Bilateral Wilms' tumors with progressive or nonresponsive disease

Background

To provide guidelines for future cooperative group trials, we reviewed the outcomes of children with bilateral Wilms' tumors (BWTs) treated on National Wilms Tumor Study-4 (NWTS-4) who had progressive or nonresponsive disease (PNRD).

Methods

NWTS-4 enrolled 3335 patients from August 1986 to September 1994 including 188 patients with BWT (5.6%). Treatment and outcome data were collected on patients with BWT. Treatment guidelines were outlined in the protocol, but patients were not on study.

Results

Thirty-eight children with BWT had PNRD. Preoperative chemotherapy was given for a median of 7 months (range, 2-29 months) before definitive resection. After the initial chemotherapy regimen, 36 children went on to a second regimen, and of these, 21 children received a third regimen before resection. Eleven patients received irradiation to one or both kidneys. Pathology at resection revealed previously undiagnosed anaplasia in 3 patients (2 diffuse and 1 focal) treated for 14, 15, and 15 months before resection. A fourth patient developed a diffusely anaplastic tumor 13 months after therapy. Other pathological findings included rhabdomyomatous (4 patients) or differentiated stromal elements (10 patients) and complete necrosis (1 patient). Ten kidneys from 7 patients lacked biopsy at presentation or pathology review of those specimens.

Conclusions

BWT patients with PNRD received prolonged courses of chemotherapy. Early and sequential biopsies to establish the reason for failure to respond should be obtained. This will identify anaplastic tumors managed best by early nephrectomy and intensive chemotherapy and will also distinguish differentiated tumors that are best managed with early resection, but less intensive therapy after nephrectomy.     

Long-term outcome of Fowler-Stephens orchiopexy in boys with prune-belly syndrome

Purpose

Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function.

Materials and Methods

A total of 41 boys were divided into 3 groups depending on the type of orchiopexy performed, namely group 1—20 with bilateral 1-stage orchiopexy, group 2—10 with unilateral 1-stage and contralateral 2-stage orchiopexy, and group 3—11 with bilateral 2-stage orchiopexy.

Results

In group 1 9 of 20 patients had good scrotal testes bilaterally, 6 had a good scrotal testis on 1 side and 3 had small testes on each side. Two boys required testosterone supplementation but 18 had normal hormonal and sexual function. In group 2 6 of 10 patients had good scrotal testes bilaterally and 4 had a good scrotal testis on 1 side. All patients underwent spontaneous puberty with good sexual function. In group 3 7 of 11 boys had good scrotal testes bilaterally and 3 had 1 good testis with normal puberty and sexual function. These 10 patients underwent spontaneous puberty with good sexual function.

Conclusions

The majority of boys with prune-belly syndrome had a satisfactory outcome after orchiopexy with division of the gonadal vessels with testicular function sufficient to induce puberty and maintain satisfactory sexual function in adult life.     

Augmentation mentoplasty with osteocartilaginous nasal graft

Objective

Augmentation mentoplasty is a common procedure, applied especially in conjunction with rhinoplasty. Although various materials have been used, autogenous nasal hump tissue has not been studied in a big series of patients. The main objective of this study was to evaluate the use of autogenous nasal hump in chin augmentation.

Study Design

Case series with chart review.

Setting

University hospital.

Subjects and Methods

The osteocartilaginous mentoplasty procedure was performed in 124 patients along with rhinoplasty in a series of 218 patients who had undergone combined rhinoplasty and mentoplasty with various techniques. The median follow-up was 58 months (range, 12-120 months) over a 10-year period. The procedure commenced under general anesthesia for all patients with large humps and poor chin projection. During the rhinoplasty procedure, the osteocartilaginous nasal hump was removed, tailored to achieve an anatomic mental form, and inserted into the mental pocket through a submental or an intraoral incision.

Results

Infection was detected in five patients, two of whom required graft removal. All other patients recovered normally along an almost painless process, without displacements or any other complaints.

Conclusion

Nasal hump in reduction rhinoplasty is a useful alternative for augmentation mentoplasty on patients with large humps and poor chin projection. It also avoids all the disadvantages of alloplastic materials and eases integration compared with other alternatives in selected cases.     

Gastrointestinal stromal tumors arising from the stomach: a report of three children

Background

Gastrointestinal stromal tumors (GIST) are a unique subset of intestinal mesenchymal tumors that behave in an aggressive fashion. They have been commonly described in adults but have been rarely observed in children.

Methods

The authors review the presentation, diagnostic workup, operative records, pathologic specimens, and outcomes of 3 children with GISTs that originated from the stomach.

Results

All 3 children presented after upper gastrointestinal bleeding from the gastric tumor. The first was a 10-year-old girl who underwent partial gastrectomy but had recurrence 8 years later requiring a second resection. She subsequently had a hepatic metastasis 8 years later requiring a third resection. The second patient was a 9-year-old girl who had an antrectomy with a Bilroth I reconstruction and was noted to have a synchronous liver metastasis that was also resected. Despite Imatinib Mesylate, she had further hepatic metastases. The third child was a 4-year-old boy who recently underwent a partial gastrectomy and has no signs of metastatic disease at this time.

Conclusions

GISTs are unusual tumors that have been rarely described in children. When they arise in the stomach, they often present after upper gastrointestinal bleeding. Diagnosis can be made by endoscopy and biopsy. GISTs require resection and close observation for hepatic metastases. Current studies are ongoing for the potential role of Imatinib Mesylate for GISTs in children.     

Wilms' tumor with intracaval thrombus in the UK Children's Cancer Study Group UKW3 trial

Background/Purpose

To define the clinical characteristics and surgical management of intracaval involvement in patients enrolled in the UKW3 trial (1991-2001), which recommended elective preoperative chemotherapy for such cases.

Methods

Cases were identified from preoperative imaging and surgical trial forms. These asked specific questions about whether the surgeon suspected intracaval extension at diagnosis or found it at nephrectomy. For tumors with Wilms' histology, original case notes were examined.

Results

Of 842 patients registered in UKW3, 730 (87%) had Wilms' tumor. Among them, 59 (8.1%) had evidence of intracaval extension, either documented at diagnosis (53) or found unexpectedly at nephrectomy (6). Intracaval extension was also seen in tumors of other histology. The level of thrombus was intraatrial (10), suprahepatic (9), retrohepatic (8), infrahepatic (26), and unknown (6). The median age at diagnosis was 3.75 years compared to 2.97 years in patients without inferior vena cava thrombus (P < .0001). Fifty-two of 59 received preoperative chemotherapy. Thirty-one (52%) needed cavotomy, and 3 (30%) with intraatrial extension required cardiopulmonary bypass. The commonest operative complication was significant hemorrhage and resulted in mortality in 3 cases.

Conclusions

Preoperative chemotherapy is a useful adjunct to shrink the tumor and thrombus. This reduces the requirement for cavotomy and cardiopulmonary bypass. Intraoperative hemorrhage remains a significant cause of operative morbidity and mortality.     

Internal mammary lymph node metastasis in breast cancer: predictive models to assist with prognostic influence

Background

Metastatic breast cancer in the internal mammary nodes (IMN) indicates a poor prognosis. Several recent epidemiological surveys have determined a reduction in survival for patients with medial compared to lateral sector tumors attributing this to a higher rate of unrecognized IMN metastasis and hence these patients are undertreated with adjuvant therapy. ^{[1], [2], [3], [4], [5] and [6]}

Aim

Through mathematical modeling based on large datasets we aim to quantify the impact on survival of IMN metastases at different tumor and axillary stages.

Methods

Mathematical models were created to estimate the survival of patients with and without IMN metastasis. It was assumed that the different rate of survival between medial and lateral sector breast cancers was a result of the differential rate of unrecognized IMN metastases with resultant under-staging and under treatment. We applied these models on a retrospective database analysis from the Surveillance, Epidemiology and End-Results (SEER) registries from 1994 to 2003.

Results

The 10-year odds of death (OOD) from breast cancer for patients with medial compared with lateral sector tumors ranged from 1.2 to 1.5 depending on stage. The predicted odds of breast cancer death for patients with unrecognized IMN metastases ranged from 2.4 to 20, with the highest OOD in the groups with small tumors and no axillary node metastasis.

Conclusions

Through modeling we have been able to predict and quantify the significantly worse survival outcomes for patients with undiagnosed IMN metastasis.     

Bilateral Wilms' tumor with anaplasia: lessons from the National Wilms' Tumor Study

Purpose

The purpose of this study was to evaluate whether initial diagnostic technique influenced the ability to identify anaplastic histology, to determine the time interval to diagnosis of anaplasia, and to delineate the incidence of discordant pathology in bilateral Wilms' tumor. We hypothesized that delay in diagnosis of anaplasia could affect time to appropriate surgery and intensive multimodality therapy.

Methods

One hundred eight-nine children were enrolled in the fourth National Wilms' Tumor Study with synchronous bilateral tumors, 27 of whom were eventually shown to have anaplastic histology. Initial diagnostic technique, time interval to diagnosis of anaplasia, and the incidence of discordant pathology were determined.

Results

Anaplasia was identified in 0 of 7 tumors by core needle biopsy, 3 of 9 tumors by open wedge biopsy, and in 7 of 9 cases by partial or complete nephrectomy. The mean duration of first chemotherapy regimen (DD or EE) was 20, 39, and 36 weeks, respectively, before anaplasia was identified at second surgery. Discordant pathology between bilateral tumors was identified on final tissue diagnosis in 20 patients. Only 4 patients had anaplastic tumors in both kidneys.

Conclusions

Core needle biopsy did not identify anaplasia in 7 of 7 children. Open biopsy or partial/complete nephrectomy identified anaplasia at initial diagnostic procedure in 10 of 18 children. Twenty of 24 patients at final tissue diagnosis had discordant pathology between the 2 kidneys. Earlier interval incisional biopsy or resection may identify anaplastic histology and limit the duration of chemotherapy targeted to favorable histology for children with bilateral Wilms' tumor and anaplasia.     

Does video-assisted minilaparotomy surgical living donor nephrectomy satisfy donors?

Purpose

Video-assisted minilaparotomy surgical (VAMS) nephrectomy is believed to provide better cosmetic outcomes than open-donor nephrectomy in healthy donors. However, the results of a few studies have influenced the opinion of donors on their physical appearance. This study investigated the satisfaction of donors after a VAMS living donor nephrectomy.

Methods

Donors who underwent VAMS living donor nephrectomy between 2009 and 2011 were requested to fill out a body image questionnaire. This questionnaire consisted of three subscales: body image scale (BS), confidence in surgery scale (CS), and hospital experience scale (HS). A total of 20 VAMS living nephrectomy donors completed the questionnaire.

Results

The study included 3 male and 17 female donors of overall mean age of 38.7 ± 12.4 years. Eight donors were unmarried (40.0%), 11 were married (55.0%), and 1 was divorced. The mean follow-up was 7.9 ± 4.5 months. The mean BS, CS, and HS scores were 41.6 ± 5.3, 21.85 ± 8.3, and 13.9 ± 2.2, corresponding to perfect scores of 50, 30, and 20, respectively.

Conclusion

The results of this study showed that VAMS nephrectomy donors tended to be pleased with their body image, operation, and hospital experiences.     

Objective measures of renal mass anatomic complexity predict rates of major complications following partial nephrectomy

Background

The association between tumor complexity and postoperative complications after partial nephrectomy (PN) has not been well characterized.

Objective

We evaluated whether increasing renal tumor complexity, quantitated by nephrometry score (NS), is associated with increased complication rates following PN using the Clavien-Dindo classification system (CCS).

Design, setting, and participants

We queried our prospectively maintained kidney cancer database for patients undergoing PN from 2007 to 2010 for whom NS was available.

Interventions

All patients underwent PN.

Measurements

Tumors were categorized into low- (NS: 4-6), moderate- (NS: 7-9), and high-complexity (NS: 10-12) lesions. Complication rates within 30 d were graded (CCS: I-5), stratified as minor (CCS: I or 2) or major (CCS: 3-5), and compared between groups.

Results and limitations

A total of 390 patients (mean age: 58.0 ± 11.9 yr; 66.9% male) undergoing PN (44.6% open, 55.4% robotic) for low- (28%), moderate- (55.6%), and high-complexity (16.4%) tumors (mean tumor size: 3.74 ± 2.4 cm; median: 3.2 cm) from 2007 to 2010 were identified. Tumor size, estimated blood loss, and ischemia time all significantly differed (p < 0.0001) between groups; patient age, body mass index (BMI), and operative time were comparable. When stratified by CCS, minor and major complication rates for all patients were 26.7% and 11.5%, respectively. Minor complication rates were comparable (26.6 vs 24.9 vs 32.8%; p = 0.45), whereas major complication rates differed (6.4 vs 11.1 vs 21.9%; p = 0.009) among tumor complexity groups. Controlling for age, gender, BMI, type of surgical approach, operative duration, and tumor complexity, prolonged operative time (odds ratio [OR]: 1.01; confidence interval [CI], 1.0-1.02) and high tumor complexity (OR: 5.4; CI, 1.2-24.2) were associated with the postoperative development of a major complication. Lack of external validation is a limitation of this study.

Conclusions

Increasing tumor complexity is associated with the development of major complications after PN. This association should be validated externally and integrated into the decision-making process when counseling patients with complex renal tumors.     

Congenital spigelian hernias and cryptorchidism

Purpose

Spigelian hernias in childhood are rare. Only 24 infants in the English literature have been identified to have spigelian hernias, and 12 of these have been associated with cryptorchidism. Spigelian hernias are more commonly seen in the adult population and are considered to be acquired because they are typically associated with trauma or other etiologies of increased intraabdominal pressure. In the infant however, the etiology remains unclear, but a congenital defect in abdominal wall development is suspected.

Methods

We discuss the presentation and treatment of 4 additional patients with spigelian hernias (2 siblings included) associated with cryptorchidism.

Results

The hernias occurred within the well-described spigelian hernia belt in the semilunar line at the level of the semicircular fold of Douglas. Of the 6 repaired spigelian hernias, 5 were closed primarily with absorbable suture similar to previously reported cases; the sixth hernia required a patch closure because of its large size. All cryptorchid testes (7) were repaired in single-stage orchiopexies.

Conclusions

Spigelian hernias are rare entities in infants. We present 4 new cases of spigelian hernias associated with cryptorchidism and, with previously reported cases, discuss the probability of a congenital origin of these hernias in infants.     

Renal angiomyolipoma in Japanese tuberous sclerosis patients

Purpose

Renal angiomyolipoma (AML) is a benign neoplasm that may grow massive in tuberous sclerosis (TS) patients. The aim of this study was to document the characteristics of renal AML in Japanese TS patients.

Methods

Medical records of 29 TS patients followed up at the authors’ center were reviewed for the presence, size, symptom, and treatment of renal AML.

Results

Twenty-four patients screened for renal AML were subdivided into 4 groups: group 0 (n = 8), no mass; group 1 (n = 5), AML less than 1 cm in diameter; group 2 (n = 4), AML 1 to 4 cm in diameter; group 3 (n = 7), AML greater than 4 cm in diameter. When present, AML always affected both kidneys and were multiple. All patients in groups 1 and 2 were symptom free, and the tumors seemed stable in size. All tumors in group 3 grew progressively causing various symptoms. Total or partial nephrectomy or transarterial embolization was performed in 5 patients with limited success.

Conclusions

AML in TS patients can be stable or aggressive. Pediatric surgeons aware of this problem should be involved in a follow-up program.     

Neoadjuvant pegylated liposomal doxorubicin in combination with cisplatin and infusional fluoruracil (CCF) with and without endocrine therapy in locally advanced primary or recurrent breast cancer

Purpose

To explore the activity of pegylated liposomal doxorubicin (PLD) as neoadjuvant therapy of breast cancer.

Methods

The combination of PLD with cisplatin and infusional fluorouracil (CCF) for 8 courses was investigated in patients with primary or recurrent T2-T4a-d N0-3 M0 breast cancer. Patients with ER and/or PgR ≥10% tumors also received letrozole (±triptorelin).

Results

Forty patients entered the study. Four patients had recurrent tumors and 13 had cT4d tumors. Overall, clinical response rate was 77.5% whereas a pathological complete response (pCR) was obtained in 3 patients (7.7%), 4 when considering bilateral tumors. Noticeably 3 pCR were observed among the 10 patients with T4d ER positive tumors (33%). Eleven patients discontinued treatment before completion of the 8 planned courses.

Conclusions

Our results indicated that CCF yielded an appreciable rate of clinical responses in a series of very locally advanced tumors and an unusually high rate of pCR in T4d ER positive tumors, suggesting an enhanced cutaneous activity of PLD.     

Use of transanastomotic feeding tubes during esophageal atresia repair

Purpose

Esophageal atresia (EA) with tracheoesophageal fistula (TEF) type C accounts for 85% of all EA. In our center, patients were previously started on total parenteral nutrition (TPN) postoperatively and oral feedings initiated only after a contrast esophagogram. Our aim is to assess the benefit of intraoperatively placed transanastomotic feeding tubes (TAFTs).

Methods

A 7-year retrospective review analyzed the outcomes of children with EATEF type C as they relate to the use of TAFT. Demographics, associated anomalies, operative findings, complications, duration of TPN, resumption of oral feeding, length of stay, and follow-up were examined.

Results

Twenty-one patients had EATEF type C. Eleven (55%) and 9 (45%) patients were identified as nonfeeding tube (NFT) and TAFT groups, respectively. There were no differences in gestational age, birth weight, associated anomalies, and interval to operative intervention or operative time. Excluding one patient with a severe cardiac malformation in the NFT group, there were no significant differences in anastomotic leak (8% vs 22%), stenosis (36% vs 22%), TPN duration (20 days vs 12 days), and cholestasis (36% vs 11%).

Conclusion

Transanastomotic feeding tube may lead to shorter TPN duration and decreased cholestasis, but a larger prospective study would be required to prove these benefits and ensure that it does not increase anastomotic leaks. This could be done through an expanded Canadian Pediatric Surgery Network study.     

Omission of routine chest x-ray after chest tube removal is safe in selected trauma patients

Background

Definitive practice guidelines regarding the utility of chest x-ray (CXR) following chest tube removal in trauma patients have not been established. The authors hypothesized that the selective use of CXR following chest tube removal is safe and cost effective.

Methods

A retrospective review of chest tube insertions performed at a level I trauma center was conducted.

Results

Patients who underwent chest tube removal without subsequent CXR had a lower mean Injury Severity Score and were less likely to have suffered penetrating thoracic injuries. These patients received fewer total CXRs and had shorter durations of chest tube therapy and shorter lengths of stay following tube removal. Subsequent reinterventions were performed more frequently in the CXR group. The annual decrease in hospital charges by foregoing a CXR was $16,280.

Conclusions

The selective omission of CXR following chest tube removal in less severely injured, nonventilated patients does not adversely affect outcomes or increase reintervention rates. Avoiding unnecessary routine CXR after chest tube removal could provide a significant reduction in total hospital charges.     

How important is rigid proctosigmoidoscopy in localizing rectal cancer?

Background

Colonoscopic localization of rectal and rectosigmoid tumors may be inaccurate. Rigid proctosigmoidoscopy has been suggested as an adjunctive technique to accurately localize rectal tumors as it may alter treatment options.

Methods

A retrospective review was performed of patients with rectal and rectosigmoid cancer from 2001 to 2006. Patients were stratified into 1 of 4 anatomic regions based on colonoscopic localization of the tumor. The distances of the tumor from the anal verge by colonoscopy were compared with distances obtained via rigid proctosigmoidoscopy.

Results

Rigid proctosigmoidoscopy localization likely changed the treatment options in 21% of lower rectal tumors, 14% of middle rectal tumors, 38% of upper rectal tumors, and 29% of rectosigmoid tumors. Overall, this modality impacted 25% of patients.

Conclusions

Rigid proctosigmoidoscopy localization of rectal tumors can significantly change treatment options and should be performed on all patients with colonoscopic localization of a cancer thought to be in the rectosigmoid or rectum.     

Extrapleural, submuscular bars placed by bilateral thoracoscopy--a new improvement in modified Nuss funnel chest repair

Background

Thoracoscopic Nuss funnel chest repair still has a significant complication rate. Bar dislocation, pneumothorax, pleural effusions, and pericarditis seem to be caused mechanical irritation by the bar. We intended to reduce these problems by further technical modification of the Nuss technique.

Methods

Of 157 prospectively followed modified Nuss repairs, the last 57 patients had the bars placed in an extrapleural position and fixed by 10 to 14 pericostal sutures under bilateral thoracoscopy.

Results

Entirely, extrapleural bar position was feasible in 53 of 57 patients. Four patients had minor holes over one of the bars, predominantly on the left side of the thorax. Pleural effusions, pneumothorax, and pain were greatly reduced, so that we discontinued the so far routine use of bilateral pleural drainages.

Conclusions

Extrapleural bar position is feasible in more than 90% of modified Nuss repairs. It reduces pleural secretion and pain, and seems to reduce pneumothorax, pulmonary bar adhesions, and pericardial effusions. The technique is easy and safe, and reduced the incidence of most complications in this early experience of 57 adolescent patients, although no sportive restrictions were imposed at all.     

What is the optimal treatment for children with primary hyperparathyroidism?

Purpose

Little information exists regarding the optimal surgical treatment of pediatric primary hyperparathyroidism. We hypothesized that primary hyperparathyroidism in children, in the absence of a family history, is caused by single-gland disease and is amenable to minimally invasive parathyroidectomy (MIP).

Methods

We reviewed the records of individuals younger than 25 years who underwent parathyroidectomy in a prospectively collected database at a single tertiary hospital from 2003 to 2009.

Results

Twenty-five patients were identified, with a mean (SD) age of 19 (3.7) years. Sixty percent had single-gland disease (n = 15). Familial disease was present in 6 patients. All of the children younger than 18 years without a family history of disease (9/9) were found to have a single-gland disease (P < .001). Seventy-eight percent of patients without a family history were successfully treated without a bilateral exploration. Average length of stay was less than 1 day with no complications or recurrences.

Conclusions

Primary hyperparathyroidism in patients younger than 18 years without a family history was uniformly caused by single-gland disease. Minimally invasive parathyroidectomy was successful in these patients and avoided the morbidity of bilateral exploration. We recommend MIP be used in pediatric patients at large referral centers with prior successful institutional experience with the technique.     

Robot-assisted laparoscopic partial adrenalectomy for pheochromocytoma: the National Cancer Institute technique

Background

Partial adrenalectomy has recently been advocated to preserve unaffected adrenal tissue during resection of pheochromocytoma.

Objective

To describe a robot-assisted laparoscopic partial adrenalectomy (RALPA) technique and to report on early functional and oncologic outcomes.

Design, setting, and participants

From 2007 to 2010, 15 RALPA were performed on 12 consecutive patients with pheochromocytoma. Follow-up data of >1 yr are available on 11 procedures. Median follow-up for the entire cohort was 17.3 mo (range: 6-45).

Surgical procedure

Positioning and port placement is designed for adequate reach and visualization of the upper retroperitoneum. The plane between the adrenal cortex and pheochromocytoma pseudocapsule is identified visually and with laparoscopic ultrasound. The tumor is dissected away from normal adrenal cortex, preserving normal adrenal tissue.

Measurements

Preoperative, perioperative, pathologic, and functional outcomes data were analyzed.

Results and limitations

Fourteen of 15 cases were completed robotically. Among 15 procedures, 4 were performed on a solitary adrenal gland. Four cases required resection of multiple tumors (up to six) with two performed in a solitary gland. The mean age of the patients was 30 yr, and the mean body mass index was 27. The mean operative time was 163 min, the median estimated blood loss was 161 ml, and the median tumor size was 2.7 cm (range: 1.3-5.5). There was one conversion to an open procedure in a patient requiring reoperation on a solitary adrenal gland. One patient who underwent RALPA on a solitary adrenal gland required postoperative steroid supplementation at last follow-up. At a median follow-up of 17.3 mo (range: 6-45), there were no recurrences or metastatic events. Study limitations include small sample size and short follow-up.

Conclusions

RALPA for the treatment of pheochromocytoma is feasible and safe and provides encouraging functional and oncologic outcomes, even in patients with a solitary adrenal lesion or multiple ipsilateral lesions.     

Influence of maternal parity, age, and ethnicity on risk of esophageal atresia in the infant in a population-based study

Background

The etiology of esophageal atresia (EA) is virtually unknown. We hypothesized that the maternal factors low parity, high age, and white ethnicity are involved.

Methods

A Swedish nationwide, population-based, case-control study was nested in a cohort of newborn children in 1982 to 2004. Among 2,305,858 deliveries, 722 cases of EA and 3610 controls were included. Conditional logistic regression was used to calculate odds ratios (ORs) and 95% confidence intervals (CIs). Matching, stratification, and multivariable regression were used to adjust for potential confounding.

Results

A more than 30% decreased risk of EA was found for mothers delivering their second (OR, 0.68; 95% CI, 0.56-0.83) or third child (OR, 0.64; 95% CI, 0.49-0.83) compared to primiparous mothers. Children of women giving birth when 35 to 40 years and older than 40 years showed a 2-fold (OR, 2.09; 95% CI, 1.09-3.99) and 3-fold (OR, 3.04; 95% CI, 1.37-6.74) increased risk of EA, respectively, compared to those of mothers younger than 20 years. This association remained when chromosomal abnormality cases were excluded (P = .004). There was a 66% increase in risk of isolated EA in children of mothers of white (OR, 1.66; 95% CI, 1.06-2.61), compared to mothers who are not of white ethnicity.

Conclusions

This study indicates an increased risk of EA in children of mothers having their first delivery, of older age, and of white ethnicity.     

Long-term risk of hypertension and chronic kidney disease in living kidney donors

Objective

The aim of this study was to assess the long-term risks of chronic kidney disease and arterial hypertension in living kidney donors.

Methods

Donors who were followed for more than 1 year after nephrectomy were included. We assessed each donor's blood pressure, urine protein, and estimated glomerular filtration rate (eGFR).

Results

The follow-up rate was 11% (154 out of 1,356 donors), only 19% of whom were followed by nephrologists. Blood pressure had increased from 113/75 to 116/77 mm Hg (P < .01), urinary protein excretion after donation did not increase, and renal function was well preserved after donor nephrectomy. However, 33 patients (21.4%) showed a decreased eGFR of <60 mL/min/1.73 m², and 3 donors developed end-stage renal disease that required renal replacement therapy.

Conclusions

The follow-up rate of living donors after donation was low, and we observed an increased risk of developing chronic kidney disease after donation.