0041-1337/01/7203-428/0
TRANSPLANTATION
Copyright © 2001 by Lippincott Williams & Wilkins, Inc.
PRIMARY HYPEROXALURIA TYPE 1: IMPROVED OUTCOME WITH
TIMELY LIVER TRANSPLANTATION: A SINGLE-CENTER REPORT
OF 36 CHILDREN
RIVKA SHAPIRO,1 IRIT WEISMANN,2 HANA MANDEL,3 BELA EISENSTEIN,1 ZIV BEN-ARI,4
NATHAN BAR-NATHAN,5 ILAN ZEHAVI,1 GABRIEL DINARI,1 AND EYTAN MOR5,6
Pediatric Gastroenterology and Pediatric Nephrology Units, Schneider Children’s Medical Center, Petach-Tikva; The
Pediatric Nephrology Unit, West Galilee Medical Center, Naharia; The Pediatric Genetic Unit, Rambam Medical Center,
Haifa; and The Liver Institute and Department of Transplantation, Rabin Medical Center, Petach-Tikva, Israel
Background. The appropriate use of liver transplan-
tation in children with type-1 primary hyperoxaluria
(PH-1) is not well established. We reviewed our expe-
rience with 36 children with PH-1, including 12 who
underwent liver transplantation.
Patients and Methods. From 1989 –1998, 36 children
from 10 families in northern Israel were diagnosed
with PH-1. Eight children presented with renal fail-
ure; seven of these eight had the severe infantile form
of the disease. One child was treated with kidney
transplantation alone. Combined liver-kidney trans-
plantation has been performed in nine children and
preemptive liver transplantation in three children. A
review of the patients’ charts for the following param-
eters was performed: age, clinical signs, and renal
sonographic findings at diagnosis, age at onset of di-
alysis, and current status. Type of transplant, pre- and
posttransplant urine oxalate excretion, current renal
function, survival, and complications were recorded
in liver recipients.
Results. Of the 23 nontransplanted children, 9 died
of complications related to severe systemic oxalosis
and 14 are alive (mean follow-up, 7.4 years), including
2 who are candidates for transplantation. The child
who underwent only kidney transplantation died of
unrelated causes. Of the 12 liver recipients, 2 died
within the first 3 months posttransplant and another
child underwent retransplantation due to hepatic ar-
terial thrombosis. At intervals after transplant rang-
ing from 6 –54 months, 10 recipients are alive (7 of the
9 recipients of combined liver-kidney transplants and
all 3 recipients of preemptive liver transplants). Mean
GFR in the 10 survivors is 77 ml/min/m2. In 9 of these
10, daily urinary oxalate excretion normalized. Renal
function has improved (mean GFR 86 vs. 58 ml/min/m2)
but renal oxalate deposits remain in the three recipi-
ents of isolated liver grafts.
Conclusions. Our decade-long experience with chil-
dren with PH-1 supports strategies for early diagnosis
and timely liver transplantation. Preemptive isolated
liver transplantation should be considered in children
who develop the disease during infancy or in those
1
Pediatric Gastroenterology and Pediatric Nephrology Units,
Schneider Children’s Medical Center.
2 the option of preemptive living-related liver transplantation
The Pediatric Nephrology Unit, West Galilee Medical Center.
3
The Pediatric Genetic Unit, Rambam Medical Center.
4
The Liver Institute.
5
Department of Transplantation, Rabin Medical Center.
6
Address correspondence to: Eytan Mor, MD, Department of Trans-
plantation, Rabin Medical Center, Petach-Tikva, 49100, Israel.
428
Vol. 72, 428–432, No. 3, August 15, 2001
Printed in U.S.A.
with slowly progressive disease when significant
symptoms develop. Combined liver-kidney transplan-
tation is suggested for children with end-stage renal
disease.
INTRODUCTION
Primary hyperoxaluria type-1 (PH-1) is an autosomal re-
cessive inborn error of glyoxylate metabolism in which exces-
sive production of oxalate results in urolithiasis and nephro-
calcinosis followed by end-stage renal failure and systemic
oxalosis (1). In the severe infantile form of the disease, renal
failure occurs in infancy, but more often, renal function de-
teriorates slowly during the first 5 years of life, despite med-
ical treatment and dietary modifications (2). In some cases,
the disease progresses slowly and the patient remains
asymptomatic until middle age (3).
In children with end-stage renal failure, control of the
disease by dialysis is limited because despite treatment,
plasma oxalate exceeds saturation and extra-renal deposi-
tion occurs (i.e., in bones, muscle, cartilage, cornea, nerves,
and blood vessels) (5). Results of kidney transplantation in
these children have been disappointing because of rapid ox-
alate deposition in the graft. Almost 80% of recipients in the
European registry lost their kidney within 3 years after
transplant (6). Liver transplantation (LTx), however, cor-
rects the metabolic defect, and combined liver-kidney trans-
plantation (LKTx) may offer prolonged kidney graft survival
(7).
In Israel, the incidence of this disease is high, with four to
eight children diagnosed each year. Many of these children
are asymptomatic, and we face the serious dilemmas of how
to time their transplant and what kind of transplant to offer.
Because of the rareness of the condition and the unpredict-
able course of the disease, there are no strict management
guidelines. However, preemptive isolated liver transplanta-
tion, which has recently been advocated (8), can avoid the
need for dialysis or for combined liver-kidney transplanta-
tion. However, should we offer such radical therapy to chil-
dren with mild symptoms early after diagnosis? Further-
more, because primary hyperoxaluria is a genetic disorder,
usually more than one sibling in a family is affected. Then,
creates yet another dilemma: which child should get the
transplant? And which parent will be the donor?
We summarize our 10-year experience with 36 children
with PH-1, with a specific focus on 12 children who under-
went liver transplantation. Although there have been several
August 15, 2001 SHAPIRO ET AL.
case reports of children with this rare metabolic disorder, as
well as reports on small numbers of patients from major
transplant centers, ours is the first report to review a rela-
tively large single-center experience. In addition, based on
our long-term experience and reports by others, we suggest a
transplant strategy developed with the perspective of the
clinicians who have cared for this large cohort from the time
of initial diagnosis, many years before transplant.
PATIENTS AND METHODS
Between 1989 –1998, 36 children from 10 families were diagnosed
with PH-1. All but one were products of consanguineous marriages
and all belonged either to the Druz or Moslem population residing in
the northern part of Israel. These children were diagnosed and
followed by a single physician (IW).
Diagnosis of PH-1 was confirmed in all cases by elevated urine
oxalate and glycolate levels and by deficiency of AGT activity in
frozen liver specimens obtained by percutaneous biopsy. Analysis of
AGT activity was performed using an immunostaining technique
against catalase and AGT (9). Upon diagnosis, all children were
managed according to commonly accepted guidelines, with overhy-
dration, dietary restriction of oxalate rich-food, and supplementation
of orthophosphate or citrate (10). Pyridoxine was prescribed only to
children with proven residual AGT activity. Dialysis was instituted
when end-stage renal failure developed.
Before 1991, kidney transplantation was the only treatment avail-
able and was performed in two children. Since 1991, when liver
transplantation became accessible, combined liver-kidney transplan-
tation has been performed in nine children (including one child who
had earlier received a kidney graft and lost it to renal oxalosis) and
preemptive liver transplantation in three children.
To prevent posttransplant oxalate deposition, forced diuresis and
dialysis was initiated immediately after combined transplant. Dial-
ysis was discontinued when renal graft function became normal,
usually toward the end of the first week. In 10 children, immuno-
suppression was based on tacrolimus and steroids. A cyclosporine-
based protocol was initiated in two children, who were later con-
verted to tacrolimus because of acute rejection and hirsutism. At
present, all 10 surviving transplant recipients are receiving tacroli-
mus-based maintenance immunosuppression.
In all patients, we recorded gender, age, and urinary oxalate levels
at diagnosis and initiation of dialysis, clinical signs and renal sonog-
raphy findings at diagnosis, and current status. Furthermore, in
transplant recipients we looked at pretransplant duration of dialysis,
type of transplant, pre- and posttransplant urinary oxalate levels,
most recent creatinine level and glomerular filtration rate (GFR),
major complications, and survival. Mean and SD values were calcu-
lated for numerical parameters. Survival was calculated using
Kaplan-Meier estimates.
RESULTS
In our cohort of 36 children with PH-1, age at diagnosis
varied from 1 month to 20 years, with a mean of 3.9 years (Table
1). There were 20 boys and 16 girls. Renal failure was the
presenting symptom in eight children. In four children, the
diagnosis was made during evaluation for failure to thrive at
infancy. Another 11 children presented with symptoms related
to urolithiasis, including recurrent urinary tract infections, he-
maturia, and renal colic. In the 13 asymptomatic children,
diagnosis was made after recognition of PH-1 in their siblings or
by ultrasonographic evidence of nephrocalcinosis.
Dialysis was instituted in 13 children, including three who
were less than 2 years of age. Seven of the eight children who
presented with renal failure had the severe infantile form of
the disease and died before the age of 5 years from compli-
429
cations related to systemic oxalosis. Another two children
(one who was asymptomatic at diagnosis and one who pre-
sented with hematuria) also died without transplantation,
due to complications related to renal failure and severe sys-
temic oxalosis. Fourteen nontransplanted children are alive,
including two awaiting transplantation. The follow-up since
diagnosis among these 14 children ranges from 3 months to
20 years (mean 7.1 years). One child, who presented with
renal failure at 9 years of age, died 2 years after isolated
kidney transplantation after a car accident.
Twelve children (four boys, eight girls) underwent 13 liver
transplants (one retransplant) (Table 2). Mean age at trans-
plantation was 7.4 years (range 1.5–17 years). Of the nine
combined liver/kidney transplants, one was performed 7
years after kidney transplantation (the renal graft was lost 2
years posttransplant from renal oxalosis). In another case,
cadaveric kidney transplantation was performed 3 months
after a segmental living-related liver transplantation. All
nine patients were on dialysis at the time of transplant
except one who was at predialytic stage (GFR, 15 ml/min/1.73
m2). The mean duration of dialysis before transplant was 1.5
years (range 5 month to 5 years).
All three patients who underwent isolated liver transplan-
tation had GFRs⬎50 ml/min/1.73 m2 at the time of trans-
plant. Two of these three children received segmental grafts
from living donors; one child was transplanted with a split
left lateral segment from a cadaveric donor. At follow-up (42,
36, and 36 months), GFR is unchanged from pretransplant
levels in one patient, improved by 20% in another patient and
slightly decreased in another (Table 2). In all three children,
nephrocalcinosis in the native kidneys remained unchanged.
Of the 12 liver transplant recipients, 2 died during the
immediate postoperative period: one of sepsis at 1 month and
the other child died of lymphoproliferative disease at 3
months posttransplant. One child who received a combined
kidney/liver transplant lost his first liver graft to hepatic
artery thrombosis and underwent a successful liver
retransplantation.
Ten transplant recipients are alive at 6 –54 months post-
transplant (mean 32.2 months). Actuarial 5-year patient and
graft survival are 83.3 and 74.9%, respectively. All survivors
have normal liver function (Table 2) except for one patient
with chronic ductopenic rejection; in this patient, liver func-
tion tests have improved recently with mycophenolate
mofetil salvage treatment. Mean GFR in the 10 surviving
patients is 77 ml/min/m2 (range 51–95 ml/min/m2). In the
nine patients with long-term follow-up (⬎1 year), daily uri-
nary oxalate excretion has returned to normal (Table 2).
DISCUSSION
Primary hyperoxaluria type-1 is a rare autosomal recessive
inborn error of glyoxylate metabolism encoded by the AGXT
gene on chromosome 2q36 –37 (2). The disorder is caused by
deficiency of liver-specific peroxysomal alanine:glyoxylate
aminotransferase (AGT) enzymatic activity. A failure of ami-
nation of glyoxylate to glycine leads to oxidation of excessive
amounts of glyoxalate to oxalate and glycolate. The excessive
production of oxalate together with the low solubility of its
calcium salt results in hyperoxaluria, producing urolithiasis,
nephrocalcinosis, renal failure, and systemic oxalosis (1).
In Europe, the reported incidence of the disease is 1:107
(11). Among the 0.6⫻106 Druz and Moslem residents of the
430 TRANSPLANTATION Vol. 72, No. 3

TABLE 1. Disease characteristics of 36 children with PH-1

Agea at Current Current
Family Name Sex Agea at Dx Clinical signs Transplant Agea at Tx
dialysis agea GFR
1 AH M 0.8 FTT 1.2 LKTx 1.5 Died 1.7 yr
1 NR F 1 FTT 5 LKTx 6 Died 6yr
1 RN F 2 Asymptomatic 3.5 LKTx 4.5 9 55
1 AR F 1 FTT No LTx 2.5 6 93
1 NS F 3 Asymptomatic No LTx 3.5 6.5 85
1 ND F 0.1 FTT No LTx 6 9 84
1 CR F 0.6 Asymptomatic 1.5 LKTx 2.5 3.8 80
2 CM M 6 Asymptomatic No No 8.5 78
2 HI M 7 Asymptomatic No LKTx 13 15 77
2 HA F 4 Asymptomatic 4.5 No Died 8yr
2 HS M 5 Asymptomatic No No 9.5 85
2 HS M 9 Hematuria 10 No Died 10 yr
2 HR M 4 Hematuria No No 6.5 96
3 KC M 4 Hematuria No No 5.5 96
3 KT F 5 Asymptomatic 6 LKTx 6.5 7 78
3 KM M 0.8 Asymptomatic No Candidate 2 60
4 NM M 2.5 Hematuria No No 5.5 78
4 NA M 1.5 Asymptomatic No No 4.5 92
5 AW F 9 Renal failure 10 KTx 11 Died 13 yr
5 AA F 20 Renal colic No No 23 112
5 AY M 5 UTI 16 Candidate 17.5 Dialysis
6 ZS F 0.1 Renal failure 0.4 No Died 1.2 yr
6 ZT F 5 Renal colic 12 LKTx 14 16 95
7 SB M 0.1 Renal failure No No Died 5 mo
7 SN M 6 Hematuria No No 25 31
7 SN F 6 Hematuria No No 23 36
8 AO M 7 Hematuria 11 LKTx 11.5 15.5 85
8 AB F 0.2 Renal failure No No Died 5 mo
8 BR M 0.1 Renal failure No No Died 6 mo
8 BA M 2 Renal failure No No Died 5 yr
8 BA F 0.2 Renal failure No No Died 3 mo
8 BB F 0.2 Renal failure No No Died 4 mo
9 GM M 1.5 Asymptomatic No No 2 96
9 GA M 0.8 Asymptomatic No No 1 90
10 AF M 8 Hematuria 9 KTx & 10&17 20.3 51
LKTx
10 AS M 12 Asymptomatic No No 23 85
a
Age is given in years. GFR, glumerular filtration rate; UTI, urinary tract infection; LTx, liver transplantation; LKTx, liver/kidney
transplantation.

northern part of Israel, 36 members of 10 families were
diagnosed with this rare disorder. This high incidence of
PH-1, 54 per 106 over a 10-year period, is probably the
highest rate reported in the literature and is related to the
consanguineous marriages that are so common among the
Druz and Moslems in that region. Similarly, in a report of 78
cases of PH-1 worldwide, a high degree of consanguinity was
observed mainly in Moslems, who represented 44% of pa-
tients in that series (12). These observations suggest that
transmission of genetic mutations plays a major role in the
high incidence of PH-1 among Moslems and Druz. In fact, in
all 10 families in our series, we identified clinical symptoms
related to PH-1 in at least one sibling. Because of this high
incidence of disease, we are presently conducting a genetic
survey in the Northern Galilee.
Despite the similar enzymatic genetic defect, there is a
wide clinical heterogenicity of PH-1 among affected individ-
uals, suggesting that nongenetic factors also contribute to
the phenotypic form of the disease (4). As with others (13), we
observed different phenotypic characteristics of PH-1 in sib-
lings in the same family. In one family, for instance, one child
had the severe infantile type of the disease although his
brother and his sister were asymptomatic (family 7).
In the past, we had no therapies to offer children with the
severe infantile form of PH-1. Today, screening of affected
families and of children with minimal symptoms permits
earlier diagnosis and institution of appropriate management.
Diagnosis is established by analysis of AGT immunoreactive
protein (9) and catalytic activity (14) in liver biopsy. Treat-
ment, directed at prevention of oxalate supersaturation and
the ensuing deposition of calcium oxalate in the kidneys and
extrarenal tissues (15), consists of high amounts of fluids
(twice the recommended daily intake), dietary restriction of
oxalate-rich food, and supplementation of orthophosphate,
citrate, and magnesium. Vitamin B6, a cofactor for AGT
activity, is indicated in patients with residual catalytic activ-
ity of this enzyme (10). Once renal insufficiency develops, the
patient is listed for liver transplantation and dialysis is in-
stituted as soon as indicated to prevent extrarenal oxalate
deposition.
There are conflicting data on the role of isolated kidney
transplantation for PH-1. The US registry reports reasonable
August 15, 2001 SHAPIRO ET AL. 431
TABLE 2. Liver transplantation for children with PH-1
Oxalate
Patient Age at Duration of Oxlate pre- Cr. post- GFR post-Tx (ml/
Sex Type of Tx post-Tx Survival Complications
name Tx dialysis (yr) Tx (mg/24 hr) Tx (mg/dl) min/1.73 m2)
(mg/24 hr)
AH M 1.5 0.4 LKTx 680 Died (3 mo) PTLD
NR F 6 1 LKTx 890 Died (1 mo) Sepsis
RN F 4.5 0.8 KTx after LRD- 388 12 1 55 Alive (54 mo)
LTx
AR F 2.5 No LRD-LTx 760 16 0.8 93 Alive (42 mo)
NS F 3.5 No LTx 1042 18 0.8 85 Alive (36 mo)
ND F 6 No LRD-LTx 1280 16 1 75 Alive (36 mo)
CR F 2.5 1 LKTx 1390 44 0.8 80 Alive (14 mo)
HI M 13 No LKTx 1875 48 0.9 77 Alive (24 mo) Chronic rejection
ZT F 14 2 LKTx 1960 21 0.8 95 Alive (22 mo)
AO M 11.5 0.5 LKTx 560 65 0.8 85 Alive (49 mo)
AF M 17 5 KTx and LKTx 1700 55 1.5 51 Alive (39 mo)
KT F 6.5 1 LKTx 890 65 1.2 78 Alive (6 mo) Hepatic artery
thrombosis
Tx, transplantation; Cr, creatinine; GFR, glumerular filtration rate; PTLD, posttransplant lymphoproliferative disease; LTx, liver
transplantation; LKTx, liver/kidney transplantation; LRD, living-related donor; HAT, hepatic artery thrombosis.

results with kidney transplantation alone, with 5-year graft
survival of 45% (16). The European registry experience
shows 3-year graft survival of less than 20% (7). The differ-
ence in outcomes between the two series probably arises from
differences in patient populations, genetic phenotypes, and
disease characteristics. Accumulated data from different cen-
ters suggest that isolated kidney transplantation may be an
appropriate therapeutic option for children without systemic
oxalosis who have some residual AGT activity (17, 18). Be-
cause the ethnic groups of the patients in our series, Moslems
and Druz, are known to have the more severe form of the
disease, namely, absence of AGT activity and early onset of
symptoms, kidney transplantation alone is not likely to offer
any therapeutic advantage. Indeed, two of our patients who
underwent kidney transplantation alone lost their grafts in
less than 3 years.
It has been suggested that combined liver/kidney transplan-
tation should be performed early enough in these children to
avoid complications of prolonged dialysis and systemic oxalosis
(7). In our series, the death of one child at 1 month posttrans-
plant was related to severe failure to thrive associated with
malnutrition and muscle wasting from systemic oxalosis. In
addition, there has recently been a tendency to offer preemptive
liver transplantation to correct the metabolic defect before the
development of end-stage renal disease (8). It has been sug-
gested that such an approach be carried out in children with
slowly declining GFR and in those with recurrent urolithiasis
despite adequate treatment (19).
Although our cohort is large compared to series reported by
others, we have too few patients to permit statistical analysis
of differences between transplanted and nontransplanted pa-
tients. Still, certain features of the groups are noteworthy.
Among the nontransplanted patients, there are two distinct
subgroups. One subgroup had extremely severe disease;
these eight patients presented with renal failure (seven of
the eight had the infantile form of the disease), and all died
before the age of 5 years. The other subgroup had mild or no
symptoms; these 12 children, mostly siblings of affected in-
dividuals, maintain normal renal function. Together, the two
subgroups that comprise the nontransplanted group repre-
sent the clinical extremes of the disease. In between lie the
patients who underwent transplant; these patients became
transplant candidates mainly because they had developed
renal failure. Based on our own experience and that of others
(7, 8, 19), we decided to offer preemptive liver transplanta-
tion to children with the infantile form and a rapidly progres-
sive course but to delay transplantation in older children as
long as their kidney function is normal. Because there is no
way to predict when renal function will begin to deteriorate,
there is no sense in performing preemptive transplant in
those patients.
All three of our children who underwent isolated liver trans-
plantation maintain their renal function between 36 – 42
months posttransplant despite the residual oxalate deposits in
their kidneys. Oxalate deposits have also been found in kidney
grafts of patients who have undergone combined liver/kidney
transplantation, with no effect on graft function (20).
Because the release of oxalate from tissues continues for
several months after successful liver transplantation (21), it is
important to dialyze these patients until renal function normal-
izes and to maintain adequate hydration and other measures
later on to prevent oxalate deposition within the kidneys (20).
In summary, we are aware that our small number of pa-
tients limits our ability to draw solid conclusions. Still, based
on our own experience in the long-term management and
follow-up of children with PH-1 and on experience reported
by other centers, we recommend an aggressive strategy for
early diagnosis and timely liver transplantation. When chil-
dren present in infancy with nephrolithiasis and nephrocal-
cinosis, it is our policy to list them for isolated liver trans-
plantation. The children are followed closely, and once their
renal function deteriorates we recommend isolated liver
transplantation. For asymptomatic children diagnosed at in-
fancy or for those with late onset of disease, we defer trans-
plantation until significant symptoms develop (e.g., nephro-
calcinosis associated with deteriorating renal function).
Urolithiasis is managed by endoscopic procedures.
Acknowledgments. The authors thank Dr. Xavier Rogiers, U.K.
Eppendorf, Hamburg, Germany, Dr. Eliezer Katz, University of Mas-
sachusetts Medical Center, Worcester, MA for the information on our
patients, and Mrs. Nancy Erlich for professional editorial assistance.
432 TRANSPLANTATION
REFERENCES
1. Danpure CJ, Purde PE. Primary hyperoxaluria. In: Scriver CR, Beaudet
AL, Sly WS, Valle D, eds. The metabolic and molecular basis of inher-
ited disease. New York: McGraw-Hill, 1995.
2. Danpure CJ. Advance in the enzymology and molecular genetics of pri-
mary hyperoxaluria type I. Prospects for gene therapy. Nephrol Dial
Transplant 1995; 10 (suppl 8): 24.
3. Gluck T, Kramer BK, Zulke C, et al. Late onset of primary oxalosis type I:
an uncommon presentation of a rare disease. Eur J Gastroenterol
Hepatol 1998; 10: 809.
4. von Schnakenburg C, Hulton SA, Milford DV, Roper HP, Rumsey G.
Variable presentation of primary hyperoxaluria type 1 in 2 patients
homozygous for a novel combined deletion and insertion mutation in
exon 8 of the AGXT gene. Nephron 1998; 78: 485.
5. Marangella M, Petrarvlo M, Cosseddu D, Linari F. Oxalate balance studies
in patients on hemodialysis for type I primary hyperoxaluria. Am J
Kidney Dis 1992; 19: 546.
6. Broyer M, Brunner FP, Brynger H, et al. Kidney transplantation in pri-
mary oxalosis: data from the EDTA registry. Nephrol Dial Transplant
1990; 5: 332.
7. Jamieson NV. The results of combined liver/kidney transplantation for
primary hyperoxaluria (PH1) 1984 –1987; The European PH1 trans-
plant registry report: J Nephrol 1998; 11 (suppl 1): 36.
8. Kemper MJ, Nolkemper D, Rogiers X, et al. Preemptive liver transplan-
tation in primary hyperoxaluria type 1: timing and preliminary results.
J Nephrol 1998; 11 (suppl 1): 46.
9. Allsop J, Jennings PR, Danpure CJ. A new micro-assay for human liver
alanine:glyoxylate aminotransferase. Clin Chim Acta 1987; 170: 187.
10. Broyer M, Jouvet P, Niaudet P, Daudon M, Revillon Y. Management of
oxalosis. Kidney Int 1996; 49 (suppl 53): S93.
11. Cochat P, Deloraine A, Rotily M, Olive F, Liponski I, Deries N. Epidemi-
ology of primary hyperoxaluria type 1. Nephrol Dial Transplant 1995;
10 (suppl 8): 3.
12. Cochat P. World-wide view of primary hyperoxaluria type 1. Abstract, 5th
Vol. 72, No. 3
Workshop on Primary Hyperoxaluria, March 1999, Kappel, Zurich
13. Oner A, Demircin G, Tinaztepe K, Bulbul M, Tezic T. Fatal outcome of
infantile oxalosis: case reports from three families and a review of the
literature. Turk J Pediatr 1998; 40: 237.
14. Rumsby G, Mandel H, Avey C, Geraerts A. Polymorphism in the human
alanine:glyoxylate aminotransferase gene and their application to the
prenatal diagnosis of primary hyperoxaluria type 1. Nephrol Dial
Transplant 1995; 10 (suppl): 30.
15. Parekh RS, Smoyer WE, Bunchman TE. Diagnosis and management of
primary hyperoxaluria type 1 in infancy. Pediatr Transplant 1997; 1:
48.
16. Scheinman JI. Recent data on results of isolated kidney or combined
kidney/liver transplantation in the U.S.A. for primary hyperoxaluria. J
Nephrol 1998; 11 (suppl 1): 42.
17. Allen AR, Thompson EM, Williams G, Watts RW, Pusey CD. Selective
renal transplantation in primary hyperoxaluria type 1. Am J Kidney
Dis 1996; 27: 891.
18. Thervet E, Legendre C, Daudon M, et al. Is there a place for isolated
renal transplantation in the treatment of primary hyperoxaluria
type 1? Experience from Paris. Nephrol Dial Transplant 1995; 10
(suppl 8): 38.
19. Haffner D, Cochat P, Otto G, Steffen H, Scharer K. When should isolated
liver transplantation be performed in primary hyperoxaluria type 1?
Follow-up report of two children.Nephrol Dial Transplant 1995; 10
(suppl 8): 47.
20. Ruder H, Otto G, Schutgens RB, et al. Excessive urinary oxalate excretion
after combined renal and hepatic transplantation for correction of hy-
peroxaluria type 1. Eur J Pediatr 1990; 150: 56.
21. Tossaint C, De Pauw L, Vienne A, et al. Radiologic and histologic improve-
ment of oxalate osteopathy after combined liver-kidney transplantation
in primary hyperoxaluria type 1. Am J Kidney Dis 1993; 21: 54.
Received Received 2 October 2000.
Revision Requested 7 June 2000.
Accepted 11 July 2000.