Acute renal failure due to obstruction in Burkitt lymphoma

 Acute renal failure in Burkitt lymphoma is commonly the result of tumor lysis syndrome. We present a 15-year-old boy who developed hypertension, seizures, and acute renal failure due to extrinsic compression of the bladder and ureters by a large retrovesical Burkitt lymphoma. The causes of acute renal failure in Burkitt lymphoma and the incidence of acute urinary obstruction in this disease are reviewed. Received: 18 May 1998 / Revised: 30 June 1998 / Accepted: 1 July 1998     

Intestinal perforation secondary to intestinal Burkitt lymphoma

Introduction and importanceSmall intestinal perforation in patients with Burkitt lymphoma is extremely rare. We present the first report of such a case.Case presentationA 53-year-old woman was admitted with abdominal pain and vomiting. Abdominal examination revealed rigidity and tenderness in the upper abdomen.Computed tomography scan showed thickening of the wall of the jejunum, intra-abdominal free gas, and ascites; the patient was diagnosed with small intestinal perforation, and underwent emergency surgery on the same day. Laparoscopic findings were a 50 mm jejunal perforation and perforation in the transverse mesocolon. A partial jejunal resection of the perforated area, partial transverse colectomy, temporary colostomy, and intra-abdominal drainage were performed. Histological examination showed diffuse infiltration of medium-sized atypical lymphocytes in the perforated area, exhibiting a “starry sky” appearance.Immunostaining results showed that the atypical lymphocytes were CD20 and virtually 100% Ki-67 positive, and CD56, CD30, and EBER negative. The lesion was identified as Burkitt lymphoma (BL). The postoperative course was favorable. On postoperative day 18 the patient began chemotherapy through the hematology department. Currently, the patient is in remission.Clinical discussionThe majority of the malignant lymphomas occurring in the digestive tract are identified in the stomach; over 90% are B-cell lymphomas and mucosa-associated lymphoid tissue lymphoma Nakamura et al. BL originating from the small intestine accounts for only about 9%.ConclusionThe incidence of BL in the small intestine is low. Pretreatment BL can lead to bowel perforation. Prompt involvement of the hemato-oncologist after definitive diagnosis, and commencing chemotherapy as early as possible after surgery, are thought to improve prognosis.     

Acute renal failure due to sarcoid granulomatous infiltration of the renal parenchyma

A 56-year-old female with evidence of multisystem involvement due to sarcoidosis presented with acute renal failure. Percutaneous renal biopsy revealed sarcoid granulomatous infiltration of the renal parenchyma. Steroid therapy resulted in a prompt improvement of renal function. A review of the literature has disclosed 16 similar cases. Pertinent clinical and laboratory data and treatment regimens are discussed.     

同种肾组织移植治疗慢性肾功能衰竭性贫血的实验研究

以Ｗｉｓｔａｒ雄性大鼠为受体，建立慢性肾功能衰竭动物模型，将鼠婴肾组织声多点植入受体双侧后肢皮下和筋膜下。结果表明，３０天后移植物的体积由１ｍｍ＾３增至４ｍｍ＾３大小，表面血管网丰富；光镜下见肾小球、肾小管结构正常。促红细胞生成素（ＥＰＯ）着色颗粒主要分布在肾小球区，移植组着色程度明显增高。血红蛋白和４促红细胞生成素随移植的时间延长而逐渐升高，实验结果提示，此方法有可能为治疗慢性肾功能衰竭性贫血提     

Acute renal failure due to malignant lymphoma infiltration

We present two cases of renal lymphoma revealed by acute renal failure (ARF), which remains a rare clinical entity. Case 1 was a 29-year-old man with an ARF. The diagnosis was a primitive kidney immunophenotype B lymphoma. The patient died after three courses of chemotherapy due to rapid spread lymphoma. The second case was a high-grade renal lymphomatous infiltration, with an unusual computer tomography image of two large kidneys compressing the stomach. Death happened early before initiating therapy. In both cases the diagnosis has been established by renal pathology. Early diagnosis is a key component of therapeutic success, however, the rapid spread of lymphoma worsened renal and vital prognosis.     

Burkitt lymphoma presenting as colonic ischemia and overwhelming sepsis

Lymphomas may occasionally present as acute surgical emergencies in children, most commonly because of obstruction, intussusception, or direct bowel invasion. We present the case of a 14-year-old adolescent boy who presented with septic shock and was found to have necrosis of the cecum because of a Burkitt lymphoma. The cause of the ischemia appeared to be mesenteric infiltration and subsequent vascular compromise of the bowel. We discuss the management of this patient and present a review of the literature.     

Chronic renal failure in Iranian children

We investigated chronic renal failure (CRF) in 166 Iranian children (95 boys and 71 girls) from July 1991 to June 1999. The mean age at onset of CRF was 7.9±4.5 years. The most common cause of CRF was congenital urological malformations (78 cases). The second most common cause of CRF was hereditary nephropathy (21%). Glomerular diseases accounted for only 10% of children who later went on to develop renal failure. High rates of cystinosis and primary hyperoxaluria were seen, and these elevated rates could be due to a high prevalence of parental consanguinity. Eighty-six patients required renal replacement therapy, of whom the majority underwent hemodialysis. The prevalence of primary reflux as a cause of CRF was high compared with reports from western countries. Earlier diagnosis and management of urinary tract infections in this group could reduce the prevalence of reflux as a cause of CRF in this population. Received: 15 May 2000 / Revised: 2 October 2000 / Accepted: 5 October 2000     

Multiple endocrine neoplasia type 1 in end-stage renal failure

A 59-year-old woman with chronic renal failure due to type 2 diabetes mellitus (DM) is presented. Her father and a brother had a history of brain tumor. Her blood urea nitrogen and serum creatinine levels were 102mg/dl and 4.5mg/dl, respectively. Her serum Ca²⁺ and Pi were within the normal range (9.4mg/dl and 5.4mg/dl, respectively). Her intact parathyroid hormone (PTH) level was 1730000pg/ml. A ^99mTc-methoxy-isobutylisonitrile scintigraphy showed high uptake in three parathyroid glands. A magnetic resonance image showed microadenoma in the pituitary gland. The serum gastrin level was high. Genetic examination revealed a mutation of the MEN1 gene (894–9 G A). From these findings, she was diagnosed with multiple endocrine neoplasia (MEN) type 1. Subsequently, a parathyroidectomy was performed successfully, a parathyroid gland was transplanted to her right forearm, and her serum Ca²⁺ level was controlled at 8.5–9.0mg/dl. It is very important to identify MEN1 if an end-stage renal disease (ESRD) patient has hyperparathyroidism with multigland involvement. Examination of the MEN1 gene may be valuable to make an accurate diagnosis and choose the appropriate therapy in some ESRD patients with hyperparathyroidism.     

Chronic renal failure in pediatrics 1996

 The Nephrology Branch of the Chilean Pediatric Society has greatly influenced the development of government health plans regarding the management and care of patients under 18 years with chronic renal failure (CRF). In order to assess the status of children with CRF in Chile up to 1996, a questionnaire was sent to all pediatric nephrologists in charge of those children. The total sample was of 227 patients under 18 years, giving a national prevalence of 42.5 and an incidence of 5.7 per million inhabitants; of these patients, 50.7% were male, 58.6% over 10 years and 15% younger than 5 years. The most frequent etiologies of CRF were: obstructive uropathy, 18.1%; hypo/dysplasia, 16.7%; reflux nephropathy, 16.7%; and glomerulopathies, 16.3%. Although 48% of patients were on conservative medical treatment, 42.2% of these were in end-stage renal disease, 22.9% were on dialysis, and 29.1% had undergone renal transplantation. Of the dialysis group, 75% were on peritoneal dialysis. Of the transplanted children, 78.8% had normal renal function, but 16.7% returned to dialysis. Three-year graft survival and patient survival were 68% and 94%, respectively. Received: 19 January 1998 / Revised: 6 July 1998 / Accepted: 16 July 1998     

Clinical implications and surgical management of intussusception in pediatric patients with Burkitt lymphoma

Background/Purpose

Intussusception as a presenting feature of Burkitt lymphoma may be associated with early stage disease, which is curable with less intensive therapy. We determined the incidence, presentation, stage, and outcome of children with Burkitt lymphoma presenting with intussusception.

Methods

The medical records of patients with Burkitt lymphoma treated at our hospital from 1962 to 2005 were reviewed, and the patients presenting with intussusception were then further analyzed.

Results

Of 189 patients with primary abdominal Burkitt lymphoma, 33 (17.5%) presented with intussusception. Their median age at diagnosis was 10 years (range, 3-19 years). Most patients presented with abdominal pain (88%) and/or nausea/vomiting (42%). Twenty-three (70%) of these 33 patients as compared with 10 of the other 156 patients with abdominal lymphoma could have complete resection of their tumor (P < .0001) and hence had low stage disease (stage II). Only 10 patients with intussusception had stage III (n = 7) or stage IV (n = 3) unresectable disease. Twenty-five of the patients remained alive in continuous complete remission for 3 months to 31 years (median, 14 years).

Conclusion

Pediatric patients with Burkitt lymphoma presenting with intussusception often have completely resectable disease and are older than general pediatric patients with intussusception.     

Chronic renal failure in Sudanese children: aetiology and outcomes

The provision of tertiary paediatric nephrology facilities has led to increasing referrals of children with chronic renal failure (CRF). We report the aetiology and outcomes over 5 years, during which period the improving socio-economic situation has allowed increasing provision of dialysis and transplantation. The records of 205 children (124 male; 60.5%) who were referred to a tertiary paediatric nephrology unit in Khartoum between 2001 and 2006 with a glomerular filtration rate of less than 50 ml/min per 1.73 m² body surface area were reviewed. The mean age at the time of diagnosis with CRF was 9.8 years (range 3 months–17 years). The aetiology was chronic glomerulonephritis in 52 (25.4%), congenital urological malformations in 36 (17.5%), urolithiasis in 19 (9.3%), hereditary nephropathy in 14 (6.8%), multisystem diseases in 4 (2%), and cause unknown in 80 (39.1%). Of the 205 children, 136 (63%) had reached end-stage renal failure, with chronic haemodialysis being undertaken in 48 (35.3%), intermittent peritoneal dialysis in 43 (31.6%), continuous ambulatory peritoneal dialysis in 17 (14.7%), and no treatment in 25 (18.4%). At the end of the study period 53 of the 205 (25.9%) remained on dialysis, 51 (25%) were on conservative treatment, 8 (3.9%) had received transplants, 48 (23.4%) had died, and 45 (22%) had been lost to follow-up. The results illustrate the geographical variations in CRF aetiology in different countries, which may be influenced by current patterns of referral as well as environmental and society factors. The large number with an unknown aetiology for their CRF requires further prospective investigation. We hope the current high mortality rate will improve with earlier referral, improved nutrition, family support, and better access to treatment modalities, especially the provision of kidney transplantation from living related donors.     

Growth charts for prepubertal children with chronic renal failure due to congenital renal disorders

Despite the high prevalence of and therapeutic attention to growth failure in children with chronic renal failure (CRF), systematic evaluation of spontaneous growth in CRF are lacking. Therefore, we collected retrospectively longitudinal growth and biochemical data in 321 prepubertal patients treated for CRF due to congenital renal disorders. Data were recorded at 3-month intervals during the first 2 years of life and 6-monthly thereafter, up to the age of 10 years. Around 100 measurements were available per age interval. Mixed-longitudinal percentile curves of height and height velocity were constructed. Moreover, a statistical comparison with the heights and height velocities of healthy children and an evaluation of the effect of biochemical parameters on growth was performed. The CRF children had normal heights at birth but dropped below the 3rd normal percentile during the first 15 months of life. Thereafter, growth patterns usually were percentile parallel, with a mean height standard deviation score (SDS) of –2.37±1.6. Height velocities were consistently lower in patients with glomerular filtration rates (GFRs) below onethird of the lower normal limit (25 ml/min per 1.73 m² for patients >1 year) than in patients with better renal function. This difference in growth rates resulted in a mean height SDS of –1.65±1.5 SDS and –2.79±1.4 SDS (age 1–10 years) in the subgroups with relatively better and worse GFR, respectively. Regression analysis confirmed that GRF was a weak but significant predictor of height velocity SDS in most age groups.Study group members: I. Rätsch (Ancona), K. Michelis, T. Kapogiannis (Athens), F. Jung, T. Lennert (Berlin I), S. Gellert (Berlin II), T. Tulassay, P. Sallay (Budapest), T. von Lilien, D. Michalk (Cologne), M.-A. von Wendt-Göknur (Erlangen), K. E. Bonzel (Essen), R. Gusmano, E. Verrina (Genova), G. Offner (Hannover), O. Mehls, A.-M. Wingen, C. Fabian-Bach (Heidelberg, coordinators), A. Appiani, A. Bettinelli (Milan), J. Feber (Prague), G. Rizzoni, S. Picca (Rome), H. J. Stolpe, M. Wigger (Rostock), J. Kist-van Holthe, E. Wolff (Rotterdam, coordinators for the centers Amsterdam, Antwerp, Groningen, Nijmegen, Rotterdam), U. Berg (Stockholm), M. Fischbach (Strasbourg), E. Dobos (Szeged), E. Balzar (Vienna), T. Neuhaus (Zurich).     

Intravascular large B-cell lymphoma presenting with anasarca-type edema and acute renal failure

Abstract

Intravascular lymphoma (IVL) is a rare extra nodal subtype (usually of B-cell origin) presenting with infiltration of large neoplastic lymphocytes into lumina of blood vessels, leading to vascular occlusion. The early diagnosis is very crucial, however it is usually diagnosed postmortem investigation in most of the cases. A 56-year-old female presented with elevated creatinine level, and anasarca-type edema that superimposed with hard, indurated, erythematous plaques extending to inguinal region, abdomen, anterior aspect of chest, and face. B-cell IVL was confirmed with skin biopsy. The patient had some degree of clinical improvement following chemotherapy. B-cell IVL presenting with anasarca edema was not previously reported in the literature. Even if its rarity, IVL should be considered in the differential diagnosis of renal failure with anasarca edema.     

Chronic renal failure due to granulomatous interstitial nephritis in sarcoidosis: Response to corticosteroid therapy

Chronic renal failure due to granulomatous interstitial nephritis in sarcoidosis is a rare phenomenon, and its response to corticosteroid therapy is not well known. We report a patient with sarcoidosis who presented with chronic renal failure and hypercalcemia, but who did not exhibit nephrocalcinosis. Renal histology findings showed the presence of noncaseating granuloma and heavy interstitial nephritis. Although hypercalcemia was remarkably improved by corticosteroid therapy, chronic renal failure, due to interstitial fibrosis and scarring, remained unchanged. This case reinforces evidence supporting the effectiveness of corticosteroid therapy in granulomatous interstitial nephritis of sarcoidosis, and suggests the need for early initiation of the therapy to avoid permanent renal dysfunction.     

Chronic renal failure in India

In a series of 2028 patients with chronic renal failure, thediseases leading to renal failure, the presence or absence ofreversible factors and their nature, and the rate of declineof renal function of the most common conditions have been describedand analysed. Seven diseases: chronic interstitial nephritis(27.85%), diabetic nephropathy (26.76%), chronic glomerulonephritis(18.20%), benign nephrosclerosis (10.06%), chronic pyelonephritis(7.29%), focal glom erulosclerosis (3.20%), and autosomal dominantpoly cystic disease of the kidneys (2.07%), accounted for 95.43%of all the patients. These diseases were studied in greaterdetail and the results are presented here. It was found thatthere was a great variation in the rate of decline of renalfunction in the different groups, with chronic glomerulonephritisand focal glomerular sclerosis progressing most rapidly, diabeticnephro pathy slightly slower, and the others at a less alarmingpace. However, once serum creatinine had reached 177 µmol/lthere was an inexorable decline in renal function and the endstage was reached in almost all patients.