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Biomedical subjects

A A de Lorimier

Publications and source records attributed to A A de Lorimier.

At least 19 recordsLinked to original sources

Alcohol, wine, and health.

BACKGROUND: For the past 20 years numerous epidemiological studies have correlated the consumption of alcohol and a variety of disease states: overall mortality, arteriosclerotic vascular diseases, hypertension, cancers, peptic ulcer, respiratory infections, gall stones, kidney stones, age-related macular degeneration, bone density, and cognitive function. METHODS: A review of these articles reveals that each of these studies has compared the outcome of individuals at various levels of alcohol consumption with that of abstainers. RESULTS: Each analysis has identified a U-shaped or J-shaped curve of reduced relative risk for a given disease state compared with abstainers. A clear definition of consumption in moderation becomes evident: for men it should not exceed 2 to 4 drinks per day, and for women it should not exceed 1 to 2 drinks per day. CONCLUSIONS: Alcohol by itself has favorable effects on the level of high-density lipoprotein cholesterol, and inhibition of platelet aggregation. Wine, particularly red wine, has high levels of phenolic compounds that favorably influence multiple biochemical systems, such as increased high-density lipoprotein cholesterol, antioxidant activity, decreased platelet aggregation and endothelial adhesion, suppression of cancer cell growth, and promotion of nitric oxide production.

Adult↗

Laparoscopic splenectomy. The initial experience at University of California, San Francisco.

OBJECTIVE: To evaluate the outcomes of patients undergoing laparoscopic splenectomy (LS) at a single institution. DESIGN: Case control. SETTING: University teaching hospital. PATIENTS: The medical records of the initial 25 consecutive patients who underwent LS at a single institution were reviewed. For comparison, a control group of 25 patients undergoing open splenectomy (OS) matched for age, diagnosis, and splenic weight were also reviewed. MAIN OUTCOME MEASURES: Data regarding operative time, blood loss, pathologic findings, complications, postoperative hospital stay, ileus duration, preoperative and postoperative hematocrit and platelet counts, blood and platelet transfusions, and hospital costs were collected. RESULTS: Twenty-five patients underwent attempted LS. Four procedures (16%) were converted to OS. Operative time averaged 3.3 +/- 0.2 hours for LS and 2.6 +/- 0.1 hours for OS (P = .001). In the LS group, a regular diet was tolerated 2.1 +/- 0.3 days after surgery (P < .001), and mean postoperative hospital stay was 5.1 +/- 0.6 days (P = .037), compared with 4.3 +/- 0.3 and 6.7 +/- 0.5 days, respectively, in the OS group. No differences were observed in blood loss, complication rate, transfusion requirement, or hospital cost. CONCLUSIONS: Compared with OS, LS requires more operative time, is comparable in blood loss, transfusion requirement, complication rate, and cost, and appears to be superior in terms of return of bowel function and hospital stay.

Adolescent↗

Sclerotherapy for venous malformations.

Of the congenital vascular abnormalities, venous malformations receive little attention and essentially no discussion of treatment. The author describes a 30-year experience with sclerotherapy, which was used for 34 venous malformations. In some cases, these lesions are localized and can be excised, but all the patients in this series had such extensive involvement of adjacent organ systems that no other treatment than sclerotherapy was tenable. Five patients had Klippel-Trenaunay Syndrome, five had head and neck involvement, two had involvement of the entire left side and the remainder had other areas affected. Sodium morrhuate, ethanolamine, sotradecol, and absolute ethyl alcohol were the sclerosing agents used. A butterfly needle was inserted into an anomalous vein, and a three-way stopcock connected to saline and the sclerosing solution was used to ensure intraluminal injection. When rapid runoff into normal venous tributaries could be a concern, a venogram on the operating table preceded injection of the sclerosing solution. Small lesions required only one treatment; widespread bulky lesions required more than 30 injections. The volume of sclerosing solution varied from 5 to 90 mL per injection course. Because of pain, general anesthesia and an overnight hospital stay were necessary. Patients with pharyngeal and/or laryngeal involvement required preliminary tracheostomy or endotracheal ventilatory support for 3 days. Complications included skin necrosis, transient nerve palsy, hemoglobinuria, and one case of anaphylaxis. Repeated aggressive treatment was required for the very large malformations because recanalization occurred. All the patients have been very satisfied with the results.

Adolescent↗

Severe constipation with diffuse intestinal myenteric hyperganglionosis.

The authors report a case of neuronal intestinal dysplasia in a 6-year-old girl. The disease is characterized by hyperplastic ganglia throughout the large and small intestine, associated with severe constipation. To better understand the pathophysiology of this disease the authors investigated the histopathologic, ultrastructural, and immunohistochemical characteristics of the intestinal tissue in this case. The hyperganglionosis was associated with immunohistochemical findings of intact expression of the neuropeptides controlling the peristaltic reflex, through lower expression of calcitonin-gene related peptide. With the recent progress in our understanding of the neural regulation of gastrointestinal function, it may now be possible to begin to understand the complex pathophysiological mechanisms underlying gastrointestinal motility disorders.

Calcitonin Gene-Related Peptide↗

Amnion inversion in the treatment of giant omphalocele.

Operative repair of giant omphaloceles remains a technical challenge to close the wide abdominal wall defect. Currently, most surgeons remove the amnion to approximate the linea alba and/or skin edges or to suture prosthetic material to the abdominal wall and cover the defect with skin flaps. In doing so, the liver commonly becomes extruded and distended with blood, compounding the problem of reducing all of the viscera within the small abdominal cavity. Furthermore, bowel obstruction from adhesions produced from opening the abdomen is a life-long threat. We describe six cases of giant omphalocele in which the amnion was left intact, and it was progressively inverted into the abdominal cavity by using the silastic silo, as it is used for gastroschisis. The birth weight of these infants ranged from 2,360 to 3,240 g. The abdominal wall defect measured 7.0 cm to 10.5 cm in width, and protruded at least 8 cm beyond the abdominal wall. The first stage of repair was to suture the silastic silo to the skin-amnion junction, and progressively reduce the bowel and liver within the abdomen. The intrabdominal pressure is monitored by nasogastric tube or by an indwelling bladder catheter to avoid pressures greater than 20 cm H2O, which might compromise intestinal and renal circulation. The second stage consisted of incising the skin/amnion junction to expose the linea alba. The linea alba was approximated while leaving the amnion intact and folding it into the abdominal cavity. This avoids entering the peritoneum or interfering with the blood flow to and from the liver.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Muscles↗

Renal vein involvement with nephroblastoma: a report of the National Wilms' Tumor Study-3.

The records of children enrolled in the National Wilms' Tumor Study-3 who had Wilms' tumor and thrombosis into the renal vein were reviewed. There were 164 patients with gross involvement of the extrarenal vein and 47 patients with microscopic involvement, an incidence of 11.3% (211 of 1,865 evaluable patients). Two-year survival rates were 90, 79 and 72% for stages II, III and IV, respectively. The most important predictors of survival were histologic pattern and stage. Complete en bloc excision of the tumor and thrombus continues to be the most effective initial management.

Child↗

A comparison of four staging systems for localized and regional neuroblastoma: a report from the Childrens Cancer Study Group.

Four major staging systems have been used to estimate the prognosis for children with local and regional neuroblastoma (NBL). Data obtained at diagnosis for 251 neuroblastoma patients from two Childrens Cancer Study Group (CCSG) studies were analyzed according to staging systems of the CCSG, St Jude Children's Research Hospital, the Pediatric Oncology Group (POG), and the Union Internationale Contre le Cancer (UICC) tumor-nodes-metastasis (TNM) system. The most significant variables were found to be age, tumor stage, extent of tumor removal, transgression of the midline by tumor infiltration, and site of primary tumor. Involvement of lymph nodes per se was not a bad prognostic sign unless associated with extension beyond the midline, the latter being the single most important prognostic variable. All four staging systems had value for prognostication and all identified with accuracy the low stage patient (stage I, stage A) who fares well (greater than or equal to 87% survival). The CCSG definition of stages II and III disease discriminated prognostic groups best among the remaining patients, and was able to identify the child with local-regional NBL with poor survival. The estimated 5-year survival rates for children with regional tumor (stage III, IIIA[N]), according to the four systems were 44%, 74%, 74%, and 74% for the CCSG, St Jude, POG, and UICC methods, respectively. We conclude that all four staging systems effectively define good-prognosis patients with localized disease but that the CCSG staging system most accurately identifies patients with regional tumor who have a poor outcome.

Child↗

Prognosis of posterior urethral valves presenting at birth.

Urethral obstruction due to posterior urethral valves (PUV) is a more lethal condition in the newborn than is commonly recognized. To study its prognosis when the diagnosis of PUV is made shortly after birth, cases of PUV presenting in the first week of life were reviewed. Eleven cases were seen at the University of California, San Francisco from 1974 to 1982. Five babies died, three within two hours of birth of respiratory insufficiency secondary to severe pulmonary hypoplasia, and two within three weeks with rapidly progressive renal failure. Of the six survivors, four exhibited respiratory difficulty (prolonged ventilatory support required in two, and bilateral pneumothoraces in two), three developed renal failure despite early urinary diversion, and one had renal tubular dysfunction. Only two infants had normal renal function after undergoing a successful series of reconstructions. Prune-belly syndrome and limb deformities were present in two surviving and two fatal cases. Although most cases of congenital hydronephrosis can be successfully treated after birth, those presenting in the newborn period represent a subset with high morbidity and mortality. With advances in prenatal diagnosis of congenital hydronephrosis, salvage of severe cases detected early in gestation may require more aggressive management in the prenatal period.

Humans↗

The pathogenesis of prune belly.

Three neonates were born with marked abdominal muscular laxity from three different conditions (posterior urethral valves, nonimmune ascites, and intestinal duplication); two had fetal abdominal distention by ascites documented in utero by obstetric ultrasonographic examination. Another fetus, whose abdominal distention was relieved at 21 weeks' gestation by in utero decompression of urinary obstruction, was born with only mild abdominal changes, which suggests that the "prune belly" phenotype can resolve if distention is relieved early enough. These observations suggest that the abdominal laxity in prune belly syndrome is a simple deformation secondary to abdominal stretching and distention during fetal development and is not an intrinsic generalized mesodermal abnormality.

Amniotic Fluid↗

Management of the fetus with an abdominal wall defect.

Eleven fetuses with omphalocele and two with gastroschisis were diagnosed by prenatal ultrasonographic examination. Intact omphalocele was accurately distinguished from gastroschisis by detection of a membranous sac covering the herniated viscera, by liver protruding from the abdomen, and by the frequent association of major structural anomalies. Gastroschisis was characterized by the absence of these findings and the presence of bowel loops floating freely in the amniotic fluid. Nine of eleven fetuses with omphaloceles had other major malformations and did not survive. Two small omphaloceles and two gastroschises were correctly diagnosed as isolated defects, free from associated anomalies. All four underwent maternal transport and were successfully managed with good outcome. Prenatal ultrasonography allows rational decisions about perinatal management by distinguishing between omphalocele and gastroschisis and by screening for associated anatomic defects. Karyotype analysis should be a part of the prenatal workup. Most isolated defects can be followed and delivered normally near term. Accurate prenatal diagnosis allows maternal transport and in a few cases may alter the timing or mode of delivery.

Adolescent↗

Colon interposition: an adjuvant operation for short-gut syndrome.

Six infants with short-gut syndrome refractory to medical management underwent isoperistaltic colon interposition (length 11.7 +/- 3.1 cm.). The abdominal catastrophes that required extensive intestinal resection were: volvulus (3), necrotizing enterocolitis (2), and gastroschisis with intestinal atresia (1). No infant had another major congenital anomaly. The average trial of attempted medical therapy prior to colon interposition was 5.5 +/- 3.6 months. There was no perioperative mortality or morbidity associated with the colon interposition. Following the colon interposition, three infants were weaned from total parenteral nutrition (TPN) in 3 +/- 1 months and all survived. In contrast, three infants could not be withdrawn from TPN and they died secondary to complications of TPN (2 from sepsis and 1 from hepatic failure). Long-term survival was associated with a greater length of small bowel remaining after the initial resection (51 +/- 12 cm v 35 +/- 24 cm), colon interposition at a younger age (3 +/- 1 months v 8 +/- 3.5 months), and a shorter duration of medical management prior to colon interposition (2.8 +/- 0.8 months v 6.7 +/- 5.0 months). All survivors are now tolerating a regular diet and having one to four formed stools per day. Normal somatic growth and developmental milestones are being achieved. The follow-up period is from 24 to 84 months. Our experience with the colon interposition in the patient with short gut syndrome has led us to conclude that when a reasonable trial of medical management has failed, a colon interposition is a safe and effective adjuvant to treatment.

Child Development↗

Congenital (infantile) hemangiopericytoma of the tongue and sublingual region.

Congenital (infantile) hemangiopericytoma is a rare lesion, previously described only in subcutaneous and central nervous system locations. The authors report here an obstructing tumor of the tongue and sublingual oral cavity, discovered at birth, in an otherwise normal female infant of 35 weeks' gestation. The tumor had a histopathologic and ultrastructural appearance similar to previous descriptions of infantile hemangiopericytoma. Clinical features in this case included rapid local recurrence after initial excision; however, after 30 months of follow-up, there has been no evidence of further recurrence or metastasis. Because this patient was treated with chemotherapy, the authors cannot determine to what extent this benign course reflects the natural history of this process or the influence of the treatment administered.

Cyclophosphamide↗

Desmoid tumors: local control and patterns of relapse following radiation therapy.

Desmoid tumors are benign neoplasms, arising from musculoaponeurotic tissues, which tend to be locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Nineteen patients with desmoid tumors underwent radiation therapy at the University of California, San Francisco, between 1970 and 1980. Fifteen patients were referred with local recurrence following one or more surgical resections. Three patients were referred for initial radiation therapy with unresectable tumors, and one patient received planned postoperative irradiation following subtotal tumor resection. At the time of treatment, 8 patients had nonresectable disease measuring greater than 10 cm. Five patients had residual tumor masses measuring 4 to 6 cm, and six had only microscopic disease following resection. The majority of patients were treated to a tumor dose of 50-55 Gy at 1.6 to 1.8 Gy per fraction. With a median follow-up of 8 years, 13 patients remained free of recurrent disease following radiation therapy. The 5 year relapse free survival was 72% with 10 patients continuing to be free of disease 5 to 11 years following therapy. Local control was not related to the amount of disease present at the time of treatment. Of the 6 patients who developed recurrent disease, only 1 patient had a true in-field recurrence. Four patients recurred at the margin of the radiation field 1 to 5 years following therapy. Of these four patients, 3 were successfully salvaged while 1 died as a result of tumor extension into a major vessel. One patient with an extensive mesenteric mass did not respond to therapy and died 1 month post irradiation. The patient with the in-field recurrence and 1 patient with a marginal recurrence were successfully treated with combination chemotherapy. Moderate dose radiation therapy to desmoid tumors can result in lasting local control when surgical resection is not possible. Post operative radiation can improve the rate of local control for patients with a high risk of recurrence. As desmoid tumors tend to be locally infiltrative, fields must be very generous to prevent marginal recurrence. Systemic chemotherapy offers an alternative to ablative surgery in the event of local failure following radiation therapy.

Adolescent↗