Glucose-6-phosphate dehydrogenase deficiency as a cause of neonatal jaundice in Zagazig university hospital

Jaundice is a common problem affecting 50% to 70% of term infants and more than 80% of preterms, its mechanism is multifactorial; increased production, impaired conjugation and impaired excretion of bilirubin. Glucose-6-phosphate dehydrogenase [G-6-PD] deficiency the most common red cell enzyme abnormality associated with hemolysis. It is also known to be associated with neonatal jaundice, kernicterus, and even death. Identify neonates suffering pathological unconjugated hyperbilirubinemia particularly due to G-6-PD deficiency in Zagazig University Hospital. Two hundred clinically jaundiced neonates were enrolled in this study. Their mean gestational age was 36.65 +/- 1.76 weeks, mean birth weight was 2.9 +/- 0.49 kg, mean age at time of admission was 6.5 +/- 3.72 days, 121 were males and 79 were females [representing 60.5% and 39.5% respectively]. In addition 60 age and sex matched healthy neonates served as a control group. AII neonates were subjected to history taking, clinical examination, and measurement of total and direct serum bilirubin. Upon plotting total serum bilirubin [TSB] on bilirubin nomogram 123 neonates were identified as having pathological hyperbilirubinemia and the remaining 77 neonates were excluded from the study in addition, 22 neonates were identified as having direct [conjugated] hyperbilirubinemia and were also excluded from the study. The remaining 101 [50.5%] neonates [61 males and 40 females] were diagnosed as having pathological unconjugated hyperbilirubinemia and were subjected to laboratory investigations in the form of; CBC, peripheral blood smear, reticulocytic count, direct Coombs' test, ABO and Rh blood grouping for mothers and neonates, liver function test, urine analysis, sepsis screen [C-reactive protein, total and differential leucocytic count and band cell count], serum TSH and T4, and G-6-PD enzyme assay. Results: Out of 101 neonates wfth pathological unconjugated hyperbilirubinemia G-6-PD deficiency was detected in 13 [12.9%] neonates, 10 of them were males and 3 were females [representing 76.9% and 23.1% respectively]. G-6-PD deficiency is an important cause of neonatal jaundice in both males and females

Effects of chichorium intybus on bilirubin.

OBJECTIVE: To evaluate the in vitro effects of chichorium intybus on bilirubin levels. METHODS: In this study the bilirubin levels in the serum of thirty neonates staying in the NICU and suffering from clinical jaundice was determined three times: first without any alterations, second after adding chichorium intybus extract and third after adding the same amount of distilled water. The results were compared using SPSS statistical software. RESULTS: Of the patients evaluated 76.7% were male and 23.3% were female. The average age was 7.35 days. The mean difference observed in unconjugated bilirubin levels in the specimens containing chichorium intybus extract was 3.84 +/- 2.38 mg/dl and in the ones containing distilled water was 2.85 +/- 2.00 mg/dl. The mean difference observed in conjugated bilirubin levels was 0.29 +/- 0.32 mg/dl in specimens containing distilled water and 0.35 +/- 0.26 in the ones containing chichorium intybus. In none of the cases the results were found to be significant. CONCLUSION: In this study chichorium intybus was found to have no significant in vitro effect on the bilirubin level reported by the laboratory. The in vivo effects of this herbal medicine must be evaluated more closely.

Diagnostic evaluation of infantile cholestasis.

OBJECTIVE: To evaluate diagnostic accuracy of some important clinical manifestations and different investigations in infantile cholestasis. MATERIAL AND METHOD: Infants diagnosed with prolong conjugated hyperbilirubinemia and admitted to Chiang Mai University Hospital between Jan 1999 and Feb 2003. Demographic and clinical data were recorded Routine biochemical tests, and serology for TORCHS infections were carried out. An abdominal ultrasonography, DISIDA scan and percutaneous/open liver biopsy were performed. Hyperechoic band at the level of portal bifurcation, named triangular cord (TC) sign was blindly assessed on ultrasonography by the same radiologist. The patients were diagnosed as BA if either operative findings of atretic common bile duct/ gallbladder or evidence of bile duct obstruction demonstrated by intraoperative cholangiography was noted RESULTS: Sixty-one patients were diagnosed as BA (n = 31) and NH (n = 30) with an average age at diagnosis of 88.6 and 63.1 days respectively. Concerning clinical presentations, only the presence of acholic stool was significantly different between BA and NH (p = 0.006). The GGT level of greater than 500 IU/L was significantly found in BA (p < 0.001). The acholic stool and GGT level more than 500 IU/L were highly specific for BA at 100 and 96.6% respectively. In addition, the sensitivity and specificity of US-TC and DISIDA scan were 87.4, 100 and 89. 7, 92.0% respectively. The accuracy for diagnosis of BA were highest by DISIDA scan (96.3) followed by US-TC (86.9), GGT level of > 500 IU/L(81.0) and acholic stool (80.3) in order CONCLUSION: There was no single laboratory investigation that could precisely make a definite diagnosis of BA. The acholic stool and GGT level of higher than 500 IU/L were highly specific for BA. The TC in ultrasound is noninvasive and easily available tests when combined with acholic stool and the GGT level is suggested plan of management.

Prognostic value of direct bilirubin in neonatal hyperbilirubinemia.

OBJECTIVE: To determine the prognostic value of indirect and direct hyperbilirubinemia in neonates with jaundice. METHODS: A cohort of 92 consecutive neonates reporting with hyperbilirubinemia to a tertiary care center were followed up till well and discharged, or, till death to assess risk and rate of mortality. RESULTS: The baseline median values of total, direct and indirect bilirubin in the cohort of 21.8, 1.6 and 18.6 mg/dl, respectively, were used as cut-offs for high and low levels. Using survival analyses i.e. Kaplan-Meier plots, logrank tests and multivariate Cox proportional hazards regression models to adjust for other strong predictors such as receipt of breastfeeding, being small for gestational age (SGA) and exchange transfusion, high direct bilirubin (> or = median value of 1.6 mg/dl) was independently associated with a higher and faster mortality. CONCLUSION: This study showed that direct bilirubin has independent and additive prognostic value and due attention should be given to newborns with raised levels.

Maternal and neonatal factors affecting physiological jaundice in western U.P.

Several maternal and fetal factors are responsible for neonatal jaundice, which is a common observation in large number of newborns. However, role of these factors in causation of this condition is not well established. Fifty pregnant mothers and their fifty two newborns were studied in the present study. Mothers with complicated pregnancy or septicemia at the time of delivery were excluded. In addition newborns with congenital or chromosomal abnormalities were excluded. Serum concentrations of bilirubin of all neonates were measured on days 1, 3 and 5. It was found to be lower on day 1, with a peak at day 3. The area under serum bilirubin level-time curve (AUC) for each neonate was also calculated. Fetal sex and birth weight were not found to significantly affect the neonatal hyperbilirubinemia. Newborn of bipara mothers were found to have significantly lower (P < 0.05) serum bilirubin level on day 1 as compared to primipara mothers only but higher (P < 0.05) on day 3 as compared to either primi or multipara mothers. Yet, AUC of serum bilirubin curve was significantly higher (P < 0.05) in newborns of bipara mothers than others. Significantly (P < 0.05) higher serum bilirubin on day 1 was also observed in preterm neonates than full term ones. However, maternal haemoglobin and mode of delivery were not shown to affect the neonatal bilirubin levels in these newborns.

Early neonatal hyperbilirubinemia using first day serum bilirubin level.

OBJECTIVE: To evaluate the predictive value of total serum bilirubin (TSB) < or =6 mg/dl at 24 +/- 6 hr postnatal age in identifying near term and term infants, who do not develop hyperbilirubinemia subsequently. DESIGN: Prospective study. SETTING: Tertiary care hospital. METHODS: All healthy neonates with gestation > or =35 weeks, in absence of significant illness or Rh hemolysis were included. TSB was estimated at 24 +/- 6 hr by micromethod using spectrophotometry. Infants were followed up clinically every 12 hr till discharge and then after 48 hr. TSB level was estimated again whenever clinical suspicion of jaundice exceeded 10 mg/dl. Primary outcome was defined as presence of hyperbilirubinemia (TSB > or= 17 mg/dl) till day five of age. RESULTS: Of the 220 infants, 213 (96.8%) were followed up. All infants were exclusively breastfed. Mean age at bilirubin estimation was 24.7 +/- 1.9 hr with mean TSB of 5.9 +/- 1.8 mg/dl. Clinically detectable jaundice was present in 164 (77%) and hyperbilirubinemia occurred in 22 (10.3%) infants. A TSB level of < or = 6 mg/dl at 24 +/- 6 hr was present in 136 (63.8%) infants and only one infant developed hyperbilirubinemia subsequently (probability < 1%). In the remaining 77 (36.1%) infants, with TSB >6 mg/dl, subsequent hyperbilirubinemia developed in 21 (27.2%) (sensitivity 95%, specificity 70.6%, positive predictive value 27.2%, negative predictive value 99.3%, likelihood ratio of positive test 3.23 and likelihood ratio of negative test 0.07). Babies with TSB levels higher than 6 mg/dl had a significant risk of developing hyperbilirubinemia (relative risk 38; 95% confidence interval 6-1675). CONCLUSION: A TSB level of < or = 6 mg/dl at 24 +/- 6 hr of life predicted neonates who would not develop hyperbilirubinemia.

High dose intravenous immunoglobulin therapy in neonatal immune hemolytic jaundice

Isoimmune hemolytic jaundice due to blood group [ABO] and Rhesus [Rh] incompatibility is an important problem in the neonatal period. A controlled study was conducted to assess the role of high dose intravenous immunoglobulin [HDIVIG] therapy in neonatal immune hemolytic jaundice. Newborn patients with ABO and/or Rh incompatibilities proved by significant hyperbilirubinemia [>15mg/dl], positive direct Coombs' test and high reticulocytic count [>6%] were randomly assigned to receive either conventional treatment measures alone, including phototherapy and exchange transfusion if needed [control group, n=10 newborns] or phototherapy with high dose i.v. immunoglobulin [1gm/kg] over 4 hours [study group, n=30 newborns] as soon as the diagnosis was established. One of the 30 patients in the HDIVIG group required exchange transfusion, while this became necessary in 3 of 10 patients in the control group [p<0.001]. The duration of phototherapy and hospitalization, in terms of hours were significantly shorter in the HDIVIG group [p<0.001]. No adverse effects of HDIVIG therapy were observed. The use of HDIVIG therapy in newborns with ABO and/or Rh hemolytic disease reduces hemolysis, serum bilirubin levels, the need for blood exchange transfusion and the duration of hospitalization

A study of hepatitis B surface antigen positive patients on haemodialysis and following transplantation.

Of 1339 patients who entered the dialysis and transplantation program, 104 (7.77%) patients were HBsAg positive. On dialysis, 15 of 104 patients (14.42%) developed icteric hepatitis (serum bilirubin > 2 mg/d1) and 7 patients (6.73%) developed anicteric hepatitis (SGOT > twice the upper limit of normal--normal 5-40 IU); one patient died of hepatic failure. Sixty five patients underwent live related transplantation. Actuarial graft survival at the end of 1 year, 2 years and 6 years were 81.67%, 73.98% and 69.36% respectively, and there was no significant difference compared to the negative group. 8 grafts (12.31%) were lost due to patient death due to infection in the HBsAg positive group compared to 27 out of 390 (6.923%) HBsAg negative patients (x2 = 1.88 P > 0.1). Post transplantation hepatic dysfunction was seen in 7 out of 65 (10.77%) patients and two patients died of hepatic failure.

Exanguinotransfusión: riesgo evitable / Exanguinotransfusion: avoidable risk

En la Maternidad Concepción Palacios realizamos este estudio prospectivo durante los meses de noviembre de 1992 hasta abril de 1993, con el objeto de verificar la hipótesis de que los neonatos sometidos a exanguinotransfusión tienen alto riesgo de morbi-mortalidad, el cual pudiera ser minimizado si formalizamos la indicación de este procedimiento. Se evaluaron todas las exanguinotransfusiones solicitadas durante este período de tiempo y encontramos que la principal indicación fue la hiperbilirrubinemia, en un 82,35 por ciento de las veces, de las cuales el 15,11 por ciento fueron de origen inmunológico. La edad de apariciómn promedio de la ictericia fue a las 27 horas de vida, con un valor de bilirrubina total indicativo del procedimiento de 17,6 mg por ciento . Las exanguinos se realizaron entre las 6 horas y los 15 días de vida con un promedio de 72 horas. Registramos una mortalidad del 20,29 por ciento , la cual es estadísticamente significante mayor que la mortalidad neonatal institucional ocurrida en nuestro hospital durante el lapso estudiado (p<0,01). El grupo de los neonatos pretérmino pequeños para su edad gestacional fue el grupo más afectado (p<0,01). Observamos también una asociación significante en cuanto a la realización de exanguinotransfusiones en el grupo de los neonatos menores de 2500 g(p<0,01). La complicación más frecuente del procedimiento fue la sepsis en el 21,95 por ciento de las veces, y los gérmenes señalados con más frecuencia fueron Klebsiella sp, y Cándida albicans. Notamos que existe premura en la indicación y posterior realización de este riesgoso procedimiento y sugerimos que un juicio integral del paciente y no sólo valores aislados de bilirrubina deben ser tomados en cuenta a la hora de realizar una exanguinotransfusión

Evaluación de los índices de flujos sanguíneos maternos y fetales en la colestasia intrahepática del embarazo / Evaluation of maternal and fetal blood flow indexes in pregnancy intrahepatic cholestasis

Nuestro objetivo fue observar si existen alteraciones de los índices de flujo sanguíneo en pacientes portadoras de una colestasia intrahepática del embarazo. Se estudiaron 51 pacientes gestantes de tercer trimestre, las que se dividieron en; grupo normal n=15, grupo con colestasia intrahepática del embarazo n=36; 21 pacientes anictéricas y 15 pacientes ictéricas, con bilirrubinemia >1,2 mg por ciento En todas se determinaron los índices de flujo en las arterias uterina, umbilicales y cerebrales fetales. No existieron diferencias significativas en la paridad, edad materna, edad gestacional, peso de nacimiento, ni apgar de los recién nacidos. Tampoco se apreciaron diferencias significativas en ninguno de los índices de flujo en los territorios mencionados. Concluimos que las pacientes portadoras de una colestasia intrahepática no presentan alteraciones de los índices de flujo evidenciables por medio de la técnica del doppler

Exchange transfusions via peripheral vessels.

During a 15 month period, partial exchange transfusions (ET) were done in 40 neonates with polycythemia, and double volume ET attempted in 7 neonates with hyperbilirubinemia via peripheral vessels. The procedure was effective and not associated with any complications for partial ET. During double volume ET minor complications were noted in 2 cases, both of whom recovered and subsequently successfully underwent supraumbilical ET. Of the 5 cases who had uneventful double volume exchanges, there was a significant drop in indirect serum bilirubin following the procedure. The mean pre-ET serum indirect bilirubin in these 5 cases was 334 mumol/L and mean post-ET level was 179 mumol/L with a mean drop of 155 mumol/L (46% drop). Technical difficulties in catheterization may be overcome with greater expertise and use of heparin to flush arterial catheters.

Spectrum of neonatal hyperbilirubinemia: an analysis of 454 cases.

A prospective study of 454 newborn babies with pathological hyperbilirubinemia revealed that in about one-third of cases (34.6%), no cause could be identified despite detailed investigations. Nearly three-fifth of infants (62.5%) had hyperbilirubinemia due to hemolytic causes. On the basis of four variables, i.e., peak serum bilirubin level, age of attaining the peak level, age of starting phototherapy and total duration of phototherapy, the cases of hyperbilirubinemia can be categorized into three groups: (a) Group I (mild) included non-hemolytic hyperbilirubinemia, i.e., idiopathic, bacterial infections, intrauterine infections and others, (b) Group II (moderate) comprised of hemolytic as well as non-hemolytic hyperbilirubinemia due to prematurity, administration of oxytocin, bruising/cephalhematoma, and (c) Group III (severe) comprised of hyperbilirubinemia due to hemolysis as a result of blood group incompatibility between the mother and the neonate and G-6-PD deficiency. Sixty six babies required exchange blood transfusion (EBT) and a total of 100 EBTs were performed. Most of the babies (80.3%) requiring exchange blood transfusion belonged to Group III. The most common cause of hemolytic hyperbilirubinemia needing exchange blood transfusion was Rh isoimmunization followed by G-6-PD deficiency and ABO isoimmunization. There was no death attributable to the procedure of exchange blood transfusion.

Deficiencia de gammaglutamil transpeptidasa en niños con hiperbilirrubinemia directa / Gammaglutamyl transpeptidase deficiency in children with direct hyperbilirrubinemia

Se presentan breves resúmenes clínicos de cinco casos de niños con hiperbilirrubinemia directa en quienes pudo comprobrarse deficiencia de gammaglutamil transpeptidasa, enfatizando en síntomas y signos clínicos - algunos de ellos rebeldes al tratamiento, como ictericia y prurito - y en los datos suministrados por laboratorio y biopsia de hígado; se concluye que como otros autores también lo aseguran, el descubrimiento de bajos niveles de gammaglutamil transpeptidasa en niños con estas características debe estimarse como indicador de mal pronóstico e incluso requerir de manera ocasional de transplante de hígado como aconteció en dos de los casos de la serie estudiada