Pregnancy outcome at 24-31 weeks' gestation: neonatal survivors.

All surviving babies of less than 32 weeks'' gestation born to mothers resident in the Northern region in 1983 were assessed. No baby was lost to follow up, and 230 long term survivors were assessed when 2 years old, by a single observer. Among 248 neonatal survivors, severe disability was present in 10 of the 37 at 24-27 weeks'' gestation (27%) and in 18 of the 211 at 28-31 weeks'' gestation (9%). On average the surviving babies required 27 days and seven days of intensive care nursing respectively. Babies who were ventilated for more than two weeks and babies who had neonatal fits had a poor prognosis. Because of postneonatal deaths only 20 of the 230 long term survivors of less than 32 weeks'' gestation were severely disabled (9%). Comparable data were obtained for all babies weighing 1500 g or less at birth, irrespective of their gestational age. Severe disability was seen in eight of the 49 neonatal survivors (16%) who had weighed 500-999 g at birth, and in 17 of the 171 survivors (10%) who had weighed 1000-1499 g. The 10 neonatal survivors who had weighed exactly 1500 g at birth were all well. Only 18 of the 212 long term survivors (8%) who had weighed 1500 g or less at birth were severely disabled. Because the period of gestation is the only variable known to the obstetrician before delivery, and disability correlates better with gestation than birth weight, future studies should concentrate on relating morbidity, mortality, and the cost of neonatal care to gestation rather than weight at birth.     

Long-term outcome of deceased donor renal transplantation in pediatric recipients: A single-center experience from a developing country

Kute VB, Trivedi HL, Vanikar AV, Shah PR, Gumber MR, Patel HV, Munjappa BC, Modi PR, Gera DN. Long‐term outcome of deceased donor renal transplantation in pediatric recipients: A single‐center experience from a developing country Abstract: RTx is best treatment for children with ESRD. Data scarcity on DDRTx outcome in children prompted us to review our experience. This study was undertaken to evaluate patient/graft survival, function vis‐a‐vis SCr, rejection episodes, and mortality in DDRTx performed in 37 children between 1998 and 2011. The most common recipient diseases leading to ESRD were congenital anomalies of kidney and urinary tract (48.6%) and chronic glomerulonephritis (18.9%). Mean recipient age was 13.8 ± 3.1 yr; 67.5% (n = 25) were men. Mean donor age was 38.8 ± 18.6 yr; 48.5% (n = 18) were men. Mean dialysis duration pre‐transplantation was 15.5 ± 3.5 months. All recipients received r‐ATG, and triple immunosuppression. Over a mean follow‐up of 3.93 ± 3.5 yr, patient and graft survival rates were 72.9% (n = 27) and 83.7% (n = 31), respectively, with a mean SCr of 1.1 mg/dL; 21.6% (n = 8) of patients had acute rejection episodes; 24.3% (n = 9) of patients had DGF. A total of 27% (n = 10) patients died, mainly owing to infections (n = 6) and cardiovascular disease (n = 3). DDRTx is a viable option for children and achieves acceptable graft function with patient/graft survival over long‐term follow‐up, encouraging use of this approach.     

Late effects of treatment for germ cell tumors during childhood and adolescence

PURPOSE: To evaluate the long-term sequelae of treatment for malignant germ cell tumors (GCT) during childhood and adolescence. PATIENTS AND METHODS: Of 128 patients treated for GCT at St. Jude Children's Research Hospital between 1962 and 1988, 73 are long-term survivors (continuously disease-free for > or =5 years after diagnosis), with a median follow-up of 11.3 years). Survivors' ages at diagnosis ranged from birth to 18.3 years (median, 9.2 years); 64% (47 patients) were female. Initial surgical resection was followed by observation for stage I germinomas (n = 2), testicular tumors (n = 13), and selected cases of ovarian or sacrococcygeal tumors (n = 2), and by radiation therapy (RT) for patients with stage II to III germinoma (n = 8). The remaining 48 patients received postoperative chemotherapy (vincristine, dactinomycin, and cyclophosphamide [VAC] +/- doxorubicin, 1962 to 1978; VAC and/or cisplatin, vinblastine, and bleomycin [PVB], 1979 to 1988). RT was added to the chemotherapy for 21 patients. Late complications involving various organ systems and their relationship to treatment were evaluated. RESULTS: More than two-thirds of long-term survivors (n = 50) had at least 1 complication, and half (n = 38) had > 1 organ system affected. The systems most often involved included the musculoskeletal (41% of survivors), endocrine (42%), cardiovascular (16% excluding those who had only abnormal chest radiograph), gastrointestinal (25%), genitourinary tract (23%), pulmonary (19%), and neurologic (16%) systems. High-frequency hearing loss occurred in 58% (11 of 19) of patients treated with cisplatin. Musculoskeletal, gastrointestinal, and urinary tract abnormalities were most frequent in patients whose treatment included RT. CONCLUSIONS: A high frequency of late effects after treatment for pediatric GCT, particularly in patients who received RT, was demonstrated. Treatment sequelae could be anticipated from the intensity and type of therapeutic modalities. Treatment-directed screening evaluations may improve quality of life in long-term survivors of pediatric GCT through timely identification of sequelae that can be prevented or ameliorated.     

Ultrasonography for thyroid screening after head and neck irradiation in childhood cancer survivors

We prospectively used ultrasonography to detect thyroid abnormalities in 96 long-term survivors of childhood cancer, who received head and neck radiation therapy at a median age of 8.9 years. The median time interval since irradiation was 10.8 years (range 5.6–22.8 years). Most survivors of leukemia received 24 Gy cranial irradiation for central nervous system prophylaxis; patients with solid tumors received between 20 and 66 Gy (median 37.5 Gy). The total evaluation included clinical history, physical examination, thyroid function tests, and thyroid ultrasonography; radionuclide scans were performed in patients whose abnormalities persisted on subsequent ultrasound exams. Clinical history and physical examination revealed thyroid abnormalities in 14 patients (15%), but ultrasound detected abnormalities in 42 patients (44%). These findings included inhomogeneity (n = 29), cysts (n = 15), and nodules (n = 22) and occurred in nearly half of patients treated with 15 Gy or more directly to the thyroid gland. Radionuclide scans confirmed the presence of thyroid nodules in 13 of 15 patients with ultrasonographic evidence of nodules. Six patients had thyroid neoplasia, including one case of papillary carcinoma. All patients with neoplasia had nodules demonstrated on ultrasonography. Our experience suggests that in childhood cancer survivors, ultrasonography is a sensitive, affordable, and noninvasive means of detecting subtle parenchymal abnormalities. We recommend thyroid ultrasonography for childhood cancer survivors who recieved head and neck irradiation. A baseline study should be obtained within 1 year of completion of therapy. The frequency of subsequent examinations should be based on the radiation dose and the patient's age at the time of irradiation. © 1997 Wiley-Liss, Inc.     

Do infants with stage IV-S neuroblastoma need treatment?

The study reports the results of treatment in 31 infants under age 12 months with stage IV-S neuroblastoma diagnosed between 1976 and 1979. All had small or undetected primary tumours associated with disease in one or more of the following sites: liver, skin, or marrow. The primary site was left adrenal (in 16), and other areas included paravertebral, mediastinum, and pelvis (in 5); in 3 the primary site could not be found. Distant disease was found in the liver (in 29), marrow (in 16), and skin (in 8). Other sites affected were pancreas, pleura, peritoneum, and regional nodes. Treatment varied according to the clinical course of the disease; most patients had very little. In 19 primary tumour was resected, in 21 the liver was irradiated with a median dose of 450 rad, and 15 received chemotherapy in courses varying between 1 month and 1 year. Nine patients had resection of the primary tumour as their only treatment and all survive; a total of 16 patients had sites of disease which regressed spontaneously. Four of 31 patients died in the first 2 months despite vigorous measures, all from some complication of the disease or its treatment. The projected 2-year survival rate is 87%. Children with this ''special'' pattern of widespread neuroblastoma fare well with little or no treatment unless early complications develop. In this study none died of late progression of their disease.     

High-grade astrocytoma in very young children

BACKGROUND: High-grade astrocytomas are rare in young children, but have been reported to have a better prognosis than similar tumors in older patients. PROCEDURE: We retrospectively reviewed the clinical characteristics, survival, and long-term sequelae for patients younger than 3 years old with high-grade astrocytoma, treated at a single institution between 1984 and 2005. RESULTS: Sixteen patients were included. Histology included anaplastic astrocytoma (n = 9), glioblastoma multiforme (n = 5), and malignant glioma (n = 2). All patients underwent biopsy or resection, followed by chemotherapy. Six patients received scheduled irradiation and six were irradiated at the time of disease progression. Ten patients are alive at a median follow-up of 11.6 years (range, 1.7-21.6 years). 5-year overall survival (OS) was 66.3% (SE 12.2%), and 5-year event-free survival (EFS) was 28.6% (SE 12.1%). Age at diagnosis was a significant predictor of the hazard of death in a Cox model (HR 2.871, 95%CI 1.015-8.123). Gender and histology did not predict OS or EFS. Trends toward improved OS were detected for patients with hemispheric tumors and those undergoing complete resection. All evaluable survivors (n = 9) had some neurocognitive impairment, with estimated overall cognitive ability ranging from significantly delayed to average; all survivors attending school (n = 5) performed below grade level on achievement testing. Seven of nine evaluable survivors had endocrine dysfunction. CONCLUSIONS: Young children with high-grade astrocytoma have better long-term overall survival than older patients, but recurrence is common, and most children require irradiation. Long-term complications are frequent and often severe.     

Insufficient growth hormone secretion is associated with metabolic syndrome after allogeneic stem cell transplantation in childhood

The aim was to evaluate whether the metabolic syndrome associates with other endocrinopathies observed after allogeneic stem cell transplantation (SCT) in childhood. Thirty-one SCT long-term survivors, transplanted for leukemia (n=26) or nonmalignant hematologic diseases (n=5) were evaluated by oral glucose tolerance test and assessment of serum lipids at a median age of 15 (range 7 to 34) years. Hyperinsulinemia, hypertriglyceridemia, and abdominal obesity were required for the diagnosis of metabolic syndrome. Growth hormone (GH) secretion was evaluated either with GH releasing hormone and arginine (n=14), clonidine (n=15), or insulin-tolerance (n=2) test. A GH peak level of <20 mU/L was considered insufficient. The thyroid and gonadal functions were assessed. Twelve patients (39%) had metabolic syndrome. Nine out of 12 (75%) patients with metabolic syndrome had insufficient GH response in provocative testing as opposed to 6/19 (31%) of those without it (P=0.02). No difference was observed in thyroid or gonadal function between patients with versus without metabolic syndrome. In conclusion, metabolic syndrome is frequently associated with insufficient GH secretion in the SCT long-term survivors. This should implicate a close follow-up of the metabolic parameters in SCT patients with either frank GH insufficiency or signs of inadequate GH response in provocative testing.     

Pulmonary function in childhood leukaemia survivors

Little is known of pulmonary function in survivors of acute lymphoblastic leukaemia (A.L.L.); this is despite the fact that some drugs used, most notably methotrexate, have well-recorded pulmonary toxicity, and the most common infections during therapy in most series are of upper and lower respiratory tract. As part of a survey of all cancer survivors attending the Royal Hospital for Sick Children in Edinburgh, 38 leukaemic patients, who had completed treatment 3 months to 14 years 6 months (median 6 years and 8 months) prior to survey were assessed with regards to their respiratory status. Each patient completed a questionnaire and had spirometry and lung volumes measured; 30 patients additionally had transfer factor for carbon monoxide (TCO) measured. There were 21 children, 11 adults, and 6 patients in the age range between child and adult. Of the 26 adults and children studied with complete data available, 17 (65%) had one or more low values for vital capacity (VC), total lung capacity (TLC), residual volume, or TCO. Mean VC, TLC and TCO were significantly lower than the mean of the predicted values (P less than .001). Gas transfer per unit lung volume (KCO) was normal in all cases. Few patients had symptoms of respiratory disease. There was an increased incidence of low TCO in patients diagnosed under 8 years of age. Impairment of lung growth could be a contributing factor to the observed abnormalities in pulmonary function. Impairment of pulmonary function in survivors of A.L.L. may be of significance for them in later life.     

Multicystic kidney dysplasia

The clinical course of 48 children (27 boys and 21 girls) with multicystic kidney dysplasia was analysed retrospectively. The patients were seen and treated at the Children's Hospital of Medical School Hannover between 1976 to 1989. There was no familial occurrence of the disease, yet in eight families various other renal diseases could be observed. In 20 patients the diagnosis of multicystic renal dysplasia was presumed by prenatal sonographic examination, in the other patients the diagnosis was established at the age between 1 day to 12 years. The first manifestations were palpable abdominal mass (n = 16), urinary tract infection (n = 4), casually because of a sonographic evaluation for other reasons (n = 4) and vomiting (n = 2). Associated malformations were found in 18 patients: cardiac malformations (n = 6), dysplasia of the other kidney (n = 5), ureter obstruction of the other kidney (n = 3), horseshoe kidney (n = 1) and others (n = 3). There was no hypertension and serum creatinine levels were normal in those children, who did not suffer from associated malformations of the other kidney. The multicystic kidney was removed operatively in 42 patients at the age of 3 days to 9.5 years (median 4 weeks). The prognosis of multicystic kidney dysplasia depends on associated renal and other malformations.     

儿童肾移植的远期预后

目的探讨儿童肾移植的长期存活情况及其对生长发育、就业、婚育的影响。方法以27例肾移植5年以上的儿童受者为研究对象作回顾性分析。结果本组受者术后1、3、5、10年人存活率分别为100%、92.6%、85.8%、68.6%,1、3、5、10年肾存活率分别为96.3%、89.2%、82.6%、66.1%,均采用以环孢素A或他克莫司为基础的二联或三联免疫抑制方案,移植后第1年体重增加4～10kg,女性增高0～2cm,男性增高2～5cm。术后接受初中以上教育者占44.4%。现存活的20例受者年龄20～39岁,男性就业率46.2%(6/13),女性57.2%(5/7),已婚12例(60%),男性生育5例,女性生育3例,子女均健康,服药不依从比例占30%,主要并发症包括高血压(40%)、高血脂(30%)、牙龈增生(30%)、多毛(25%)、肝功能损害(20%)、单纯疱疹(15%)、糖尿病(5%)、白内障(5%)。死亡7例,死亡年龄21～39岁,分别死于感染(28.6%)、肝功能损害(28.6%)、心衰(42.8%)。结论肾移植是终末期肾功能衰竭儿童的最佳治疗方法,应重视受者的不依从行为,合理使用免疫抑制剂。呼吁社会各界对移植受者给予就业等多方面的支持。     

A study of children and adolescents referred to a rapid diagnosis breast clinic.

AIM: Children and adolescents referred to a rapid diagnosis breast clinic were analysed to study the conditions these patients were referred with, their management, and the final outcome. METHODS: All new patients aged 18 or under seen in a breast clinic over a 39-month period to June 2004 were identified and their case notes were reviewed. RESULTS: Among 6930 new referrals, 88 (1.3 %) were aged 18 or under and 86 patients were included in the study (median age 16 yrs, range 8 - 18 yrs). Five patients were aged less than 12 and were referred with physiological breast buds. In girls aged 13 - 18 years, most were referred for an apparent palpable abnormality. Thirty percent had a family history of breast carcinoma and 35 % were on the oral contraceptive pill. Thirty-three patients (38 %) had a clinical examination only. Others, in addition to the clinical examination, had an ultrasound (US) (n = 24, 28 %), fine needle aspiration cytology (FNAC)/core biopsy (CB) (n = 5, 6 %) or both US and FNAC/CB (triple assessment) (n = 24, 28 %). No malignant disease was found. No diagnostic biopsies were performed. Only 5 patients had surgery, four for fibroadenoma and one for sebaceous cyst. CONCLUSIONS: Patients aged 18 years or under do not constitute a major workload problem for rapid diagnosis breast clinics. Malignant disease remains rare. A significant proportion of patients require only clinical examination and reassurance. The diagnosis can usually be made without open biopsies and less invasively using FNAC or CB.     

Risk of Progressive Kidney Damage after Acute Leukemia

ABSTRACT. In order to evaluate potential long-term renal sequelae of childhood leukemia, we studied 62 consecutive patients successfully treated for acute leukemia in 1971–83. At the time of this follow-up study they had been off therapy for 1–9 years and they were all in complete remission. Relative renal length was measured from X-ray films of intravenous pyelograms. Median relative renal length prior to the start of chemotherapy was + 1.0 SD (range −1.5 to + 4.0, n = 35), at discontinuation of therapy + 0.5 SD (range −1.5 to + 2.2, n = 22), and at follow-up −0.3 SD (range −3.9 to + 2.6, n = 61). The mean calculated decrease in relative kidney size was 0.1 SD unit per year during the follow-up time. The median glomerular filtration rate was 110 ml/min/1.73 m² (range 70 to 164). Six of 60 patients had glomerular filtration rates below 85 ml/min/1.73 m². Three patients had some evidence of tubular dysfunction documented by increased excretion of urinary amino acids and/or beta-2-micro-globulin or by reduced concentrating capacity. In spite of these abnormalities, we conclude that in most long-term survivors of childhood leukemia renal size and function are relatively well preserved. However, slightly reduced glomerular filtration rates in some patients indicated renal damage. A longer follow-up time is needed to find out whether the decrease in relative renal length is still continuing.     

Papillary thyroid carcinoma: Demographics,treatment, and outcome in eleven pediatric patients treated at a single institution

We describe 11 cases (8 females, 3 males) of papillary thyroid carcinoma in children treated at St. Jude Children's Research Hospital over a 33-year period, and review the literature. Ages ranged from 7–25 years (median, 16 years). Six patients had primary papillary thyroid carcinoma. Five patients had secondary papillary thyroid carcinoma after treatment of Hodgkin's disease (n = 2), acute lymphoblastic leukemia (n = 2), and neuroblastoma (n = 1) with chemotherapy and cervical radiation. The typical presentation was either cervical lymphadenopathy or a thyroid mass of short duration. Treatment consisted of thyroidectomy, cervical lymph node dissection, and postoperative thyroid hormone replacement (n = 11), parathyroid reimplantation (n = 1), ¹³¹I ablation (n = 4), external-beam irradiation (n = 1), and chemotherapy with doxorubicin (n = 1) or carboplatin and topotecan (n = 1). Nine patients are alive without evidence of disease 3.0–22.4 years from diagnosis. One patient has persistent but stable disease 17.3 years after diagnosis. One patient relapsed with metastatic lung disease 0.8 years after the initial diagnosis. He continues to do well after a brief but unsustained complete radiographic remission of disease to combination chemotherapy with carboplatin and topotecan. Our review supports excellent long-term outcome for primary or secondary papillary thyroid carcinoma in pediatric patients, although complications may require close follow-up in a multidisciplinary setting. Med. Pediatr. Oncol. 28:433–440, 1997. © 1997 Wiley-Liss, Inc.     

Comparison of Tc-99 measurement of glomerular filtration rate vs. calculated creatinine clearance to assess renal function pretransplant in pediatric patients undergoing hematopoietic stem cell transplantation

We compared the results of Tc-99 evaluation of glomerular filtration rate (GFR) vs. the calculation of the creatinine clearance (CCrC) as a predictor for the development of renal insufficiency in pediatric patients following hematopoietic stem cell transplantation (HSCT). We reviewed 95 consecutive patients receiving autologous (n = 37) or allogeneic (n = 58) HSCT at Children's Memorial Hospital between January, 1995 and February, 1998. Diagnoses included leukemia (n = 43), solid tumor (n = 27), bone marrow failure syndrome (n = 12), non-malignant disease (n = 8), CNS tumors (n = 5) and immunodeficiency (n = 3). Tc-99 GFR was compared with a calculated creatinine clearance derived from the Schwartz formula (CCrC) prior to HSCT. These measures of renal function were compared with the patient's subsequent clinical course to determine if patients who developed renal insufficiency of sufficient magnitude as to require continuous veno-venous hemofiltration (CVVH) or dialysis, could have been identified. Overall comparison of the two methods of evaluation of renal function showed low correlation with values obtained by CCrC, which were consistently higher in most patients (r-value 0.01 in the regression analysis and a p = 0.08 95% CI -24.15 to 1.48). When stratified for age, correlation between the two methods was excellent only in children younger than 5 yr of age p = 0.02 95%, CI 0.032-0.49). Eleven patients required therapy with CVVH or dialysis but neither CCrC nor Tc-99 GFR prior to transplant predicted this event. Patients who received TBI were statistically more prone to develop renal insufficiency than those without TBI (p < 0.0001, 95% CI 0.25-0.008). Neither the Tc-99 GFR nor the CCrC was predictive of the development of renal insufficiency in HSCT patients as the majority of patients who required dialysis had normal Tc-99 GFR prior to transplant. The characteristics found in the patients who developed renal insufficiency and required dialysis include: the use of total body irradiation as part of the transplant-conditioning regimen (p < 0.0001) and the use of continuous infusion CSA (p = 0.04).     

Heart transplantation for dilated cardiomyopathy.

Between 1988 and 1994, 23 patients underwent heart transplantation for dilated cardiomyopathy. The age of the 13 boys and 10 girls was from 8 months to 16 years (mean 7.1 years). Selection criteria included failure to thrive despite maximal antifailure treatment and/or intravenous inotrope dependence. The aetiology of cardiomyopathy was idiopathic (n = 13), congenital (n = 3), anthracycline induced (n = 4), Barth''s syndrome (n = 1), and maternal systemic lupus erythematosus (n = 2). The waiting period of heart transplantation ranged from one day to 147 days (mean 22 days). Maintenance immunosuppression included cyclosporin, azathioprine, and prednisolone. Follow up after transplantation was from one month to 62 months (median 27 months) with a mean actuarial survival of 95% at one year and 87% at three years. Four patients developed coronary artery disease, one of whom died as a consequence 15 months after heart transplantation. Heart transplantation has emerged as an acceptable therapeutic option, at least in the short term, for patients with dilated cardiomyopathy.     

Risk of progressive kidney damage after acute leukemia

In order to evaluate potential long-term renal sequelae of childhood leukemia, we studied 62 consecutive patients successfully treated for acute leukemia in 1971-83. At the time of this follow-up study they had been off therapy for 1-9 years and they were all in complete remission. Relative renal length was measured from X-ray films of intravenous pyelograms. Median relative renal length prior to the start of chemotherapy was +1.0 SD (range -1.5 to +4.0, n = 35), at discontinuation of therapy +0.5 SD (range -1.5 to +2.2, n = 22), and at follow-up -0.3 SD (range -3.9 to +2.6, n = 61). The mean calculated decrease in relative kidney size was 0.1 SD unit per year during the follow-up time. The median glomerular filtration rate was 110 ml/min/1.73 m2 (range 70 to 164). Six of 60 patients had glomerular filtration rates below 85 ml/min/1.73 m2. Three patients had some evidence of tubular dysfunction documented by increased excretion of urinary amino acids and/or beta-2-microglobulin or by reduced concentrating capacity. In spite of these abnormalities, we conclude that in most long-term survivors of childhood leukemia renal size and function are relatively well preserved. However, slightly reduced glomerular filtration rates in some patients indicated renal damage. A longer follow-up time is needed to find out whether the decrease in relative renal length is still continuing.     

Body composition and bone mass in survivors of childhood cancer

BACKGROUND: The number of survivors of childhood cancer has increased. Several studies in children and adults have shown relationships between lean mass (LM), fat mass (FM), and bone mineral content (BMC). The objective of the study was to examine the association between body composition and bone mass in young survivors of childhood cancer. METHODS: Sixty-eight postpubertal participants (31 females and 37 males) aged between 15.5 and 27 years who were at least 5 years after completion of treatment for leukemia (n = 30), lymphoma (n = 28), or solid tumors (n = 10) were studied. Anthropometry was performed and dual energy X-ray absorptiometry (DXA) was used to assess BMC in the total body (T) and lumbar spine (S), FM, and LM. RESULTS: There were no observed differences in age or time for cessation of treatment. Body mass index (BMI) was a strong determinant of bone mass in both sexes. TBMC correlated positively with LM (males r = 0.9 and females r = 0.76; P < 0.0001, respectively) and with FM (r = 0.54; P < 0.01 in males and r = 0.8; P < 0.00001 in females). SBMC correlated with LM in both sexes (in males r = 0.77 and in females r = 0.64; P < 0.0001, respectively) but only in females, SBMC also correlated positively with FM (r = 44 P = 0.03). There were no differences between patients who received radiation and those who did not. CONCLUSIONS: The associations between bone mass and body composition differ by sex and skeletal site, however, they are similar in survivors of childhood cancer and compared to healthy individuals during growth. Further prospective research is needed in cancer survivors to determine the long-term effect of anti-cancer therapy on body composition and bone mass.     

Computerized tomography of the brain in children with chronic renal failure

We performed computerized tomography (CT) of the brain in 22 patients, 2 to 18 years of age, with stable chronic renal failure (n = 6), on dialysis (n = 14) and after renal transplantation (n = 2). None suffered from a systemic disease known to affect the central nervous system (CNS) and none had overt CNS dysfunction at the time of the CT examination. The most striking pathological finding was brain atrophy, which was present in 13 patients (59%). In two patients cortical infarcts were present and one patient was found to have a hypodense area in the basal ganglia. The brain atrophy could not be related to the type of basic renal disease, the age of the patient, corticosteroid dosage, the duration of the renal failure or the presence of hypertension However, the mode of treatment, i.c. hemodialysis, seemed to be a risk factor. We assume that metabolic derangements and/or the accumulation of toxic substances due to the uremic state may be responsible for the brain atrophy in young patients with CRF. Recurrent osmotic changes of the brain during hemodialysis may aggravate the process of brain atrophy.     

Familial Hashimoto's thyroiditis with kidney impairment.

A 12 year old boy and his two sisters with Hashimoto''s thyroiditis and renal impairment were studied. Three generations of this family had autoimmune thyroid disease: Graves'' disease was diagnosed in the first generation, and the second and third generations had thyroid enlargement with abnormal thyroid function and immunological abnormalities. The disease in this family could not be explained simply by the types of human leucocyte antigens found. Renal disease in autoimmune thyroid disease is uncommon, treatment difficult, and the prognosis unknown. The proteinuria disappeared in all three children during the three and a half years of follow up, which implies that the renal impairment may be transitory in some patients.