To date, the only known effective treatment for celiac disease is a strict gluten-free diet for life. We reviewed the literature to evaluate the upper limit for gluten content in food, which would be safe for patients with celiac disease. Patients with celiac disease should limit their daily gluten intake to no more than 10–50 mg. Most health authorities define gluten-free products as containing less than 20 parts per million gluten.
Stents are widely use in endoscopic urological procedures. One of the most important indications is the treatment of urinary tract strictures. Allium™ Medical has introduced several types of stents for the treatment of different types of urinary tract strictures, based on anatomic location. All the stents are made of nitinol and coated with a co-polymer that reduces encrustations. These stents are self-expandable and have a large caliber and a high radial force. They have different shapes, designed especially for the treatment of each type of stricture. One of the most important features of Allium-manufactured stents is the ease of removal, due to their special unraveling feature. The company has introduced the Bulbar Urethral Stent (BUS) for treatment of bulbar urethral strictures; a rounded stent available in different lengths. Initial data on 64 patients with bulbar urethral stricture treated with the BUS showed a significant improvement in symptoms, with minimal complications and few adverse events. For treatment of prostate obstruction in patients unfit for surgery or unwilling to undergo a classical prostatic surgery, the Triangular Prostatic Stent (TPS) was introduced, which has a triangular shape that fits in the prostatic urethra. Its body has a high radial force attached to an anchor (which prevents migration) through a trans-sphincteric wire (which reduces incontinence rate). Initial data on 51 patients showed significant improvement in symptoms and in urinary peak flow rate, with a relatively small number of complications. The Round Posterior Stent (RPS) was designed for treatment of post radical prostatectomy bladder neck contracture. This short, round stent has an anchor, which is placed in the bladder neck. This stent being relatively new, the clinical data are still limited. Ureteral strictures can be treated with the Ureteral Stent (URS), which is round-shaped, available in different lengths, and has an anchor option (for very distal or very proximal strictures). We have previously published data on 107 URSs inserted in patients with ureteral stricture due to several etiologies, including patients who failed previous treatment. All patients were asymptomatic for a long period of follow-up after stent removal, with only one case of re-stenosis. In this paper, we review the urological “covered” stents produced by Allium Medical with the relevant clinical data available at the present time.
Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of “Gaucher cells.” These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.
TO THE EDITOR
We read with interest the report by Yeshayahu about four minors who were diagnosed late with non-COVID-19 diseases during the COVID-19 pandemic (Rambam Maimonides Med J 2021;12:e0017. doi: 10.5041/RMMJ.10431 ). We would like to emphasize that, firstly, such delays are not limited to minors, and secondly, that also in minors should we distinguish the administrative and the physiological meanings of the term “child” and hence distinguish administratively defined “chil¬dren” who bodily are already mature from those young patients who bodily are indeed still children.
Coronavirus disease 2019 (COVID-19) is a global respiratory disease with unique features that have placed all medical professionals in an alarming situation. Preeclampsia is a hypertensive disorder of pregnancy affecting 8%–10% of India’s pregnant population. Assuming that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) enters host cells through the angiotensin-converting enzyme 2 (ACE2) receptor, the resulting symptoms are due to vasoconstriction, caused by disturbances in the renin–angiotensin system (RAS). Other features of preeclampsia include endothelial dysfunction due to placental ischemia, leading to imbalances in angiogenic and antiangiogenic factors which result in increased blood pressure, proteinuria, altered hepatic enzymes, renal failure, and thrombocytopenia, amongst others. The increased prevalence of preeclampsia that was seen among mothers with SARS-CoV-2 infection might be due to misdiagnosis, as COVID-19 and preeclampsia have coincidental medical features. The major similarities of SARS-CoV-2-infected and preeclamptic women are a rise in pro-inflammatory cytokines, and increased serum ferritin and thrombocytopenia. Therefore, differential diagnosis might be difficult in pregnant women with COVID-19 who present with hypertension and proteinuria, thrombocytopenia, or elevated liver enzymes. The most promising markers for earlier diagnosis of preeclampsia is soluble endoglin (sEng), pregnancy-associated plasma protein-A (PAPP-A), soluble fms-like tyrosine kinase 1 (sFlt-1), and placental growth factor (PlGF). Due to placental hypoxia, sFlt-1 will be overproduced, thus inhibiting PlGF, and this alteration will be observed in the circulation five weeks or more before the onset of symptoms. The sFlt-1/PlGF ratio may also be modified via infectious states, but unregulated levels of those mediators are related to placental insufficiency. Hence, pregnant women with COVID-19 may develop a preeclampsia-like syndrome that might be differentiated properly by angiogenic markers to avoid unnecessary interventions and induced preterm labor.
Background: Patellar instability comprises a group of pathologies that allow the patella to move out of its trajectory within the trochlear groove during walking. Symptomatic patients who need surgery commonly undergo soft tissue procedures such as medial patellofemoral ligament repair to strengthen the ligaments that hold the patella in place. However, soft-tissue repairs may be insufficient in patients suffering from patellar maltracking, which is characterized by an unbalanced gliding of the patella within its route. In these patients, a different approach is advised. We aim to provide the mid-term clinical outcomes of the Fulkerson distal realignment operation in selected patients with non-traumatic patellar maltracking.
Methods: The clinical outcomes of the Fulkerson distal realignment operation performed in 22 knees of 21 patients were evaluated by a self-administered subjective International Knee Documentation Committee (IKDC) score and the Tegner–Lysholm knee scoring scale.
Results: Before surgery, the median IKDC score was 52, and the median Tegner–Lysholm score was 56. Following surgery (mean follow-up 48 months, range 24–156), the median IKDC and the Tegner–Lysholm scores were 67 and 88, respectively. The improvement was statistically significant (P=0.001 and P=0.002 for IKDC and Tegner–Lysholm scores, respectively). Associated procedures included patella microfracture due to grade III–IV cartilage lesion (International Cartilage Repair Society grading system) in four patients, retinacular releases in three patients, medial capsular augmentations in two patients, and medial patellofemoral ligament reconstruction in two patients. One patient with Ehlers–Danlos disease required excessive medialization of the tibial tuberosity. Surgery-related complications occurred in three patients.
Discussion: Surgical correction of patellar maltracking with Fulkerson distal realignment combined with associated procedures in individual patients was associated with an increase in subjective and functional clinical scores at medium-term follow-up. Particular attention should address pathologies associated with patellar maltracking and managed accordingly.
Level of evidence: 4c (case series).
In the management of malignant thyroid disorders, the standard primary treatment is thyroidectomy, a surgical resection of the thyroid gland. This procedure has been performed for over a century. Hence, it comes as no surprise that it is not only exceedingly well-described in the literature. This issue of Rambam Maimonides Medical Journal includes an article by Chaturvedi et al. that challenges the standard widely-practiced clinical inclination toward surgery as the first and best option for all patients with early thyroid cancer. This editorial discusses the issues raised by the authors and points out the importance of ongoing research to determine when standards of care should be modified in the light of low-risk disease.
Background: Transthyretin (TTR), also known as prealbumin, has been suggested as an indicator of protein and nutritional status.
Objective: The aim of this study was to examine the maternal and umbilical cord (UC) TTR in relation to intrauterine growth, and the serum TTR of preterm infants in relation to nutritional status and growth.
Methods: After application of exclusion criteria, 49 preterm infants (mean gestational age and birth-weight 32.9±2.9 weeks and 1822±556 g) were included in the study. Transthyretin was sampled at birth and on days 14, 28 or at discharge with growth parameters and nutritional laboratories.
Results: Mean UC and maternal TTR were positively correlated (8.5±2.4 mg/dL and 20.4±7.0 mg/dL, r=0.31, P=0.07). Umbilical cord TTR was neither an index of maturity nor of intrauterine growth. Umbilical cord TTR was higher in females (9.4±2.6 versus 7.6±1.8 mg/dL, P=0.015). Maternal TTR was lower in twin pregnancies (16.8±4.9 versus 22.5±7.3 mg/dL, P=0.007). Although TTR levels gradually increased over time in correlation with post-menstrual and chronological ages (r=0.24, P=0.011 and r=0.40, P<0.001, respectively), there was no correlation to weight gain (r=0.10, P=0.41), nutritional status, protein intake, or laboratories. The only significant correlations were between TTR and glucose and triglycerides levels (r=0.51, P<0.001 for both).
Conclusions: Although TTR levels increased over time, we could not demonstrate significant correlations between TTR and indices of the nutritional status in preterm infants at birth or during the neonatal course.
Purpose: This case series analyzed the appropriateness of computed tomography (CT) and magnetic resonance imaging (MRI) for visualization of rhinoorbitocerebral mucormycosis (ROCM) patterns associated with type 2 diabetes (T2D) post-recovery from coronavirus disease 2019 (COVID-19).
Methods: The study included 24 patients with invasive ROCM after having recovered from COVID-19. All patients underwent CT examinations and microbiological and histological verification; 5 patients underwent MRI.
Results: The CT and MRI patterns noted in our patients revealed involvement of skull orbits, paranasal sinuses, large arteries, and optic nerves, with intracranial spread and involvement of the cranial base bones. Using brain scan protocol for CT provided better soft-tissue resolution. We found that extending the MRI protocol by T2-sequence with fat suppression or STIR was better for periantral fat and muscle evaluations.
Conclusion: Computed tomography of the paranasal sinuses is the method of choice for suspected fungal infections, particularly mucormycosis. However, MRI is recommended if there is suspicion of orbital, vascular, or intracranial complications, including cavernous sinus extension. The combination of both CT and MRI enables determination of soft tissue invasion and bony destruction, thereby facilitating the choice of an optimal ROCM treatment strategy. Invasive fungal infections are extremely rare in Europe; most of the related data are provided from India and Middle Eastern or African nations. Hence, this study is notable in its use of only diagnosed ROCM cases in Russia.
Background: Blunt traumatic brain injury (bTBI) and uncontrolled hemorrhagic shock (UCHS) are common causes of mortality in polytrauma. We studied the influence of fresh frozen plasma (FFP) resuscitation in a rat model with both bTBI and UCHS before achieving hemorrhage control.
Methods: The bTBI was induced by an external weight drop (200 g) onto the bare skull of anesthetized male Lewis (Lew/SdNHsd) rats; UCHS was induced by resection of two-thirds of the rats’ tails. Fifteen minutes following trauma, bTBI+UCHS rats underwent resuscitation with FFP or lactated Ringer’s solution (LR). Eight groups were evaluated: (1) Sham; (2) bTBI; (3) UCHS; (4) UCHS+FFP; (5) UCHS+LR; (6) bTBI+UCHS; (7) bTBI+UCHS+FFP; and (8) bTBI+UCHS+LR. Bleeding volume, hematocrit, lactate, mean arterial pressure (MAP), heart rate, and mortality were measured.
Results: The study included 97 rats that survived the immediate trauma. Mean blood loss up to the start of resuscitation was similar among UCHS only and bTBI+UCHS rats (P=0.361). Following resuscitation, bleeding was more extensive in bTBI+UCHS+FFP rats (5.2 mL, 95% confidence interval [CI] 3.7, 6.6) than in bTBI+UCHS+LR rats (2.5 mL, 95% CI 1.2, 3.8) and bTBI+UCHS rats (1.9 mL, 95% CI -0.2, 3.9) (P=0.005). Similarly, non-significant increases in blood loss were observed in UCHS+FFP rats (P=0.254). Overall mortality increased if bleeding was above 4.5 mL (92.3% versus 8%; P<0.001). Mortality was 83.3% (10/12) in bTBI+UCHS+FFP rats, 41.7% (5/12) in bTBI+UCHS+LR rats, and 64.3% (9/14) in bTBI+UCHS rats.
Conclusion: The bTBI did not exacerbate bleeding in rats undergoing UCHS. Compared to LR, FFP resuscitation was associated with a significantly increased blood loss in bTBI+UCHS rats.
