This document discusses the approach to a case of neonatal liver failure. It begins by describing the case presentation and initial workup. Key points include that metabolic diseases are a common cause of neonatal liver failure. The document then discusses priorities in management, which include stabilizing life-threatening issues and starting investigations. Common causes like galactosemia, tyrosinemia, and HSV hepatitis are discussed. Newer concepts in management like N-acetylcysteine are also covered. Finally, the case is summarized as mitochondrial DNA depletion syndrome caused by a novel mutation in the DGUOK gene.
pediatrics emergency, hypoglycemia of infancy.
Glucose level can drop if:
There is too much insulin in the blood (hyperinsulinism). Insulin is a hormone that pulls glucose from the blood.
The baby is not producing enough glucose.
The baby's body is using more glucose than is being produced.
The baby is not able to feed enough to keep glucose level up.
pediatrics emergency, hypoglycemia of infancy.
Glucose level can drop if:
There is too much insulin in the blood (hyperinsulinism). Insulin is a hormone that pulls glucose from the blood.
The baby is not producing enough glucose.
The baby's body is using more glucose than is being produced.
The baby is not able to feed enough to keep glucose level up.
case presentation of hypoglycemia, Approach to hypoglycemia, pathophysiology, differential diagnosis, treatment and management, comparison and case presentation
Gestetional hypertension, Preeclampsia and Eclampsiasunil kumar daha
Please find the power point on Gestetional hypertension, Preeclampsia and Eclampsia . I tried to present it on understandable way and all the contents are reviewed by experts and from very reliable references. Thank you
Pregnancy induced hypertension introduction
Classification of pregnancy induced hypertension
Preeclampsia -
Definition
Criteria for diagnosis of preeclampsia,
Epidemiology of preeclampsia,
Risk factors of preeclampsia,
Pathogenesis of preeclampsia,
Pathophysiology of preeclampsia,
Course of preeclampsia,
Complications of preeclampsia,
What is HELLP ?
Management of preeclampsia at home, at hospital, during labour, during puerperium,
Management of acute fulminant preeclampsia
Hypercalcaemia is a common disorder we doctors from all faculties face in day to day clinical practice. This was a presentation done by me to give you an update regarding hypercalcaemia and it's management.
High blood pressure during pregnancy poses various risks, including: Decreased blood flow to the placenta. If the placenta doesn't get enough blood, your baby might receive less oxygen and fewer nutrients. This can lead to slow growth (intrauterine growth restriction), low birth weight or premature birth.
This slide contains neonatal jaundice by including real case senario and nursing management through by passing definition, pathophysiology and diagnosis modality
case presentation of hypoglycemia, Approach to hypoglycemia, pathophysiology, differential diagnosis, treatment and management, comparison and case presentation
Gestetional hypertension, Preeclampsia and Eclampsiasunil kumar daha
Please find the power point on Gestetional hypertension, Preeclampsia and Eclampsia . I tried to present it on understandable way and all the contents are reviewed by experts and from very reliable references. Thank you
Pregnancy induced hypertension introduction
Classification of pregnancy induced hypertension
Preeclampsia -
Definition
Criteria for diagnosis of preeclampsia,
Epidemiology of preeclampsia,
Risk factors of preeclampsia,
Pathogenesis of preeclampsia,
Pathophysiology of preeclampsia,
Course of preeclampsia,
Complications of preeclampsia,
What is HELLP ?
Management of preeclampsia at home, at hospital, during labour, during puerperium,
Management of acute fulminant preeclampsia
Hypercalcaemia is a common disorder we doctors from all faculties face in day to day clinical practice. This was a presentation done by me to give you an update regarding hypercalcaemia and it's management.
High blood pressure during pregnancy poses various risks, including: Decreased blood flow to the placenta. If the placenta doesn't get enough blood, your baby might receive less oxygen and fewer nutrients. This can lead to slow growth (intrauterine growth restriction), low birth weight or premature birth.
This slide contains neonatal jaundice by including real case senario and nursing management through by passing definition, pathophysiology and diagnosis modality
nausea and vomiting in pregnancy is very common. it may be a manifestation of some medical - surgical - gynecological complications. hyperemesis gravidarum is a severe type of vomiting in pregnancy which has got deleterious effects on the health of the mother. it is a very important topic and it is also a topic in obstetrics. we should encourage and help young mothers to identify the symptoms. please read it and get knowledge about nausea and vomiting in pregnancy. stay tuned.
Dr Anil Arora address the liver diseases that are specific during pregnancy. The presentation contains case discussions on diagnosis, treatments & take home messages
Wilsons disease and hepatitis dr. abhamoni baroSanjeev Kumar
Case: Prolonged acute hepatitis – is there more to it?
Presenter: Abhamoni Baro
Moderator: Ashish Bavdekar
Panelists: Prakash Vaidya, Harshad Devarbhavi, Seema Alam
Hepatomegaly with seizures and hepatitis in a family dr. rajesh kumar meenaSanjeev Kumar
Case: Hepatomegaly with seizures and hepatitis in a family
Presenter: Rajesh Kumar Meena
Moderator: Vidyut Bhatia
Panelists: Seema Alam, Alka Jadhav, Anshu Srivastava
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
New Drug Discovery and Development .....NEHA GUPTA
The "New Drug Discovery and Development" process involves the identification, design, testing, and manufacturing of novel pharmaceutical compounds with the aim of introducing new and improved treatments for various medical conditions. This comprehensive endeavor encompasses various stages, including target identification, preclinical studies, clinical trials, regulatory approval, and post-market surveillance. It involves multidisciplinary collaboration among scientists, researchers, clinicians, regulatory experts, and pharmaceutical companies to bring innovative therapies to market and address unmet medical needs.
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
HOT NEW PRODUCT! BIG SALES FAST SHIPPING NOW FROM CHINA!! EU KU DB BK substit...GL Anaacs
Contact us if you are interested:
Email / Skype : kefaya1771@gmail.com
Threema: PXHY5PDH
New BATCH Ku !!! MUCH IN DEMAND FAST SALE EVERY BATCH HAPPY GOOD EFFECT BIG BATCH !
Contact me on Threema or skype to start big business!!
Hot-sale products:
NEW HOT EUTYLONE WHITE CRYSTAL!!
5cl-adba precursor (semi finished )
5cl-adba raw materials
ADBB precursor (semi finished )
ADBB raw materials
APVP powder
5fadb/4f-adb
Jwh018 / Jwh210
Eutylone crystal
Protonitazene (hydrochloride) CAS: 119276-01-6
Flubrotizolam CAS: 57801-95-3
Metonitazene CAS: 14680-51-4
Payment terms: Western Union,MoneyGram,Bitcoin or USDT.
Deliver Time: Usually 7-15days
Shipping method: FedEx, TNT, DHL,UPS etc.Our deliveries are 100% safe, fast, reliable and discreet.
Samples will be sent for your evaluation!If you are interested in, please contact me, let's talk details.
We specializes in exporting high quality Research chemical, medical intermediate, Pharmaceutical chemicals and so on. Products are exported to USA, Canada, France, Korea, Japan,Russia, Southeast Asia and other countries.
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
Novas diretrizes da OMS para os cuidados perinatais de mais qualidade
2 neonatal liver failure
1. Approach to a case of
neonatal liver failure
Dr Meet Leuva
2nd year resident, V S hospital
Dr Manoj K Ghoda
Consultant Gastroenterologist
Ahmedabad
2. This is the story of Tanya....
..........And what she taught us
There is always more to learn
Lessons learnt
3.
4. Neonatal Liver Failure
At another hospital
• 1st child of 2degree consanguineous
parents
• IUGR
• NVD
• Hypoglycemia (sugar 35mg) onD1
Hypoglycemia soon after birth: Perinatal events, Galactosemia, GSDs, Organic
acidemia, PHHI
Hypoglycemia starting late in infancy or early childhood: FAOD, PHHI, HFI, Insulinoma,
GSDs
5. Readmitted* (at the same hospital) NNU D5
•Vomiting
•Dehydration
•“Rapid” breathing
* Lesson: Repeated admission should raise the suspicion of metabolic
disease
6. •Metabolic acidosis with pH of 7.1 and high
anion gap
•Jaundice: SBR 25 mg., mainly conjugated
•Transaminitis; ALT 600, AST 500, GGT 500
•Coagulopathy, INR 4, PT 40 sec (10.3), not
corrected by IV Vit K
•S. Albumin 2.1 , Glob 1.8
•Ammonia 375 µmol/L ( up to 180 at this age)
Anion gap = AG = [Na+] + [K+] - [Cl-] - [HCO3-].
Normal range: 8 to 16 mmol/L
Anion gap metabolic acidosis: Consider organic acidopathy, lactic acidosis, and
ketoacidosis. These are not measured in above equation and hence they produce a
“gap”
7. •Hypoglycemia, blood sugar 35 mg%
•RFTs were normal, Urea 23, creat 1.0
•CBC: Mild normochromic, normocytic anemia, Hb 11.2,
WCC 14,000, Plt 2,30,000, reticulocytes 2%
•Urine reducing substances ( non glucose) negative;
•USG: Normal size and echo texture, collapsed GB, CBD
could not be visualized, IHBR normal, portal vein and
splenic vein normal
8. Sepsis work up:
WCC 14,000 with 70% polys
CRP: 5 ( up to 1 mg/L)
Blood culture obtained for aerobic, anerobic; and fungal
culture
Urine microscopy normal, culture awaited.
CXR: NAD
9. What is your working diagnosis here?
What would be your priorities here?
10. Diagnosed as Acute Liver Failure.
Neonatal liver failure can be defined as "failure* of
the synthetic function of liver within 4 weeks
of birth".
Encephalopathy is not essential for the diagnosis.
*Uncorrectable coagulopathy with INR >1.5 in
patients with hepatic encephalopathy, or INR> 2.0 in
patients without encephalopathy.
11. Why Acute Liver Failure in neonates assume
significance?
Acute liver failure (ALF) is potentially devastating process[1–3]
It progresses fast resulting in death or transplant if not
arrested with appropriate treatment
Lesson
Once ALF it is diagnosed there is no time to loose.
13. Priorities in such cases are as follows
Simultaneously...
•Correct any immediate life threatening
complication/ events
•Start investigations to arrive at a diagnosis and
treat accordingly; or
•Arrange for a speedy and safe transport if your
centre is not geared for such emergencies
14. What are the likely causes of neonatal
liver failure?
Etiology of neonatal liver failure
In neonates and infants, metabolic diseases are the
main cause of ALF.[1,9] Amongst non-metabolic causes,
HSV is common and it is an emergency.
In older children, viruses, drug-induced hepatotoxicity
and autoimmune hepatitis are the most common
identified causes of ALF.
15. Do all causes of neonatal liver failure
have same intensity or timeframe of
presentation?
16. •Neonatal Hemochromatosis
•Zellweger syndrome
•Mitochondrial liver diseases
May be present at birth
•Tyrosinemia
•Galactosemia
•Hemophagocytic Lymphocytic syndrome
•HSV hepatitis
May take few days to weeks to manifest
Lesson: Time of presentation may provide clue as
to the underlying etiology
18. Metabolic Diseases: When should the
alarm bell ring?
if you see a child with…..
• Intermittent illness
• Recurrent unexplained vomiting.
• Failure to thrive.
• Aversion to certain food or the illness starting with
particular food.
19. When to suspect IEM ?
Or a child with….
• Rapid deterioration for no obvious reasons.
• Rapidly progressive encephalopathy of obscure
origin.
• Hypotonia, seizures, especially if hard to control.
20. When to suspect IEM ?
Or if the child has….
• Apnea or respiratory distress.
• Sepsis, particularly with E. coli
• Unusual odor.
• Jaundice.
• Dysmorphic features.
• Organomegaly.
21. •Sugar
•Urinary reducing
substances, urinary
ketones,
•Ammonia
•Urea
•Total Galactose, Galactose
phosphate/ Gal-1-T,
Metabolic tests which may be required and are
now available....
•Lactate
•Ferritin and transferrin
saturation
•Triglycerides
•Alpha- fetoprotein
•Acyl carnitine profile
•Plasma aminoacids
•Urinary organic acids
•Urinary GAG estimation
22. What basic treatment a pediatrician needs
to start irrespective of the cause if
metabolic cause is suspected?
23. The basic principles for treatment of suspected
metabolic disorders are*:
•Prevent catabolism –
•Use IV dextrose.
•Limit the intake of the offending substance - if possible, through
manipulation of the diet. Limit protein in urea cycle disorders, fatty
food in FAOD
Lesson:
Stabilize the patient without bothering to find out the cause of
illness if the patient is acutely ill.
*Hoffman and Zschocke
25. How and when to suspect HSV hepatitis?
•Could be confused with bacterial sepsis.[30,34]
•Vesicular skin lesions are the most predominant symptom
consistent with neonatal herpes.
•Later on....DIC, hepatitis, pneumonitis, and seizures.[11,29,35]
Lesson: HSV hepatitis is an emergency and
once suspected treatment is mandatory
26. Hemophagocytic lymphohistiocytosis (HLH)
•Rare but potentially fatal.
• Fever, hepatosplenomegaly, pancytopenia, lymphadenopathy
•Cutaneous involvement occurs in as many as 65% of patients.
•An inherited more prevalent with parental consanguinity
27. Non metabolic investigations of neonatal liver
failure:
Infectious :
culture from cutaneous lesions or oropharynx for HSV
Viral markers
cytomegalovirus PCR, IgM varicella zoster virus, IgM Epstein-
barr virus, HIV 1 and 2,
Hemophagocytosis
Serum triglyceride, cholesterol, ferritin and bone marrow biopsy
28. What are the new concepts in management of
Liver failure ?
29. Newer concepts in management of Liver
failure...
To prevent hepatocyte damage
•N-acetyl cysteine (NAC) infusion in non-acetaminophen
causes of ALF [20] @ 100 mg/kg/d in all cases of ALF
irrespective of the etiology.
30. •To prevent cerebral edema
•Prophylactic infusion of 3% saline to maintain sodium at 145-
155 mmol/L ....is preferred over mannitol.
•If neurological signs .. or ICP is above 25 mm Hg, a bolus of IV
mannitol (0.25-1 g/kg, 20%). Repeated if serum osmolality is
less than 320 mosmol/L.
•Hyperventilation with reduction of pCO2 to <35 mmHg.....in
patients with impending herniation where mannitol therapy
fails [23].
•No ....hypothermia, prophylactic phenytoin or corticosteroids in
the management of raised ICP in ALF
31. Sedation:
•Should be avoided (unless there is a back-up plan for
ventilation) to prevent worsening or assessment of
encephalopathy.
•If sedation is mandatory, 1-2 mg/kg of propofol can be
given.
32. Managing Hyperammonemia:
•Sodium Benzoate/ Butyrate: 250 mg/Kg in 5-10% D, loading
over 90 mins followed by 250-500mg/Kg over 24 hours.
•Arginine:
•Carnitine: Do not give Carnitine if Cardiomyopathy is suspected.
•Single oral dose of N-Carbamyl glutamate if NAGS deficiency is
suspected or in organic acidemia
•If these doesn’t work hemofiltration (CRRT) not simple dialysis
33. Coagulopathy
•No routine correction
•FFP if significant bleeding, or while performing invasive
procedure or in situations of extreme coagulopathy with INR>7.
•Cryoprecipitate ... For hypofibrinogenemia (<100 mg/dL).
•Recombinant factor VIIa is beneficial in patients with prolonged
INR despite FFP, who are volume overloaded [26].
•No Platelet transfusion unless platelet count of 10-20,000 or
significant bleeding with platelet count <50,000/mm3 [5,27].
34. Empirical antibiotics ... where infection or the likelihood of
impending sepsis is high e.g.
cultures positive,
progression of, or advanced stage (III/IV) HE,
refractory hypotension,
renal failure,
presence of SIRS (temperature >38°C or <36°C, WCC
>12,000 or <4,000/mm3, tachycardia).
•...for patients listed for liver transplantation (LT),
•A third-generation cephalosporin, vancomycin/teicoplanin, and
fluconazole are recommended.
•Fungal infections, particularly Candida albicans, in one third of
patients with ALF.
36. Coming back to our case...
Transferred to our unit
• Appeared very sick
• Conservative Rx for ALF
• Rx for NNH (IVIG) (Intrauterine growth
restriction (IUGR) occurs in 25% of infants
with neonatal hemochromatosis)
• Glucose @ 7mg/kg/min
What is
going on
here
37. Coming back to our case....
• Plasma Amino Acids
– Normal
• Organic Acids
– Lactate moderately increased (16
mMol).
– Increased TCA cycle metabolites
• Acylcarnitines – within normal
limits for neonate
38. Neonatal Liver Failure
• Further investigation for
mitochondrial disease
• MRI / MRS of Brain
– Reported structurally
normal
– Normal appearance of
basal ganglia
– Lactate peak in BG
• Muscle & Liver Bx
– Intrafibre lipid / steatosis
39. Neonatal Liver Failure
• Muscle & Liver mtDNA
– 37% mean in liver
– 45% mean in muscle
• Nuclear DNA tests
– POLG1 common
mutations negative
– Homozygous for a novel
mutation in DGUOK
• Diagnosis =
MITOCHONDRIAL DNA
DEPLETION SYNDROME
secondary to mutation
in DGUOK gene
41. Summary:
•Neonatal liver failure is an emergency
•Most of the causes are metabolic
•Galactosemia, Tyrosinemia, Neonatal
hemochromatosis, HSV hepatitis and
Hemophagocytosis are treatable and must be
actively looked for
•Familiarize your self with newer therapies
45. Pediatric ALF
(a) evidence of liver dysfunction within 8 weeks of onset of
symptoms (with or without overt symptoms)
(b) uncorrectable coagulopathy with INR >1.5 in patients with
hepatic encephalopathy, or INR> 2.0 in patients without
encephalopathy; and
(c) no evidence of chronic liver disease either at presentation or
in the past.
Vidyut Bhatia, Ashish Bavdekar and Surender Kumar Yachha for the Pediatric Gastroenterology
Chapter of Indian Academy of Pediatrics.
Management of Acute Liver Failure in Infants and Children: Consensus Statement of the Pediatric
Gastroenterology Chapter, Indian Academy of Pediatrics. Indian Pediatr 2013;50: 477-482
46. Management in the Intensive Care Unit
•A central venous line preferred.
•The fluids titrated as per requirement.
Vasoactive drugs if required.
47. Monitoring in ICU
(a) vital signs, including blood pressure every 4 hours, more frequently in an
unstable child
(b) continuous oxygen saturation monitoring
(c) neurological observations/coma grading, electrolyte, arterial blood
gases, blood sugar every 12 hourly (more frequently in an unstable
child); prothrombin time should be monitored 12 hourly till patient
stabilizes or decision to perform a transplant is taken
(d) daily measurements of liver span and prescription review
(e) liver function tests, blood urea, serum creatinine, calcium and phosphate
at least twice weekly. Surveillance of blood and urine cultures should be
done during the course of illness.
48. Electrolyte disturbances and glucose
homeostasis:
•Hyponatremia, hypokalemia, hypocalcemia, hypophosphatemia and
hypomagnesemia are commonly observed.
•Persistent hyponatremia and hypoglycemia are poor prognostic parameters.
•Patients with ALF are at an increased risk for hypoglycemia secondary to
failure of hepatic gluconeogenesis, hyperinsulinemia and secondary bacterial
infections. But remember, hyperglycemia could be equally dangerous as it
increases osmotic pressure and may be damaging to brain
•Intravenous fluids should be tailored in accordance to electrolyte, sugar and
renal status of the patient.
Editor's Notes
Tanya was admitted at another hospital and one day 1 she had hypoglycemia. Nothing much was made of her hypoglycemia and was discharged next day.
While at home Tanya was mot well at all with excessive crying and persistent vomiting and hence was readmitted.
She had high anion gap acidosis and deep conjugated hyperbilirubinemia, with transaminitis and gross synthetic function derangement; as shown here.
She had hypoglycemia but otherwise CBC and Renal functions were normal. USG was unremarkable, although CBD could not be visualized.
Sepsis work up was done. A large number of admissions with neonatal jaundice, up to 1/3 in some studies, are due to cholestasis of inflammation, i.e. Secondary to infections; which when adequately treated results in resolution of jaundice. Therefore sepsis work up is an integral part of investigations for neonatal jaundice.
Even in a short span of life, neonates behave differently to various insults and therefore presentations for various etiologies could be distinctly different.
Some metabolic diseases affect crucial liver functions and mother can’t protect the child and hence they manifest in utero with IUGR or something like that.
Other diseases are such that mother provides protection till birth and hence these metaboic derangements take time to manifest.
If you see a neonatal failure or for that matter any other disease in this age group, what makes you suspect metabolic diseases?
Now that you have hopefully stabilized the patient and have some clue as to the broad category of underlying etiology; so how will you proceed further from here?
First panel shows routine tests which have metabolic significance and except tests for Galactosemia all are universally available.
Some steps are needed immediately without proof of metabolic etiology or precise nature of the metabolic etiology to prevent damage or death
IV dextrose switches off almost all metabolism and the treatment of choice. Dextrose may worsen condition only in pyruvate carboxylase deficiency which it self appears to be very uncommon
normal but overactive histiocytes and lymphocytes that commonly appears in infancy, although it has been seen in all age groups.
Lactulose, once the main stay of treatment for hyperammonemia is out of favor now.
Management of intracranial hypertension. Cerebral edema is one of the most important cause of death; nearly 80% develop cerebral edema and one has to treat it proactively.
Usually hyperammonemia is not life threatening on its own in liver failure and sodium benzoate which is available as oral powder, will take care of it. Sod butyrate is not availble easily. Both provide alternative pathwa for ammonia detoxification.
Arginine, carnitine and N-Carbamyl glutamate are mainly required in life threatening hyperammonemia of Urea Cycle Disorders
Avoid correcting coagulopathy unless mandatory because you will loose a good marker for prognostication
No routine antibiotics unless as specified above
Because of high lactate and increased TCA/ Krebb’s cycle metabolite our focus was now on mitochondrial liver diseases. Normalacyl carnitine profile makes Faaty Acid Oxidation Disorders unlikely