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Biomedical subjects

K J Welch

Publications and source records attributed to K J Welch.

32 records · Page 2Linked to original sources

Pulmonary agenesis (aplasia), esophageal atresia, and tracheoesophageal fistula: a different treatment strategy.

This is a report of a patient with esophageal atresia, distal tracheoesophageal fistula, and right pulmonary agenesis who survived for 10 months. Death was due to progressive respiratory failure following a complicated clinical course. Several lessons were learned. First, the remaining lung is at high risk. Since the number of respiratory units in these infants is already decreased, the loss of more units has a more profound effect than in an otherwise healthy child. Second, while the establishment of esophageal continuity is important, all attempts to perform an esophagoesophagostomy should be abandoned if doing so further jeopardizes the solitary lung. Third, since all of these children have some degree of tracheomalacia, early tracheostomy should be considered. The tracheostomy will not only act as a tracheal stent but will also avoid progressive laryngeal pathology, which would result from long-term endotracheal intubation.

Abnormalities, Multiple↗

Juxtapancreatic intestinal duplications with pancreatic ductal communication: a cause of pancreatitis and recurrent abdominal pain in childhood.

Pancreatic duplications with ductal communications should be included in the differential diagnosis of any child presenting with recurrent abdominal pain of unknown etiology and should be considered as a possible cause of pancreatitis in childhood. Such duplications most likely arise from nonregressing diverticula of the pancreatic bud during embryologic development. Their clinical presentation is unique from other duplications because of their anatomic association with the pancreatic duct. Pain and weight loss are the major presenting complaints, although many patients have nausea and vomiting. Serum chemistries, in particular the serum amylase, are usually normal and are of little help in the differential diagnosis. Radiographic evaluation has not been particularly helpful in the past. ERCP, ultrasonic examination, and CT scan show great promise, however. Operative intervention should be tailored for the individual patient. The operation performed will depend upon operative findings. Intraoperative pancreatograms or cystograms are very helpful in differentiating these cysts from others at the time of operation. Pathologically, most of the duplications have a thickened muscular coat that usually has some evidence of inflammation. They are usually lined with gastric mucosa. Except in the most severe cases, the pancreas is histologically normal, suggesting that most of the pain experienced by these patients is secondary to inflammation within the duplication. The inflammatory response may completely destroy the mucosal lining and cause fibrosis within the muscular coat of the duplication. In those instances, these lesions cannot be differentiated from pancreatic pseudocyst. This may account for some of the "idiopathic" pseudocysts reported in the literature.

Abdomen↗

Retroperitoneal germ cell tumors in childhood. A clinical and pathologic study of 11 cases.

During the last 54 years at the Children's Hospital 11 children (10 female, 1 male) had been treated for an extragonadal germ cell tumor arising in the retroperitoneum. There were eight teratomas (five mature, three immature), two endodermal sinus tumors and one tumor with a mixture of both components. Abdominal pain or discomfort was the usual presenting complaint, with the average age at diagnosis being 18 months. On physical examination, each child had a palpable abdominal mass usually localized in the upper quadrants. The finding of calcification, bone or teeth, on radiologic study was most helpful in establishing a preoperative diagnosis of teratoma. The preferred treatment for children with mature and immature teratomas is complete surgical resection; decisions regarding adjuvant therapy for patients with immature tumors must be evaluated on an individual basis. The prognosis remains guarded for children with unresectable primaries or those with endodermal sinus tumor. Of three tumor-related deaths, two were due to metastatic endodermal sinus tumor and one resulted from extensive local growth by immature teratoma. Successful management of children with endodermal sinus tumor requires surgery aimed at debulking or complete resection in combination with aggressive adjuvant chemotherapy.

Child, Preschool↗

Pectus excavatum in children: pulmonary scintigraphy before and after corrective surgery.

Regional distribution of pulmonary function was evaluated preoperatively and postoperatively with xenon-133 perfusion and ventilation scintigraphy in 17 patients with pectus excavatum. Ventilatory preoperative studies were abnormal in 12 of 17 patients, resolving in seven of 12 postoperatively. Perfusion scans were abnormal in ten of 17 patients preoperatively; six of ten showed improvement postoperatively. Ventilation-perfusion ratios were abnormal in ten of 17 patients, normalizing postoperatively in six of ten. Symmetry of ventilation-perfusion ratio images improved in six out of nine in the latter group. The distribution of regional lung function in pectus excavatum can be evaluated preoperatively to support indications for surgery. Postoperative improvement can be documented by physiological changes produced by the surgical correction.

Adolescent↗

Mediastinal germ cell tumors in childhood. A clinical and pathological study of 21 cases.

Twenty-one patients aged 16 years or less had been treated for a primary mediastinal germ cell tumor at the Children's Hospital, Boston Massachusetts, during the last 54 years. There were 13 boys and eight girls with the average age at diagnosis being 7 years (range 2 weeks to 16 years). Twelve mediastinal germ cell tumors were classified as pure teratoma, five contained embryonal carcinoma admixed with other germ cell components, and four were pure embryonal carcinoma. Of 12 patients with pure teratoma, 10 underwent complete surgical resection and were alive and well 1 to 13 years later; two children left untreated died of complications related to local tumor growth. Complete surgical resection was possible for only two of nine patients with embryonal carcinoma; both received adjuvant therapy and were alive and well 3 and 20 years later. Seven patients received radiation and/or chemotherapy but died of residual or metastatic disease. Successful treatment for children with embryonal carcinoma requires an operation aimed at either debulking or complete resection (if possible) coupled with early and aggressive combination chemotherapy. The role of radiation in primary therapy remains undefined with regard to curative intent.

Adolescent↗

Sacrococcygeal germ cell tumors in childhood: an updated experience with 118 patients.

The histopathology of 118 sacrococcygeal germ cell tumors (SGCT) was correlated with clinical presentation, therapeutic management, and prognosis. There were 97 teratomas (78 mature, 19 immature), 19 embryonal, and 2 anaplastic carcinomas. Mature and immature teratomas usually presented externally (AAP type I) permitting relatively early detection and surgical removal. The immature group was significantly larger (11.6 cm vs. 7.5 cm) with a greater incidence of subsequent embryonal carcinoma (16%). The embryonal and anaplastic carcinomas were diagnosed at a later age (average 21 mo) and had a substantial presacral or endopelvic component (AAP types II-IV); this group had the worst prognosis with no survivors. Our data suggest that combined analysis of clinical findings and histopathology may help to identify some of the children at risk for early recurrence.

Carcinoma↗

Identification of hazardous organic chemicals in fish from the Ashtabula River, Ohio, and Wabash River, Indiana.

Composite fish samples from the Wabash River, IN, and the Ashtabula River, OH, were analyzed by conventional pesticide procedures for PCBs, DDTs, chlordane components, and hexachlorobenzene. Additional aliquots of each sample were processed by gel permeation chromatography and were analyzed with gas chromatography--mass spectrometry by using both electron impact and negative chemical ionization modes. These analyses resulted in the identification of a series of chlorinated alkanes, chlorinated alkenes, chlorinated alkylamines containing 2--5 carbons, and polychlorinated styrenes in the Ashtabula River sample, and a series of chlorinated norbornenes, pentachlorobenzyl alcohol, and pentachlorophenol in the Wabash River sample.

Animals↗

Surgical treatment of thymic cysts and neoplasms in children.

Based on experience with 17 patients and review of the literature, complete excision is possible for all benign thymic cysts and neoplasms. Recurrence is rare (2%). Most malignant thymomas in children are epithelial (73%). As with adults, prognosis depends on accurate surgical staging and aggressive surgical intervention. Even the largest tumors can be completely excised removing portions of lung, pleura, diaphragm, and pericardium in continuity as required. Cardiopulmonary bypass may be necessary when the innominate veins or the superior vena cava is involved. Irradiation is required for stage II and stage III lesions. Stage IV patients require irradiation and chemotherapy.

Adolescent↗

Non-obstructive megacystis and refuluxing megaureter in pre-teen enuretic boys with minimal symptoms.

We herein describe 4 older boys with non-obstructive, non-neurogenic refluxing megacystis-megaureter syndrome. These patients require immediate investigation and aggressive surgical intervention, the goal being total reconstruction of the bladder and upper tracts in continuity. This goal was achieved in 3 patients with followup in excess of 4 years. Failure to recognize the syndrome, as with our fourth patient, may result in improper classification and treatment. A plea is made for urological investigation of pernicious nocturnal enuresis in older boys and in all children with a history of urinary tract infection.

Child↗