Ellin Lieberman

Renal homotransplantation in children

Twenty-three children, aged 2 to 17 years, received 24 renal homotransplants from both live related donors and cadaver donors. Twenty-two children and 19 kidneys are surviving 1 to 32 months after transplantation. The clinical course of renal transplantation in children is described.

A Rat Model for the Study of Growth Failure in Uremia

Extract: The growth of children with chronic renal disease is poor and the cause of this stunting is not known. Various factors have ben implicated and it is difficult to evaluate their relative importance in clinical studies. Accordingly, there is a need for an animal model, preferably one which enables the effect on growth of a number of factors to be studied separately and over a reasonably short period of time. The growth and food intake of male and female rats rendered uremic by 5/6 nephrectomy was observed between 40 and 70 days of age for male rats and between 35 and 70 days of age for female rats. Final mean body weight for males with uremia (243 g ± 32 g) was significantly less than for control males (323 g ± 24 g); final mean body weights for female rats were also significantly different (172 g ± 17 g; 223 g ± 21 g). The differences in body weight were apparent from 50 days onwards. Final tail length was significantly less in female uremic rats compared with their control subjects (173 mm ± 8 mm; 183 mm ± 7 mm). Uremic rats matched for body weight with control rats consumed significantly fewer calories; for both groups the average difference was about 15%. Multiple regression analysis of weight gain against age and calorie intake suggests that there may be an increase in the calorie cost of growth in rats with uremia, but these findings require confirmation in paired feeding studies.Speculation: These studies suggest that this rat model can be used for the investigation of alterations in energy balance, body composition, and metabolic functions in uremia. It should be possible to study the effects of single variables in the pathogenesis of growth retardation by appropriate manipulations halfway through the growth period.

Thrombosis, nephrosis, and corticosteroid therapy

Thromboemboli occurred in 5 children receiving corticosteroid therapy for idiopathic nephrosis; 3 had fatal pulmonary thromboemboli and 2 survived peripheral involvement. The factors related to thromboemboli in children with nephrosis are considered and discussed in relationship to the known and possible effects of corticosteroid therapy.

HEPARIN THERAPY IN THE HÆMOLYTIC-URÆMIC SYNDROME

Abstract Eight children with haemolytic-uraemic syndrome received heparin intravenously. An early return of renal function despite prolonged thrombocytopenia was commonly observed. Detailed coagulation studies in two anuric patients showed raised levels of factors I, v, and VIII concomitant with thrombocytopenia and increased fibrinolytic activity. No consumption of clotting factors was demonstrated. These findings, together with previously documented histopathological alterations, suggest that the haemolytic-uraemic syndrome may be an example of localised intravascular coagulation. Six patients survive without clinical evidence of renal disease. One child is hypertensive with impaired kidney function. The only fatality occurred after initial improvement and cannot be directly ascribed to the basic disease or the anticoagulant therapy.

Hypertension secondary to a renin-producing juxtaglomerular cell tumor

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Rhabdomyolysis with severe hypernatremia

Three children with severe hypernatremia presented with profound generalized weakness and biochemical evidence of rhabdomyolysis and myoglobinuria. These findings in combination have not been previously reported, to our knowledge, in children with severe hypernatremia. Unusual complications included respiratory failure in one child and cardiac arrhythmias in two children. All three children had acute renal insufficiency; one required peritoneal dialysis.

Renal hypertension in children.

Preliminary results of this retrospective-prospective analysis of renal hypertension in 110 children indicate that hypertension may be secondary to a wide variety of acute progresive, and chronic renal diseases which may be either congenital or acquired. Affected children may be detected at any time from infancy through adolescence. Symptoms usually associated with acute glomerulonephritis (i.e., headache, swelling, nausea, vomiting, anorexia, fatigue, dizziness, and fever) occur in both acute and chronic renal diseases associated with hypertension. Headache and swelling are the most common symptoms in this series. Peripheral edema, rales, and increased heart size were found in between 10 and 25% of these children. Differential diagnosis may be approached by a consideration of causes of acute and chronic hypertension. The child with chronic renal disease usually presents with a long history of fatigability, poor growth, and pallor, and laboratory tests reveal elevation of the creatinine and BUN along with anemia, hypocalcemia, and hyperphosphatemia. In contrast, the child with acute renal disease and hypertension presents with a history of prior good health followed by the abrupt onset of signs and symptoms of renal disease; laboratory tests usually reveal modest elevations of creatinine and BUN, anemia is unusual, an abnormal urinalysis is common, and serum calcium and phosphorous levels are usually normal. Renovascular and asymmetric renal parenchymal disease represent uncommon but important conditions because surgery may be curative. Treatment may be surgical, medical, or combined. Surgical conditions include renal trauma, hydronephrosis, asymmetric renal disease, and renal arterial disease. Adequate blood pressure control without medication can be expected following surgery in instances of unilateral involvement with a normal contralateral kidney. Meticulous assessment of the contralateral kidney is needed to determine that it is normal. If surgery is unsuccessful or is not indicated, pharmacologic therapy is initiated with a stepwise regimen starting with the mildest agent (e.g., thiazides) and then adding additional antihypertensive drugs when adequate blood pressure control has not yet been achieved. The goal of therapy is the lowest, safest, tolerated blood pressure levels. Long-term, carefully designed studies of antihypertensive agents for children with renal hypertension are not available. The need for collection and critical analysis of data concerning the clinical course of children with renal hypertension is evident from a review of the literature and from the preliminary data presented in this series. The presentation of such information and a critique of outcome variables will provide a basis for program planning for affected children and improvement in patient care where indicated.

Human Immunodeficiency Virus-Associated Kaposi’s Sarcoma in a Pediatric Renal Transplant Recipient

An 11-year-old boy developed Kaposis sarcoma and progressive T lymphocyte deficiency 5 years after cadaveric kidney transplantation for end-stage renal disease. He had received 17 individual red blood cell transfusions prior to and during transplantation in 1980. Human immunodeficiency virus (HIV) was cultured from blood in cerebrospinal fluid and HIV antibodies were detected with enzyme immunoassay and immunoblot techniques. The recipient of the donors other kidney was well and HIV antibody-negative. The patient was treated with etoposide with excellent although transient regression of tumor. Allograft function has remained stable despite minimal immunosuppressive therapy and the need for high-dose anticonvulsant therapy. This case represents the first pediatric patient with acquired immune deficiency syndrome (AIDS) and Kaposis sarcoma following kidney transplantation.

Renal artery stenosis in pediatric transplant recipients.

From 1967 through 1985, 400 cadaveric transplants were performed at Children Hospital of Los Angeles. Of these 400, 31 were later identified as having renal artery stenosis. No live related graft developed RAS. Of the 31 grafts, 11 were from donors less than 2 years of age. The major feature suggesting stenosis was hypertension: either persistent or a sudden exacerbation often associated with hypertensive encephalopathy. In individuals with hypertension without obvious cause, renal angiography should be promptly conducted under controlled conditions to avoid complications. The stenotic lesion involved 13 end-to-end and 19 end-to-side arterial anastomoses. Surgery for revascularization of RAS was performed in 21 of 31 with success or improvement in 14, no change in 2, and graft loss in 5. Percutaneous transluminal angioplasty was performed in 4. Two were unsuccessful, 1 was successful and 1 graft was lost. The 7 remaining patients were treated medically.

Bacteriologic relapse during ampicillin treatment of Hemophilus influenzae meningitis

A v t P I c I L L I N is a safe and effective agent in the t r ea tmen t of Hemophilus influenzae meningitis . 1-~ No strains of Hemophilus influenzae which are resistant to ampicil l in have been isolated in the extensive studies by Wehr le and associates. 4, 5 However, 3 instances of bacter iologic relapse in HemophiIus influenzae meningit is t rea ted with ampici l l in have been r epor t ed2 -s I n one case of meningit is t rea ted by the oral route, 6 relapse was a t t r ibu ted to inadequa te concent ra t ion of ampici l l in in the cerebrospinal fluid. In ano ther pat ient , s relapse was also a t t r ibu ted to inadequa te concent ra t ion of the ant ibiot ic in the cerebrospinal fluid. I n a th i rd repor t 7 relapse was a t t r ibu ted to sequestra t ion of organisms at a site (cellulitis or e thmoidi t is) inaccessible or only par t ia l ly accessible to the drug; the second lumbar punc tu re in this last pa t ien t revealed ne i ther cells nor organisms. T h e following is the case repor t of an addi t ional infant who had a bacter iologic re-