Biomedical subjects

G T Zwiren

Publications and source records attributed to G T Zwiren.

13 recordsLinked to original sources

PubMed1991

Ovarian tumors in relapsing acute lymphoblastic leukemia: a review of 23 cases.

Five cases of relapsing acute lymphocytic leukemia (ALL) presenting as an ovarian tumor have been treated at this institution, representing the largest reported series. In a review of the literature we identified 18 additional cases of ovarian leukemic relapse. Together, these 23 patients form the basis for this report. Abdominal pain is the most common presenting symptom of ovarian leukemia. An abdominal mass is usually palpable, and at least four patients had hydronephrosis. Nine patients had documented bilateral ovarian involvement; however, bilateral disease was not a poor prognostic sign. Most ovarian relapses occurred more than 36 months after the original diagnosis of ALL, with these "late'h relapsers responding more favorably to treatment than "early" relapsers. Definitive statements can not be made from a retrospective review of 23 case reports; however, salpingooophorectomy had no obvious advantage over simple biopsy, and there was no obvious advantage to the routine use of radiation therapy. Most failures in treating ovarian leukemia occurred within 2 years. Most failures were systemic rather than local, illustrating the need for aggressive multiagent systemic chemotherapy. Survival after ovarian leukemic relapse is possible, with eight of the 23 patients alive and in complete continuous remission following the ovarian relapse (median follow-up since relapse, 42 months; range, 2 to 135+ months). With the use of more intensive chemotherapy in recent protocols, the frequency of ovarian leukemic relapses appears to be decreasing. At this institution, no child with ALL diagnosed in the 1980s has subsequently developed an ovarian relapse.

Adolescent↗

PubMed1991

Modern treatment of cloacal exstrophy.

We have treated 12 newborns with cloacal exstrophy (10 classical and 2 variants) since 1980. Eleven infants underwent repair and they are all surviving; the one nonoperated infant died of pulmonary hypoplasia. There are six genetic females and six genetic males, five of whom are being raised as females. The initial operation consisted of separating the bowel from the bladder to create an intestinal stoma; closing the omphalocele; and reapproximating (5), closing (4), or leaving the exstrophied bladder undisturbed (2). The importance of creating a "tailgutostomy" instead of an ileostomy to prevent problems with diarrhea, dehydration, and acidosis is emphasized. There have been 71 subsequent operations in these 11 patients (28 general surgical, 25 urological, 9 neurosurgical, and 9 orthopedic). Quality of life, rather than survival, is now the major issue facing patients with cloacal exstrophy. All of these patients except one are neurologically normal. The defect is closed in all patients. Three patients wear no appliances, 7 wear one (gastrointestinal [GI]), and one wears two (GI and genitourinary [GU]). We developed a scoring system to analyze bowel and bladder continence: voluntary control = 3; control with an enema program or intermittent catheterization = 2; incontinence with a well-functioning stoma = 1; and incontinence without a stoma = 0. The best continence score is 6 (GU + GI). We currently have 7 patients with a continence score of 1 (colostomy + incontinent bladder); 1 with a score of 2 (ileostomy + incontinent ureteroenterostomy); 2 with a score of 4 (enema program + continent urinary diversion); and 1 with a score of 5 (enema program + continent bladder).(ABSTRACT TRUNCATED AT 250 WORDS)

Bladder Exstrophy↗

PubMed1989

Repair of pectus excavatum using a substernal metal strut within a Marlex envelope.

In a retrospective review, 82 children aged 3 to 16 years who had repair of pectus excavatum from 1963 to 1987 were divided into three groups according to type of repair: those without a metal substernal strut, those with a metal substernal strut, and those with a metal substernal strut within a Marlex envelope. In group 1 (n = 50) there were five recurrences; in group 2 (n = 18) and group 3 (n = 14) there were no recurrences. There was migration of the substernal strut in eight patients in group 2 (44%) and three patients in group 3 (21%). There were minor wound infections in groups 1 and 2 only. There were no deaths. Results of this study suggest that the use of a substernal strut significantly reduced recurrence and that the addition of a Marlex envelope around the strut reduced migration of the strut with no associated complications.

Adolescent↗

PubMed1986

Biliary atresia: an evolving perspective.

From 1967 to 1984, 50 of our patients with extrahepatic biliary atresia had surgical exploration. Of 40 biliary drainage procedures, bile drained in 21 (52%). Thirty-four patients had portoenterostomy, three had portocholecystostomy, and the most recent six patients had a valved hepatoduodenal conduit. Successful biliary drainage was related to the presence of microscopic ducts at the porta hepatis in 20 of 21 infants. Twenty patients are alive, 12 from two to six years postoperatively (one with a liver transplant). Seven have normal serum bilirubin values. Height and weight exceed the 50th percentile in 5/15 patients studied. Multiple episodes of cholangitis have occurred in 11 patients with portoenterostomy and two with hepatoduodenal conduits. In 12 patients, hemorrhage from the stoma necessitated closure of the stoma before 1 year of age. Five of the six patients with hepatoduodenal conduit are alive two years postoperatively.

Bile Ducts↗

PubMed1985

Characteristics of children with endoscopically proved chronic bronchitis.

This study evaluated by chart review the clinical, allergic, pathologic, and immunologic characteristics of 20 children found to have chronic bronchitis (CB) by bronchoscopic evaluation, including 13 children in whom CB was documented by endobronchial biopsy. Two additional children likely to have had CB also are described. In this study group, all of the patients were believed to have asthma as well. Chronic bronchitis was predicted by chronicity of symptoms and incomplete response to bronchodilators and corticosteroids, but it was not predicted by a history of allergy or laboratory evidence of systemic infection or inflammation. In the children described herein, CB often was associated with an IgG subclass abnormality. Bronchoscopic evaluation documented CB and provided biopsy and secretion samples. Although the histomorphic findings were heterogeneous, patients with CB usually had white blood cells present on Gram stains of secretions in addition to mononuclear cell infiltrates on biopsy specimens. Chronic bronchitis in these children seems to be distinct from CB in adults. Further studies will be needed to define CB in children.

Adolescent↗

PubMed1985

Recurrence of Wilms tumor after apparent cure.

The recurrence of Wilms tumor after a 5-year disease-free interval is rare. We present two patients who had recurrent disease after a disease-free interval of greater than 7 years. Three additional patients, registered with the National Wilms Tumor Study who had a recurrence after 5 years, are also described. Of these three patients, two had nephroblastomatosis. Because more patients are achieving long-term survival, careful surveillance after apparent "cure" is recommended, particularly if nephroblastomatosis is identified in the original nephrectomy specimen.

Child, Preschool↗

PubMed1975

A complex pattern of disposition of phenytoin in severe intoxication.

A 5-year-old child developed phenytoin (diphenylhydantoin, DPH) toxicity after receiving 500 mg of the drug daily for 3 weeks. Plasma, urine, and duodenal fluid were collected for assay of DPH and its metabolites. The peak plasma concentration of DPH was 108 mug/ml, and the decline in plasma level did not fit first-order kinetics. The para-hydroxy, meta-hydroxy, and dihydrodiol metabolites of DPH were measured in urine; duodenal aspirate contained both DPH and the para-hydroxy metabolite. Plasma pH may affect distribution of DPH since in vitro binding of DPH to human albumin increased as pH increased.

Child, Preschool↗

PubMed1975

Significance of absent vas deferens at hernia repair in infants and children.

An absent vas deferens may be associated with unilateral renal agenesis or undiagnosed cystic fibrosis. Such patients should be further evaluated with a sweat chloride and intravenous pyelogram. The finding of absent vas deferens during exploration for an undescended testis does not provide absolute evidence for absence of the testis, since these structures have independent embryologic origins.

Child↗

PubMed1974