Israel Medical Association Journal

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.    

Background: Hodgkin’s lymphoma is a distinct primary solid tumor of the immune system that shows wide variation in incidence among different geographic regions and among various races. It was previously suggested that susceptible people living in certain parts of Israel had a higher risk of HL[1] because of exposure to unidentified environmental factors in these regions. Compared with other parts of Israel, these regions were characterized by a higher proportion of Israeli-born Jews.

Objectives: To study time trends in the incidence rate of HL in Israeli-born Jews of all age groups during the years 1960–2005.

Results: A total of 4812 Jewish cases of HL were reported to the Israel Cancer Registry during the study period 1960–2005. There has been a persistent increase in the age-standardized incidence rate of HL, all subtypes pooled, in Israeli-born Jews in both men and women. The age distribution pattern in both genders was bimodal in all periods. The highest incidence was observed in the 20–24 year age group: for women (9.13 per 100,000 per year) during the period 1988–1996, and for men (6.60 per 100,000 per year) during the period 1997–2005.

Conclusions: The reported incidence level of HL in Israeli-born young adult Jews in Israel has increased in recent years to high levels compared with other western countries. Our findings suggest a cohort effect to unidentified factors affecting Israeli-born young adult Jews in Israel.

Background: In view of the rising prevalence of obesity, the identification of young adult populations at risk is important for the formulation of intervention and prevention programs.

Objectives: To assess demographic and behavioral factors associated with an increase in body mass index in young healthy adults and to identify the incidence of overweight/obesity in this population.

Methods: Data on anthropometric measures, demographic characteristics, and health behaviors were collected retrospectively for a representative sample of young Israeli adults (11,391 men, 11,280 women) on their release from military service (age 20–22 years) between 1989 and 2003. The incidence of overweight (BMI[1] < 25-< 30 kg/m²), incidence of obesity (BMI ≥ 30 kg/m²), and increase in BMI during military service were calculated.

Results: The average increase in BMI during military service was 1.11 kg/m² in males and 1.08 kg/m² in females. A greater increase was positively associated with low paternal education and smoking cessation, and negatively associated with high physical activity. Twelve percent of subjects with a normal BMI on recruitment became overweight, and 21.7% of overweight subjects became obese. On multivariate logistic regression analysis, a higher incidence of overweight was associated with low education level (in both the subject and his or her father) in both genders, and non-use of oral contraceptives and low level of physical activity in females.

Conclusions: BMI appears to increase significantly during early adulthood. Intervention programs should be targeted specifically at subjects with low education or who started smoking before age 18, and physical activity (especially among females) should be encouraged.

Background: The new syndrome, known as PFAPA, of periodic fever characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy  has been described only in pediatric patients. It usually begins before the age of 5 years and in most cases resolves spontaneously before age 10. 

Objectives: To describe a series of adults with PFAPA syndrome.

Methods: This 6 year retrospective descriptive study includes all newly diagnosed incident adult cases aged 18 years and over referred to our center with symptomatology suggestive of PFAPA syndrome. Patients’ medical records were reviewed for past history of the disease, demographic characteristics, symptoms and signs, course of the disease, laboratory findings, and outcome following corticosteroid therapy. The comparison group included our pediatric cohort children (N=320, age 0–18 years) followed for the last 14 years (1994–2008).

Results: Fifteen adult patients were diagnosed with PFAPA syndrome. Episodes of fever occurred at 4.6 ± 1.3 week intervals, beginning at the age of 20.9 ± 7.5.  All patients had monthly attacks at the peak of the disease, with attacks recurring at 4–8 week intervals over the years. Between episodes the patients were apparently healthy, without any accompanying diseases. Attacks were aborted by a single 60 mg dose of oral prednisone in all patients.

Conclusions: This study reports the presence of PFAPA syndrome in adult patients. Although the disease is rare, an increased awareness by both patients and family physicians of this clinical syndrome has resulted in more frequent diagnosis in adult patients.
 

Background: Posterior glottic stenosis is a complication of prolonged intubation, manifesting as airway stenosis that may mimic bilateral vocal cord paralysis. It presents a variety of features that mandate specific surgical interventions.

Objectives: To summarize our experience with PSG[1] and its working diagnosis.

Methods: We conducted a retrospective review of a cohort of adult patients with PGS operated at the Sheba Medical Center between 1994 and 2006.

Results: Ten patients were diagnosed with PGS, 6 of whom also had stenosis at other sites of the larynx and trachea. Since 2000, all patients underwent laryngeal electromyographic studies and direct laryngoscopy prior to surgery. Surgical interventions included endoscopic laser procedures (in 2 patients), laryngofissure and scar incision (in 1), laryngofissure with buccal mucosa grafting (in 3) or with costal cartilage grafting (in 1), laryngofissure with posterior cricoid split and stenting (in 1); one patient was not suitable for surgery. Postoperative follow-up included periodical fiberoptic endoscopies. Voice analysis was evaluated by the GRBAS grading. Seven patients were successfully decannulated within one to three procedures. Voice quality was defined as good in 7 patients, serviceable in 2 and aphonic in 1.

Conclusions: Posterior glottic stenosis may be isolated or part of complex laryngotracheal pathologies. Electromyographic studies and direct laryngoscopy must be included in the diagnostic workup. Costal cartilage or buccal mucosa grafts are reliable, safe and successful with respect to graft incorporation and subglottic remodeling.

Background: Transcobalamin II is a serum transport protein for vitamin B₁₂. Small variations in TC-II[1] affinity were recently linked to a high homocysteine level and increased frequency of neural tube defects. Complete absence of TC-II or total functional abnormality causes tissue vitamin B₁₂ deficiency resulting in a severe disease with megaloblastic anemia and immunologic and intestinal abnormalities in the first months of life. This condition was described in hereditary autosomal-recessive form. Low serum TC-II without any symptoms or clinical significance was noted in relatives of affected homozygotes.

Objectives: To study 23 members of a four-generation family with hereditary vitamin B₁₂ deficiency and neurologic disorders.

Methods: Thorough neurologic, hematologic and family studies were supplemented by transcobalamin studies in 20 family members.

Results: Partial TC-II deficiency was found in 19 subjects. Apo TC- II (free TC-II unbound to vitamin B₁₂) and total unsaturated B₁₂ binding capacity were low in all tested individuals but one, and holo TC-II (TC-II bound by vitamin B₁₂) was low in all family members. The presentation of the disease was chronic rather than acute. Early signs in children and young adults were dyslexia, decreased IQ, vertigo, plantar clonus and personality disorders. Interestingly, affected children and young adults had normal or slightly decreased serum vitamin B₁₂ levels but were not anemic. Low serum B₁₂ levels were measured in early adulthood. In mid-late adulthood megaloblastic anemia and subacute combined degeneration of the spinal cord were diagnosed. Treatment with B₁₂ injections resulted in a significant improvement. The pedigree is compatible with an autosomal-dominant transmission. This family study suggests a genetic heterogeneity of TC-II deficiency.

Conclusions: We report the first family with a hereditary transmitted condition of low serum TC-II (partial TC-II deficiency) associated with neurologic and mental manifestations in childhood. Partial TC-II deficiency may decrease the amount of stored cobalamin, resulting in increased susceptibility to impaired intestinal delivery of cobalamin and predisposing to clinically expressed megaloblastic anemia at a later age. Partial TC-II deficiency should be suspected in families with megaloblastic anemia and in individuals with neurologic and mental disturbances – despite normal serum vitamin B₁₂ levels. Low serum UBBC[2] and apo TC-II should confirm the diagnosis. Early vitamin B₁₂ therapy may prevent irreversible neurologic damage.

Background: Cystic fibrosis is no longer a terminal illness of childhood and mean survival is now over 30 years. Adult patients with atypical CF[1] are increasingly being diagnosed. In Israel, all patients are still followed in pediatric centers.

Objectives: To describe our experience with adult CF, stressing the importance of adult-related health and psychosocial issues.

Methods: Twenty-five CF patients aged 20–50 years, constituting 44% of the 57 patients followed at our center, were analyzed for pulmonary and extrapulmonary features and management.

Results: Nineteen were diagnosed as children and 6 as adults. Nineteen were pancreatic-insufficient and 6 were pancreatic-sufficient, including 5 diagnosed as adults. Pulmonary status was usually stable, with forced expiratory volume in 1 second 66.3 ± 21% (mean ± SD) and no difference between pancreatic-sufficient and insufficent patients. The latter had more hemoptysis, Pseudomonas infection, intestinal obstruction, liver disease and diabetes. Two patients died of malignancy and two of advanced lung disease. A majority received continuous inhaled and oral antibiotics, bronchodilators, Dnase, physiotherapy and periodic home intravenous antibiotics. Psychosocial functioning was excellent: 60% were employed, 36% were married and 40% had children (none with CF). Patients diagnosed as adults had mild multisystem disease or isolated severe lung disease.

Conclusions: CF adults generally have a good quality of life. Advances in understanding the CF defect and a plethora of new treatment modalities bode well for the future. Patients must be maintained in optimal condition to reap the benefits, and there is an urgent necessity for adult physicians to develop expertise in CF.

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Background: Acute pharyngitis in children is one of the most frequent illnesses for which primary care physicians are consulted. It is caused more frequently by viruses than by bacteria, but it is difficult to differentiate the causative agent by clinical signs alone. Group A Streptococcus accounts for 30% of children with a sore throat, and only in these cases is antibiotic therapy definitely indicated. However, the frequency and symptomatology of streptococcal pharyngitis in adults is not well established.

Objectives: To examine the clinical features that could distinguish sore throat caused by b-hemolytic group A Streptococcus in adults.

Methods: Patients aged over 16 years old (n=207) who presented with a sore throat to community clinics were examined and throat cultures were taken. The microbiologic confirmation of Group A Streptococcus was correlated with symptoms and clinical signs by univariate analysis.

Results: About 24% of the patients with positive cultures were younger individuals. Chills, absence of cough, pain in swallowing, absence of rhinitis, headache, vomiting, tonsillar exudate, oral malodor, fever >38°C and sweats had high sensitivity but low specificity for streptococcal pharyngitis. Univariate analysis suggested that chills and pharyngeal exudate had the greatest predictive value for streptococcal pharyngitis (P = 0.044, odds ratio 2.45; P = 0.001, OR[1] 5.49, respectively). When compared with a published scoring method (Centor criteria), large inconsistencies were found.

Conclusion: Our adult population had a relatively high prevalence of group A Streptococcus, and their presentation differed from that of pediatric patients. In primary care, a throat swab culture is not necessary in adults with a low score (0–1 points).

Background: Some studies have indicated a possible link between cigarette smoking and hearing loss.

Objectives: To analyze the association between smoking and hearing loss, other than that induced by noise, and to characterize the type of HL impairment found in smokers.

Methods: We conducted a retrospective cross-sectional study in 13,308 men aged 20±68 (median 34.6 years) who underwent a hearing test as part of a routine periodic examination. For each subject, age, smoking status (current, past or non-smokers) and number of cigarettes per day were noted and a hearing test was performed. The test was performed in a sealed, soundproof room by an experienced audiologist and included pure tone audiometry of 250±8,000 Hz. The audiograms were analyzed and subjects were accordingly divided into two groups: those with HL and at least one of the following impairments in at least one ear: sensorineural, conductive or mixed; and those with no hearing loss (control). Audiograms showing HL typical to noise exposure were excluded.

Results: The prevalence of any type of HL among subjects <35 years was 4.5%, compared to 10.5% among those >35 years (P < 0.0001). A significantly higher incidence of any type of HL was found in current (11.8%) and past smokers (11.7%) than in non-smokers (8.1%) (P < 0.0001). The risk increment of the smoking status for developing HL among subjects under age 35 was 43%, and 17% among those above 35 years. Both mild, flat, sensorineural impairment and conductive impairment were found to be associated particularly with smoking (odds ratio 2.2 and 1.9, respectively).

Conclusions: The incidence of HL unrelated to noise exposure is higher in smokers than in non-smokers, and in young adults the effect is greater.