndrome.

Abnormal sonographic findings were obtained in all cases of infantile idiopathic nephrotic syndrome (IINS). Hyperechoic renal parenchyma, subcapsular hypoechoic band and loss of cortical medullary differentiation were the commonest abnormalities. There was no specific sonographic difference between the different types of IINS. The subcapsular hypoechoic band is also not diagnostic for IINS. Abnormal sonographic findings indicate renal biopsy to identify specific types of IINS.

The outcome of percutaneous renal biopsy in children: an analysis of 120 consecutive cases.

One hundred and twenty percutaneous renal biopsies performed in 104 patients aged 1 month to 15 years were analysed. Ultrasound examination was used for localization of the kidneys. Adequate renal tissue was obtained in 103 biopsies, with an overall success rate of 85.8%. The most frequent complication was gross haematuria, which occurred in 32 biopsies, but only 5 children required blood transfusion. Nineteen patients developed perirenal haematoma; 2 of these were symptomatic. Arteriovenous fistula was diagnosed in 2 patients. One patient died secondary to intestinal perforation. Serious complications were noted in patients with chronic renal failure. The success and complication rates in 10 patients less than 1 year of age were comparable with the rest of the group. The frequency of serious complications on renal biopsy in the present study was slightly higher than in the more developed countries.