Two-step transplantation for primary hyperoxaluria: A winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases.

[primary hyperoxaluria type 1]

Several transplant strategies for PH 1 have been proposed , and LT is performed to correct the metabolic defects . The patients with PH 1 often suffer from ESRD and require simultaneous LKT , which leads to a long wait due to the shortage of suitable organ donors . Five patients with PH 1 underwent LDLT at our institute . Three of the five patients were under dialysis before LDLT , while the other two patients were categorized as CKD stage 3 . An isolated LDLT was successfully performed in all but our first case , who had complicated postoperative courses and consequently died due to sepsis after retransplantation . The renal function of the patients with CKD stage 3 was preserved after LDLT . On the other hand , our second case with ESRD underwent successful LDKT six months after LDLT , and our infant case is waiting for the subsequent KT without any post-LDLT complications after the early establishment of PD . In conclusion , a two -step transplant strategy may be needed as a life-saving option for patients with PH 1 and may be possible even in small infants with systemic oxalosis . While waiting for a subsequent KT , an early resumption of PD should be considered from the perspective of the long -term requirement of RRT .

Diseases
Validation

Diseases presenting "first case" symptom

achondroplasia

adrenal incidentaloma

allergic bronchopulmonary aspergillosis

alpha-thalassemia

aniridia

aromatase deficiency

canavan disease

carcinoma of the gallbladder

child syndrome

cholangiocarcinoma

classical phenylketonuria

coats disease

cohen syndrome

congenital toxoplasmosis

cushing syndrome

cutaneous mastocytosis

dedifferentiated liposarcoma

dentin dysplasia

dracunculiasis

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

esophageal adenocarcinoma

esophageal carcinoma

fabry disease

familial mediterranean fever

focal myositis

gm1 gangliosidosis

harlequin ichthyosis

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

junctional epidermolysis bullosa

kabuki syndrome

krabbe disease

lamellar ichthyosis

legionellosis

liposarcoma

locked-in syndrome

malignant atrophic papulosis

monosomy 21

neonatal adrenoleukodystrophy

oculocutaneous albinism

omenn syndrome

papillon-lefèvre syndrome

pendred syndrome

pleomorphic liposarcoma

primary effusion lymphoma

primary hyperoxaluria type 1

proteus syndrome

pyomyositis

systemic capillary leak syndrome

thoracic outlet syndrome

von hippel-lindau disease

waldenström macroglobulinemia

well-differentiated liposarcoma

werner syndrome

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

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