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: Transthyretin (TTR)-associated familial amyloid polyneuropathy (FAP) is an autosomal dominant multisystem disease with neurological and extra-neurological manifestations. It is caused by various mutations in the TTR gene leading to the formation of insoluble amyloid.

Objectives: To describe the clinical and genetic findings in patients with TTR-associated FAP in Israel.

Methods: We evaluated eight patients clinically and genetically during the years 2006 to 2011.

Results: At onset, all the patients exhibited sensory loss of the lower and upper limbs, five patients experienced muscle pain, and one patient had lower limb weakness. Five patients had autonomic nervous system manifestations, and four demonstrated evidence of amyloid cardiomyopathy. Nerve conduction studies showed sensorimotor axonal neuropathy in all patients. Sural nerve biopsies were obtained in five patients; only three biopsies revealed amyloid deposit. In four patients of Yemenite descent, genetic analysis of the TTR gene demonstrated ser77tyr mutation. One patient of Tunisian descent and one Ashkenazi patient harbored the val30met mutation. One patient of Iranian descent showed val32ala mutation, and another Ashkenazi patient showed phe33leu mutation.

Conclusions: TTR-associated FAP is a progressive and fatal disease that exists in the Israeli population and is unproportionally common among Yemenite Jews. This disease may be under-diagnosed and should be considered in the differential diagnosis of any patient with rapidly progressive neuropathy, especially with autonomic involvement or extra-neural features. The absence of amyloid in nerve biopsy should not rule out the diagnosis.  
 

Background: Human milk produced during prolonged lactation (> 1 year) is extraordinarily rich in fat and has a higher energy content than human milk produced during short lactation.

Objectives: To estimate the fatty acid (FA) profile of human milk and to test the hypothesis that the proportion of C12 and C14 (two dietary saturated FA known to most promote hypercholesterolemia) in human milk during prolonged lactation is similar to that in short lactation.

Methods: We conducted a cross-sectional study of 30 mothers of term infants lactating for more than 1 year as compared with 25 mothers of full-term infants who lactated for 2–6 months. Milk was collected by manual expression in mid-breastfeeding.

Results: The two groups did not differ in maternal height, weight, body mass index, diet, infant birth weight and gestational age, but mothers in the prolonged lactation group were significantly older. There was a significant correlation between lactation duration and C12 or C14. The percentage of all FA combined (except for C12 and C14) decreased significantly over time. In contrast, C12:0 and C14:0 combined increased significantly during lactation (R2 = 10.0%, P < 0.03).

Conclusions: Women who lactated for more than 1 year had higher C12 and C14 FA percentages in their milk than women who lactated for 2–6 months.

Background: Myotonic dystrophy type 2 (DM2) is an autosomal dominant, multisystem disorder caused by a CCTG tetranucleotide repeat expansion located in intron 1 of the zinc finger protein 9 gene (ZNF9 gene) on chromosome 3q 21.3.

Objectives: To describe the clinical, electrophysiologic and pathologic findings in patients with myotonic dystrophy 2.

Methods: We evaluated 10 patients genetically, clinically and electrophysiologically during the years 2007 to 2008.

Results: All patients were of Jewish European ancestry. Among affected individuals, eight patients had symptoms of proximal muscle weakness, two had muscle pain, and two exhibited myotonia. On physical examination six patients had severe weakness of hip flexor muscles. Seven individuals underwent cataract surgery, and cardiac involvement was seen in one case. On the initial electromyographic (EMG) examination five patients demonstrated myotonic discharges; repeated studies showed these discharges in nine cases. Six muscle biopsies showed non-specific pathological changes. Seven patients had an affected first-degree relative with either a diagnosed or an undiagnosed muscular disorder, consistent with an autosomal dominant trait.

Conclusions: DM2 may often present with proximal muscle weakness without myotonia. EMG may initially fail to show myotonic discharges, but these discharges may eventually show in most cases on repeated EMG. Thus, DM2 may be underdiagnosed and should be included in the differential diagnosis of adult patients of Jewish European ancestry presenting with proximal lower limb weakness.
 

Background: Control of diabetes is challenging, and frequent treatment changes are needed. 

Objective: To study the effect of the recommendation to start insulin glargine or insulin determir (long-acting insulin treatment, LAI) at discharge from hospital, on glucose control in the community setting.

Methods: Included were type II diabetes patients who were referred to and received a consultation from the hospital diabetes clinic during their hosptialization, as part of a routine consultation for diabetes management. During the visit, all patients were recommended long-acting insulin-based treatment, as inpatient treatment and at discharge. Follow-up was done by the primary physician in the community or by a community-based diabetes clinic. Glycosylated hemoglobin, glucose levels and other laboratory tests were obtained from the community health records before hospitalization and 6–12 months later. Medical treatment was ascertained by reviewing the actual usage of prescriptions.

Results: Eighty patients (58% males, mean age 64.1 ± 12.7 years) were included in the analysis. HbA1c levels were 10.1 ± 2.4% before admission, but improved significantly at follow-up (8.6 ± 2.2%, P < 0.001). Seventy-one percent of the patients were taking the LAI treatment and the rest were using non-LAI medications. Changes in diabetes control were similar between the LAI and non-LAI groups (HbA1c was reduced by 1.5 ± 3.2% and 1.9 ± 3.1% respectively). The rate of repeated admissions was also similar, averaging at 1.3 admissions for both groups, the minority of which were related to glucose control.

Conclusions: Insulin glargine or determir-based treatment does not show any superiority over other anti-diabetes treatment. It is our opinion that this treatment should be used as tailored therapy and should not be recommended routinely to all patients.
 

Background: The criteria for tonsillectomy for recurrent tonsillitis were established by prospective studies in the pediatric population and are applied to adults as well. No studies have been conducted to assess whether these guidelines are followed. 

Objectives: To examine the eligibility for tonsillectomy of tonsillectomized patients who were referred because of recurrent acute tonsillitis.

Methods: A retrospective case series in an ambulatory military otolaryngology clinic was conducted, and the medical records of 44 tonsillectomized patients who suffered from throat infections in the year before surgery were analyzed. The number of tonsillar infections that met the referral criteria was counted.

Results: The average number of throat infections that met the referral criteria was 1.89 per year. The average number of visits to the clinic due to upper respiratory tract infection was 12.92 (range 2–36) per year. The average number of visits for any cause was 45.13 (range 6–64) per year. One patient with eight documented throat infections met the criteria of more than six infections in the last year.

Conclusion: Although the referral criteria were not strictly met, we speculate that surgery was probably beneficial. This study shows that the indications for tonsillectomy referral are not strictly followed, and that new criteria for referral of adults for tonsillectomy need to be established.