Safety of different cord clamping strategies in the early postnatal period.

BACKGROUND: This study aimed to evaluate the effect of ICC (cord clamping within the first 15 s), DCC (delayed cord clamping at 60 s), and cut-UCM (cut-umbilical cord milking, cord clamping within the first 15 s) groups on oxygen saturation (SpO2), heart rate (HR), and perfusion index (PI) up to 10 min after birth in newborn infants. METHODS: We conducted this randomized clinical trial in the delivery unit of a University Hospital with 189 infants born between 35 and 42 weeks of gestation. Participants were randomly assigned to one of three groups: ICC, DCC, and cut-UCM. The primary outcomes measured were SpO2, HR, and PI at the 1st, 3rd, 5th, and 10th minutes after birth. We utilized ANOVA and Bayesian calculations in this study. RESULTS: There was no difference between the ICC, DCC, and cut-UCM groups in SpO2, HR, and PI values at the 1st, 3rd, 5th, and 10th minutes of life, which did not significantly alter between the three groups in one-way ANOVA. Bayesian repeated-measure ANOVA calculations showed that SpO2 and heart rate results at the 1st, 3rd, 5th, and 10th minutes did not differ between ICC, DCC, and cut-UCM techniques with strong evidence. At the 3rd minute, PI was slightly higher in the DCC and cut-UCM groups compared to the ICC group, with anecdotal evidence. We found no difference between DCC and cut-UCM regarding the 3rd-minute PI, with moderate evidence. CONCLUSION: Umbilical clamping procedures (ICC, DCC, and cut-UCM) did not affect SpO2 and HR in the first ten minutes of life, but 3rd-minute PI values were slightly higher in DCC and cut-UCM compared with ICC among late preterm and term neonates.

Regional pulmonary oxygen saturations in late preterm and term infants with respiratory distress at birth.

BACKGROUND: Measurement of regional pulmonary oxygen saturation by near-infrared spectroscopy is a novel monitorization method. This study aimed to determine the early regional pulmonary oxygen saturations in neonates with respiratory distress. METHODS: This observational study was conducted at the delivery room in infants above 35 weeks of gestation who developed respiratory distress immediately after birth. Preductal oxygen saturation (Covidien Nellcor®) and regional oxygen saturations of both apical (raSO2) and basal regions (rbSO2) of right lung were measured (Covidien INVOS®) within the first 15 min of life and compared to those of healthy neonates. RESULTS: Of the 165 infants included to the study, 15 were late preterm and 55 developed respiratory distress. Infants with respiratory distress had significantly lower gestational age and birth weight. Regional pulmonary oxygenations at both apex and basal lung areas were positively correlated with SpO2 in all infants. The rbSO2 was significantly lower than raSO2 until 10th minute of life regardless of respiratory distress. The fractionized tissue oxygen extraction of both apical and basal lung areas was significantly higher in infants with respiratory distress until 5th minute of life. CONCLUSION: This study is one of the pioneer studies evaluating the early pulmonary oxygenation values of infants with respiratory distress. Oxygenation of apical lung regions are better than basal areas. Higher fractionized tissue oxygen extraction showed the impaired pulmonary perfusion in infants with respiratory distress.

Prongs or Mask for Nasal Continuous Positive Airway Pressure in Neonates: Which One Is More Comfortable?

BACKGROUND: Nasal continuous positive airway pressure (NCPAP) is a common mode of respiratory support in neonatal intensive care units. Our objective was to compare whether NCPAP given with nasal prongs compared with a nasal mask reduces the pain scores in preterm infants with respiratory distress. METHODS: Preterm infants on NCPAP due to respiratory distress were included in the study. All infants received NCPAP via the Infant Flow SiPAP. The COVERS pain scale was used to score the infants' pain. Each infant was studied alternating between nasal prongs and a nasal mask. Heart rate, respiratory rate, oxygen saturation, and transcutaneous CO2 (tcCO2) were monitored. Blood pressure and the infants' pain scores were determined every 30 minutes and the average of measurements was taken. RESULTS: The median (interquartile range) values of pain scores, respiratory rates, oxygen saturations, tcCO2 levels, and systolic blood pressures differed significantly and favored the nasal mask. CONCLUSIONS: This study demonstrates that continuous positive airway pressure via a nasal mask leads to a significant reduction in pain scores without altering the respiratory parameters of babies. On the basis of this study, it is possible to conclude that NCPAP applied via nasal mask may be a good alternative to NCPAP applied via nasal prongs.

Plerixafor in autologous stem cell transplantation: Does it affect engraftment kinetics?

Plerixafor increases stem cell mobilization by reversibly binding to the chemokine receptor CXCR4. In our study, we examined the results of mobilization with plerixafor and granulocyte colony-stimulating factor (G-CSF) and revealed their effects on autologous stem cell transplantation (ASCT) engraftment kinetics. The study included all cases of ASCT performed in the Adult Bone Marrow Transplantation Unit of xxx University between January 2014 and January 2022. It included a total of 300 patients. The total number of CD34 + cells collected was 7.44 ± 4.19 in patients with plerixafor and 9.53 ± 6.09 in patients without plerixafor. The mean neutrophil and platelet engraftment took longer in plerixafor-mobilized patients (neutrophil: 12 ± 4.1 vs. 10.2 ± 2.7 days; platelet: 21.6 ± 13.9 vs. 14.2 ± 5.9 days; p = 0.008 and p = 0.002). The number of febrile neutropenia attacks was significantly higher in plerixafor-mobilized patients (p = 0.04). In the chemo-mobilized patient subgroup, plerixafor-mobilized patients experienced more febrile neutropenia attacks (p = 0.04). The mean time to both neutrophil and platelet engraftment was longer in patients mobilized with plerixafor. In the subgroup of patients with MM, the mean time to platelet engraftment was longer in patients mobilized with plerixafor. Plerixafor and its effect on engraftment kinetics should be evaluated with further studies in a larger population with survival analysis.

The Factors Affecting the Postnatal Bonding of Mothers Whose Babies were Admitted to the Neonatal Intensive Care Unit During the COVID-19 Pandemic.

OBJECTIVE: COVID-19 pandemic has created challenges for sick preterm babies and their parents. This study aimed to explore the factors affecting the postnatal bonding of mothers who were not permitted to visit and touch their babies who were in the neonatal intensive care unit during the COVID-19 pandemic. MATERIALS AND METHODS: This is a cohort study conducted in a tertiary neonatal intensive care unit in Turkey. The participants consisted of mothers who were offered full rooming in with their baby (group 1, n = 32) and mothers whose newborns had been admitted to the neonatal intensive care unit immediately after delivery and were hospitalized for at least 7 days (group 2, n = 44). The Turkish versions of Beck Anxiety Inventory, Edinburgh Postpartum Depression Scale, Adjustment Disorder-New Module 8, and Postpartum Bonding Questionnaire were applied to mothers. Tests were performed once in group 1 at the end of the first postpartum week (test1) and twice in group 2 before the baby was discharged from the neonatal intensive care unit (test1) and 2 weeks after the discharge (test2). RESULTS: None of the Beck Anxiety Inventory, Edinburgh Postpartum Depression Scale, Adjustment Disorder-New Module 8, and Postpartum Bonding Questionnaire scores was abnormal. Although the scales were within normal ranges, Postpartum Bonding Questionnaire 1 and Postpartum Bonding Questionnaire 2 had statistically significant correlation with gestational week (r = -0.230, P = .046; r = -0.298, P = .009), Edinburgh Postpartum Depression Scale score (r = 0.256, P = .025; r = 0.331, P = .004), hospitalization (r = 0.280, P = .014; r = 0.501, P < .001), and neonatal intensive care unit anxiety (r = 0.266, P = .02; r = 0.54, P < .001). Postpartum Bonding Questionnaire 2 had statistically significant correlation with birth weight (r = -0.261, P = .023). CONCLUSION: Low gestational week and birth weight, increased maternal age, maternal anxiety, high Edinburgh Postpartum Depression Scale scores and hospitalization negatively affected maternal bonding. Although all self-reporting scale scores were low, being in the neonatal intensive care unit and not being able to visit (touch) the baby is a major stressor.

Intestinal perforation after intravitreal low dose ranibizumab injection for the treatment of type 1 retinopathy of prematurity: A case report.

PURPOSE: To report a newborn patient with gastrointestinal (GIS) perforation after intravitreal ranibizumab (RBZ) treatment. CASE REPORT: The patient was born at 31 gestational week and hospitalized with the diagnosis of small for gestational age and prematurity. In the follow up he underwent GIS surgery due to necrotizing enterocolitis (NEC) and was diagnosed with retinopathy of prematurity (ROP). At 43 weeks of postmenstrual age, he developed intestinal perforation after 12 h of the second low-dose RBZ injection. According to our knowledge, this is the first report of GIS perforation due to low-dose intravitreal RBZ treatment in an infant with severe ROP. CONCLUSION: The risk of GIS perforation should be taken into consideration during the application of intravitreal vascular endothelial growth factor antagonist agents, especially in newborns with previous GIS surgery and a history of NEC, and these patients should be carefully monitored for GIS complications.

Should Fathers Also Do Kangaroo Care?

OBJECTIVE: The aim of the study is to compare the effect of maternal and paternal Kangaroo care (KC) on vital signs and cerebral oxygen saturation (rSO2) parameters. METHODS: This randomized study included 35 preterm infants born at 32 weeks gestation or earlier. The body temperature, respiratory rate, heart rate, oxygen saturation (sPO2), and cerebral oxygen saturation (rSO2) were measured at the postnatal 35th week of gestation during three periods: in the incubator before KC, during KC with the mother or father, and in the incubator after KC. The measured parameters of the three periods were compared within each group (mother or father) and between the groups (mother and father). RESULTS: There was no statistically significant difference between the vital signs during the three periods, both in the mother and father groups, and between the parents.The rSO2 values increased significantly in both parents (p <0.001) during and after KC. When we compared parents, rSO2 values were significantly higher during (p = 0.015) and after (p = 0.046) mother KC. CONCLUSION: The difference between M-KC and F-KC can be ignored as the cerebral near infra-red spectroscopy values are within normal limits. The active participation of fathers in KC should be encouraged by health professionals in all NICUs. KEY POINTS: · * The vital signs during and after kangaroo care, both in the mother and father groups, were similar.. · * The rSO2 values increased significantly in both parents during and after kangaroo care, favoring the mother.. · * This difference can be ignored as the cerebral near-infra-red spectroscopy values are within normal limits in both groups, and the participation of fathers in kangaroo care should be encouraged..

Regional pulmonary oxygen saturations immediately after birth.

BACKGROUND: Partial oxygen saturation (SpO2) increases within minutes during transition from the intrauterine to extrauterine life. This study aims to determine the postnatal course of pulmonary regional oxygen saturation (rSO2) measured by Near-Infrared Spectroscopy (NIRS). METHODS: We conducted an observational study at the delivery room in infants above 35 weeks of gestation who did not need resuscitation and did not develop respiratory distress. Preductal pulse oximetry (Covidien NellcorTM) and right pulmonary apex oxygen saturation (raSO2) and basal oxygen saturation (rbSO2) (Covidien INVOSTM) were measured, starting from the postnatal third minute of life, until the 15th minute. The correlations between SpO2 and pulmonary rSO2 were analyzed. RESULTS: Of the 110 infants included in the study, 87 were term and 23 were late preterms. The gestational age and birth weight were 38.5 ± 1.36 weeks and 3285 ± 508 g, respectively. Median (5th-95th percentile) raSO2 and rbSO2 were 79% (58-95%) and 78% (46-95%) at the third minute, respectively. The rSO2 values measured from both sides increased and reached a steady-state around postnatal 9 min, similar to SpO2 values. The pulmonary NIRS values were significantly higher for babies born by C-Section compared to babies born by vaginal delivery (p < 0.05). CONCLUSION: We found that rSO2 measurements increased within minutes in the postnatal period in late preterm and term babies without respiratory distress and reached a plateau at the postnatal 9th minute. The normal values obtained from this preliminary study may be used to predict the prognosis of cases with respiratory distress.

Atypical Teratoid Rhabdoid Tumor in a Newborn: Can IVF Be a Risk Factor?

BackgroundIn recent years, with the widespread use of assisted reproductive technologies, questions have arisen regarding the possible relationship between these infertile parents with assisted conception procedures and childhood cancers. Case report: We present a 23-day-old newborn conceived by in vitro fertilization (IVF) with a 53 × 46 × 38 mm intracranial mass detected by magnetic resonance imaging on the 15th postnatal day. The mass, removed on 23rd postnatal day, was an Atypical Teratoid Rhabdoid Tumor (ATRT), WHO grade 4. Conclusions: As far as we know, this is the only neonatally detected ATRT. Further studies are needed to investigate whether there is a causal relationship between IVF and childhood cancers.

Computed Tomography-guided Percutaneous Drainage of Pneumomediastinum in a Newborn: A Case Report.

BACKGROUND: Neonatal pneumomediastinum is seen in 2.5 per 1000 live births and is mostly managed conservatively. An intervention is essential in cases with tension pneumomediastinum. Ultrasonography-guided (USG-guided) relief of pneumomediastinum has been reported in newborns. There are no reported cases of computed tomography-guided (CT-guided) drainage of pneumomediastinum in neonates. CASE PRESENTATION: A newborn girl born at 34 weeks of gestation was intubated due to respiratory distress and received intratracheal surfactant treatment. Pneumomediastinum was detected at the chest X-ray on the 6th postnatal hour. On the second postnatal day, the patient's oxygen needs increased, tachypnea and subcostal retractions recurred, so it was decided to intervene. USG-guided drainage of the pneumomediastinum was attempted twice but was unsuccessful. Percutaneous drainage with CT guidance was performed successfully. CONCLUSION: This report aims to emphasize that CT-guided intervention of pneumomediastinum can be an effective alternative in a newborn if USG-guided intervention fails.

The Use of the "Early Weight Loss Nomograms" as Compared to a Standard Weight Loss Percentage Protocol Will Decrease Postnatal Formula Supplementation.

Introduction: We compared the number of babies who needed formula supplementation, based on the "Early Weight Loss Nomograms," with the hypothetical outcomes that would have occurred in the same cohort if they had been managed according to a "weight loss percentage" protocol. Subjects and Methods: This study included 308 newborns. Supplemental formula was provided to babies whose weight loss was more than the 95th percentile according to the "Early Weight Loss Nomograms." Pathological weight loss was defined as when a weight loss was >5% at the 24th hour or >8% at the 48th hour. The number of babies who would have needed formula supplementation according to those two strategies were compared. Results: The mean postnatal first-second day weight losses for vaginal and cesarean deliveries were 3.06% versus 4.7% and 4.5%, versus 5.8%, respectively, and were significantly higher for babies born by cesarean section (p = 0.001). We found that 89.4% of vaginal deliveries and 89.2% of babies born by cesarean section were exclusively breastfed when the nomograms were in use. If the daily weight loss strategy would be applied instead of the nomograms to the study cohort, the rate of exclusive breastfeeding would be significantly lower for babies born by cesarean section (64.2% versus 89.2%) (p = 0.001). Conclusions: The use of the Early Weight Loss Nomograms will decrease the rate of formula supplementation.

Clinical Efficacy of Ruxolitinib Monotherapy and Haploidentical Hematopoeitic Stem Cell Transplantation in a Child with Philadelphia Chromosome-like Relapsed/Refractory acute lymphoblastic leukemia.

INTRODUCTION: (Ph-like) ALL is a subset of leukemia which has a gene expression profile similar to Ph+disease, but without the presence of BCR-ABL1 translocation. CASE DESCRIPTION: We reported an exceptional case of a child with relapsed Ph-like ALL with IKZF1 gene deletion treated with high-dose ruxolitinib as monotherapy, after multi-agent chemotherapy. He remains in continued MRD-negative leukemia remission with full donor chimerism at 12 months post-HSCT. DISCUSSION: The circumstance that makes our case featured is the usage of ruxolitinib as monotherapy. This report, we believe, is a pioneering report for a frequent disease with a high risk of failure for the outcome.

Acute lung injury after exchange transfusion in two newborns with Glucose-6-phosphate dehydrogenase deficiency.

Transfusion-related lung injury (TRALI) is a condition that develops suddenly within the first six hours after a blood transfusion and it is one of the most important causes of blood transfusion-related mortality. There are few data in the literature about TRALI in the neonatal period. We present two newborn patients who developed TRALI after exchange transfusion due to high bilirubin levels. Our first case was a late preterm LGA baby and was on CPAP. The baby was intubated due to sudden deterioration after the exchange transfusion. Our second case was born at term and, an exchange transfusion was performed on the 5th day of life. He developed respiratory distress unexpectedly soon after the exchange transfusion and was intubated. Glucose-6- phosphate dehydrogenase (G6PD) deficiency was detected in both of our cases. We wanted to emphasize that TRALI should be considered in the differential diagnosis of respiratory distress that develops soon after a transfusion in the newborn period and to draw attention to that TRALI may develop more frequently in patients with G6PD deficiency.

Eltrombopag for treatment of thrombocytopenia after allogeneic hematopoietic cell transplantation in children: Single-centre experience.

Delayed recovery of thrombocytopenia is a well-known complication after allogeneic HSCT. Eltrombopag (ELT), a thrombopoietin receptor agonist (TRAs), induces platelet maturation and release. Mostly conducted in adults, some of the previous studies have shown that ELT seems to enhance platelet recovery for post-allogeneic HSCT thrombocytopenia, appears efficacious, and offers transfusion independence. To evaluate the safety and efficacy of ELT in pediatric patients with prolonged isolated thrombocytopenia (PIT) or secondary failure of platelet recovery (SFPR) after alloHSCT. Retrospective analysis of childhood patients who received treatment with ELT for persistent thrombocytopenia after alloHSCT between May 2016 and August 2019. We evaluated the safety and efficacy of ELT in 18 childhood patients with PIT or SFPR after alloHSCT. Eltrombopag (50 mg/d) treatment was started in all patients, above 6 years of age and 20 kg weight, who had thrombocytopenia despite neutrophil engraftment on the 30th day of HSCT. Our objective was to decrease the need for platelet transfusion and have a platelet count of more than 50 000/µL. The overall response rate was 77.7%. The median time to achieve a platelet level above 30 000/µL and 50 000/µL was 21 and 44 days, respectively. In four patients, platelet count never reached 30 000/mm3 . In two patients, the treatment was discontinued due to grade 3 hepatotoxicity. Our study supports the efficacy and relative safety of ELT use for the treatment of PIT and SFPR seen after alloHSCT in children.

Cinacalcet as a First-Line Treatment in Neonatal Severe Hyperparathyroidism Secondary to Calcium Sensing Receptor (CaSR) Mutation.

INTRODUCTION: Neonatal severe hyperparathyroidism (NSHPT) is a rare cause of neonatal hypercalcemia caused by a loss of function mutation in the calcium-sensing receptor (CaSR). Hypercalcemia in NSHPT can be life-threatening. Maintenance of serum calcium within a safe range is the primary goal of treatment through hydration, forced diuresis, and bisphosphonate treatment, nevertheless most cases require parathyroidectomy. We report a case with NSHPT diagnosed on the first day of life (DoL) and successfully treated with cinacalcet as the first-line treatment from the 2nd DoL up to the age of 18 months. CASE REPORT: A full-term baby evaluated for weight loss at postnatal 14th hour and found to have hypercalcemia (14.4 mg/dL, reference range [RR]: 8.0-11.3). Despite hydration and diuresis, hypercalcemia persisted. Further evaluation revealed a parathyroid hormone (PTH) level of 1,493 pg/mL (RR: 15-65) and urine Ca/Cr of 0.09 mg/mg (RR: 0.03-0.81). Cinacalcet treatment was initiated on the 2nd DoL with the diagnosis of NSHPT due to hypocalciuric hypercalcemia and elevated PTH level. Ca levels decreased to normal levels on the 7th DoL. She was discharged from hospital at postnatal day 15 on cinacalcet treatment and still continued at 18 months of age. Sequencing of CaSR revealed a novel homozygous c.1836G>A (p.G613E) mutation in the patient, for which the parents and sister were heterozygous. CONCLUSION: This case represents the youngest age at cinacalcet initiation and the longest duration without parathyroidectomy in a homozygous NSHPT and demonstrates that cinacalcet is an effective first-line treatment in patients who are responsive to this treatment modality and allows avoiding/delay in surgical intervention in NSHPT.

A single center cohort of 40 severe COVID-19 patients who were treated with convalescent plasma

Background/aim: A SARS-Cov2 infection which was first arised from Wuhan in December 2019 and named as COVID-19. Still there lacks either a specific treatment or a vaccine to treat COVID-19. Convalescent plasma (CP) was previously used successfully to treat SARS-CoV-1 and MERS infections. Health authority in Turkey has published a guideline to integrate this promising option in the treatment process of patients who are prone to high risk of developing severe COVID 19. Materials and Methods: Forty consecutive patients who had received CP at our center were included in the study. Demographics, COVID-19 specific parameters, biomarkers to detect the severity of COVID-19 infection and outcome variables were collected retrospectively. The correlation between outcome variables and the independent predictors of the outcome were reported. Results: Median age of the patients was 57.5 and 72.5% were male. At least one COVID-19 PCR test was confirmed to be positive in 75% of patients. Remaining 25% had a Chest-CT which was reported to be compatible with an ongoing COVID-19. All patients (100%) were classified as having severe COVID-19 infection. Over a half of the patients harbored an oxygen saturation of less than 90 despite of a continuous 5 L/min support of O2. 82.5% of the patients had a need for mechanical ventilation and 45.5% had a need for invasive mechanical ventilation. Nine out of 10 patients who have received CP outside ICU have totally recovered from COVID-19 at a median of 9 days, and a half of the patients who needed invasive mechanical ventilation were successfully free of mechanical ventilation support and managed to recover from COVID-19. Conclusion: According to the results of this study, CP is an efficient conjunct to conventional therapy against COVID-19 with a favorable safety profile.

Neurons from human mesenchymal stem cells display both spontaneous and stimuli responsive activity.

Mesenchymal stem cells have the ability to transdifferentiate into neurons and therefore one of the potential adult stem cell source for neuronal tissue regeneration applications and understanding neurodevelopmental processes. In many studies on human mesenchymal stem cell (hMSC) derived neurons, success in neuronal differentiation was limited to neuronal protein expressions which is not statisfactory in terms of neuronal activity. Established neuronal networks seen in culture have to be investigated in terms of synaptic signal transmission ability to develop a culture model for human neurons and further studying the mechanism of neuronal differentiation and neurological pathologies. Accordingly, in this study, we analysed the functionality of bone marrow hMSCs differentiated into neurons by a single step cytokine-based induction protocol. Neurons from both primary hMSCs and hMSC cell line displayed spontaneous activity (≥75%) as demonstrated by Ca++ imaging. Furthermore, when electrically stimulated, hMSC derived neurons (hMd-Neurons) matched the response of a typical neuron in the process of maturation. Our results reveal that a combination of neuronal inducers enhance differentiation capacity of bone marrow hMSCs into high yielding functional neurons with spontaneous activity and mature into electrophysiologically active state. Conceptually, we suggest these functional hMd-Neurons to be used as a tool for disease modelling of neuropathologies and neuronal differentiation studies.

Clinical and laboratory awareness for an under recognized pathogen in newborn meningitis: Mycoplasma Hominis: a case report.

BACKGROUND: Mycoplasma hominis is a well-known bacterium colonizing the genito-urinary tract. It may cause pneumonia, bacteremia, abscesses, chronic lung disease, and rarely meningitis during the newborn period. CASE: A preterm infant with a birth weight of 885 grams was born at 27 weeks of gestation and had respiratory distress syndrome needing mechanical ventilation. Spontaneous intestinal perforation and grade four intraventricular hemorrhage was diagnosed on day three. CONCLUSION: M. hominis was accepted as the causative agent of meningitis in this case report.

Squamous cell carcinoma development in Fanconi anemia patients who underwent hematopoietic stem cell transplantation.

We examined SCC development of 24 FA patients, who received HSCT from HLA-matched relatives. In our BMT center, we applied low-dose CY + LFI + ATG (n:13) as conditioning regimen for FA patients between 1992 and 1999, and CY + BU + ATG (n:11) between 1999 and 2002. The aim of this study was to investigate SCC development after HSCT and examine features of the follow-up patients. The 10-year overall survival (OS) of the group with LFI + regimen was 43%, whereas the group without LFI regimen was 60%. There was a statistically significant relationship between infections (viral/bacterial) and overall survival (Fisher's Exact test P < .001). Five out of 13 long-term (>1 year) surviving patients developed SCC in the HNSCC (n:4) and esophagus (n:2) region (a patient with oral SCC developed a second primary esophageal SCC). The SCC rate in our FA patients was 38%, four of the SCC patients were transplanted with irradiation used conditioning regimens, three of them had acuteGvHD (Grade II-III), only one developed chronic GvHD. The interval between HSCT and SCC diagnosis was median 13 (range 6-18) years, the age for the development of cancer was median 21 (range 15-32) years. Survival after SCC was low, median 6 months (range 6-12), due to delayed SCC diagnosis, tumor progression under therapy and treatment-related toxicities of the usually reduced RT and/or CT.