This paper reports a pair of siblings with congenital short-bowel syndrome (CSBS) complicated with intestinal malrotation. Case 1 was born with a birth weight of 2 550 g and a length of 48 cm. On September 10, 2017, emergency Ladd's procedure and appendectomy were performed on the infant 23 days after birth due to intestinal obstruction at the Women and Children's Hospital, School of Medicine, Xiamen University. The small intestine of the infant had a total length of 65 cm. Postoperative enteral and parenteral nutrition supports were provided for six months. Whole exome sequencing revealed a homozygous variant (NM 024769; nucleotide deletion in the exon 3-5) in the CLMP gene (chr11:122953792-122955421), with the parents being the heterozygous carriers but without phenotype. Case 2, the younger sibling of Case 1, was born in the same hospital on March 20, 2020, with a birth weight of 2 932 g and a body length of 49 cm. Prenatal single-gene sequencing on the amniotic fluid identified the same gene variation as his sister's. The baby boy received Ladd's procedure and appendectomy on the second day after birth which found the length of his small intestine was 51 cm. Full enteral nutrition was achieved six months after the operation. Both cases were followed up for 12 months. The body weight and length of Case 1 were both below the first percentile (< P1). The body weight of Case 2 was 8.03 kg ( P3- P5) and the length was 76.0 cm ( P25- P50).

Objective:To investigate the clinical value of open abdomen therapy in non-traumatic critically patients.Methods:The retrospective cohort study was conducted. The clinical data of 23 non-traumatic critically patients who underwent open abdomen therapy in 5 hospitals in China from July 2015 to July 2024 were collected. There were 17 males and 6 females, aged 70(range, 24-84)years. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers. Repeated measurement data were analyzed using the repeated ANOVA, and pairwise comparison within groups was conducted using the least significant difference method. The Boruta algorithm was applied for analyzing variables related to survival outcomes. Results:(1) Treatment of patients undergoing open abdomen therapy. ① The intra-abdominal pressure, lactate, heart rate, central venous pressure, mean arterial pressure, sequential organ failure assessment score of 23 patients from preoperation to postoperative day 3 were changed from (19.7±5.4)mmHg (1 mmHg=0.133 kPa), (6.1±1.9)mmol/L, (120±14)beats/minutes, (13.1±4.3)cmH 2O (1 cmH 2O=0.098 kPa), (58.8±6.8)mmHg, 13.2±1.8 to (10.6±1.3)mmHg, (2.3±0.6)mmol/L, (95±10)beats/minutes, (8.8±2.0)cmH 2O, (75.2±8.5)mmHg, 10.1±1.6, respectively, showing significant differences in the time effect of changes in the above indicators ( Ftime=46.40, 29.19, 24.91, 11.84, 27.81, 11.71, P<0.05). ② The oxygenation index, total intake, total output of 23 patients from preoperation to postoperative day 3 were changed from (255.0±54.2)mmHg, (5388±1562)mL, (2 520±630)mL to (291.7±25.0)mmHg, (2 886±866)mL, (3 221±923)mL, respectively, showing significant differences in the time effect of changes in the above indicators ( Ftime=7.61, 13.83, 2.97, P<0.05). ③The daily caloric intake, daily protein supplementation of 23 patients from preoperation to postoperative day 3 were changed from (465±116)kcal, (18±5)g to (1 628±472)kcal, (60±18)g, respectively, showing significant differences in the time effect of changes in the above indicators ( Ftime=40.31, 41.23, P<0.05). (2) Patients outcomes after open abdomen therapy. Of 23 patients, 18 cases survived and 5 cases died. The duration of intensive care unit stay and duration of hospital stay of 23 patients were 26(range, 5-82)days and 40(range, 5-98)days. Twelve of 23 patients received renal replacement therapy for 12 (range, 5-32)days. Time of pain and sedation management, mechanical ventilation, antimicrobial therapy, vasopressor therapy of 23 patients were 13(range, 5-74)days, 12(range, 5-74)days,20(range, 5-50)days, 6(range, 2-35)days. (3) Analysis of variables related to survival outcomes for patients after open abdomen therapy. Results of Boruta analysis showed that postoperative high-output enteric fistula, postoperative bile fistula, postoperative intra-abdominal hemorrhage, postoperative enteric air fistula, and preoperative mean arterial pressure were significantly associated with survival outcomes. Conclusions:Open abdomen therapy is effective in the treatment of non-traumatic critically patients. Postoperative high-output enteric fistula, postoperative bile fistula, postoperative intra-abdominal hemorrhage, postoperative enteric air fistula, and preoperative mean arterial pressure reduction are significantly associated with survival outcomes.

Objective:To investigate the clinical value of open abdomen therapy in non-traumatic critically patients.Methods:The retrospective cohort study was conducted. The clinical data of 23 non-traumatic critically patients who underwent open abdomen therapy in 5 hospitals in China from July 2015 to July 2024 were collected. There were 17 males and 6 females, aged 70(range, 24-84)years. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers. Repeated measurement data were analyzed using the repeated ANOVA, and pairwise comparison within groups was conducted using the least significant difference method. The Boruta algorithm was applied for analyzing variables related to survival outcomes. Results:(1) Treatment of patients undergoing open abdomen therapy. ① The intra-abdominal pressure, lactate, heart rate, central venous pressure, mean arterial pressure, sequential organ failure assessment score of 23 patients from preoperation to postoperative day 3 were changed from (19.7±5.4)mmHg (1 mmHg=0.133 kPa), (6.1±1.9)mmol/L, (120±14)beats/minutes, (13.1±4.3)cmH 2O (1 cmH 2O=0.098 kPa), (58.8±6.8)mmHg, 13.2±1.8 to (10.6±1.3)mmHg, (2.3±0.6)mmol/L, (95±10)beats/minutes, (8.8±2.0)cmH 2O, (75.2±8.5)mmHg, 10.1±1.6, respectively, showing significant differences in the time effect of changes in the above indicators ( Ftime=46.40, 29.19, 24.91, 11.84, 27.81, 11.71, P<0.05). ② The oxygenation index, total intake, total output of 23 patients from preoperation to postoperative day 3 were changed from (255.0±54.2)mmHg, (5388±1562)mL, (2 520±630)mL to (291.7±25.0)mmHg, (2 886±866)mL, (3 221±923)mL, respectively, showing significant differences in the time effect of changes in the above indicators ( Ftime=7.61, 13.83, 2.97, P<0.05). ③The daily caloric intake, daily protein supplementation of 23 patients from preoperation to postoperative day 3 were changed from (465±116)kcal, (18±5)g to (1 628±472)kcal, (60±18)g, respectively, showing significant differences in the time effect of changes in the above indicators ( Ftime=40.31, 41.23, P<0.05). (2) Patients outcomes after open abdomen therapy. Of 23 patients, 18 cases survived and 5 cases died. The duration of intensive care unit stay and duration of hospital stay of 23 patients were 26(range, 5-82)days and 40(range, 5-98)days. Twelve of 23 patients received renal replacement therapy for 12 (range, 5-32)days. Time of pain and sedation management, mechanical ventilation, antimicrobial therapy, vasopressor therapy of 23 patients were 13(range, 5-74)days, 12(range, 5-74)days,20(range, 5-50)days, 6(range, 2-35)days. (3) Analysis of variables related to survival outcomes for patients after open abdomen therapy. Results of Boruta analysis showed that postoperative high-output enteric fistula, postoperative bile fistula, postoperative intra-abdominal hemorrhage, postoperative enteric air fistula, and preoperative mean arterial pressure were significantly associated with survival outcomes. Conclusions:Open abdomen therapy is effective in the treatment of non-traumatic critically patients. Postoperative high-output enteric fistula, postoperative bile fistula, postoperative intra-abdominal hemorrhage, postoperative enteric air fistula, and preoperative mean arterial pressure reduction are significantly associated with survival outcomes.

Objective To study the clinical effect of different tracheal intubation in patients with severe brain injury.Methods 82 patients with severe brain injury were selected and randomly divided into observation group and control group,each group had 41 cases.The observation group was given the dexmedetomidine induction combined with surface anesthesia on the basis of endotracheal intubation,and the control group was given traditional endotracheal intubation.The effects of the two groups were compared.Results The intubation time of the observation group was (26.7 ±13.8)s,which was significantly lower than (41.6 ±17.9)s of the control group(t =4.221,P =0.000).There were no differences in cardiovascular response indices between the two groups before intubation(t =0.122,P =0.903;t =0.296,P =0.768;t =1.128,P =0.263).After 10min,HR and MAP levels were significantly lower in the observation group than those in the control group(t =3.326,P =0.0.001;t =2.354,P =0.021).In the observation group,HR,MAP after intubation were lower than before intubation(t =2.548,P =0.013;t =3.626,P =0.000),the SpO2 of the two groups was higher than that before intubation(t =30.622,P =0.000;t =38.797,P =0.000),there were no differences in HR and MAP before and after intubation in the control group(t =0.846,P =0.400;t =1.824,P =0.072).There were no differences between the two groups before intubation(t =0.183,P =0.856;t =0.000,P =1.000;t =1.132,P =0.261),CK -MB,cTnT,BNP were significantly lower than those in the control group after 12h,respectively(t =2.030,P =0.046;t =2.264,P =0.026;t =3.785,P =0.000).CK -MB, cTnT,BNP were significantly lower than the control group after intubation in the two groups(t =7.845,P =0.000;t =8.591,P =0.000;t =22.757,P =0.000;t =5.525,P =0.000;t =2.264,P =0.000;t =22.149,P =0.000).The BCS and SS scores in the observation group were significantly higher than those in the control group(t =5.739,P =0.000;t =5.264,P =0.000).Conclusion The dexmedetomidine induction combined with topical anesthesia based uplink tracheal intubation scheme has significant effect,safety is good,it is worth of clinical application.

The toxicity of HCN inhaled via the respiratory tract in dogs and the therapeutic effects of DMAP and AmNO2 for such form of HCN intoxication were studied. The LD50 of HCN when inhaled was 850.4?80.4?g/kg.When the dosage of 2?LD50 of HCN was given to the experimental animals, the signs of intoxication developed rapidly. Uneasiness, struggling, strident barking, and respiratory excitation were observed several seconds after the exposure. Subsequently there was spasm of the extensors and rigidity of the extremities. Finally general inhibition and paralysis ensued. Respiration and heart beat stopped 6 and 7 minutes after exposure. If DMAP (3.25 mg/kg intramuscularly) or AmNO2 (2 ampules through inhalation) was administered 15 seconds after exposure, the survival rate of the animals was 90% or 100% respectively.When the dosage of 4?LD50 of HCN was inhaled and the two drugs were administered 45 seconds after exposure, the survival rate of the animals treated with DMAP remained as high as 90% (9/10) .But the survival rate of those treated with two ampules of AmNO2 or even three ampules reduced to 20% or 40% respectively.The amount of HCN inhaled during respiratory intoxication, the problems of early diagnosis and the administration of DMAP at the site of accident were analyzed and discussed.