Adenocarcinoma of the ileum during pregnancy

Adenocarcinoma of the ileum was discovered at abdominal exploration of a 26-yr-old primigravida in her 30th wk of pregnancy. The operation, which consisted of resection of the tumor and creation of an anus praeter, was followed by premature delivery of a baby that succumbed to respiratory distress. The patient died 7 mth after the operation, due to obstruction resulting from tumor recurrence. No comparable case could be found in the literature.     

Are patient-reported outcome measures biased by method of follow-up? Evaluating paper-based and digital follow-up after lumbar fusion surgery

Background Context

Long-term follow-up of patient-reported outcome measures (PROM) is essential in both modern spinal care and research. Lack of time and staff are commonly reported barriers to implementing long-term follow-up of PROM. Automated and digital follow-up systems for PROM collection are seeing widespread use, yet their validity and comparative effectiveness have never been evaluated.

A mutation update on the LDS‐associated genes TGFB2/3 and SMAD2/3

The Loeys–Dietz syndrome (LDS) is a connective tissue disorder affecting the cardiovascular, skeletal, and ocular system. Most typically, LDS patients present with aortic aneurysms and arterial tortuosity, hypertelorism, and bifid/broad uvula or cleft palate. Initially, mutations in transforming growth factor‐β (TGF‐β) receptors (TGFBR1 and TGFBR2) were described to cause LDS, hereby leading to impaired TGF‐β signaling. More recently, TGF‐β ligands, TGFB2 and TGFB3, as well as intracellular downstream effectors of the TGF‐β pathway, SMAD2 and SMAD3, were shown to be involved in LDS. This emphasizes the role of disturbed TGF‐β signaling in LDS pathogenesis. Since most literature so far has focused on TGFBR1/2, we provide a comprehensive review on the known and some novel TGFB2/3 and SMAD2/3 mutations. For TGFB2 and SMAD3, the clinical manifestations, both of the patients previously described in the literature and our newly reported patients, are summarized in detail. This clearly indicates that LDS concerns a disorder with a broad phenotypical spectrum that is still emerging as more patients will be identified. All mutations described here are present in the corresponding Leiden Open Variant Database.     

Heart Rate Variability Predicts Severe Hypotension after Spinal Anesthesia for Elective Cesarean Delivery

Background: Hypotension due to vasodilation during subarachnoid block (SAB) for elective cesarean delivery may be harmful. Heart rate variability (HRV), reflecting autonomic control, may identify patients at risk of hypotension.

Methods: Retrospectively, HRV was analyzed in 41 patients who were classified into one of three groups depending on the decrease in systolic blood pressure (SBP): mild (SBP > 100 mmHg), moderate (100 > SBP > 80 mmHg), or severe (SBP < 80 mmHg). Prospectively, HRV and hemodynamic data of 19 patients were studied. Relative low frequency (LF), relative high frequency (HF), and LF/HF ratio were analyzed.

Results: Retrospective analysis of HRV showed a significantly higher sympathetic and lower parasympathetic drive in the groups with moderate and severe compared with mild hypotension before SAB (median, 25th/75th percentiles): LF/HF: mild: 1.2 (0.9/1.8), moderate: 2.8 (1.8/4.6), P < 0.05 versus mild; severe: 2.7 (2.0/3.5), P < 0.05 versus mild. Results were confirmed by findings of LF and HF. Prospectively, patients were grouped according to LF/HF before SAB: low-LF/HF: 1.5 (1.1/2.0) versus high-LF/HF: 4.0 (2.8/4.7), P < 0.05; low-LF: 58 +/- 9% versus high-LF: 75 +/- 10%, P < 0.05; low-HF: 41 +/- 10% versus high-HF: 25 +/- 10%, P < 0.05. High-risk patients had a significantly lower SBP after SAB (76 +/- 21 vs. 111 +/- 12 mmHg; P < 0.05).     

Combined Radiochemotherapy with Paclitaxel in the Treatment of Malignant Glioma

Background: Paclitaxel is a radiosensitizing agent that shows a synergistic effect with radiation in malignant glioma cells. A phase I/II trial was performed in order to evaluate both feasibility and outcome of combined radiochemotherapy in malignant glioma. Patients and Methods: 56 patients were included in the trial. Paclitaxel was administered on days 1-4, 15-18 and 29-32 18 to 24 hours prior to irradiation. Radiotherapy with 60 Gy in conventional fractionation was initiated on day 2. 31 patients were included in a dose-escalation trial starting with 20 mg/m²/d and increasing up to 60 mg/m²/d in 10-mg steps. Additional 25 patients were treated with 50 mg/m²/d. Results: The regimen was very well tolerated with minimal subjective impairment such as sickness or weakness. Maximal tolerable dose was 50 mg/m²/d, with neutropenia being the dose-limiting toxicity. There were four allergic reactions and five thromboembolisms; furthermore, one patient with a normal blood count died due to pneumonia. The median survival was 12 months, and significantly favorable factors were a young age, a lower tumor grade (grade 3) and a complete resection. Varying the paclitaxel dose had no influence on outcome. Conclusions: Combined radiochemotherapy with paclitaxel in the treatment of malignant glioma was well tolerated. Maximal tolerable dose was 50 mg/m²/d. There was no increase in survival compared with results reported in the literature. Hintergrund: Paclitaxel ist eine strahlensensibilisierende Substanz, die auch bei Gliomzellen eine synergistische Wirkung zeigt. Wir führten eine Phase-I/II-Studie durch, um die Toxizität und erste Ergebnisse der kombinierten postoperativen Radiochemotherapie bei malignen Gliomen zu evaluieren. Patienten und Methode: 56 Patienten wurden in die Studie eingeschlossen. Paclitaxel wurde an den Tagen 1-4, 15-18 und 29-32 18 bis 24 Stunden vor der Bestrahlung gegeben. Diese erfolgte mit 60 Gy in konventioneller Fraktionierung ab Ta 2. 31 Patienten nahmen an der Dosiseskalationsstudie teil, die mit Paclitaxel 20mg/m²/d bis 60 mg/m²/d in 10-mg-Schritten durchgeführt wurde. Weitere 25 Patienten wurden mit 50 mg/m²/d behandelt. Ergebnisse: Das Regime wurde sehr gut vertragen. Es trat keine Übelkeit oder Reduktion des Allgemeinzustandes auf. Die maximal tolerable Dosis war 50 mg/m²/d. Dosislimitierend war eine Grad-4-Neutropenie bei 60 mg/m²/d (Tabelle 1). Vier allergische Reaktionen und fünf thromboembolische Ereignisse waren zu verzeichnen. Ein Patient starb an einer Pneumonie bei normalem Blutbild. Das mediane Überleben betrug 12 Monate. Signifikant günstige prognostische Faktoren waren junges Patientenalter, Grad-3-Tumoren (Abbildung 1) und komplette Resektion (Abbildung 2). Die Paclitaxeldosis hatte keinen Einfluss auf das Therapieergebnis. Schlussfolgerung: Die kombinierte Radiochemotherapie mit Paclitaxel war gut verträglich. Die maximal tolerable Dosis betrug 50 mg/m²/d. Im Vergleich zu Literaturdaten konnte keine Verbesserung des Überlebens festgestellt werden.     

Retarded hand growth due to a hemodialysis fistula in a young girl

Long-term presence of an arteriovenous hemodialysis fistula (AVF) may lead to alterations in hand perfusion. In the case reported here, a 14-year-old girl developed pain associated with hand ischemia 5 years after a successful kidney transplantation. At age 8 years, she required a period of hemodialysis using an autogenous left upper arm AVF. Compared to the healthy right hand, a smaller ischemic left hand was observed in the presence of a patent AVF. Access flow was 1400 ml/min. Seldinger angiography demonstrated a stenotic brachial artery, and duplex measurements indicated a reversed blood flow in the radial artery. AVF ligation abolished the ischemic symptoms. Distal hypotension due to an impaired arterial inflow combined with a low resistance elbow AVF may result in chronic hypoperfusion of acral portions of the extremity and growth retardation. Access ligation is advised in children with an optimal renal transplant function and a patent elbow AVF suffering from lowered distal tissue perfusion.