Circumcision bleeding rates in patients diagnosed with delta-storage pool deficiency later in life.

BACKGROUND: Male circumcision is a common procedure, generally performed during the newborn period. Bleeding is an uncommon but feared complication of circumcision. Few reports have evaluated circumcision bleeding rates in patients with bleeding disorders. OBJECTIVES: To study circumcision bleeding rates in male subjects who were diagnosed later in life with delta-storage pool deficiency (δ-SPD). METHODS: We retrospectively reviewed the medical records of male subjects (≤18 years of age) who were circumcised without hemostatic prophylaxis prior to δ-SPD diagnosis from 2000 to 2020. Bleeding rates and severity were the main outcomes evaluated. We collected demographic data, bleeding scores using a validated assessment tool, laboratory data, and platelet electron microscopy results. A descriptive analysis was performed. RESULTS: Twenty-five male subjects were included. The median bleeding score at presentation was 3.5 (range: 2-9). The diagnosis was confirmed using platelet electron microscopy. A value of <2 dense granules/platelet was considered abnormal. Circumcision was performed at a median age of 2 days (range: 1 day to 4 months). None of the subjects had intraoperative or postoperative bleeding. With a bleeding rate of zero, we suggest that newborn males with a family history of δ-SPD may be safely circumcised without hemostatic prophylaxis.

A phenomenological three-parameter model for predicting the extent of outpatient palliative care.

Whether a patient receives general or specialized outpatient palliative cancer care rarely follows clear criteria, leading to undertreatment or overtreatment. Detailed scores exist to predict prognosis, but not treatment requirements, leaving caregivers to follow their intuition. As a phenomenological indicator incorporating possibly important subjective information, intuition may in fact be a helpful tool. In this prospective observational study, a score to estimate three global dimensions of patients' resources was applied: Medical prognosis, feeling of strength and feeling of support. The score results were correlated with the actual amount and effort of care required during the subsequent palliative care time. This phenomenological score correlated well with the performance index and the Hospice and Palliative care Evaluation score. Whilst various individual items correlated significantly with the score or its constituent parameters, there was no uniform coherent pattern, reflecting the complexity of palliative care and the potential value of this predictive tool.

Postoperative tonsil/adenoidectomy bleeding management in patients with diagnosed bleeding disorders.

OBJECTIVE: Tonsil/adenoidectomy (T/A) is a commonly performed procedure with an average post-tonsillectomy bleed (PTB) rate between 3 and 5%. Patients with bleeding disorders (BDs) are believed to have an increased risk of PTB. We hypothesize that our medical management of BD patients using a combination of DDAVP/antifibrinolytic agents has a similar PTB rate to control patients. This study suggests a standardized protocol for patients with BDs to avoid PTB. METHODS: A retrospective cohort study was completed for patients with BD who underwent tonsillectomy or T/A at Promedica Toledo or Flower Hospital between 2013 and 2020. Exclusion criteria included incomplete records, diagnosis of BD after surgery, and inability to find age and sex matched control. We defined the control group as patients who underwent T/A without BD. The following variables were collected: age, sex, medical history, BD severity, medications, type of surgery, indication for surgery, estimated blood loss (EBL), pre/postoperative medications, PTB status, and post-PTB intervention. RESULTS: A total of 164 patient charts were reviewed. There were 82 patients in both cohorts. The BDs represented were platelet function disorder (80.5%), von Willebrand disease (14.6%), and others such as Factor VII and IX deficiency (4.9%). Of the BD patients included, 13.4% had severe disease. There was no significant difference between the age, sex, EBL, and PTB rates. Of the 8 BD patients with PTB, 62% bled 9-10 days postoperatively and none had severe disease. CONCLUSION: Our protocol to prevent PTB in patients with BDs produced similar bleed rates to control patients in this study. Further studies are required to assess postoperative length of antifibrinolytic treatment in BD patients. LEVEL OF EVIDENCE: III.

Outcome of young children with high-grade glioma treated with irradiation-avoiding intensive chemotherapy regimens: Final report of the Head Start II and III trials.

PURPOSE: To report the final analysis of survival outcomes for children with newly diagnosed high-grade glioma (HGG) treated on the "Head Start" (HS) II and III protocols with chemotherapy and intent to avoid irradiation in children <6 years old. PATIENTS AND METHODS: Between 1997 and 2009, 32 eligible children were enrolled in HS II and III with anaplastic astrocytoma (AA, n = 19), glioblastoma multiforme (GBM, n = 11), or other HGG (n = 2). Central pathology review was completed on 78% of patients. Patients with predominantly brainstem tumors were excluded. Patients were to be treated with single induction chemotherapy regimen C, comprising four cycles of vincristine, carboplatin, and temozolomide. Following induction, patients underwent marrow-ablative chemotherapy and autologous hematopoietic cell rescue. Irradiation was used for patients with residual tumor after consolidation or >6 years old or at the time of tumor progression. RESULTS: The 5-year event-free survival (EFS) and overall survival (OS) for all HGG patients were 25 ± 8% and 36 ± 9%, respectively. The EFS at 5 years for patients with AA and GBM were 24 ± 11% and 30 ± 16%, respectively (P = 0.65). The OS at 5 years for patients with AA and GBM was 34 ± 12% and 35 ± 16%, respectively (P = 0.83). Children <36 months old experienced improved 5-year EFS and OS of 44 ± 17% and 63 ± 17%, compared with children 36-71 months old (31 ± 13% and 38 ± 14%) and children >72 months old (0% and 13 ± 12%). CONCLUSIONS: Irradiation-avoiding treatment strategies should be evaluated further in young children with HGG given similar survival rates to older children receiving standard irradiation-containing therapies.

Hyponatremia after desmopressin (DDAVP) use in pediatric patients with bleeding disorders undergoing surgeries.

Desmopressin (DDAVP) 1-deamino-8-D-arginine vasopressin is used in patients with bleeding disorders, including mild factor VIII deficiency, types 1 and 2 von Willebrand disease, and platelet function defects, undergoing surgeries to help control bleeding. We conducted a retrospective chart review of bleeding disorder patients undergoing inpatient surgery at Toledo Children's Hospital, OH, from 2005 to 2009. Our study population included 107 patients aged 2 to 19 years with platelet function defects and von Willebrand disease. Our study aimed to evaluate the extent of hyponatremia caused by DDAVP and to propose a safe and effective treatment regimen for these patients. The mean change in sodium level before and after DDAVP was statistically significant within each age group. Thirteen patients had second dose of DDAVP withheld, and 11 patients had postoperative sodium levels ≤ 130 mEq/L. There were 2 patients with significant complications: a 6-year-old with postoperative bleeding and a 2-year-old with post-DDAVP tonic-clonic seizures. We conclude that DDAVP causes significant hyponatremia, despite appropriate fluid restrictions. On the basis of our analysis, we recommend monitoring sodium levels before each dose of DDAVP and fluid restriction. These patients should be observed in the hospital setting after DDAVP administration for complications such as seizures and postoperative bleeding.

Primary squamous cell carcinoma of lung in a 7-year-old boy: a case report.

We are reporting a rare case of a primary squamous cell carcinoma of lung with skeletal metastases in a 7-year-old boy. The patient received chemotherapy with initial improvement but eventually died of the disease. A brief literature review is also presented.

Role of non-operative management of spleen injury in patients with hemophilia: report of two patients with review of literature.

The non-operative management (NOM) of hemodynamically stable patients with splenic trauma is currently well accepted, yet non-operative therapy has rarely been attempted in coagulopathic patients. Two cases of successful NOM of splenic trauma in patients with hemophilia are presented with a review of the English medical literature.