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Biomedical subjects

E Freud

Publications and source records attributed to E Freud.

At least 55 records · Page 3Linked to original sources
PubMed1993

The value of precise preoperative localization of colonic arteriovenous malformation in childhood.

Massive hemorrhage from the gastrointestinal tract in a 12-yr-old boy caused by a congenital atypically located colonic arteriovenous malformation is described. Guided and "clean" resection of the involved colon was possible due to preoperative selective angiography, which proved to be the most efficient diagnostic tool. Histologic documentation of this rare pathology in childhood is presented, and the classification and features of the disease are briefly reviewed.

Angiography↗
PubMed1992

Familial chronic recurrent pancreatitis in identical twins. Case report and review of the literature.

Familial presentation of chronic recurrent pancreatitis in childhood is rare. The etiology of this illness is obscure, and its hereditary properties are not well defined. Simultaneous occurrence of chronic recurrent pancreatitis in identical twins with the same clinical presentation and similar typical pancreatographic abnormalities is exceptional. Twin sisters, aged 9 years, were admitted to the hospital because of recurrent attacks of pancreatitis. Ultrasound examination revealed an enlarged irregular pancreatic duct in both girls, and endoscopic retrograde cholangiopancreatography showed a distorted duct with multiple strictures and dilatations similar to a "chain of lakes" pattern. Both patients underwent longitudinal pancreatojejunostomy within a month. The therapeutic regimen and preoperative and surgical treatment of such patients are discussed, as is the optimal timing of intervention.

Child↗
PubMed1992

Subtotal splenectomy in Gaucher's disease: towards a definition of critical splenic mass.

Partial splenectomy is currently the favoured surgical approach for Gaucher's disease. Preservation of splenic tissue is indicated to prevent susceptibility to overwhelming postsplenectomy sepsis, to delay the massive deposition of glucocerebroside in the liver and bones, and to relieve symptoms of hypersplenism. Controversial issues and technical problems related to partial splenectomy for Gaucher's disease are discussed and a definition of critical splenic mass considered.

Gaucher Disease↗
PubMed1992

Neonatal intestinal perforation due to congenital defects in the intestinal muscularis.

Congenital defect of the muscular layer of the small intestine is a rare cause of spontaneous bowel perforation in premature infants. During the last 12 years we have observed four similar cases. We describe the most recent one, a premature infant who developed two abdominal events. On her 2nd day of life, spontaneous perforation of the distal ileum due to focal absence of the muscular layer occurred. Several weeks later she developed the typical clinical and histological picture of necrotizing enterocolitis. The clinical and histological characteristics of the two different conditions are compared, and the 24 cases reported in the literature are discussed. We conclude that focal absence of intestinal musculature may be not such a rare entity as is commonly believed.

Female↗
PubMed1992

Long-term follow-up of partial splenectomy in Gaucher's disease.

Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.

Adolescent↗
PubMed1991

Inflammatory pseudotumor in childhood. A diagnostic and therapeutic dilemma.

We describe a case of inflammatory pseudotumor of the lower abdomen in a young child. The urinary bladder and abdominal wall were involved, and the pseudotumor simulated a malignant sarcomatous tumor. Surgical excision was undertaken, and the outcome was favorable. This rare tumor simulates a true neoplasm both clinically and morphologically and presents a diagnostic and therapeutic dilemma. Our case and a review of the literature indicate that an effort should be made to perform a radical excision. This is considered the treatment of choice except if it requires a mutilating procedure.

Abdominal Muscles↗
PubMed1991

Hepatic iron overload: quantitative MR imaging.

Iron deposits demonstrate characteristically shortened T2 relaxation times. Several previously published studies reported poor correlation between the in vivo hepatic 1/T2 measurements made by means of midfield magnetic resonance (MR) units and the hepatic iron content of iron-overloaded patients. In this study, the authors assessed the use of in vivo 1/T2 measurements obtained by means of MR imaging at 0.5 T using short echo times (13.4 and 30 msec) and single-echo-sequences as well as computed tomographic (CT) attenuation as a measure of liver iron concentration in 10 severely iron-overloaded patients with beta-thalassemia major. The iron concentrations in surgical wedge biopsy samples of the liver, which varied between 3 and 9 mg/g of wet weight (normal, less than or equal to 0.5 mg/g), correlated well (r = .93, P less than or equal to .0001) with the preoperative in vivo hepatic 1/T2 measurements. The CT attenuation did not correlate with liver iron concentration. Quantitative MR imaging is a readily available noninvasive method for the assessment of hepatic iron concentration in iron-overloaded patients, reducing the need for needle biopsies of the liver.

Adolescent↗
PubMed1987

Congenital disseminated neurofibromatosis: a "benign" diagnosis with malignant prognosis.

A neonate with congenital disseminated neurofibromatosis involving skin, subcutaneous tissues retropharyngeal, retroperitoneal and urinary bladder is presented. The massive presentation precluded radical surgical approach. This seems to be the first report of congenital widely disseminated neonatal neurofibromatosis in the English literature.

Abdominal Neoplasms↗
PubMed1985

Familial meconium ileus with normal sweat electrolytes.

Meconium ileus (MeI) is the presenting symptom in 10 to 15 percent of infants with cystic fibrosis (CF), but it is a rare phenomenon in patients who do not have this disease. We report four Bedouin siblings who had MeI with no laboratory evidence of CF.

Electrolytes↗
PubMedDate not supplied

Decreased insulin-like growth factor-I receptor sites on circulating mononuclear cells from children with acute leukemia.

Insulin-like growth factor-I (IGF-I) is a known mitogen for various cell types, including those of the hematopoietic cell system. To study the role of IGF-I in the neoplastic process of leukemia in children, the authors have determined the number of IGF-I binding sites on circulating mononuclear cells of children with acute leukemia as compared to normal children, using binding assays. The IGF-I binding sites per cell on peripheral mononuclear cells of children with leukemia decreased compared to those of the control group (411 +/- 73 and 1334 +/- 227, respectively, p < .001), while their affinity increased (Kd = 0.14 +/- 0.04 and 0.43 +/- 0.16, respectively, p = .05). Furthermore, in the patients, the number of the IGF-I binding sites was significantly lower in the subgroup of the peripheral mononuclear cells, which included lymphocytes and monocytes, as compared to their number in the peripheral blast cells (254 +/- 43.6 and 536 +/- 98.6, respectively, p = .02). A significant reduction was found in serum GHBP levels in the patients as compared to the controls (28.21 +/- 1.93 and 35.83 +/- 2.90, respectively, p = .02), while serum IGF-I and growth hormone levels were similar in patient and control groups. These results suggest a possible involvement of IGF-I in childhood acute leukemia, but further studies are needed to establish whether IGF-I plays a role in this disease.

Adolescent↗
PubMedDate not supplied

A plea for incidental appendectomy in pediatric patients with malignancy.

The evaluation of right lower quadrant (RLQ) abdominal pain in pediatric patients with malignancy can be difficult. However, since the mortality rate from peritoneal infections in these patients is very high, the differential diagnosis of RLQ peritoneal irritation, mainly of acute appendicitis (AA) versus neutropenic enterocolitis (NE), is crucial. Three cases of pediatric patients with malignancy demonstrating these difficulties are represented to enlighten this problem. The first patient died of multiorgan failure after operation for perforated appendicitis without generalized peritonitis. The second had a severe life-threatening postoperative complication because of delayed diagnosis of acute appendicitis. The third patient with malignant pelvic spread, underwent an unnecessary abdominal exploration for suspected AA. In all these cases and probably in many others, the clinical outcome could have been different if a previous incidental appendectomy had been performed during the primary abdominal operation. Incidental appendectomy in oncologic patients is recommended to facilitate the differential diagnosis of RLQ pain and to exclude the diagnosis of AA.

Adolescent↗
PubMedDate not supplied

Anal stenosis with megarectum: an unusual complication of a perineal burn.

A case of a 3-year-old boy with anal stricture and megarectum is described. Overflow incontinence and soiling were corrected by local flaps and skin graft and were maintained by frequent dilatations after downward traction of the whole anus was performed. The options of immediate and late treatment of such burns and the colostomy procedure are discussed.

Anus Diseases↗