CD38 is a multifunctional enzyme expressed in a variety of mammalian tissues, its catalytic activity was involved in a wide range of physiological processes. Based on the reported inhibitor of human CD38 NADase, 33 purine derivatives were designed and synthesized. The biological activity assay showed that compounds 20 and 38 exhibited almost the same extent of inhibitory activities on human CD38 NADase as the lead compound H2. The results also revealed that small substituents at C-6 of purine ring gave no obvious effect on inhibitory activity, but phenylpropionyl moiety at N-2 could affect the binding mode of the compound with CD38. This study provides a reliable basis for future rational design of inhibitors for CD38.

Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease caused by mutations in the solute carrier family 26 member 3 (SLC26A3) gene on chromosome 7q31. Affected neonates are vulnerable to dehydration, electrolyte imbalance in the form of hyponatremia, metabolic alkalosis, failure to thrive, or even death if left untreated. Genetic testing for mutations should be considered if the clinical diagnosis remains uncertain because early diagnosis and appropriate management are critical to the disease course in CLD. Several mutations have been reported in Korean patients with CLD, with the most common being the c.2063-1G>T mutation. Here, we report the case of a neonate with prenatally suspected CLD with confirmed novel mutations in the SLC26A3 gene (c.2147C>G; p.Ala716Gly).

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

CD38 is a multifunctional enzyme expressed in a variety of mammalian tissues, its catalytic activity was involved in a wide range of physiological processes. Based on the reported inhibitor of human CD38 NADase, 33 purine derivatives were designed and synthesized. The biological activity assay showed that compounds 20 and 38 exhibited almost the same extent of inhibitory activities on human CD38 NADase as the lead compound H2. The results also revealed that small substituents at C-6 of purine ring gave no obvious effect on inhibitory activity, but phenylpropionyl moiety at N-2 could affect the binding mode of the compound with CD38. This study provides a reliable basis for future rational design of inhibitors for CD38.
ADP-ribosyl Cyclase 1 ; antagonists & inhibitors ; Enzyme Inhibitors ; chemical synthesis ; chemistry ; Humans ; Purines ; chemical synthesis ; chemistry

OBJECTIVE: To assess the influence of reflex sympathetic dystrophy (RSD) on functional status and recovery of the hemiplegic upper extremity in stroke patients. METHOD: Retrospective chart review was performed in 561 patients. Among 561 stroke patients, 116 subjects were recruited and classified into two groups: patient group, 43 cases with RSD; control group, 73 cases without RSD. Upper extremity function was assessed based on feeding, dressing and personal hygiene scores of the modified Barthel index at the beginning of rehabilitation treatment and at the time of discharge. Causes of stroke and length of stay were recorded. Median nerve-somatosensory evoked potential studies were performed and assessed. RESULTS: The incidence of RSD was 7.7% and the time to development of RSD was 62.3+/-34.1 days after the onset of stroke. There was no significant difference in functional status between two groups at initial and final evaluation. The upper extremity function had improved in both groups although the length of stay was longer in patient group. SSEP abnormalities were more frequent in the patient group. CONCLUSION: The presence of well-managed RSD affected neither the functional status nor the functional recovery of upper extremity in stroke patients.
Bandages ; Evoked Potentials ; Evoked Potentials, Somatosensory ; Humans ; Hygiene ; Incidence ; Length of Stay ; Reflex Sympathetic Dystrophy* ; Reflex* ; Rehabilitation ; Retrospective Studies ; Stroke* ; Upper Extremity*

PURPOSE: This study investigated predictive factors for severe neonatal thrombocytopenia, which greatly increases the need for intensive care and is associated with a high mortality rate in premature infants. Factors adopted for prompt identification of at-risk newborns include blood test results and birth history. This study analyzed the relationship between the presence of severe neonatal thrombocytopenia and the mortality rate. The causes of thrombocytopenia in premature infants were also examined. METHODS: This retrospective study evaluated 625 premature infants admitted to the neonatal intensive care unit (NICU) at Chung-Ang University Medical Center. The neonates were classified into 3 groups according to the severity of thrombocytopenia: mild (100×10⁹/L≤platelet < 150×10⁹/L), moderate (50×10⁹/L≤platelet < 100×10⁹/L), or severe (platelet < 50×10⁹/L). Analysis of blood samples obtained at the onset of thrombocytopenia included platelet count, white blood cell (WBC) count, hemoglobin level, hematocrit level, absolute neutrophil count, and high-sensitivity C-reactive protein level. RESULTS: Of the 625 premature infants admitted to our NICU, 214 were detected with thrombocytopenia. The mortality rate in thrombocytopenic neonates was 18.2% (39/214), whereas a mortality rate of only 1.0% was observed in non-thrombocytopenic neonates. The major causes of thrombocytopenia were perinatal insufficiency and sepsis in premature infants. Severe thrombocytopenia was noted more frequently in premature infants with higher WBC counts and in those with a younger gestational age. CONCLUSION: Platelet count, WBC count, and gestational age are reliable predictors for severe neonatal thrombocytopenia. The major causes of thrombocytopenia were perinatal insufficiency and sepsis in premature infants.
Academic Medical Centers ; C-Reactive Protein ; Classification ; Critical Care ; Gestational Age ; Hematocrit ; Hematologic Tests ; Humans ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Leukocytes ; Mortality ; Neutrophils ; Platelet Count ; Reproductive History ; Retrospective Studies ; Sepsis ; Thrombocytopenia ; Thrombocytopenia, Neonatal Alloimmune