תוצאת חיפוש

The Effect of Gastro-Esophageal Reflux Therapy on Respiratory Diseases in Children

Y. Levin¹, A. Mandelberg², A. Gornstein³, F. Srour³, S. Reif⁴

¹The Sackler Faculty of Medicine, Tel-Aviv University, ²The Unit of Pediatric-Pulmonology, Wolfson Hospital, ³The Unit of Pediatric-Surgery, Wolfson Hospital, ⁴The Unit of Pediatric-Gastroenterology, Dana Children's Hospital

In order to examine the effect of reflux therapy on Hyper Reactive Airway Disease (HRAD) and apnea severity, 107 children, 78 with HRAD and 29 with apnea, underwent pH monitoring in the Pediatric Surgery Unit of Wolfson Hospital and the Dana Children's Hospital during the years 1995-1998. Pathological reflux was defined by means of the Boix-Ochoa and RI (Reflux Index) scores. In patients with positive reflux, anti-reflux treatment was initiated. Prior to and following pH monitoring, the respiratory status of all patients (both with and without reflux) was evaluated by a pediatric pulmonologist employing commonly used scores to determine severity.

Results: Subject age ranged between one day and 15 years (mean: 15.44±29 months, median: 6.37 months). In HRAD, following anti-reflex treatment the reflux positive group showed a significant score improvement, from an average of 2.9±1.1 units to 1.54±1.2 units (p<0.0001); a decrease in the number of patients treated with oral corticosteroids (p<0.01); a close to significant decrease (p=0.069) in the average dose of inhaled corticosteroids; and a decrease in the number of patients using bronchodilators (p=0.042). The reflux-negative group, not treated for reflux, displayed no significant improvement, with only a decrease in the severity scores from 2.44±1.0 to 1.78±1.2 units (p=0.14), and no change on any of the other parameters.

In apnea, all patients improved, from an average score of 2.34±0.77 to 0.03±0.19 units (p<0.0001), with no significant difference between the reflux positive and the reflux negative groups.

In view of these findings, it is postulated that anti-reflux therapy may have an additive effect on HRAD severity, beyond that of spontaneous respiratory improvement. We therefore find it appropriate for every severe HRAD patient (frequent exacerbations or high corticosteroid dose) to undergo pH monitoring in order to treat those with proven reflux. In respect to apnea, we cannot attribute any significance to the existence of reflux or to anti-reflux treatment.

Metallic Stents for Acute Colonic Obstruction

M. Itkin, E. Atar, H. Neuman, D. Kravarosic, Z. Fuko, M. Kaz

Radiology and General Surgery B Depts., Golda Campus; and General Surgery Dept., Beilinson Campus; Rabin Medical Center, Petah Tikva

The traditional treatment of acute colonic obstruction, usually caused by malignant tumor, is a 2-stage surgical procedure. The first operation is emergent and includes primary tumor resection and end colostomy followed by scheduled colostomy closure. These operations are associated with high mortality and morbidity. Recently, insertion of self-expanding metallic stents for temporary colonic decompression has been was introduced. With this new technique colostomy can be avoided in the acute phase.

In advanced colonic cancer stent-insertion is the only palliative treatment. It is done in the radiology department under fluoroscopic guidance. We present 2 cases of malignant colonic obstruction treated successfully by stent insertion.

A Molecular Method of Diagnosis of Congenital Adrenal Hyperplasia

Shoshana Israel, Chaim Brautbar

Tissue Typing Unit, Hadassah Medical Center, Jerusalem

Congenital adrenal hyperplasia (CAH) is caused mainly by deficiency of the 21-hydroxylase enzyme. The disease may appear in the classical salt-losing, simple virilizing forms or as a mild, nonclassical form. 21-hydroxylase is encoded by the CYP21B gene on the short arm of chromosome 6, in the midst of the human leukocyte antigen (HLA) complex, between HLA Class I and Class II regions.

We describe a method for identifying mutations in the CYP21B gene. It is based on amplification of the gene using the polymerase chain reaction and identification of mutations with sequence-specific oligo-probes. The mutations identified were: V281 and P30L responsible for nonclassical CAH, and I2 splice, Q318X, I172N, cluster E6, and a deletion including 8bP in the third exon (8bP del) responsible for the classical form of CAH.

We also analyzed 2 families affected with the classical form of CAH which demonstrate possible complications in genotyping. Typing for HLA haplotypes can be helpful in certain cases, as demonstrated in 1 of the families presented. In this case it was necessary to distinguish between 2 possible genotypes: 1 with the mutations in tandem on 1 chromosome and the other with the mutated genes on both chromosomes. HLA haplotyping enabled the assignment of the mutations to the relevant chromosomes and thus allowed correct genetic counseling.

The other family demonstrated the importance of CYP21B genotyping in individuals with the nonclassical form of CAH. This form may consist of 1 mild and 1 severe mutation, representing a serious potential for transmitting the classical form of CAH.

Pulmonary Adenocarcinoma in Myasthenia Gravis - Auto-Immunity and Late Development of Malignancy

Yitzhak Rosen, Yair Levy, Yehuda Shoenfeld

Dept. of Internal Medicine B, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

We report a 75-year-old man with myasthenia gravis for many years, who was hospitalized because of cough, fever, and dyspnea. Chest x-ray revealed a bilateral pleural effusion. Adenocarcinomatous cells were found in the pleural fluid. Computerized tomography of the chest showed widespread pulmonary dissemination of the tumor.

The relationship between myasthenia gravis, an autoimmune disease involving the motor end-plate, and malignancy (thymoma) has been widely recognized. Current literature documents few reports of lung malignancies with concurrent development of myasthenia gravis. A tentative explanation, based on current research, is provided for the possible role of myasthenia gravis and the late development of lung cancer. Moreover, a model for the autoimmune phenomenon and the development of late malignancies will be provided with explicit explanations. It is important to search for occult, developing malignancies in newly diagnosed autoimmune diseases.

Psychological Treatment for Paruresis (Bashful Bladder)

Baruch Elitzur

Psychiatric Clinic, Ichilov Hospital, Tel Aviv Medical Center

Paruresis, or bashful bladder, is a functional disorder that involves inability to urinate in the presence of others. The symptom meets the DSM-IV diagnostic criteria for simple phobia or social phobia. Although the prevalence of paruresis is relatively high (7% in 1 study and 32% in another), there are few reports of its treatment. The treatment of choice presented in the literature is cognitive-behavioral.

We describe a multidimensional model used in treating 3 men who suffered from paruresis. Promotion of relaxation, mental imagery, paradox, gestalt, metaphor, cognitive-behavioral and psychodynamic techniques were used. After 5 treatment meetings, 2 younger men, 18- and 24-years old, respectively, were able to overcome the condition, while a 50-year-old got only partial relief.

Prader-Willi Syndrome: Medical, Emotional and Cognitive Facets

Varda Gross-Tsur, Yael E. Landau

Neuropediatric Unit, Shaare Zedek Medical Center, Jerusalem

Prader-Willi syndrome, first described in 1956, is characterized by marked hypotonia, hyperphagia, severe obesity, short stature, hypogonadism, orthopedic problems, breathing- related sleep disorders, mild to moderate mental retardation and behavioral abnormalities. The incidence of this syndrome, an expression of a genetic imprinting error in chromosome 15, is 1:10,000-1:25,000.

We describe the medical, emotional and cognitive parameters of 34 patients in our multidisciplinary clinic for Prader-Willi syndrome. Their ages range from 5 months to 40 years and 20 are males. Excessive weight gain started at the age of 6 years, increasing to 170-370% of that predicted by height and age and short stature started after the age of 12. All males have hypogonadism; 6 patients have scoliosis. Breathing-related sleep disorders have occurred in 15.

Children above the age of 8 years underwent neuropsychological assessment: half (9/18) have borderline intelligence while a quarter have low-normal intelligence and the remainder mild to moderate mental retardation. Behavioral and social problems are common, and become more prominent during adolescence. ADHD was diagnosed in 10/18.

Laparoscopic Adrenalectomy 

G. Zamir, D. Hazzan, D.J. Gross, S. Lyass, O. Jurim, E. Shiloni, P. Reissman

Depts. of Surgery, Endocrinology and Metabolism, Hadassah University Hospital, Ein-Kerem, Jerusalem

Constant advances and increasing experience in laparoscopic surgery renders it applicable for adrenal surgery. The wide exposure required for open adrenal surgery makes this minimally invasive procedure an attractive and advantageous alternative.

Between 1996-1999, we performed 35 laparoscopic adrenal-ectomies in 30 patients 20-72-years old. Indications included: Conn's syndrome - 14, pheochromocytoma - 11, Cushing's syndrome - 6, nonfunctioning adenoma - 3, and metastatic sarcoma - 1.

5 underwent bilateral laparoscopic adrenalectomy. In 3 (8.5%) the procedures were converted to open operations. Overall morbidity was 13% and there was no mortality. Mean operative time was 188 minutes, but only 130 in our last 10 cases. Mean hospital stay was 4 days and they returned to normal activity an average of 2 weeks later.

According to our study and previous reports, laparoscopic adrenalectomy is feasible and safe and it may soon become the procedure of choice for adrenal tumors.

Diabetic Ketoacidosis during the Ramadan Fast 

I. Friedrich, Y. Levy

Dept. of Medicine A, HaEmek Medical Center, Afula, and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

We report a 15-year-old Muslim boy with insulin-dependent diabetes mellitus (IDDM) who presented with diabetic ketoacidosis (DKA) during the Muslim Ramadan month of day-time fasting. DKA apparently occurred due to omitting pre-lunch insulin combined with dehydration and overeating during the permitted sunset-to-sunrise meals.

It is well-known that fasting accelerates development of lipolysis and ketosis and increases glucagon levels. Thus, these pathophysiological aberrations related to fasting in ketosis-prone patients, in conjunction with fasting, endanger metabolic control in IDDM.

Extraperitoneal Laparoscopic Adrenalectomy

J. Kuriansky, A. Saenz, D. Rosin, E. Astudillo, L. Fernandez-Cruz

Dept. of General Surgery and Transplantation, Sheba Medical Center, Tel Hashomer, and Dept. of Surgery, Hospital Clinic, Barcelona

Laparoscopic adrenalectomy has recently been shown to be safe and effective for a variety of benign adrenal tumors, such as aldosterone adenoma, pheochromocytoma, nonfunctioning adenoma, Cushing's adenoma and Cushing's syndrome. Recently, laparoscopic surgeons adopted an extraperitoneal approach in which a space is created by the introduction of a trocar with an inflatable balloon tip. This technique with a minimal access procedure provides direct access to the adrenal glands without interfering with intraperitoneal organs.

Laparoscopic adrenalectomy using the extraperitoneal approach was completed in 18/20 consecutive patients. Mean operative time was 95 min (range 80-120) and median hospital stay 3 days. Blood transfusion was not required, and there were no postoperative complications.

Alpha-Amino Adipic Aciduria: a Rare Psycho-Motor Syndrome

Ben-Ami Sela, Tamar Massos, Dov Fogel, Joseph Zlotnik

Pathological Chemistry and Child Development Institutes, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

A 3.5-year-old boy with developmental motor retardation, hypotonicity, and severe speech disturbance had alpha-amino adipic acid in his blood and very high levels in his urine. In only 20 cases has this catabolite of lysine and hydroxylysine been found in high concentrations in urine, due to enzymatic block.

The clinical features associated with alpha-amino adipic aciduria may include mental retardation, developmental and motor delay, learning difficulties, convulsions, speech problems and ataxia. 3 siblings had milder symptoms of psychomotor delay and intermediate degrees of alpha amino-adipic aciduria, suggesting that the described developmental deficits could be related to this metabolite or its derivatives.

Tension-Free Vaginal Tape Procedure for Female Stress Incontinence

David Gordon, Asnat Groutz, Joseph Lessing

Lis Maternity Hospital, Sourasky-Tel Aviv Medical Center

We evaluated the safety and efficacy of the tension-free vaginal tape procedure (TVT) in 20 consecutive women with urodynamically proven genuine stress incontinence. Mean operating time under spinal analgesia was 37.2‏13 minutes and 80% were discharged within 24 hours. There were 5 uncomplicated, intraoperative bladder perforations, but no defects in healing nor tape rejection. The objective cure rate was 95%, while the subjective rate was 100%. Although follow-up was short (9-16 months), the TVT method seems to be safe and effective for stress urinary incontinence.

Premature Birth and Cognitive Functioning in Adolescence

Ofra Lubetzky, Avi Weitzman, Izhak Gilat, Samuel Tyano

Levinsky College of Education and Geha Hospital, Tel Aviv and Sackler Faculty of Medicine, Tel Aviv University

Premature infants are considered a high-risk population for developing cognitive dysfunction. Studies have indicated lower cognitive performance among elementary school children born prematurely. We focused on cognitive functioning of such adolescents. This age was chosen because of its critical importance in the development of the individual.

50 adolescents aged 14-16 years born prematurely were compared with 50 born at full-term and matched for gender, age and socioeconomic status. All subjects attended regular schools and did not suffer severe neurological disorders. Cognitive functioning was measured by the Bender-Visual Motor Gestalt Test and by 3 subtests from the Wechsler Intelligence Scale for Children (revised WISC-R test).

Results revealed that prematurely born adolescents scored lower than those born at term on all measures of cognitive performance. The results are discussed in terms of their developmental meaning and of therapy for the prematurely born.

Retinal Lesion due to Excessive Exposure to Sunlight

Irene Krasniz, Itzchak Beiran, Benjamin Miller

Dept. of Ophthalmology, Rambam Medical Center and Bruce Rappaport Faculty of Medicine, Technion, Haifa

Retinal damage caused by direct exposure to the sun's rays is well recognized by the ophthalmic community. Although functional ability in solar retinopathy is usually regained within weeks, some suffer long-term visual impairment. Anatomic damage to the retina, even in those who regain full vision, is permanent. We describe 2 cases of solar retinopathy, 1 of which remained with permanent loss of vision. The role of medical education in preventing damage from solar retinal hazards is stressed.

Breast Conservation: Safe for Early Breast Cancer

Gil Bar-Sella, Georgetta Fried, Zipora Brotman, Anna Ravkin, Riva Borovik, Abraham Kuten

Dept. of Oncology, Rambam Medical Center; Dept. of Oncology, Lin Medical Center; and Rappaport Faculty of Medicine, Technion, Haifa

Between 1981-1993 581 women with primary breast cancer were treated by breast conservation. Their mean age was 56‏12 years and 63% were postmenopausal and 37% pre- or perimenopausal. The median follow-up time was 56 months. 45% had pathological Stage I disease, 49% Stage II, 2.5% Stage III and 3.5% clinical Stage I-II disease. 54% of lesions were excised with good margins, 10% with close margins (<0.5 cm), 9% with microscopic residual, 3% with macroscopic residual, and in 24% margins were not reported. Adjuvant therapy, consisting of combination chemotherapy and/or hormones, was given to 69%.

Radiotherapy, usually 50 Gy tangential photon irradiation to the whole breast, was given to 564 (97%); an electron or photon "boost" to the tumor with a median dose of 17.5 Gy was given to 378 (65%). Most of those with positive nodes received 50 Gy to the lymphatic drainage system.

1 year after radiotherapy cosmetic results were rated as "good" or "excellent" in 80%, "moderate" in 17% and "poor" in 3%. The 5-year actuarial survival was 97% in Stage I and 88% in Stage II. 37 patients (6.5%) developed breast recurrence; 11 of these (2%) had simultaneous distant metastases. 5 (<1%) developed axillary or supraclavicular lymph node metastases, and 81 (14%) developed distant metastases. Most local recurrences were in those younger than 40, and in those with primary tumors >1.75 cm.

The satisfactory level of local control achieved is attributed to the high doses of radiation (up to 75 Gy) administered to those with high risk lesions.

אברהם דומב

המח' לכימיה תרופתית, ביה"ס לרוקחות, הפקולטה לרפואה, האוניברסיטה העברית, ירושלים