De novo amyloidosis in a renal transplant patient

Systemic AA amyloidosis is a serious complication of many chronic inflammatory disorders and chronic infections. Renal involvement is seen in the majority of the patients and can lead to end‐stage renal disease. Renal transplantation can be performed in these patients; however, amyloidosis can recur in the transplanted kidneys. On the other hand, de novo AA amyloidosis in renal transplant patients has been rarely reported. We report a 17‐yr‐old patient with end‐stage renal disease due to genitourinary anomalies who developed recurrent pyelonephritis after transplantation. Three yr after transplantation, renal biopsy was performed for proteinuria and AA amyloidosis was identified in the renal allograft. Although rare, chronic infections might cause de novo amyloidosis in renal transplant patients. Therefore, amyloidosis should be kept in mind in those types of patients who present with proteinuria.     

Racial disparities in pediatric kidney transplantation in New Zealand

Racial disparities in transplantation rates and outcomes have not been investigated in detail for NZ, a country with unique demographics. We studied a retrospective cohort of 215 patients <18 yr who started renal replacement therapy in NZ 1990–2012, using the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). Primary outcomes were time to first kidney transplant, death‐censored graft survival, and retransplantation after loss of primary graft. Europeans and Asians were most likely to receive a transplant (92% and 91% transplanted within five yr, respectively), and Pacific and Māori patients were less likely to receive a transplant than Europeans (51% and 46%, respectively), reflecting disparities in live donor transplantation. Pacific patients were more likely to have glomerulonephritis and FSGS. Pacific patients had five‐yr death‐censored graft survival of 31%, lower than Māori (61%) and Europeans (88%). No Pacific patients who lost their grafts were re‐transplanted within 72 patient‐years of follow‐up, whereas 14% of Māori patients and 36% of European and Asian patients were retransplanted within five yr. Current programs to improve live and deceased donation within Māori and Pacific people and management of recurrent kidney disease are likely to reduce these disparities.     

Successful renal transplantation following prior bone marrow transplantation in pediatric patients

Improving survival rates following pediatric bone marrow transplantation (BMT) will likely result in greater numbers of children progressing to end-stage renal disease (ESRD) because of prior chemotherapy, irradiation, sepsis, and exposure to nephrotoxic agents. Renal transplantation remains the treatment of choice for ESRD; however, the safety of renal transplantation in this unique population is not well established. We report our experience with living related renal transplantation in three pediatric patients with ESRD following prior BMT. Two patients with neuroblastoma and ESRD because of BMT nephropathy, and one patient with Schimke immuno-osseous dysplasia and ESRD because of immune complex mediated glomerulonephritis and nephrotic syndrome. Age at time of BMT ranged from 2 to 7 yr. All patients had stable bone marrow function prior to renal transplantation. Age at renal transplant ranged from 8 to 14 yr. All three patients have been managed with conventional immunosuppression, as no patient received a kidney and BMT from the same donor source. These patients are currently 7 months to 6 yr status post-living related transplant. All have functioning bone marrow and kidney transplants, with serum creatinine levels ranging 0.6-1.2 mg/dL. There have been no episodes of rejection. One patient with a history of grade III skin and grade IV gastrointestinal-graft-vs.-host disease (GI-GVHD) prior to transplantation, had a mild flare of GI-GVHD (grade I) post-renal transplant and is currently asymptomatic. The incidence of opportunistic infection has been comparable with our pediatric renal transplant population without prior BMT. One patient was treated for basal cell carcinoma via wide local excision. Renal transplantation is an excellent option for the treatment of pediatric patients with ESRD following BMT. Short-term results in this small population show promising patient and graft survival, however long-term follow-up is needed. Pre-existing immune system impairment and bone marrow function should be taken into consideration when weighing different immunosuppressive agents for renal transplantation. Patients who have undergone renal transplantation following BMT are at high risk for opportunistic infections and malignancy, and need life-long medical surveillance.     

Kidney and liver transplantation in children with fibrocystic liver–kidney disease: Data from the US Scientific Registry of Transplant Recipients: 1990–2010

The natural history and survival of children with fibrocystic liver–kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US children with fibrocystic liver–kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver–kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re‐transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty‐nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.     

Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report

Backes AN, Tannuri ACA, de Mello ES, Gibelli NEM, de Castro Andrade W, Tannuri U. Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report. Abstract: Neoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donor–recipient serostatus, especially in relation to the Epstein–Barr virus. The patient was a two‐yr‐old female child with biliary atresia who underwent a liver transplantation from a female cadaver donor. Two adults received kidney transplants from the same donor. Nine months after transplantation, one of the adult recipients developed an urothelial tumor in the kidney graft. Imaging tests were repeated monthly in the liver‐transplanted child and revealed no abnormalities. However, one yr and two months after the transplantation, the patient developed episodes of fever. At that time, imaging and liver biopsy showed a clear cell tumor of urothelial origin in the graft and the disease was limited to the liver. The patient underwent liver retransplantation, and she is currently free of tumor recurrence. Although rare, the occurrence of tumors in the post‐transplant period from cadaver donors, without previously diagnosed tumors, is one of the many problems encountered in the complex world of organ transplantation.     

End‐stage renal disease due to ARPKD in the first months of life: Transplantation or dialysis? – Two case reports

Beil S, Drube J, Gluer S, Lehner F, Ehrich JHH, Pape L. End‐stage renal disease due to ARPKD in the first months of life: Transplantation or dialysis? – Two case reports.
Pediatr Transplantation 2010: 14:E75–E78. © 2009 John Wiley & Sons A/S. Abstract: ARPKD with renal insufficiency during the first months of life is a clinical challenge. We report on two children with ARPKD with massively enlarged kidneys requiring renal replacement therapy in early infancy. Patient 1 developed pulmonary insufficiency due to massively enlarged kidneys. At the age of six months the girl was listed for KT as “high urgency” on the Eurotransplant waiting list. A kidney from a deceased donor was pre‐emptively transplanted and simultaneous nephrectomy performed. No postoperative complications were observed, and the patient was discharged from in‐patient care 42 days after transplantation. Unexpectedly, she died at the age of one yr due to cerebral vascular spasms of unknown origin. Patient 2 was transferred at the age of three months to our clinic with life‐threatening pulmonary insufficiency. Pre‐emptive KT was not possible; therefore, bilateral nephrectomy was performed and PD begun. The boy is still doing well on PD one yr later. Pre‐emptive KT and bilateral nephrectomy followed by PD are two options for infants with ARPKD and excessive kidney enlargement. PD could be complicated and in some cases become impossible by peritoneal damage during nephrectomy. On the other hand, KT covers a high risk of infections caused by immunosuppression. The decision, which method to choose, should be driven by the individual situation of the patient and the expertise of the center.     

Renal transplant donation from a deceased cardiac graft recipient: A case for marginal donors

Abstract:  We describe a case of dual kidney transplant in a highly sensitized 18-yr-old male patient followed for chronic renal failure secondary to focal and segmental glomerulosclerosis. The donor was a six-yr-old boy who had received a cardiac transplant at the age of three months and had been treated with CNIs for over five yr. Renal biopsy performed before the transplant revealed focal sclerosis in 30–40% of the donor's glomeruli. Considering the long expected time on the waiting list for this highly sensitized boy and the absence of prohibited antigens in the donor, we decided to perform the transplant. To increase renal mass, both kidneys were transplanted. Clinical course was good but the patient developed proteinuria, which improved with ramipril. We performed two renal biopsies in the first nine months post-transplant which showed a progressive increase of the glomerulosclerosis. Despite the histology, renal function remains good 24 months after transplant. This is the first report of a transplant using a kidney from a donor who had received long-term CNI treatment. Short-term outcome is satisfactory. We suggest that marginal donors could also be considered for donation in pediatric age for selected groups of patients.     

Kidney transplants for children under 1 year of age - a single-center experience

From September 20, 1970 to October 24, 2001, we performed 46 kidney transplants in infants under 1 yr old at the University of Minnesota. This article reviews the preoperative care, surgical technique, and immunosuppression. Recipients included 16 females and 30 males; the youngest recipient was 6 wk old. The mean pretransplant height was 62.8 cm, which increased to 77 cm at 1 yr post-transplant and to 104 cm at 5 yr. We used 40 living donors (all but 1 were related to the recipient) and 6 cadaver donors. The overall actuarial graft survival was 85% at 1 yr and 70% at 5 yr. In the cyclosporine era, graft survival improved to 91% at 1 yr and 80% at 5 yr. Death with function was the most common cause of graft loss (n = 5), followed by biopsy-proven chronic rejection (n = 4), biopsy-proven recurrent disease (n = 3), and graft thrombosis (n = 2). Patient survival was 91% at 1 yr and 86% at 5 yr. In the cyclosporine era, patient survival was 100% at 5 yr and 85% at 10 yr. We concluded that an early transplant is the best treatment option for infants under 1 yr old with chronic renal failure. Whenever possible, adult living kidney donors should be used.     

Towards minimizing immunosuppression in pediatric liver transplant recipients

Abstract:  Immunosuppression regimens after liver transplantation focus mainly on preventing rejection and subsequent graft loss. However, in children, morbidity and mortality rates from infections exceed those from rejection after transplant, and immunosuppression can hinder growth, renal function, and graft tolerance. We hypothesized that early steroid withdrawal, with a primary aim of TAC monotherapy would yield no penalty in terms of rejection and graft loss, while reducing risks of infection and maximizing growth. We prospectively evaluated 64 consecutive pediatric liver transplant recipients. One yr patient/graft survival was 93/90%, respectively. At one yr post-transplant, 75.4% of patients were on TAC monotherapy. No deaths or graft losses were caused by infection. Sixty-one percent of patients had at least one episode of rejection, most within three months following transplant and 3.8% were treated for chronic rejection. One non-compliant adolescent died from chronic rejection. CMV, EBV, and lymphoproliferative disease rates were 3.1%, 5.3%, 1.8%, respectively. Pretransplant and one yr post-transplant glomerular filtration rates were unchanged. One yr improved catch-up growth was observed. We conclude that immunosuppression minimization after pediatric liver transplant yields no serious complications from rejection, and might confer advantages with respect to infection, renal function, growth, and is deserving of wider application and study.     

Successful medical treatment of EBV smooth muscle tumor in a renal transplant recipient

Belingheri M, Comoli P, Locatelli F, Baldanti F, Martina V, Giani M, Ferraresso M, Cro L, Edefonti A, Ghio L. Successful medical treatment of EBV smooth muscle tumor in a renal transplant recipient.
Pediatr Transplantation 2010: 14:E101–E104. © 2009 John Wiley & Sons A/S. Abstract: EBV is associated with various malignancies in patients with acquired or induced immune impairment. EBV‐SMT is very uncommon in immunocompromised patients, and a kidney localization has been described only anecdotally. We report the case of a 17‐yr‐old kidney transplant recipient diagnosed as having an EBV‐SMT inside the renal graft, which was successfully managed by minimizing isolated immunosuppression.     

Post‐renal transplant erythrocytosis: A case report

PTE is defined as hematocrit >51% or hemoglobin >17 g/dL after renal transplantation. Risk factors include native kidneys with adequate erythropoiesis pretransplant, smoking, renal artery stenosis, and cyclosporine treatment. We report the case of a 14‐yr‐old female kidney transplant patient, with triple therapy immunosuppression and stable graft function who developed PTE at 12 months post‐transplant with hemoglobin 17.3 g/dL, hematocrit 54.2%, stable graft function, and normotensive with normal cardiac echocardiogram and erythropoietin levels. The only risk factor found was tobacco use. As she had no spontaneous improvement, enalapril treatment was started at 19 months post‐transplant with a hemoglobin level of 17.5 g/dL and hematocrit 53%; by 23 months post‐transplant, hemoglobin lowered to 15 g/dL and hematocrit to 44.5% and continued to be in normal range thereafter. PTE is a rare condition in childhood and can be successfully treated with enalapril.     

Isolated unilateral cytomegalovirus retinitis: A rare long‐term complication after pediatric liver transplantation

Squires JE, Sisk RA, Balistreri WF, Kohli R. Isolated unilateral cytomegalovirus retinitis: A rare long‐term complication after pediatric liver transplantation. Abstract: To highlight the rare yet devastating complication of CMV retinitis in a minimally immunosuppressed patient eight yr after liver transplantation for biliary atresia. A 22‐yr‐old female status‐post deceased donor liver transplant at age 13 secondary to biliary atresia receiving single agent immunosuppression presented with acute, unilateral, profound decrease in visual acuity. The patient was diagnosed to have acute onset unilateral CMV retinitis. Retinal examination uncovered classical appearance of retinal whitening and retinal hemorrhages with extensive macular involvement. CMV retinitis can occur as a late complication following liver transplantation. Additionally, CMV retinal disease can occur in the absence of laboratory evidence of CMV infection and independent of additional clinical features suggesting CMV disease. Currently, there is no standard of care regarding screening for CMV retinitis, and thus, further research is needed to define the need for potential changes in current clinical practices and post‐transplant screening protocols.     

Long‐term remission of recurrent severe anemia as a result of parvovirus B19 infection in a pediatric renal transplant recipient

Shen Q, Xu H, Cao Q, Zhou L‐J, Xu J, Fang X‐Y, Ge J. Long‐term remission of recurrent severe anemia as a result of parvovirus B19 infection in a pediatric renal transplant recipient.
Pediatr Transplantation 2011: 15: E76–E79. © 2010 John Wiley & Sons A/S. Abstract: We studied a case of recurrent PV‐B19‐associated anemia in a renal transplant child with long‐term remission induced by baseline immunosuppression adjusted and intensive IVIG therapy. This was a 15‐yr‐old boy. Seven wk after transplantation, he experienced acute rejection, which was treated with high‐dose steroids, ATG, and plasmapheresis. Ten wk after transplantation (three wk after rejection), his hemoglobin dropped to 54 g/L and serum PV‐B19 PCR was positive. After therapy with IVIG and conversion from mycophenolate mofetil to rapamycin, anemia resolved. But the patient had fever on the fourth day of IVIG with mild pulmonary edema and rise in serum creatinine. Two months after the first course of IVIG, anemia recurred and a second course of IVIG (preadministration methylprednisolone) was given, which was followed by the resolution of anemia without side effect and recurrence two months later again. Baseline immunosuppression was adjusted with dual immunosuppression and low doses including prednisolone and tacrolimus. At the same time, monthly course of IVIG was repeated four times. Within the next 23 months, anemia did not recur and renal function remained stable. In conclusion, PV‐B19‐associated anemia can be recurrent in immunocompromised children and baseline immunosuppression should be carefully adjusted to control PV‐B19 infection.     

BK virus nephropathy complicated with meningoencephalitis after kidney transplantation

BK disease is an opportunistic infection in organ transplant recipients and patients with other cellular immunodeficiencies. To the best of our knowledge, we report the second case of BK meningoencephalitis associated with nephropathy in a kidney transplant recipient. A 15‐yr‐old boy underwent a cadaveric kidney transplant without complications; however, 11 wk after the transplantation, he was admitted to the hospital for graft dysfunction and cytopenia, which were confirmed by BK nephropathy (plasma viral replication and histological evidence). Four days after his hospital admission, he developed a high‐grade fever and headache. CSF analysis revealed pleocytosis with a positive PCR for BK virus. Reduction in immunosuppression and supportive care conducting cycles of immunoglobulin and cidofovir were successful in treating the patient. BK meningoencephalitis should be considered in kidney transplant recipients who present with signs and symptoms of meningoencephalitis.     

Allograft diabetic nephropathy may progress to end-stage renal disease

Mesangial expansion and glomerular basement membrane thickening characteristic of diabetic nephropathy recur in diabetic recipients of renal allografts from non-diabetic donors but progression to renal failure is minimally documented. Three female renal allograft recipients (aged 40, 62 and 73 yr), who developed end-stage renal disease (ESRD) due to recurrent diabetic nephropathy (two patients) and de novo diabetes (one patient) are reported. Onset of proteinuria, uncontrolled hypertension, azotemia, renal allograft pathologic findings and the need for hemodialysis were analyzed. None of the kidney donors (one cadaver, two living related) had known diabetes or perturbed glucose metabolism pre-transplantation. The three patients presented had different varieties of diabetes; type 1, type 2 and new onset diabetes after transplantation (NODAT). In each subject, proteinuria was detected by dipstick at a mean of 8.3 yr (range 8-9) post-transplantation and increased to the nephrotic range (3.7-4.8 g/day) inducing hypoalbuminemia and azotemia. A histopathologic diagnosis of allograft diabetic nephropathy was made in a mean of 11.7 yr (range 10-14), based on glomerular basement membrane thickening, nodular and diffuse intercapillary glomerulosclerosis, arteriolosclerosis, and tubular atrophy with marked tubular basement membrane thickening characteristic of advanced diabetic nephropathy. All three patients manifested uremia and resumed hemodialysis. Two patients died from sepsis within 2 months and one patient died 2.5 yr later after resumption of maintenance hemodialysis. We infer that recurrent or de novo diabetic nephropathy in renal allografts follows a clinical decade-long course irrespective of diabetes. Reports of ESRD due to allograft diabetic nephropathy (ADN) have been limited because of shorter survival of diabetic transplant recipients and few kidney biopsies performed in patients with chronic allograft dysfunction. The occurrence of allograft diabetic nephropathy in some, but not all patients, however, suggests that individual genetic variability modulates disease expression.     

Hodgkin's lymphoma after post-transplant lymphoproliferative disease in a renal transplant recipient

Lymphoid malignancies such as post-transplant lymphoproliferative disease (PTLD) are a major complication of solid organ transplantation. Hodgkin's lymphoma (HL) is not part of the typical spectrum of PTLD, but has rarely been reported as a separate complication. We report a case of HL occurring after previous PTLD in a renal transplant recipient. A 9-yr-old girl with end-stage autosomal recessive polycystic kidney disease received a cadaveric renal transplant at 1 yr of age. She developed polymorphic PTLD localized to the bone marrow at 6 yr post-transplant. She was treated with reduction of immunosuppression and alpha-interferon. No chemotherapy or anti-B cell antibody was administered. The PTLD resolved and kidney graft function remained stable. At 9 yr post-transplant, she presented again with fever of 2 wk duration, associated with enlarged lymph nodes at multiple sites. A lymph node biopsy revealed the presence of classic Reed Sternberg cells positive for CD15, CD30 and EB RNA. She was treated with standard combination chemotherapy for HL with COPP/ABV. All immunosuppressive agents were discontinued except for low dose prednisone. The patient had an excellent response, with resolution of her lymphadenopathy and maintenance of stable graft function. RS like cells have been reported in the setting of PTLD, but these cells possess an activated B cell phenotype, are EBV negative and CD15 negative. True HL following PTLD has been reported in only three previous cases, with good response to standard chemotherapy in each.     

Primary focal segmental glomerulosclerosis--long-term outcome after pediatric renal transplantation

Recurrence of the primary disease is a significant issue in pediatric renal transplantation (RTx). According to data reported by the North American Pediatric Renal Transplantation Cooperative Study, patients with focal segmental glomerulosclerosis (FSGS) as primary renal disease have a recurrence rate of 30% after the first RTx. The relative risk of an early graft loss because of recurrent disease is increased to 1.6-3.1 in pediatric patients with FSGS. In a German open multicenter study, which was initiated to investigate mycophenolate mofetil (MMF) after pediatric RTx [Transplantation 2001:71:638, Transplantation 2003:75:454], patients with FSGS were evaluated for recurrence rate, risk factors for recurrence, long-term graft function, glomerular filtration rate and transplant survival. All patients received immunosuppression with MMF, cyclosporine A and prednisone without induction therapy. Renal function and survival data for FSGS patients were compared with the results of patients with other primary renal diseases within the same study population. Among 86 patients transplanted between 1996 and 1999 eight patients suffered from FSGS as primary disease. Recurrence was diagnosed in two of the eight patients. One out of these two patients lost his graft as a result of recurrence. Risk factors such as time between diagnosis and end stage renal disease (ESRD) and age at onset did not predict recurrence. A three-year patient survival in the FSGS group was 100%, graft survival 87% vs. 97% in the non-FSGS group. Acute rejections occurred in three out of eight FSGS patients and in 37 out of 78 among the non-FSGS group. Long-term renal function, calculated using mathematical modeling based on glomerular filtration rate (GFR) data during 3 yr after RTx, was similar in FSGS patients - including a patient who had recurrence with a functioning graft - and those without FSGS. In patients with FSGS, recurring disease after RTx remains an important cause of graft loss (one of two patients in this population) even under modern immunosuppressants. Nevertheless, the immunosuppressive regimen used was associated with a similar graft survival rate and long-term renal function of FSGS patients compared with patients with other primary diseases.     

Rejection is less common in children undergoing liver transplantation for hepatoblastoma

To compare the incidence of acute histologically proven rejection in children who have had a liver transplant for hepatoblastoma with a control group of children transplanted for biliary atresia (EHBA). A retrospective case notes based study was performed. Twenty patients were identified with hepatoblastoma who were transplanted at a single unit between 1991 and 2008. These were matched as closely as possible for age, gender, year of transplant and type of immunosuppression used to the control group transplanted for biliary atresia (n = 60). There was a significant decrease in rate of acute rejection as assessed by the rejection activity index (RAI) in the hepatoblastoma group (75% vs. 50%, respectively, p < 0.04). Chronic rejection was rare in both groups, but twice as common in the biliary atresia group. Equal levels of immunosuppression were achieved in both groups. Renal function was noted to be reduced one yr post‐transplant in both groups, as previously reported. A modified immunosuppression regimen could be considered in children with hepatoblastoma undergoing liver transplantation.     

Bone marrow engraftment and associated dermatologic sequelae in a three‐yr‐old after liver transplantation

We present a case of a three‐yr‐old child with a history of multisystem Langerhans cell histiocytosis treated with systemic chemotherapy, who developed progressive liver failure and received an orthotopic split liver transplant while continuing on chemotherapy. One month following transplant, he developed acute graft‐vs.‐host disease of the skin and gastrointestinal tract. Peripheral blood chimerism studies post‐transplant demonstrated an increasing predominance of donor lymphocytes and granulocytes. Shortly after, the patient developed vitiligo, and two yr after transplantation, the patient developed skin manifestations of psoriasis. We discuss and review the current literature, which demonstrates that chimerism following liver transplantation is rare and in our patient may be related to his profound immunosuppression around the time of liver transplant as well the development of acute graft‐versus‐host disease. While autoimmune disease can occur after solid organ and stem cell transplant, our patient developed skin manifestations of autoimmunity after liver transplantation, which is also rarely described.     

Radiotherapy‐induced hyperthyroidism in a cystinotic kidney transplant patient with Hodgkin lymphoma

Guzzo I, Di Zazzo G, Grossi A, Greco M, Dello Strologo L. Radiotherapy‐induced hyperthyroidism in a cystinotic kidney transplant patient with Hodgkin lymphoma.
Pediatr Transplantation 2011: 15:e56–e59. © 2010 John Wiley & Sons A/S. Abstract: Hypothyroidism is a well‐known complication of cystinosis. PTLD incidence in pediatric renal transplant population ranges between 1 and 4.5%. We describe the case of a young cystinotic patient who developed hyperthyroidism after radiotherapy for Hodgkin lymphoma. He is a 23‐yr‐old male who was diagnosed with cystinosis at the age of two. He developed renal failure and other extrarenal complications but never presented hypothyroidism. At the age of 12, he received a successful kidney transplant from a cadaveric donor. Two yr later, EBV‐positive Hodgkin lymphoma was diagnosed and chemotherapy and radiotherapy were administered. He achieved remission. Eight yr later, autoimmune hyperthyroidism secondary to previous radiation was detected, and he slowly became symptomatic. Clinical symptoms and laboratory data spontaneously normalized. This is the first case of a cystinotic patient developing hyperthyroidism. Thyroid disorders, especially hypothyroidism, have been reported in association with neck irradiation. Hypothyroidism would have been considered to be a late complication of cystinosis and not a consequence of radiotherapy. Thyroid hormones, clinical examination, and history evaluation for thyroid dysfunction should be periodically monitored after neck radiotherapy. The thyroid should always be excluded from the irradiation fields. Multidisciplinary interaction in difficult cases should be encouraged.