Liver Failure with Coagulopathy in an Infant with Tyrosinemia

Moshe Nussinovitch, Gadi Campino, Rivka Shapira, Benjamin Voluvitz, Jacob Amir

Department of Pediatrics, Schneider Children's Medical Center of Israel, Petah Tikva, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

Tyrosinemia is an inherited autsomal recessive condition. We present a 5 week-old boy with this disorder. He was admitted because of a fever, vomiting and lethargy. The laboratory tests confirmed a coagulopathy with prolonged prothrombin time (PT), partial thromboplastin time (PTT) and a decreased serum fibrinogen. The a-fetoprotein level was markedly elevated. To confirm the diagnosis of tyrosinemia, quantitative urinary succinylacetone was measured. Although overt liver failure with coagulopathy may be part of the representation of tyrosinemia, a significant coagulopathy in the absence of overt signs of liver disease has not been emphasized as a clue to the diagnosis of this condition.

Male Genital Self Mutilation - A Cause of Hematuria

P. Schwartzman, Z. Viner

Department of Familyu Medicine, Community Health Section, Ben-Gurion University, The Negev, Beer-Sheva, Israel

Pathological self-mutilation is defined as a deliberate destruction of body tissue without conscious intent. It is sometimes associated with several pathologies such as mental disorders and mental retardation.

We report a non-psychotic male, with borderline mental retardation who practiced sporadic moderate genital self-mutilations as a masturbation technique and presented as a case of recurrent gross hematuria.

Ent of Femoral Artery Pseudo-Aneurysms 

G. Szendro, A. Klimov, A. Lennox, B. Jonathan, L. Avrahami, B. Yechieli, M. Griffin, S. Yurfest, Y. Charach, L. Golcman, A.N. Nicolaides

Vascular Surgery Dept., Soroka Medical Center, Beer Sheba; Irvine Laboratory for Cardiovascular Investigation and Research, St. Mary's  Hospital, Imperial College Medical  School, London; Vascular Laboratory, Soroka Medical Center, Beer Sheba; and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

The femoral artery remains the most used peripheral site for radiological catheter access. With a greater number of both diagnostic and therapeutic procedures being performed by interventional radiologists and cardiologists, and with larger catheters being used for stenting and endovascular grafting, the incidence of iatrogenic pseudo-aneurysms reported has reached as high as 0.5-2%. Ideally, they should thrombose spontaneously. However, when this does not occur, management options include: observation, ultrasound-guided obliterative compression, direct thrombin injection, embolization, stent graft insertion, and very rarely- surgery.

During a 7-year period (1992-1999) we treated 131 cases of femoral artery false aneurysms. Until 1998 ultrasound-guided compression-obliteration, with a 95% success rate, was our method of choice. Since 1998, direct thrombin injection, with 100% success in 24 cases, has become our preferred method. It is pain-free, fully successful even in anticoagulated patients, and is currently our treatment of choice.

Minocycline Pneumonitis and Eosinophilia

Noam Fink, Meir Mouallem

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

Pneumonitis with eosinophilia is one of the less common and severe adverse effects of minocycline. The disease evolves in days or weeks from the beginning of treatment, and is usually characterized by dyspnea, fever and bilateral infiltrates in the chest X-ray. With cessation of the antibiotic, and sometimes adding cortico-steroids, clinical and roentgenological resolution follow.

We present a case given minocycline for folliculitis and 3 weeks later fever, cough and shortness of breath developed. The clinical and roentgenological course was consistent with minocycline pneumonitis accompanied by eosinophilia.

Preflight Assessment by Hypoxic Inhalation Test in Cardio-Pulmonary Patients 

J. Lebzelter, G. Fink, E. Kleinman, I. Rosenberg, M.R. Kramer

Pulmonology Institute, Rabin Medical Center, Beilinson Campus, Petah Tikva

Flying may expose passengers to hypoxic conditions, which may induce hypoxemia, particularly in those with chronic heart and/or lung disease. Onset of dyspnea, wheezing, chest pain, cyanosis and right heart failure can lead to urgent need for oxygen during flight. The hypoxia inhalation test (HIT) provides a safe and simple means of identifying those who may develop hypoxemia during flight.

We report our experience with 48 self-reporting patients who underwent HIT prior to pre-planned air travel. They inhaled for 15-minute periods a reduced oxygen concentration (F₁O₂ 15%) under normobaric conditions, during which O₂ saturation was monitored by pulse oximeter; electrocardiogram, blood pressure and symptoms were also monitored. O₂ saturation of 85% (PaO₂ 50 mm Hg) was considered a positive test. In the 8 cases (17%) with a positive test, 5 had chronic obstructive pulmonary disease and 3 had cardiovascular and/or combined heart-lung disease.

We calculated predicted O₂ partial pressure in altitude (PaO₂ ALT) and compared it to actual results in the 8 patients with a positive HIT. In 5, use of the predicted formula would have under-diagnosed the hypoxemia that developed during the HIT. Thus, the results of the HIT changed treatment strategy in these patients. We recommend that patients with positive tests use O₂ (2LPM or 4LPM) during flight.

HIT is practical and of potential benefit in the objective assessment of patients with various degrees of heart, lung or combined heart-lung disease. Clinicians should be aware of the relative risk of hypoxia during flight in such patients, and of the value of HIT in identifying them, leading to increase in its use.

High Dose Endobronchial Brachy - Therapy for Malignant Airway Obstruction

Alex Yarmolovsky, Alan Katz, Eyal Fenig, G. Fink, Daniel Bendayan, Aaron Sulkes, Mordechai R. Kramer

Institutes of Pulmonology and Oncology, Rabin Medical Center, Beilinson Campus, Petah Tikva, and Sackler Faculty of Medicine, Tel Aviv University

Brachytherapy is especially suitable for palliative treatment of endobronchial tumors adjacent to internal organs that might be damaged by intensive external beam radiation, but are easily accessed with a flexible bronchoscope. This treatment is mostly palliative.

30 patients underwent such palliative high-dose endobronchial brachytherapy to alleviate malignant airway obstruction. With the aid of a flexible fiberoptic bronchoscope an endobronchial catheter was inserted adjacent to the tumor and treatment delivered using a 192Ir remote afterloader for 5-10 min, in 2 or 3 sessions.

There was symptomatic improvement in 26 (86%) and objective improvement in 27 (90%). 1 patient died of hemoptysis.

Sacrospinous Ligament Fixation for Vaginal Vault Prolapse

M. Neuman, B. Zuckerman, O. Lavie, U. Beller

Division of Gynecological Surgery and Oncology, Shaare Zedek Medical Center, Jerusalem

Sacrospinous ligament fixation is a well-known method for correction of vaginal vault prolapse. The procedure is primarily indicated after hysterectomy and as a prophylactic measure for total uterine prolapse.

8 women with post-hysterectomy vaginal vault prolapse aged 48-72 years, were referred 1-25 years following primary surgery. Sacrospinous ligament fixation was elected to enable simultaneous correction of cystocele and rectocele, and to preserve sexual function. All operations were completed without significant complications.

In 6 of the 8 patients located for long-term interview, 1 reported mild bulging of the introitus, another mild urinary stress incontinence, urgency and frequency and 1 reported frequency only. Sexual function in 4 was without complaints. Defecation in all patients was normal.
We conclude that this operation is safe and effective. We encourage gynecologic surgeons to consult an experienced tutor prior to performing this procedure, as this type of surgery is quite dangerous and many gynecologists are not familiar enough with it.  

Apomorphine for "Off-Periods" in Parkinson's Disease

J. Zoldan, D. Merims, A. Kuritzky, I. Ziv, E. Melamed

Dept. of Neurology, Rabin Medical Center, Beilinson Campus, Petah Tikva and Sackler Faculty of Medicine, Tel Aviv University

After 3-5 years of continuous use of 1-dopa preparations for Parkinson's disease, 25%-50% of patients develop side-effects such as the "on-off" phenomenon and involuntary movements that markedly impair function. One cause of these manifestations is evidently a disturbance in the absorption of 1-dopa.

We attempted to avoid this problem by using subcutaneous injections. Apomorphine is a rapid-acting dopamine agonist which causes a return from "off" to "on" within minutes.

We present the results of a trial of subcutaneous injections of apomorphine in 22 Parkinsonian patients (12 males, 10 females) with severe motor fluctuations. During 5 days prior to the apomorphine all received Motilium (domperidone, 60 mg/d) to prevent nausea and vomiting. All were hospitalized initially to determine optimal dosage and to teach them the technique of self-injection.

2 to 4 mg of apomorphine were injected 1 to 3 times daily for 2 to 12 months. In 17 patients (80%) "off" periods were reduced without significant side-effects. Apomorphine seems to be effective, tolerable treatment for shortening 1-dopa induced "off" periods.

Elastofibroma, a Rare Cause of Snapping Scapula Syndrome

Ilan Cohen, Yehuda Kolender, Josephine Isakov, Aaron Chechick, Yitzhak Meller

Dept. of Orthopedic Surgery, Sheba Medical Center, Tel Hashomer and Depts. of Orthopedic Oncology and Pathology, Sourasky Medical Center, Tel Aviv

Scapular pain is a common complaint in daily orthopedic practice. A different type of scapular discomfort, the snapping scapula syndrome that occurs when smooth gliding motion of the scapula upon the chest wall is interfered with is much less common.

We studied the syndrome of periscapular pain and discomfort, and present a rare etiology: elastofibroma dorsi, a unique, benign, soft tissue-tumor with unique characteristics.

Elastofibroma appears deep to the lower scapular pole, is often bilateral, and consists of a mixture of collagen, elastic fibers and fibroblasts. We present 6 cases, in 3 men and 3 women aged 51-65.

Clozapine in the Treatment of Schizophrenia

M. Mark, R. Magnezi, O. Luxenburg, M. Siebzehner, J. Shemer

Israel Center for Technology Assessment in Health Care, Sackler Faculty of Medicine, Tel Aviv University

Schizophrenia is associated with brain abnormalities and is typically evidenced by disorganized speech and behavior, delusions, and hallucinations; it usually requires extended hospitalization. Its incidence in the western world is estimated at 4-7 cases/10,000/year.

A method of shortening hospitalization and improving level of functioning is the use of unique medication, including clozapine, which has been in use in Israel for the past 6 years.

We report 327 patients who participated in a community rehabilitation program and were treated with clozapine. They were compared with 417 patients who corresponded to the guidelines of the Director of Mental Health Services for treatment with clozapine, but were not treated with it for reasons not defined in the guidelines.

The study included those 25-44 and 45-64 years of age and according to the division of the population of patients hospitalized in both government and private hospitals. The project demonstrates the savings from use of clozapine as opposed to the alternative of hospitalizing these patients. It also shows the complexity and difficulty in assimilating new technologies, in relation to the influence of social considerations and supplier/insurer accounting on the patterns of technological assimilation.

Malignant Eccrine Poroma

R. Silfen, A. Amir, M. Feinmesser, D.J. Hauben

Dept. of Plastic and Reconstructive Surgery, and Pathology Dept., Rabin Medical Center (Beilinson Campus), Petah Tikva

Malignant eccrine poroma (MEP) is rare and both clinical and histologic diagnosis is often difficult. Therefore, diagnosis is sometimes delayed or even incorrect. We report a case in a 70-year old man with MEP of the leg. He demonstrated typical MEP behavior and the problems of differential diagnosis.

Multicenter Community-Based Trial of Amlodipine in Hypertension

C. Yosefy, J.R. Viskoper, Y. Leshem, Y. Rav-Hon, G.I. Rosenberg, E. Yaskil

(Representing the 39 Investigators of Project AML-IL-95-001, WHO Collaborative Center for Prevention of CV Diseases) Ben-Gurion University of the Negev, Beer Sheba; Barzilai Medical Center, Ashkelon; Hypertension Clinic, Kupat Holim Afula; Statistics Consulting Unit, Haifa University; and Promedico Ltd., Petah Tikva

The safety and efficacy of Amlodipine (AML) for mild to moderate hypertension was evaluated in a "real life" setting. This open non-comparative trial included 123 men and 143 women (age 30-91 years, mean 59.4). All had sitting diastolic blood pressure (DBP) between 95 and 115 mmHg, confirmed in most by 2 baseline measurements, 2 weeks apart.

Eligible patients were given AML 5 mg daily as add-on or monotherapy and were evaluated 4 weeks later. If DBP was then >90 mmHg, the daily dose was raised to 10 mg; those with <90 mmHg remained on 5 mg. AML was continued for 8 weeks. Other BP-lowering drugs were unchanged.

Of the original 266 patients 22 (8.2%) withdrew due to adverse events (AE), and others were protocol violators, lost to follow-up or withdrew, leaving 211 available for efficacy analysis. In this major group BP was reduced from 165±15/101±4 to 139±11/83±5 after 12 weeks of AML (p<0.05). The reduction was greater in those under 70 years, from 173±12/100±5 to 142±12/80±4 (p<0.05). In those with BMI>30 kg/m², BP decreased from 165±15/101±5 to 140±12/83±5 (p<0.05).

Mean change in heart rate was -1.5 bpm (p<0.05). Mean final AML dose was 5.5 mg/day. The most common AML-related AE requiring cessation of the drug was pedal edema in 2.6% of the 266 patients; in 3.7% it persisted during therapy. Other AE occurring in >1% were dizziness in 1.8%, headache 1.5%, flushing 1.1% and fatigue 1.1%.

We conclude that AML is an effective and well-tolerated antihypertensive suitable for most hypertensive patients.

Limited Percutaneous Surgical Drainage in Severe Neonatal Necrotizing Enterocolitis in Low Birth Weight Prematures

Robert Finaly, Zahavi Cohen, Vadim Kapuller, Agneta Golan, Edna Kurtzbart, Abraham Mares

Depts. of Pediatric Surgery and Neonatology, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

The usual treatment of complicated neonatal necrotizing enterocolitis (NEC) is resection of the necrotic bowel, lavage of the peritoneal cavity and diversion enterostomy. Low-birth-weight premature neonates with this condition are in special danger if general anesthesia and full exploratory surgery is contemplated.

A relatively simple alternate procedure is percutaneous insertion under local anesthesia of a soft abdominal drain, most often in the right lower quadrant. The procedure is done in the neonatal intensive care unit without moving the whole set-up to the operating room.

4 such cases have been treated within the past year. 3 were discharged home as they did not require additional surgical treatment, not having developed intestinal stenosis or obstruction. 1 recovered from the acute episode, but succumbed to a severe intraventricular hemorrhage and respiratory failure 7 days after the procedure.

Our limited but most gratifying experience, in addition to similar experience of others, encourages us to recommend this simple surgical approach in the very sick low-birth-weight premature with fulminant NEC.

Malignant Lymphoma of the Bladder

Y. Mor, Z. Dotan, J.H. Pinthus, I.S. Engelberg, J. Golomb, J. Ramon

Depts. of Urology and Pathology, Chaim Sheba Medical Center, Tel Hashomer

Urinary tract lymphoma is usually reported when the secondarily stem is affected by widespread non-Hodgkin lymphoma. We describe an 83-year-old woman who presented with secondary lymphoma of the bladder 3 years after diagnosis when it initially infiltrated her breast. Treatment included local transurethral excision followed by chemotherapy, during which she died of disseminated disease.

Nerve Palsies Following Prolonged Use of Limb Tourniquets

G. Volpin, R. Said, W. Simri, B. Grimberg, M. Daniel

Depts. of Orthopedic Surgery and Neurology, Western Galilee Hospital, Nahariya

Nerve paralysis following the use of tourniquets, regular or pneumatic, for limb surgery is rare. We describe a 19-year-old male soldier who had tourniquets applied for 3 1/4 hours to his arm and both legs due to penetrating injuries. As a result, he suffered palsy of the radial nerve and both common peroneal nerves. Nerve palsy in such cases has not been described in the literature. It is not clear whether the cause is direct mechanical pressure on the nerve, nerve ischemia, or a combination of both.

We recommend that tourniquets should not be used continuously for more than 2 hours. If evacuation of the injured is delayed, the medical team should consider loosening tourniquets for short intervals or changing for a pressure bandage. This is providing the patient's condition is stable and bleeding does not start again on release of the tourniquet.