Israel Medical Association Journal

Background: Pain, fatigue and functional disability are common key outcomes in most rheumatologic disorders. While many studies have assessed the outcomes of specific disease states, few have compared the outcomes of various rheumatic diseases.

Objectives: To assess how the intensity and rating of pain, fatigue and functional disability vary among groups of patients with various rheumatic disorders receiving standard care. 

Methods: In a cross-sectional study conducted in a hospital-based rheumatology unit, standard clinical and laboratory data were obtained and all patients filled out questionnaires on pain, fatigue and daily function. The analysis concentrated on visual analogue scales (VAS) using specific statistical methods.

Results: A total of 618 visits of 383 patients with inflammatory as well as non-inflammatory rheumatic disorders were analyzed. Fibromyalgia patients had significantly higher VAS scores compared to all other groups. On the other hand, patients with polymyalgia rheumatica demonstrated significantly lower VAS scores compared to all other groups of patients. Patients with psoriatic arthritis also demonstrated relatively low VAS scores. VAS scores were lower in patients with inflammatory disorders as compared to patients with non-inflammatory disorders.

Conclusions: Our results suggest a spectrum of outcome intensity in various rheumatic disorders receiving standard care, ranging from fibromyalgia patients who report distinctive severity to patients with inflammatory disorders who are doing relatively well as compared to patients with non-inflammatory disorders. The findings emphasize the need to explore the underlying mechanisms of pain and fatigue in patients with non-inflammatory rheumatic disorders. 

 

Background: Childhood attention deficit hyperactivity disorder (ADHD) is a chronic health problem with significant risk for long-term morbidity in adulthood.

Objectives: We examined long-term outcomes of ADHD in a population-based sample of childhood ADHD cases prospectively assessed as adults.

Methods: Long-term outcomes for 70 adults who were diagnosed with ADHD during childhood were examined and compared with data on the general population.

Results: Most subjects admitted to persistence of ADHD-related symptoms in adulthood, despite discontinuation of regular medical treatment and follow-up. Areas most severely affected by past and ongoing symptoms included driving performance and incidence of motor vehicle accidents, and rates of marriage stability over time. Relatively unaffected were occupational and academic achievements and military service. 

Conclusions: There is a need for outreach and better services for adults who were previously diagnosed with ADHD. 

 

Abstract

Background: Laryngeal cleft (LC) is a rare congenital anomaly manifesting in a variety of symptoms including swallowing disorders and aspirations, dyspnea, stridor and hoarseness. The mild forms (types I-II) may be underdiagnosed, leading to protracted symptomatology and morbidity. 

Objectives: To evaluate the diagnostic process, clinical course, management and outcome in children with type I-II laryngeal clefts.

Methods: We conducted a retrospective case analysis for the years 2005–2012 in a tertiary referral center.

Results: Seven children were reviewed: five boys and two girls, aged from birth to 5 years. The most common presenting symptoms were cough, aspirations and pneumonia. Evaluation procedures included fiber-optic laryngoscopy (FOL), direct laryngoscopy (DL) and videofluoroscopy. Other pathologies were seen in three children. Six children underwent successful endoscopic surgery and one child was treated conservatively. The postoperative clinical course was uneventful in most of the cases.

Conclusions: Types I-II LC should be considered in the differential diagnosis of children presenting with protracted cough and aspirations. DL is crucial for establishing the diagnosis. Endoscopic surgery is safe and should be applied promptly when conservative measures fail. 

Background: The VEGFR/PDGFR inhibitor sunitinib was approved in Israel in 2008 for the treatment of metastatic renal cell carcinoma (mRCC), based on an international trial. However, the efficacy of sunitinib treatment in Israeli mRCC patients has not been previously reported.

Objectives: To report the outcome and associated factors of sunitinib treatment in a large cohort of Israeli mRCC patients.

Methods: We conducted a retrospective study of an unselected cohort of mRCC patients who were treated with sunitinib during the period 2006–2013 in six Israeli hospitals. Univariate and multivariate analyses were performed to determine the association between treatment outcome and clinicopathologic factors.

Results: We identified 145 patients; the median age was 65 years, 63% were male, 80% had a nephrectomy, and 28% had prior systemic treatment. Seventy-nine percent (n=115) had clinical benefit (complete response 5%, n=7; partial response 33%, n= 48; stable disease 41%, n=60); 21% (n=30) were refractory to treatment. Median progression-free survival (PFS) was 12 months and median overall survival 21 months. Factors associated with clinical benefit were sunitinib-induced hypertension: [odds ratio (OR) 3.6, P = 0.042] and sunitinib dose reduction or treatment interruption (OR 2.4, P = 0.049). Factors associated with PFS were female gender [hazard ratio (HR) 2, P = 0.004], pre-sunitinib treatment neutrophil to lymphocyte ratio ≤ 3 (HR 2.19, P = 0.002), and active smoking (HR 0.19, P < 0.0001). Factors associated with overall survival were active smoking (HR 0.25, P < 0.0001) and sunitinib-induced hypertension (HR 0.48, P = 0.005). To minimize toxicity, the dose was reduced or the treatment interrupted in 39% (n=57). 

Conclusions: The efficacy of sunitinib treatment for mRCC among Israeli patients is similar to that of international data.

Background: A growing number of Eritrean and Sudanese refugees seek medical assistance in the labor and delivery ward of our facility. Providing treatment to this unique population is challenging since communication is limited and pregnancy follow-up is usually absent.

Objectives: To compare the perinatal outcome of refugees and Israeli parturients.

Methods: The medical and financial records of all refugees delivered between May 2010 and April 2011 were reviewed. Perinatal outcome was compared to that of native Israeli controls.

Results: During this period 254 refugees were delivered (2.3% of deliveries). Refugees were significantly younger and leaner. They had significantly more premature deliveries under 37 weeks (23 vs. 10, P = 0.029) and under 34 weeks gestation (9 vs. 2, P = 0.036) with more admissions to the neonatal intensive care unit (15 vs. 5, P = 0.038). Overall cesarean section rate was similar but refugees required significantly more urgent surgeries (97% vs. 53%, P = 0.0001). Refugees had significantly more cases of meconium and episiotomies but fewer cases of epidural analgesia. There were 2 intrauterine fetal deaths among refugees, compared to 13 of 11,239 deliveries during this time period (P = 0.036), as well as 7 pregnancy terminations following sexual assault during their escape. Sixty-eight percent of refugees had medical fees outstanding with a total debt of 2,656,000 shekels (US$ 767,250).

Conclusions: The phenomenon of African refugees giving birth in our center is of unprecedented magnitude and bears significant medical and ethical implications. Refugees proved susceptible to adverse perinatal outcomes compared to their Israeli counterparts. Setting a pregnancy follow-up plan could, in the long run, prevent adverse outcomes and reduce costs involved in treating this population.

Objectives: To investigate whether the change in protocols over the years resulted in an outcome difference at two hospitals in Israel.

Methods: We thoroughly reviewed the records of 153 patients from Sheba Medical Center and Soroka Medical Center, of whom 106 comprised the study group. The patients were divided into two groups according to the treatment protocol used: 40 patients with the 1989/93 protocol and 66 with the 1999/2003 protocol. Outcome was analyzed for the two groups.

Results: We found a significant difference in disease-free survival (DFS) between the two groups for B cell-ALL (B-ALL) patients who achieved complete remission after induction. There was no difference in overall survival. We did not find any difference in outcome for T cell-ALL patients or for CD20-positive patients.

Conclusions: In our retrospective analysis, GMALL 99/2003 led to a better DFS for B-ALL patients who were in complete remission after induction. This is possibly related to the differences in medications between the protocols, but may also be due to better supportive care. Despite the proven advantage of the newer protocols regarding overall survival, in our experience there was no other significant difference between the two regimens. 

Objectives: To determine if the decrease rate of serum alkaline phosphatase (SAP) levels during the first month of neoadjuvant chemotherapy could serve as a predictive indicator of tumor necrosis and clinical outcome.

Methods: We analyzed the medical files of 53 osteosarcoma patients (19 females, 34 males) (median age 16 years, range 8–24); the disease was metastatic in 12 and localized in the other 41.

Results: The histological responses were good in 38 patients (71.7%) and poor in 15 (28.3%). At a median follow-up of 50 months, 34 patients (64.2%) had no evidence of disease and 19 (35.8%) had died from the disease. High levels of SAP at diagnosis correlated with worse survival (P = 0.002). There was no difference in overall survival between patients whose SAP decrease rate was > 25% and those with a rate < 25% (P = 0.14). Among female patients, "rapid" SAP responders had better survival than "slow" responders (P = 0.026). In patients with metastases the SAP decrease rate was positively correlated with survival (P = 0.042).

Conclusions: There was no evidence that "rapid" SAP responders had a higher percentage of tumor necrosis than "slow" responders, although female "rapid" SAP responders had a better prognosis than "slow" responders. Patients with metastases at presentation and "rapid" SAP response had better prognoses.

Objectives: To determine the outcome of RA with respect to disease activity and the rate of remission, as measured by the DAS-28, in a real-world inception cohort.

Methods: We conducted an observational cross-sectional study of a single-center real-world inception cohort of 101 consecutive patients being treated for RA in 2009–2010 in a rheumatology outpatient clinic. Patients were managed at the discretion of the attending rheumatologist with the goal of achieving remission. DAS-28 scores were calculated and analyzed by clinical and treatment variables derived from the medical files.

Results: Mean patient age was 58.6 ± 13.4 years and mean duration of disease 10.7 ± 7.9 years. Disease remission (DAS-28 < 2.6) was achieved in 26.7% of patients and low disease activity (> 2 .6 DAS-28 < 3.2) in 17%. Monotherapy with a conventional disease-modifying anti-rheumatic drug (C-DMARD, 21% of patients at last follow-up) was associated with a significantly lower mean DAS-28 score and C-reactive protein level than combined C-DMARD treatment (79% of patients), and with shorter disease duration than combined treatment with C-DMARDs or C-DMARD(s)+biological DMARD (40% of patients). Rheumatoid factor and anti-cyclic citrullinated peptide positivity had no effect on DAS-28 scores. Time from diagnosis was inversely correlated with DAS-28 scores.

Conclusions: The achievement of low disease activity and remission in a significant portion of our inception cohort of patients with RA suggests that the treat-to-target strategy is feasible and effective in routine clinical practice. 

Objectives: To evaluate the course and management of visual and pregnancy outcomes of consecutive pregnancies with IIH.

Methods: The medical records of women with IIH in consecutive pregnancies were reviewed for neuro-ophthalmological findings, management, and visual and pregnancy outcomes.

Results: The study group comprised eight women with at least two consecutive pregnancies (mean age 27.3 ± 5.3 years). The mean duration of IIH prior to the first pregnancy was 3.4 ± 3.16 years. One woman with IIH pre-pregnancy symptoms and three women with clinical features of IIH during the second trimester of pregnancy (gestational week 21.7 ± 4.04) were treated with acetazolamide (250 mg every 8 hours). Symptoms resolved, resulting in uncomplicated first deliveries for all four. The first deliveries of four other women were by cesarean section due to obstetric indications. Only one woman developed symptoms and signs of IIH during her second pregnancy and was thus treated with acetazolamide. Two women who completed three pregnancies had no IIH symptoms during their pregnancies. The course and outcome of those pregnancies were normal.

Conclusions: IIH apparently does not worsen or even become symptomatic in consecutive pregnancies. The appropriate management of IIH in pregnant women is similar to management for non-pregnant women; neither the course nor the obstetric outcome of first and consecutive pregnancies is influenced by the presence of IIH.

Objectives: To evaluate the upper extremity injury pattern of attacks on civilian targets, comparing bomb explosion injuries to gunshot injuries and their functional recovery using standard outcome measures.

Methods: Of 157 patients admitted to the hospital between 2000 and 2004, 72 (46%) sustained explosion injuries and 85 (54%) gunshot injuries. The trauma registry files were reviewed and the patients completed the Disabilities of Arm, Shoulder and Hand Questionnaire (DASH) and Short Form-12 (SF-12) after a minimum period of 1 year.

Results: Of the 157 patients, 72 (46%) had blast injuries and 85 (54%) had shooting injuries. The blast casualties had higher Injury Severity Scores (47% over a score of 16 vs. 22%, P = 0.02) and higher percent of patients treated in intensive care units (47% vs. 28%, P = 0.02). Although the Abbreviated Injury Scale score of the upper extremity injury was similar in the two groups, the blast casualties were found to have more bilateral and complex soft tissue injuries and were treated surgically more often. No difference was found in the SF-12 or DASH scores between the groups at follow up.  

Conclusions: The casualties with upper extremity blast injuries were more severely injured and sustained more bilateral and complex soft tissue injuries to the upper extremity. However, the rating of the local injury to the isolated limb is similar, as was the subjective functional recovery.

Objectives: To describe and identify clinical characteristics, prognostic factors and visual outcome in a group of patients with OGI in southern Israel.

Methods: We conducted a retrospective review of all cases of OGI examined in the ophthalmology department at Soroka University Medical Center, Beer Sheva, Israel, from 1996 to 2005. A total of 118 eyes with OGI were detected and analyzed statistically. We recorded demographic data, cause of injury, initial visual acuity (VA), associated globe morbidity and injuries, Ocular Trauma Score (OTS), surgical procedures, postoperative complications, and final VA.

Results: The mean age of the study group was 36.1 years and included 84% males. The median follow-up was 13.3 months (range 6–66 months). The annual incidence of open globe injuries was 3.1 cases/100,000. In 84 cases (71%) the mechanism of open eye injury was laceration. Most of the injuries were work related (45%). Bilateral injury was observed in two patients. An intraocular foreign body was observed in 45 eyes (38%). Primary surgical repair was performed in 114 eyes. Six patients (5.1%) had complications with post-traumatic endophthalmitis and 12 patients (10.1%) underwent evisceration or enucleation. Clinical signs associated with poor visual outcomes included reduced initial VA, eyelid injury, and retinal detachment at presentation.

Conclusions: In our study population the most important prognostic factors in open globe injury were initial VA, eyelid injury and retinal detachment.

Objectives: To present a case series of femoral hematogenous osteomyelitis in adults, a rare condition that is difficult to diagnose and may cause major morbidity and mortality.

Methods: We reviewed three cases of femoral hematogenous osteomyelitis that occurred between 2007 and 2009. The course of the disease, physical findings, imaging modalities, laboratory analysis, culture results and functional outcomes were recorded.

Results: In all cases the diagnosis was delayed after symptoms were first attributed to radicular-like pain or lateral thigh pain due to an inflammatory non-infectious source. In all cases infection was caused by an unusual or fastidious bacterium. The pathogen was Haemophilus aphrophilus in one case, and Streptococcus specimens were found in the other two. Pathological fracture occurred in two of the cases despite culture-specific antibiotic treatment and a non-weight bearing treatment protocol. It took five surgical interventions on average to reach full recovery from infection, but residual disability was still noted at the last follow-up.

Conclusions: Clinicians should be aware that although femoral hematogenous osteomyelitis is a rare condition in adults, its ability to mimic other pathologies can result in delayed diagnosis and major morbidity. In our series the pathogen was different in each case and was cultured only from the infected site. Pathological fracture is a devastating complication but we do not recommend prophylactic stabilization at this point.