Translating Fecobionics Into a Technique That Addresses Clinical Needs for Objective Perineal Descent Measurements.

INTRODUCTION: Perineal descent is a phenomenon associated with anorectal dysfunction. It is diagnosed by defecography but subjected to manual measurements on the images/videos and interobserver bias. Fecobionics is a simulated feces for assessing important physiological parameters during defecation. Here, we translate Fecobionics into a new method for estimation of perineal descent based on electronic signals from the embedded inertial measurement units (IMUs). METHODS: A displacement measurement method by a combined zero-velocity update and gravity compensation algorithm from IMUs was developed. The method was verified in a robot model, which mimicked perineal descent motion. RESULTS: The method correlated well with the reference (R = 0.9789) and had a deviation from the peak displacement (range 0.25-2.5 cm) of -0.04 ± 0.498 cm. The method was further validated in 5 human experiments with comparison to the benchmark defecography technology (R = 0.79). DISCUSSION: The proposed technology is objective, i.e., electronic measurements rather than by fluoroscopy or MRI. The development may impact clinical practice by providing a resource-saving and objective technology for diagnosing perineal descent in the many patients suffering from anorectal disorders. The technology may also be used in colon experiments with Fecobionics and for other gastrointestinal devices containing IMUs such as ingestible capsules like the Smartpill.

Minimally invasive right colectomy with transrectal natural orifice extraction: could this be the next step forward?

BACKGROUND: The transvaginal natural orifice specimen extraction (NOSE) approach for right-side colon surgery has been proven to exhibit favorable short-term outcomes. However, thus far, no study has reported the advantages of transrectal NOSE for right-side colon surgery. The aim of this study was to compare the technical feasibility, safety, and short-term outcomes of minimally invasive right hemicolectomy using the transrectal NOSE method and those of conventional mini-laparotomy specimen extraction. METHODS: A study was conducted on consecutive patients who had minimally invasive right hemicolectomy either for malignancy or benign disease at Chang Gung Memorial Hospital, Linkou, Taiwan, between January 2017 and December 2018. The patients were divided into two groups: conventional surgery with specimen extraction using mini-laparotomy and NOSE surgery. Surgical outcomes, including complications, postoperative short-term recovery, and pain intensity, were analyzed. RESULTS: We enrolled 297 patients (151 males, mean age 64.9 ± 12.8 years) who had minimally invasive right hemicolectomy. Of these 297 patients, 272 patients had conventional surgery with specimen extraction through mini-laparotomy and 25 patients had NOSE surgery (23 transrectal, 2 transvaginal). The diagnosis of colon disease did not differ significantly between the conventional and NOSE groups. Postoperative morbidity and mortality rates were comparable. The postoperative hospital stay was significantly (p = 0.004) shorter in the NOSE group (median 5 days, range 3-17 days) than in the conventional group (median 7 days, range 3-45 days). Postoperative pain was significantly (p = 0.026 on postoperative day 1 and p = 0.002 on postoperative day 2) greater in the conventional group than in the NOSE group. CONCLUSIONS: NOSE was associated with acceptable short-term surgical outcomes that were comparable to those of conventional surgery. NOSE results in less postoperative wound pain and a shorter hospital stay than conventional surgery. Larger studies are needed.

The relationship between parental genetic or phenotypic divergence and progeny variation in the maize nested association mapping population.

Appropriate selection of parents for the development of mapping populations is pivotal to maximizing the power of quantitative trait loci detection. Trait genotypic variation within a family is indicative of the family's informativeness for genetic studies. Accurate prediction of the most useful parental combinations within a species would help guide quantitative genetics studies. We tested the reliability of genotypic and phenotypic distance estimators between pairs of maize inbred lines to predict genotypic variation for quantitative traits within families derived from biparental crosses. We developed 25 families composed of ~200 random recombinant inbred lines each from crosses between a common reference parent inbred, B73, and 25 diverse maize inbreds. Parents and families were evaluated for 19 quantitative traits across up to 11 environments. Genetic distances (GDs) among parents were estimated with 44 simple sequence repeat and 2303 single-nucleotide polymorphism markers. GDs among parents had no predictive value for progeny variation, which is most likely due to the choice of neutral markers. In contrast, we observed for about half of the traits measured a positive correlation between phenotypic parental distances and within-family genetic variance estimates. Consequently, the choice of promising segregating populations can be based on selecting phenotypically diverse parents. These results are congruent with models of genetic architecture that posit numerous genes affecting quantitative traits, each segregating for allelic series, with dispersal of allelic effects across diverse genetic material. This architecture, common to many quantitative traits in maize, limits the predictive value of parental genotypic or phenotypic values on progeny variance.

Congenital varicella syndrome as an unusual cause of congenital malformation: report of one case.

Intrauterine infections with varicella-zoster virus following maternal varicella in early pregnancy and resulting in congenital malformations are rare. Herein we report a child with congenital varicella syndrome characterized by low birth weight, cicatricial scarring, hypoplasia of both lower extremities with joint contracture, congenital hip dislocation, corneal opacity, atresia of the sigmoid colon and a rarely associated cloaca anomaly. The varicella IgG remained positive after she was seven months old. Her mother developed chickenpox at the 14th week of gestation. The purpose of this article is to raise pediatricians' index of suspicion for congenital varicella syndrome when an infant is born with multiple congenital malformations with an apparent history of maternal varicella infection.

Comparison of hemocytometer leukocyte counts and standard urinalyses for predicting urinary tract infections in febrile infants.

OBJECTIVES: To compare the accuracy of standard and hemocytometer white blood cell (WBC) counts and urinalyses for predicting urinary tract infection (UTI) in febrile infants. METHODS: Enrolled were 230 febrile infants < 12 months of age. All urine specimens were obtained by suprapubic bladder aspiration and microscopically analyzed by the standard urinalysis (UA) and by hemocytometer WBC counts simultaneously, and quantitative urine cultures were performed. Receiver-operating characteristic (ROC) curves were constructed for each method of UA. The optimal cutoff point of the UA test in predicting UTI was determined by ROC analysis. RESULTS: There were 37 positive urine cultures of at least 1,000 CFU/ml. Of these 37 patients, 9 females and 28 males, 1 had a positive blood culture (Escherichia coli). Thirty (81%) of the positive urine cultures had a bacterial colony count > or = 100,000 colony-forming units/ml, whereas the remaining had between 1,000 and 50,000 colony-forming units/ml. The area under the ROC curve for standard UA was 0.790 +/- 0.053, compared with 0.900 +/- 0.039 for hemocytometer WBC counts (P < 0.05). For hemocytometer WBC counts, the presence of < or =10 WBC/microl appeared to be the most useful cutoff point, yielding a high sensitivity (83.8%) and specificity (89.6%). Standard UA, with a cutoff point of 5 WBC/high power field, had a lower sensitivity (64.9%) and similar specificity (88.1%). The hemocytometer WBC counts showed significantly greater sensitivity and positive predictive value (83.8 and 60.8%, respectively) than the standard urinalysis (64.9 and 51.1%, respectively) (P < 0.05). The accuracy, specificity and likelihood ratio of hemocytometer WBC counts were also greater than that of standard UA (88.7, 89.6 and 8.08% vs. 84.3, 88.1 and 5.44%). CONCLUSION: Hemocytometer WBC counts provide more valid and precise prediction of UTI in febrile infants than standard UA. The presence of > or =10 WBC/microl in suprapubic aspiration specimens is the optimum cutoff value for identifying febrile infants for whom urine culture is warranted.

Urinary tract infection in febrile infants younger than eight weeks of Age.

OBJECTIVE: To assess the usefulness of laboratory parameters, including peripheral white blood cell (WBC) count, C-reactive protein (CRP) concentration, erythrocyte sedimentation rate (ESR), and microscopic urinalysis (UA), for identifying febrile infants younger than 8 weeks of age at risk for urinary tract infection (UTI), and comparison of standard UA and hemocytometer WBC counts for predicting the presence of UTI. METHODS: A total of 162 febrile children <8 weeks of age were enrolled in this prospective study. All underwent clinical evaluation and laboratory investigation, including WBC count and differential; ESR; CRP; blood culture; a lumbar puncture for cell count and differential, glucose level, protein level, Gram stain, and culture; and a UA and urine culture. All urine specimens were obtained by suprapubic aspiration and microscopically analyzed with standard UA as well as with hemocytometer WBC counts. Quantitative urine cultures were performed. Sensitivity, specificity, accuracy, likelihood ratios, and receiver operating characteristic (ROC) curves were determined for each of the screening tests. RESULTS: There were 22 positive urine culture results of at least 100 colony-forming unit/mL. Eighteen of these 22 patients were males, and all were uncircumcised. There were significant differences for pyuria >/=5 WBCs/hpf, pyuria >/=10 WBC/microL, CRP >20 mg/L, and ESR >30 mm/hour between culture-positive and culture-negative groups (P <.05). The ROC area for hemocytometer WBC count, standard UA, peripheral WBC count, ESR, and CRP concentration were.909 +/-.045,.791 +/-.065,.544 +/-.074,. 787 +/-.060, and.822 +/-.036, respectively. The ROC curve analysis indicates that the CRP, ESR, and standard UA were powerful but imperfect tools with which to discriminate for UTI in potentially infected neonates. Hemocytometer WBC counts had the highest sensitivity, specificity, accuracy, and likelihood ratios for identifying very young infants with positive urine culture results. For all assessments, hemocytometer WBC counts were significantly different, compared with the standard urinalysis. ESR, CRP, and peripheral WBC counts were not helpful in identifying UTI in febrile infants. CONCLUSION: UTI had a prevalence of 13.6% in febrile infants <8 weeks of age. The CRP, ESR, and standard UA were imperfect tools in discriminating for UTI, and the sensitivity of these laboratory parameters was relatively low. Hemocytometer WBC count was a significantly better predictor of UTI in febrile infants.

Thyroid function in the sick very low-birth-weight infants.

This study was undertaken to determine postnatal changes in thyroid function in very low-birth-weight (VLBW) infants. A total of one-hundred VLBW infants participated. Serial examination of serum levels of thyroxine (T4), free T4, triiodothyronine (T3), and thyroid-stimulating hormone (TSH) was performed in the neonatal period. A total of eighty-nine infants survived to discharge, while eleven died during hospitalization. Transient hypothyroxinemia was found in forty-one (46.1 percent) of the survivors. One of the surviving infants had primary hypothyroidism. His data was excluded from the analysis. In the other eighty-eight surviving infants, TSH levels were within normal limits throughout the six-week study period. T4 and free T4 values decreased after the first day of life, reaching a nadir at one week of age, followed by progressive increases. The mean cord blood T3 level was very low; the serum T3 value increased progressively in the postnatal period. We found a correlation between low T4 and free T4 values and mortality and neonatal illness. Hypothyroxinemia was associated with critical illness. In conclusion, the postnatal changes in thyroid function in VLBW infants were characteristic, with transient hypothyroxinemia being common in these infants. Further investigation of the relationship between thyroid function, death, neonatal illness, and developmental outcome is warranted.

Neonatal vallecular cyst: report of eleven cases.

BACKGROUND: Vallecular cyst is fairly uncommon in neonates and infants. Although benign in nature, it may cause severe airway obstruction and even death. This study retrospectively analyzed the clinical manifestations of vallecular cyst in neonates and discussed its management. METHODS: From June 1993 through January 1997, 11 cases of vallecular cyst were collected and reviewed retrospectively. There were 8 male infants and 3 female infants. Their clinical manifestations, age at the onset of symptoms, age at diagnosis, and surgical management were analyzed. Fibrolaryngoscopy was used for preoperative diagnosis and postoperative follow-up. RESULTS: The infants' initial presentations were inspiratory stridor, respiratory distress, noisy breathing, feeding difficulty, and failure to thrive. There was a high incidence of patients with coexisting signs of laryngomalacia (10/11). Ten patients received laryngomicrosurgery with CO2 laser for deroofing the cyst. Additional supraglottoplasty was performed at the same time in 4 patients with laryngomalacia type A + C and in one patient with severe laryngomalacia type C. Their symptoms all resolved soon after surgery. The phenomenon of laryngomalacia also disappeared. There has been no recurrence up to the present. CONCLUSION: Although fairly uncommon, vallecular cyst should be included in the differential diagnosis of congenital laryngeal stridor in neonates. The use of fibroendoscopy can promptly diagnose vallecular cyst and any synchronous airway lesions. Although most of the synchronous laryngomalacia (type C) in this study was secondary to vallecular cyst, we suggest that supraglottoplasty be taken into consideration during cyst deroofing when the signs and symptoms of laryngomalacia type A are also present.

Home oxygen therapy for chronic lung disease in very low-birth-weight infants.

Prolonged hospitalization in infants suffering from chronic lung disease who require continuous oxygen therapy can be avoided by oxygen administration at home. In the period from August 1995 to September 1996, 155 very low-birth-weight (VLBW) infants were consecutively admitted to the neonatal intensive care unit of Mackay Memorial Hospital. Of the 155 infants, 72% (111/155) survived to discharge. However, 34% (38/111) of the survivors developed chronic lung disease. Twenty-three infants with chronic lung disease underwent home oxygen therapy after 107.0 +/- 43.6 days of hospitalization. The mean duration of home oxygen therapy was 4.3 +/- 3.4 months. In the first year after discharge, 91% of the patients required rehospitalization. One patient died during the fourth hospitalization. Follow-up information on growth and development at one year of corrected age was available for 19 patients. Five of the 19 patients had a body weight below the 5th percentile. Five of the 19 infants were mentally retarded and 12 of the 19 patients had significantly delayed motor development. In conclusion, carefully supervised home oxygen therapy permits safe early discharge of selected VLBW infants with chronic lung disease. Their somatic and psychomotor development should be carefully followed up.

Atrial flutter in the neonate and early infancy.

Atrial flutter is a rare arrhythmia in the neonate and early infancy. We retrospectively reviewed the clinical presentations, treatment and outcome of seven patients who presented clinically with atrial flutter. The age of onset ranged from 1 day to 3 months. Atrial flutter was diagnosed in the first 3 days of life in 4. Three cases presented as atrial flutter with 2:1 atrioventricular conduction and the remaining 4 with variable AV block. Heart failure was present in 3 patients and 6 patients showed normal intracardiac structure on echocardiography. Electrical cardioversion was attempted as the first treatment in 4 cases, followed by digoxin in three of the four. Digoxin was given as an initial therapy in 2 patients. One patient recovered spontaneously without treatment. In the 6 patients who received therapy, 5 converted to normal sinus rhythm within 2 days. The remaining patient had ventricular ectopic beats for about 4 months. Only 2 cases were maintained on oral digoxin for at least 4 months after discharge. No patient had a recurrence of atrial flutter during the follow-up period which ranged from 6 months to 7 years. We conclude that there is a good long-term prognosis for atrial flutter in the neonate. Digoxin and DC cardioversion may be effective as initial therapy. Long-term digoxin prophylaxis after conversion to sinus rhythm may be not necessary.

Prenatal and neonatal intussusception.

Intussusception found in the 1st month of life is rare and usually discussed as one entity, neonatal intussusception, but in fact, includes the intussusceptions occurring both prenatally and neonatally, of which the clinical presentations and results are different. Four full-term babies with prenatal intussusception presenting as intestinal atresia (IA) and three premature babies with neonatal intussusception mimicking necrotizing enterocolitis (NEC) are presented. Prenatal intussusception, as one of the causes of IA produces prominent signs of intestinal obstruction immediately after birth. Preoperative evaluation usually fails to yield a definitive diagnosis, but surgery is usually performed in time and is successful. In neonatal intussusception, full-term infants usually have a pathological lead point and the colon is almost always involved. A barium enema is thus useful in diagnosis. Premature babies, on the other hand, rarely have a colonic component, and the clinical features are insidious and similar to NEC. This results in diagnostic confusion that may lead to a dangerous delay in appropriate surgical correction. A high level of suspicion about this condition in cases diagnosed presumptively with NEC is important. Serial abdominal sonograms may be helpful in the early diagnosis of neonatal intussusception.

Frequency of associated anomalies in congenital hypoplasia of depressor anguli oris muscle: a study of 50 patients.

Aside from congenital heart disease, anomalies associated with unilateral hypoplasia of the depressor anguli oris muscle have not been well-documented in large series. We evaluated the associated anomalies in 50 infants or children with this disorder (male:female = 2:1) and found accompanying anomalies in 35 (70%) of 50 cases. They included anomalies of the head and neck (48%), heart (44%), skeleton (22%), genitourinary tract (24%), central nervous system (10%), gastrointestinal tract (6%), and miscellaneous minor anomalies (8%). Nearly half of our cases (22/50) had at least 2 associated systemic anomalies. Failure to thrive and psychomotor retardation were found in 5 (10%) and 3 (6%) patients, respectively, on follow-up. Three infants died neonatally of severe heart disorders, and the other one died of central nervous system anomalies. The above findings indicate that a thorough search for associated anomalies, particularly in the cardiovascular system, should be performed in all newborns with asymmetric crying face.

Congenital rickets--a patient report.

We present a premature newborn with congenital rickets, born to a mother with untreated chronic renal insufficiency. X-ray films showed blurred metaphyseal ends and decreased bone density in the femurs and ribs. With treatment including calcium, phosphate, and vitamin D, her rickets healed and she grew normally.

Clinical experience with early enteral feeding in very-low-birth-weight infants.

The primary objective of this study was to evaluate the safety and benefit of early enteral feeding in very-low-birth-weight (VLBW) infants without parenteral nutrition. Weight gain, feeding intolerance, nosocomial infection rate and a postnatal growth curve were recorded for 61 VLBW premature infants who were admitted to the Neonatal Intensive Care Unit of Mackay Memorial Hospital from September 1, 1995 to February 28, 1997. Nine infants were unable to complete the study and three were excluded because of severe bronchopulmonary dysplasia; therefore only 49 infants could be evaluated. They were divided into two groups based on birth weight: 1001 gm to 1250 gm (Group A, mean birth weight 1153 +/- 64 gm, mean gestational age 29.0 +/- 2.2 weeks), and less than or equal to 1000 gm (Group B, mean birth weight 911 +/- 82 gm, mean gestational age 27.1 +/- 1.5 weeks). They received breast milk or premature formula by intermittent nasogastric or continuous nasogastric feeding. Growth was followed over the first 30 postnatal days. Group A reached 100 kcal/kg/day of enteral feeding at a mean age of 17 days as compared with a mean age of 20 days for group B. Infants regained their birth weight at 20 and 25 days in Groups A and B, respectively. By the 30th postnatal day, weight gain exceeded birth weight by 218.2 +/- 143.1 gm and 95.3 +/- 81.5 gm in groups A and B respectively. No definite episodes of necrotizing enterocolitis (NEC) developed. Two cases of Escherichia coli sepsis and one of Klebsiella sepsis occurred. The conclusion was that early enteral feeding in very-low-birth-weight infants does not increase the risk of NEC. It was also demonstrated that enteral feeding alone can produce biphasic postnatal growth curves in very-low-birth-weight infants. Although early enteral feeding was well tolerated in the study infants, the occurrence of feeding intolerance in some (36%) would suggest that additional parenteral nutrition may benefit some infants until full enteral feeding can be achieved.

Cutis marmorata telangiectatica congenita associated with an elevated maternal serum human chorionic gonadotrophin level and transitory isolated fetal ascites.

A 34-year-old woman with an abnormal maternal serum screening result and a Down syndrome risk of 1:60 calculated from a maternal serum alpha-fetoprotein (AFP) value of 1.4 multiples of the median (MoM) and a human chorionic gonadotrophin (hCG) level of 4.32 MoM at 18 weeks' gestation was found to have isolated fetal ascites at 23 weeks' gestation. Spontaneous resolution occurred 10 weeks after the initial presentation. After birth, the neonate had generalized cutis marmorata telangiectatica congenita (CMTC), large vascular plaques on the scalp with superficial ulceration and crusts, a small atrial septal defect, a patent ductus arteriosus, hepatomegaly, micrognathia, seizures, an abnormal electroencephalogram, congenital retinal detachment, glaucoma and widely spaced toes. Our patient illustrates that CMTC in utero may be associated with a markedly elevated maternal serum hCG level as well as transitory isolated fetal ascites. However, such associations can be coincidental and further collaborative studies and cases will be necessary before it can be determined that a disproportionately elevated hCG level and transitory isolated fetal ascites are predictive of CMTC in utero.

Cutis marmorata telangiectatica congenita with cerebral and ophthalmic anomalies: report of one case.

A term male neonate had an uncommon congenital vascular disorder of the skin covering the whole body and extremities. These skin lesions were characterized by mottling and persistent telangiectasia. A skin biopsy showed nevus vascularis reticularis which was recognized as cutis marmorata telangiectatica congenita. In addition to the cutaneous involvement, the patient also had fetal ascites, pleural effusion, patent ductus arteriosus, glaucoma, retinal detachment in the left eye, and telangiectasia in the right eye. Neonatal focal seizure developed and expired on the 34th day of life. The cerebral image studies suggested vascular anomalies with secondary cerebral parenchymal damage. Cutis marmorata telangiectatica congenita may be solitary but is frequently associated with other developmental defects. However, the specific findings of cerebral and ophthalmic lesions in our case were rather unusual.

Pancreatitis in children--experience with 43 cases.

UNLABELLED: Pancreatitis in children is not common and can be associated with severe morbidity and mortality. We encountered 43 children, ranging in age from 2 to 18 years, with pancreatitis over the past 10 years. The diagnosis of pancreatitis was made in those patients who showed: (1) significant intra-operative pathology or; (2) clinical findings of pancreatic inflammation and laboratory confirmation. More than one third (16 cases) of the cases were due to trauma, other causes included systemic disease (10), structural disease (8), and toxins or drugs (4). Five cases were classified as idiopathic. Most of the patients presented with abdominal pain (95%) and vomiting (56%). Jaundice was found in 7 patients and an abdominal mass in 2. Morbidity included pseudocyst (10), relapse (4), hyperglycaemia (4) and miscellaneous problems. Eight (50%) of the patients with trauma and 6 (86%) of the patients with structural diseases required surgery. Other patients were managed conservatively with bowel rest, gastric decompression, intravenous fluid and total parenteral nutrition. One case had a fatal outcome. All the survivors did well in long term follow up. Relevant literature has been reviewed and the sensitivity of various diagnostic modalities compared and discussed. A lesser known association between pancreatitis and structural anomalies such as choledochal cyst is discussed. To our knowledge, the present review is the first on pancreatitis in Chinese children. CONCLUSION: Pancreatitis can occur from a wide variety of causes and may result in severe complications. Early diagnosis, close monitoring and aggressive intervention are mandatory to reduce morbidity and mortality.

The VATER association: analysis of forty six cases without karyotyping.

Forty-six patients with two or more features of the VATER association admitted to the Mackay Memorial Hospital from May, 1983 to Mar, 1992 were retrospectively enrolled in this study. We compared the incidence of major features with that reported in the literature. Imperforate anus, congenital heart disease, and renal anomalies were the three most common major features in our study. Thirteen patients died. Heart failure was the major cause of mortality. We noted many patients associated minor features, and among them there was a relatively high ratio of cleft lip, cleft palate, and hypospadius. The overall outcome and development were good among the survivals. We suggest that children who have any congenital anomaly included in the VATER association should get a careful examination and evaluation of their heart, genitourinary tract, limbs and vertebrae. Early correction if possible is indicated in such patients.

Urinary tract infection in infants less than 2 months of age.

Ninety-five infants, less than 2 months of age, diagnosed as urinary tract infections, from July 1984 to June 1991, were reviewed. Their urinary cultures, obtained either by suprapubic puncture or via catheterization, all had bacterial colony counts of over 10(5)/ml. In this survey, males predominated (91.6%). Fever and gastrointestinal problems were the two most prevalent signs. E. coli was the most common causative organism, and gentamicin was the most effective antibiotic. Vesicoureteral reflux (VUR), the most common anomaly, was found in one-third (25/76) of patients on voiding cystourethrography, with 20% being high grade (Gr. IV or Gr. V). Eleven cases (11%) had bacteremia, and one case had bacterial meningitis. Sixty-seven cases were followed up in our hospital and seven of them had second infections within a year of their first UTI. The mean period between episodes was less than two months. All these patients had urinary tract anomalies and received oral chemoprophylactic drugs for variable lengths of time. Five of the seven recurrences were caused by resistant bacilli. Continuous oral antibiotic prophylaxis and regular follow-up examinations were the rules of prevention for further infection and future renal impairment. These preventive methods are especially important in young infants with UTI.