Adam, Elameen

Objective To evaluate cancer risk in individuals with cleft lip and/or cleft palate (CL/P). Design Retrospective chart review of patients with CL/P, including abstraction of information on cancer diagnoses was conducted. Cancer risk was compared with national population-based estimates using standardized incidence ratios (SIRs) and exact binomial tests. Subgroup analyses compared demographic and clinical characteristics between patients with and without cancer. Setting Study was performed at a single tertiary academic center with multidisciplinary surgical, pediatric, and genetic care. Patients/Participants Total of 2121 patients with CL/P from 1970 to 2024 with complete medical documentation were included. Main Outcome Measures Main outcomes included prevalence of cancer diagnoses, childhood cancer incidence, and differences in cancer occurrence by age, cleft type, syndromic status, and family history. Results Total of 144 patients (6.8%) had pathologically confirmed cancer diagnoses. Twelve childhood cancers were identified, with 10 cases presenting before age 15. Children younger than 15 with CL/P had a significantly elevated cancer risk (SIR 4.7; 95% confidence interval: 1.8-7.6; P < .00001). Pediatric cancers occurred mainly in syndromic cases. Cancer prevalence was higher in cleft palate only and cleft lip/palate cases compared with cleft lip only ( P < .05). Cancer occurrence was not higher in adults with CL/P compared to the general population. Conclusions Cancer risk is not uniformly elevated over the lifespan in individuals with CL/P. Increased cancer risk is observed in our pediatric cohort. Clinical care should include careful assessment of childhood cancers, syndromic features, and family history, with consideration for genetic counseling and multidisciplinary follow-up.

ABSTRACT Calcific tendinopathy is a common musculoskeletal disorder marked by calcium deposition within tendons, often accompanied by pain, impaired mobility, and in severe cases, ossification. However, the mechanisms underlying tendon mineralization remain poorly understood. In contrast to pathological human calcification, turkeys naturally develop tendon mineralization during growth without signs of inflammation or ossification. This study had two phases: an observational phase to assess onset and progression of calcification, and an interventional phase to evaluate the role of mechanical loading. In the observational phase, flexor tendons were collected from turkeys aged 1–16 weeks in a serial sacrifice study and analyzed by Von Kossa staining and biomechanical testing. Calcification began at 7 weeks, localized within organized collagen matrices, progressed through Weeks 8–14, and peaked by 16 weeks. In the interventional phase, nine female turkeys were randomized at 4 weeks into a transection group ( n = 3), sham group ( n = 3), and control group ( n = 3). Transection at the muscle–tendon junction eliminated mechanical loading. At 16 weeks, tendons were analyzed by histology, quantitative RT‐PCR, ICP‐MS, and biomechanical testing. Transected tendons showed a near‐complete absence of mineralization, reduced expression of osteogenic markers (osteopontin, osteocalcin, Runx‐2), and significantly lower calcium, magnesium, and phosphorus levels. Biomechanical testing indicated reduced stress relaxation, stiffness, and Young's modulus in the transected group. Tendon calcification in turkeys functioned as a mechanically regulated, nonpathological process. The study established a relevant model for early tendon mineralization and offered a basis for developing load‐dependent therapies for human tendon disease.

Objective This study aimed to identify specific causes, clinical features, and contributing factors associated with early childhood mortality in a vulnerable subset of patients with cleft lip and/or palate (CL/P) who did not survive to undergo surgical repair. Design This was a retrospective chart review study. Setting The study was conducted at a single tertiary medical center. Patients/Participants We identified 26 liveborn CL/P patients who died without cleft repair from 1970 to 2024. Their data was compared to a control group of 2070 surgically repaired CL/P patients from the same institution. Main Outcome Measures Our primary goal was to characterize the clinical profile of patients experiencing prerepair mortality. Secondary outcomes included identifying causes of death, maternal and prenatal risk factors, and comparing these findings with the surgically repaired patients. Results All 26 unrepaired patients (100%) had genetic syndromes or isolated severe congenital anomalies, a significant difference compared to 24% in the surgically repaired group ( P < .0001). Trisomy 13 (30.8%), holoprosencephaly (23.1%), congenital cardiac defects (11.5%), and trisomy 18 (11.5%) were the leading causes of death. The median age at death for unrepaired patients was 25 days (interquartile range 4-125 days), significantly lower than the 61.9 years in the control group ( P < .0001). Multivariable logistic regression showed that genetic syndromes or severe congenital anomalies were strongly associated with early mortality ( P < .0001). Notably, all unrepaired patients who were born in the United States after 2005 had accurate prenatal diagnoses, which allowed clinical planning before the patients were born. Conclusions Prerepair mortality in CL/P is significantly driven by underlying genetic syndromes or congenital anomalies. For surgeons, this highlights the importance of genetic testing and prenatal care to guide appropriate surgical management. Considering a wait in surgical intervention until patients are approximately 4 months old may allow for better survival assessment and informed decisions for these high-risk individuals.

ABSTRACT Introduction Acute Compartment Syndrome (ACS) is a severe trauma caused by elevated intra-muscle-compartment pressure (ICP). The current standard method for diagnosis is to insert a needle into the muscle sterilely under anesthesia. However, to secure the environment is sometimes not easy and leads to delays in diagnosis. Recently, we have focused on shear wave ultrasound elastography (SWE) as an alternative, which can be done concisely in unclean environment and without anesthesia. We would like to report the usefulness of SWE for ACS diagnosis using 2-pedal walking turkey model recently developed in our lab. Materials and Methods A total of 32 1-year-old Bourbon turkeys were used. 5% solution of chicken albumin was infused continuously into the tibialis cranialis (TC) muscle using IV pump. The ICP was increased stepwise from 0 to 50 mmHg. During the rising of ICP, the correlation between values of SWE (kPa) and ICP (mmHg) was measured. After the ICP reached 50 mmHg, half of the turkeys were maintained at this pressure for 2 hours and the rest for 6 hours. After infusion, a fasciotomy was performed on the half turkey. Half of the turkeys were euthanized after 2 weeks and the rest after 6 weeks. SWE of TC muscle and walking gait data on turkeys using a portable walkway system were measured weekly until euthanasia. At euthanasia, isometric tetanic muscle force (ITF) tests to TC muscle and histological evaluations were performed. Results SWE value (kPa) was highly significantly correlated to the actual ICP (mmHg) (R2 = 0.91). Stance of ACS side leg were significantly extended, and swing of the control side shortened from the second to the third week after ACS in the 6 hours infusion-no-fasciotomy group (P &lt; 0.05*). ITF was significantly reduced mainly in the 6 hours infusion group (P &lt; 0.05*). Histological evaluation revealed that in the 6 hours infusion and 6 weeks survival group, both the muscle fiber and intercellular distances were significantly expanded (P &lt; 0.05). Conclusion SWE seems to be a substitute measure of ICP in diagnosing ACS. With regard to our in vivo ACS model using turkey, survival at 50 mmHg ICP for 6 hours and 6 weeks post ACS would be an appropriate situation.