GlutaricAciduriatypeII,isaninbornerrorofmetabolism,autosomalrecessivedueto

defectsofmitochondrial
electrontransportchainfromdehydrogenationreactioncatalizedbyMCAD,SCAD,LCADand
VLCADaswellasGlutarylCoADehydrogenase.
Thesedeficiencyproduceillnessinnewbornperiodcharacterizedbyacidosis,hypoglycaemia,
coma,hypotoniaandcardiomyopathy
Someaffectedneonateshavehadfacialdysmophicfeatures,polycystickidneys.
CaseReport
GlutaricAciduriaTypeIIMultipleAcylCoADehydrogenation
Deficiencies
*NabilBarqouni
Diagnosis:
CaseReport:
Canbemadefromurinaryorganicacidfattyacid
oxidationblock(Ethylmalonateanddicarboxylicacid)lysine(glutarate)andbranchchainaminoacids
Most severlyaffectedinfants havenot survivedtheneonatal periodSecondarycarnitine deficiencyis
present.Threeweeksoldfemaleneonate,productoffullterm,NSVD,inprivateclinicB.Wt2.8kgof
multigravidamotherandcansangionousparents.ShewasadmittedinSCBUinEGHwithintractable
diarrhea,perianalskinpealing,nofever,novomiting.Clinicalexamination,shewasdehydrated,acidotic
breathing,loosingwt2.4kgperianalmacerateddermatitis.
Familyhistoryrevealedtwoinfantswereexpiredwithsimilarproblem(boy,girl)at(47months)butno
medicalreportorfinaldiagnosis.
BabywassuspectedwithInbornErrorOfMetabolism(IEOM)
Initiallabinvestigationshowedmetabolicacidosis,hypoglycemia,normalammonia,bloodculturewas
negative.
Blood urine for Aminoacid, organic acid chromatography were done and blood zinc level, and skin
culturefibroblast.
InvestigationwererevealedOrganicacidsprofileshowedraisedlevelsofEthylmalonicacid
507mmol/molcrn(N<14.6),Methylsuccinicacid40,(N<8.8),Malic361,(N<38),Glutaricacid46,
(N<5.3)
2Hydroxyglutaricacid110,(N<69.5),Adipicacid102,(N<34),Fumaricacid93,(N<14).Skinculture
fibroblast confirmed diagnosis. These result indicates metabolic disease of mitochondrial disorder,
glutaricaciduriatypeIIorSCAD.
BabywasgiveninSCBU,I.V.Fluids,antibiotic,bicarbonate,Lcarnitine,Multivitamin.
ThebabywasimprovinganddischargefromSCBUandfollowupinOPDclinic.
At3monthsoldadmittedwithgeneralizedoedema,oliguria,respiratorydistress.
Clinicalexaminationrevealedsevereanasarca,ascitis,admittedinPICU
Investigationrevealedhypoalbuminemiaandgrossproteinuria,hypercholesterolaemia,hypocalcaemia.
Infanttreatedascongenitalnephroticsyndromewithmethylprednison,I.V.humanalbumin,mechanical
ventilation,captopril,diureticsbutpatientdeterioratedandexpiredwithin10days.
Inborn error of metabolism IEOM is an autosomal recessive was noted increasing in Palestinian
populationmostlikelyduetoconsanguinitywhichincreasedinruralareasespeciallynorthandsoutharea
inGaza.
TheincidenceofIEOMisveryrareespeciallyglutaricAciduriatypeII,thiscasewassubmitteddueto
rarediseaseanddiagnosedwasconfirmedbybloodandurineandorganicacidchromatographyandskin
culturefibroblastweredoneinShebaMedicalcenter,ourpatientalsohadcongenitalnephroticsyndrome
most likely (Finish) type, due to some genetic disease and IEOM Case was given treatment mostly
supportiveandcoenzyme,Carnitin,multivitaminriboflavin,Methylpredison,captoprilbutunfortunately
mostofthesepatientswerenotsurvived.

References:
1Rudolph'sPediatrics;21thEd:15181521,2003
2Harrison'sPrinciplesOfInternalMedicine;15thEd:1046
1052,2001.
3Nelson'sTextbookOfPediatrics;17thEd:754760,2004
4Forfar&ArneilTextbookofpediatricsixthEdition2003
5PediatricClinicsofNorthAmerica;41:783818,1994
6Appletonandlangeneonatologypage328Neoatologybook
GordonB.Avery622
MBBCH(Ainshamsuniversity)
DCH(RCPI)Dublin
DCH(RCPE)Edinburgh
Jordanian+ArabandSaudiboardinpaediatric
Partograph
The Partograph is a tool that can be used by midwifery personnel to assess the progress of labor and to
identify when intervention is necessary. Studies have shown that using the partograph can be highly
effective in reducing complications from prolonged labor for the mother (postpartum hemorrhage, sepsis,
uterine rupture and its sequelae) and for the newborn (death, anoxia, infections, etc.). Nurse midwife,
medical doctor and nurse uses it. This is used to assist in making the correct decision about transfer,
Caesarean section, or other life-saving interventions.You use it when you monitor all stages of labor of all
women arriving at the maternity or health facility.

A partograph must be started only when a women is in labor. In the latent phase (cervix dilatation not
more than 2 cm), she should have two or more contractions in 10 minutes, each lasting 20 seconds or
more. In the active phase (cervix dilatation more than 3 cm), she should have one or more contractions in
10 minutes, each lasting 20 seconds or more.

The partograph is used to plot the following parameters for the progress of labor: cervical dilatation,
descent of fetal head, and uterine contractions. It will also be used for monitoring fetal conditions with the
following parameters: fetal heart rate, membranes and liquor and moulding of fetal skull. Additionally, the
partograph can be used to monitor maternal condition: pulse, blood pressure, temperature, urine, drugs,
IV fluids, and oxytocin.

1. Cervical dilatation:

The rate of dilatation of the cervix changes during labor, this is represented by the bold lines in the graph.

Dilatation of the cervix is measured by the diameter in cm. This is recorded with an X in the center of
the partograph, at the intersection of vertical and horizontal lines. The vertical scale represents dilatation
by 10 squares of 1 cm each. The horizontal scale represents time by 24 squares of one hour each.
When labor goes from latent to active phase, the dilatation must be plotted on the alert line. The latent
phase should normally not take longer than 8 hours. When admission takes place in the active phase, the
dilatation is immediately plotted on the alert line.

If progress is satisfactory, the plotting of the cervical dilatation will remain on or to the left of the alert
line (see graph).

2. Descent of fetal head:

Descent of the fetal head may not take place until the cervix has reached about 7 cm of dilatation. This is
measured by abdominal palpation and expressed in number of finger widths (fifths of the head) above
the pelvic brim. It is also recorded in the central part of the partograph with an "O".

Example:

Admission time was 13:00, the dilatation of the cervix was 1 cm and the head was 5/5 above the
pelvic brim.
At 17:00, the dilatation was 5 cm (active phase), and the head was 4/5 above the brim.

Labor is now in active phase. Cervical dilatation is immediately transferred to the alert line;
descent of the head and time are transferred to the vertical line intersecting the 5 cm line on the
alert line.

At 20:00, the cervix was fully dilated (10 cm), and the head was only 1/5 above the pelvic brim.
 The total length of the first stage of labor observed in the unit was 7 hours.

Reference: A joint effort of Management Sciences for Health and the United Nations
Childrens Fund;http://erc.msh.org/quality/pstools/psprtgrf.cfm ;
http://www.unfpa.org/emergencies/manual/3a2.htm
Basic Emergency Obstetric and Newborn Care (BEmONC )
or Comprehensive Emergency Obstetric and Newborn Care
(CEmONC) Supervision Checklist

This is a supervision checklist for Emergency Obstetric and Newborn Care sites to help
monitor: (1) the infrastructure and environment, (2) the equipment and material, (3) the
mananagement of antenatal care clients, patients during labor and delivery, as well as
postnatal mothers and babies, (4) family planning, and (5) postabortion care.

Reference: Malawi Ministry of Health Organization; http://www.k4health.org/toolkits/malawi-

mnh/basic-emergency-obstetric-and-newborn-care-bemonc-or-comprehensive-emergency

Improvement of the Implementation Procedures and Management Systems

for Health Facilities
Enhancement Grant of the DOH Enhancement Grant of the DOH

The Improvement of the Implementation Procedures and Management Systems for

Health Facilities
Enhancement Grant of the DOH study addresses the need to identify the difficulties
encountered by
the DOH in implementing the program for the efficient allocation of funds for
facilities across the
country. It assesses the indicators used in choosing which facilities should be
targeted for upgrading
to ensure equity in the allocation of funds. This study specifically aims to map and
examine the
rationale for the choice of facilities that will be upgraded through Health Facilities
Enhancement
Grant (HFEP). It also lays-out some policy options that can be considered to improve
equity and
efficiency in allocation of funds.

The DOH implemented the HFEP with the main goal of improving the delivery of
basic, essential and
as well as specialized health services. The project envisions revitalization of primary
health care
facilities and the rationalization of the various levels of hospitals to decongestend-
referral hospitals.1
Facilities will be upgraded to make them more responsive to the need of the
catchment area, to
provide Basic Emergency Obstetric and Newborn Care (BEmONC) and
Comprehensive Emergency
and Newborn Care (CEmONC) services to the population, and to strengthen the
health facility referral
system or network.
Specifically, the HFEP aims to upgrade priority BHSs and RHUs to provide BEmONC
services for the
reduction of maternal mortality; to upgrade government hospitals and health
facilities in provinces to
make them more responsive to the health needs of the catchment population; to
upgrade lower level
facilities to be able to accommodate nursing students and to establish gate-keeping
functions to
avoid congestion in higher level hospitals, and; to expand the services of existing
tertiary hospitals to
provide higher tertiary care and as teaching, training hospitals.

Reference: DOH Department Order no. 2008-0162 entitled, Guidelines and Procedures for the
Implementation of the Government Hospital Upgrading
Project under the CY2008 Health Facilities Enhancement Program Funds of the DOH dated 7 July 2008.