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Biomedical subjects

Kenneth W Gow

Publications and source records attributed to Kenneth W Gow.

At least 19 recordsLinked to original sources

Natural history of thyroid nodules in survivors of pediatric Hodgkin lymphoma.

BACKGROUND: Survivors of Hodgkin lymphoma and other patients who receive neck irradiation are at increased risk of thyroid cancer. Ultrasonography provides an inexpensive and non-invasive method of thyroid screening, but the clinical significance of thyroid nodules detected by ultrasound screening is uncertain. PROCEDURE: We reviewed the demographics, clinical characteristics, method of detection, and outcome of patients who developed thyroid nodules after treatment for pediatric Hodgkin lymphoma at our institution. One radiologist reviewed all imaging studies. RESULTS: Sixty-seven children treated for Hodgkin lymphoma from 1962 to 2001 developed thyroid nodules. The study group represented 9,024 person-years of follow-up after the diagnosis of Hodgkin lymphoma and 581 person-years after diagnosis of a thyroid nodule. A median of 10.5 years (range, 0.2-24.8 years) elapsed between the diagnoses of Hodgkin lymphoma and thyroid nodule(s). All but one patient had received neck irradiation for Hodgkin lymphoma, with a median thyroid radiation dose of 35 Gy (range, 12-45 Gy). Thyroid nodules were found to be malignant in seven patients (10%), at a median of 16.2 years (range, 8.4-23.7 years) after diagnosis of Hodgkin lymphoma. Only one malignancy was found through screening ultrasonography. All patients with thyroid cancer remained disease-free at 0.4-16.2 years of follow-up. CONCLUSIONS: Thyroid nodules are common in Hodgkin lymphoma survivors treated with neck irradiation, but the majority of these lesions have an indolent clinical course and do not undergo malignant transformation. Only patients with a palpable mass or clinical symptoms need more extensive evaluation, including Doppler-flow ultrasonography and fine-needle aspiration.

Disease-Free Survival↗

Extracorporeal membrane oxygenation for support of children after hematopoietic stem cell transplantation: the Extracorporeal Life Support Organization experience.

PURPOSE: Extracorporeal membrane oxygenation (ECMO) is a means of respiratory and hemodynamic support for patients failing conventional therapies. Children requiring hematopoietic stem cell transplantation who develop complications during therapy may require ECMO. Such patients pose medical and ethical challenges for clinicians considering initiation of ECMO. The authors review the outcomes of these patients and propose recommendations. METHODS: The Extracorporeal Life Support Organization Registry was queried for all patients younger than 18 years with an International Classification of Diseases, Ninth Revision, or Current Procedural Terminology code related to bone or stem cell transplant. RESULTS: Nineteen children in the registry met inclusion criteria. The median age was 9.6 years (7 months to 17.5 years). Initiation of ECMO was for pulmonary support (n = 17), cardiac support (n = 1), or cardiopulmonary resuscitation (n = 1). The median duration of ECMO support was 5.1 days (range, 30 hours to 42 days). Pulmonary infections included 3 parainfluenza, 2 Pneumocystis carinii, 1 influenza A, and 1 respiratory syncytial virus. Overall, 15 (79%) died during their ECMO run, whereas only 4 (21%) survived to come off ECMO. Furthermore, of those who survived their ECMO run, only one patient survived to discharge from the hospital. Risk factors for death on ECMO include development of renal complications and development of multiorgan dysfunction. CONCLUSION: Patients who require ECMO for cardiopulmonary support after hematopoietic stem cell transplantation have a poor prognosis. Clinicians must be cautious in presenting this option to parents and present them with appropriate expectations in this high-risk population.

Adolescent↗

Gene therapy: future or flop.

Many pediatric diseases have now reached a therapeutic plateau using standard therapy. Gene therapy has emerged as an exciting new means to achieve specific therapeutic benefit. Although there have been important and promising breakthroughs in recent clinical trials, there have been some serious setbacks that have tempered this initial excitement. In this review, we discuss the important developments in the field of gene therapy as it applies to various pediatric diseases and relate the recent successes and failures to the future potential of gene therapy as a medical therapeutic application.

Female↗

Left-sided gastroschisis.

Gastroschisis is a periumbilical, abdominal wall defect arising to the right of the umbilicus. We describe the clinical course of a left-sided gastroschisis in a term female born at 39 weeks' gestation. To our knowledge, there have been only 14 left-sided abdominal wall defects reported in the literature. We discuss our case and review the literature to try to determine if there is any clinic difference between right- versus left-sided lesions to make recommendations as to management.

Colon↗

Video-assisted thoracoscopic surgical excision of cystic lung disease in children.

PURPOSE: Video-assisted thoracoscopic surgical (VATS) technique for resection of cystic lung disease (CLD) may offer some advantages when compared with thoracotomy in children. METHODS: From September 1999 to August 2004, 6 pediatric patients underwent VATS for CLD. Patients were chosen for VATS based upon surgeon's choice. Data are expressed as mean +/- SD. The Children's Healthcare of Atlanta institutional review board approved this study. RESULTS: The types of lesions included congenital cystic adenomatoid malformations (n = 1), extrapulmonary sequestrations (n = 3), congenital lobar emphysema (n = 1), and bronchogenic cyst (n = 1). The extent of resection included lobectomy (n = 2) and excision (n = 4). Age and weight were 11.8 +/- 18 months (range 6 days to 4 years) and 7.5 +/- 3.6 (range 4.0-14.0) kg, respectively. Operating time was 103 +/- 70 (range 38-223) minutes. Chest tube duration was 1.2 +/- 0.8 (range 0-2) days. Morphine use on the first postoperative day was 0.2 +/- 0.3(range 0.05-0.20) mg/kg. Length of stay was 2.5 +/- 1.9 (range 1-6) days. There were no conversions to thoracotomy and no complications. CONCLUSION: VATS technique appears to be a safe and effective technique in managing CLD in children of all ages. More patients, however, need to be studied.

Bronchogenic Cyst↗

Renal artery pseudoaneurysm secondary to blunt trauma treated with microcoil embolization.

BACKGROUND: Renal artery pseudoaneurysms are rarely described sequelae of blunt abdominal trauma. Interventional radiological advances have allowed such lesions to be managed nonoperatively. METHODS: The authors review the presentation, diagnostic evaluation, and hospital course of an 11-year-old girl who developed a right renal artery pseudoaneurysm 14 days after blunt abdominal trauma. RESULTS: An 11-year-old girl fell off a horse onto her right flank. She sustained multiple right hepatic lobe lacerations and a complex fracture of the upper pole of the right kidney. Her initial hospital course was uncomplicated, and she was discharged after an uneventful 6-day course. The child did well for 2 weeks, until she developed right back pain and gross hematuria. A computed tomography scan revealed a right renal artery pseudoaneurysm. Angiography confirmed the presence of a pseudoaneurysm, which was fed by a single segmental branch originating from the renal artery. The artery was successfully occluded with a single platinum microcoil, which was demonstrated by the absence of contrast flow into the pseudoaneurysm. The patient recovered and was discharged shortly after the procedure. She initially had intermittent pain and hematuria, which resolved. Follow-up computed tomography scans have shown resolution of both the renal and hepatic lesions. CONCLUSIONS: Renal artery pseudoaneurysms that arise after blunt abdominal trauma in the pediatric population may be safely and effectively managed with arterial embolization, thereby avoiding extensive surgical interventions.

Accidental Falls↗

Laparoscopic cystogastrostomy for the treatment of pancreatic pseudocysts in children.

Pancreatic pseudocysts (PPSs) are common sequelae of pancreatitis and pancreatic trauma. The management is based upon the pseudocyst size and presence of symptoms. Those requiring intervention are often drained using several available options. The use of laparoscopic cystogastrostomy for large and recurrent PPSs has been described in adult patients as a less morbid alternative to open drainage procedures. This technique is considered a novel approach in children. We describe 2 children who had PPSs amenable to laparoscopic cystogastrostomy. The first was an 11-year-old girl who had blunt abdominal trauma from a bicycle handlebar. The second patient was a 7-year-old girl who developed idiopathic pancreatitis. Briefly, 2 ports were placed through the anterior abdominal and gastric walls, and into the lumen of the stomach. This intraluminal placement provided access to the posterior gastric wall. Using electrocautery diathermy, an incision was made through the posterior gastric wall and into the adjacent pseudocyst to obtain complete and unobstructed drainage. Both children tolerated the procedures well with resolution of their PPSs. The patients were each discharged on the fourth postoperative day and have been asymptomatic on 2 years follow-up. Laparoscopic cystogastrostomy is a safe and effective alternative to open cystogastrostomy for the minimally invasive management of PPSs in the pediatric population.

Child↗

Laparoscopic adrenalectomy for neuroblastoma in children: a report of 6 cases.

Neuroblastoma, a neoplasm of the sympathetic nervous system, is the most common solid extracranial tumor of childhood. Complete tumor resection has been shown to improve outcomes in both low- and high-risk neuroblastoma. The efficacy of laparoscopic adrenalectomy (LA) for metastatic lesions, benign tumors, and small neuroblastomas (<20 mm in diameter) is well established. However, the role of LA for large neuroblastomas is unclear. The authors describe the technical aspects of LA in 6 children with adrenal neuroblastomas greater than 2 cm and summarize their outcomes. All 6 patients underwent successful LA with removal of their tumors and surrounding lymph nodes. The specimens were placed in a laparoscopic specimen retrieval bag and morsulated in situ. The pathologic specimens were adequate for biologic factor analysis and histology. Mean operative time for all cases was 146 minutes. The average size of the adrenal tumors resected was 3.3 x 3.7 x 3.3 cm, and there were no intraoperative or perioperative deaths. All children, except the fifth patient who stayed for chemotherapy, were discharged home within 48 hours after surgery and are well. Our preliminary data suggest LA is a safe treatment option for neuroblastoma tumors greater than 2 cm in the select children.

Adrenal Gland Neoplasms↗

A unique case of renal carcinoma with Xp11.2 translocations/ TFE3 gene fusions in a 3-year-old child, with coexistent von Hippel-Lindau gene mutation.

Renal cell carcinomas (RCCs) are rare in the pediatric population; when they occur, a significant percentage are associated with specific cytogenetic abnormalities and germline mutations. These include mutations in the von Hippel-Lindau ( VHL) gene and translocations involving the TFE3 transcription factor gene on Xp11.2. Here we report a case of a 3-year-old child with a large renal mass. Histologic examination of the tumor showed a predominantly nested growth pattern with some papillary foci. Cytogenetic analysis revealed a karyotype of t(X;1)(p11.2; p34.3), consistent with a TFE3-associated RCC. Interestingly, sequencing of the patient's VHL gene revealed a single point mutation, previously seen in a subgroup of patients with von Hippel-Lindau disease. This is the first reported case, to our knowledge, of t(X;1)-associated RCC in a patient with concurrent VHL gene mutation.

Artificial Gene Fusion↗

Is the use of laparoscopy to determine presence of contralateral patent processus vaginalis justified in children greater than 2 years of age?

PURPOSE: Contralateral inguinal hernia exploration in cases of unilateral inguinal hernia remains a controversial topic. The authors have been using the in-line laparoscopic technique of contralateral evaluation for unilateral inguinal hernia in children less than 2 years of age. Because of the case of the procedure and lack of morbidity, we decided to expand the use of this procedure up to age 8 years in January 2000. The purpose of this study is to evaluate if the incidence of contralateral hernia in children greater than 2 years justifies the procedure. METHODS: This is a retrospective study of all children who underwent contralateral exploration for unilateral inguinal exploration over a 20-month period. The procedure was offered routinely to all patients up to age 8 years. During the repair, the contralateral inguinal ring was examined laparoscopically using the in-line technique for the presence of a contralateral hernia. The incidence of contralateral hernia was determined, and the results were stratified by age. Patients who underwent unilateral inguinal hernia repair without laparoscopic contralateral exploration or bilateral inguinal hernia repair without laparoscopic contralateral explorations were excluded from the study. RESULTS: A total of 284 laparoscopic explorations were performed. Positive explorations were seen in 65 of 171 (38%) of children less than 2 years of age, 19 of 101 (20%) of children 2 to 8 years of age, and 1 of 12 children greater than 8 years of age (8%). There were no operative complications. CONCLUSIONS: Laparoscopic contralateral exploration is safe and effective. Because of the low morbidity, the risk to benefit ratio warrants its use in children up to 8 years of age. This sample size is too small to make any meaningful statement in children older than 8 years.

Child↗

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.

Abdominal Pain↗

Brachial artery pseudoaneurysm in a 6-week-old infant.

Trauma to the wall of an artery may lead to the development of a pseudoaneurysm. There are infrequent case reports of children who have developed pseudoaneurysms after vascular access attempts. The options for management are limited in children and even more so in neonates. We describe the presentation and management of a 6-week-old infant who had attempts at insertion of an intravenous catheter as a newborn. She presented with an enlarging mass of the right upper extremity with no palpable radial pulse on examination. Workup included radiographs, Doppler ultrasound, and magnetic resonance imaging that established the diagnosis of pseudoaneurysm of the brachial artery. She underwent a repair of the right brachial artery by identifying the site of the arterial injury and oversewing the defect in the wall. Intraoperatively, she had good arterial flow with a return of the radial pulse. The patient did well immediately postoperatively and has been followed for more than a year with normal pulses and growth of the upper extremity. Patients that have had previous attempts at vascular access and subsequently develop a mass in the area of puncture should be worked up for the potential of a pseudoaneurysm. Doppler ultrasound and magnetic resonance imaging may help with diagnosis. Primary repair is advocated in this injury to ensure adequate growth of the limb.

Aneurysm, False↗

Pseudoangiomatous stromal hyperplasia of the breast in two adolescent females.

The majority of breast lesions that present in adolescent girls are benign, with most being fibroadenomas. Rarely, a large and rapidly growing breast mass may be found to be the more recently described entity named pseudoangiomatous stromal hyperplasia (PASH). The pathogenesis of this lesion is attributed to hyperplasia of stromal myofibroblasts in response to hormonal stimuli. To date, PASH has rarely been described in adolescence. We describe the presentation of PASH in two adolescent patients. The first is a 12-year-old girl who found a mass in her left breast 3 months prior to presentation. An excision of an 11.5 x 10 x 3.5 cm lesion weighing 347 g was performed via breast-conserving incisions. The second patient is a 16-year-old girl who also had a rapidly enlarging left breast mass removed in a similar fashion. This mass measured 12 x 11 x 6 cm and weighed 460 g. Both tumors were noted to have a smooth capsule. Histologic appearance consisted of the typical features of PASH; fibrous stroma containing numerous anastomosing slit-like spaces; some compressed and others with discernible lumina. Both patients had complete excisions and have since not experienced recurrence. Each has had excellent cosmetic results with symmetrical breast development since their resection. Pseudoangiomatous stromal hyperplasia is a rare tumor that arises in the breast. These tumors may grow quickly and often are mistaken for fibroadenomas, phylloides tumor, or angiosarcoma. They must be resected with careful attention to resection around the capsule of the tumor with breast conservation as a goal. Long-term follow-up is necessary, as some have been reported to recur.

Adolescent↗

Are localized intestinal perforations distinct from necrotizing enterocolitis?

BACKGROUND/PURPOSE: Localized intestinal perforation (LP) is thought to be a distinct entity when compared with perforation associated with necrotizing enterocolitis (NEC). Studies have indicated that LP is more amenable to percutaneous drainage and associated with a better outcome. We sought to determine whether LP and NEC could be distinguished based on clinical parameters alone. METHODS: A retrospective review of 40 neonates with gastrointestinal perforations between January 1990 and May 1998 was performed. All had undergone laparotomy and had histologic specimens available for evaluation. RESULTS: Twenty-one neonates had necrotizing enterocolitis (NEC), and 19 had localized perforation (LP) based on histologic criteria. More neonates with LP were exposed to prenatal indomethacin (37% v 5%; P <.05), received intravenous dexamethasone (42% v 10%; P <.05), had umbilical artery catheters (63% v 14%; P <.05), and had a higher white blood cell (WBC) count (27.1 +/- 23.1 v 14.3 +/- 11.5; P <.05). More neonates with NEC had pneumatosis intestinalis (47% v 11%; P <.05). No significant differences existed in enteral feeding (16% LP v 38% NEC) or overall mortality rate (37% LP v 38% NEC). No statistical differences in the timing of perforation or clinical presentation were found. CONCLUSIONS: NEC and LP are difficult to distinguish based on clinical parameters alone. The authors did find associations between LP and prenatal indomethacin, intravenous dexamethasone, umbilical artery catheters, and a higher WBC count. Mortality rate and clinical outcome were nearly identical in both groups. Pneumatosis intestinalis, thought to be pathognomonic for NEC, was seen on abdominal radiograph in 2 babies with histologically proven LP.

Adult↗