Pediatrics 2

A 10-year-old boy presents with Coca-Cola–colored urine and edema of his lower extremities. On physical examination, the patient has a blood pressure of 185/100 mm Hg.He does not appear to be in any distress. His lungs are clear to auscultation, and his heart has a regular rate and rhythm without any murmurs, gallops, or rubs. His past medical history is remarkable for a sore throat that was presumed viral by his physician 2 weeks before. What is the Diagnosis and etiology of this disease?

Acute post streptococcal Glomerulonephritis

Follows infection with nephrogenic strains of group A beta-hemolytic strepto- cocci of the throat (mostly in cold weather) or skin (in warm weather). Spread through droplets.

Mention the age range involved and clinical presentation involved in this case presentation?

Most 5–12 years old (corresponds with typical age for strep throat).

• 1–2 weeks after strep pharyngitis or 3–6 weeks after skin infection (impetigo) .

• Ranges from asymptomatic microscopic hematuria to acute renal failure, oedema, hypertension, hematuria (classic triad) .( Dark urine (brown -tea-or coca coloured).

• Pharyngitis

• Periorbital edema, Generalised oedema

• Hypertension, oliguria

• Circulatory congestion e.g dyspnea

• Non specific sympptoms -general malaise, anorhexia, fever, abdominal or flank pain, nausea and vomiting.

• Unusual presentation can be Atypical HUS, Refractory hypoxic respiratory failure, Seizures and other central nervous system, Severe nephritis including acute kidney injury and heamatologic anomalies.

What are the clinical investigations you’ll request for this patient?

• Urinalysis—RBCs, RBC casts, protein 1–2 +, polymorphonuclear cells

• Mild normochromic anemia (hemodilution and low-grade hemolysis)

• Low C3 (returns to normal in 6–8 weeks)

• Need positive throat culture or increasing antibody titer to streptococcal anti- gens; best single test is the anti-DNase antigen

• For diagnosis of prior Strep infection, use streptozyme (slide agglutination), which detects antibodies to streptolysin O, DNase B, hyaluronidase, streptokinase, and nicotinamideadenine dinucleotidase.

• Consider biopsy only in presence of acute renal failure, nephrotic syndrome, absence of streptococcal or normal complement; or if present >2 months after onset

What are the complications of this disease?

Hypertension

Acute renal failure

Congestive heart failure

Electrolyte abnormalities

Acidosis

Seizures

Uremia

What are the treatment of this this disease? (in-patient, if severe)

• Antibiotics for 10 days (penicillin)

• Sodium restriction, diuresis

• Fluid and electrolyte management

• Control hypertension (calcium channel blocker, vasodilator, or angiotensin- converting enzyme inhibitor)

You are working in a Paediatric Out Patient clinic, You encountered Selma, a 12 year old girl of Asian descent. She describes a two month history of general malaise and decreased energy. Her mother states that her daughter has felt “feverish” on and off for the last 2 months. She used to play soccer regularly, but she has stopped participating not only because she feels “too run down” but she has also been having pain and stiffness in her left knee. Last week when she was out in the sun with her classmates she developed a Butterfly rash on her cheeks. What would your approach be to this patient??

What is the diagnosis of this patient and definition?

SLE is a systemic multi-system autoimmune disease characterized by the presence of autoantibodies and multiorgan system involvement.

What are the 11 criteria of this diagnosis?

M- Malar rash: this rash is a fixed erythematous rash over the malar eminences – commonly called a ‘butterfly rash’. It can be flat or raised, and tends to spare the naso- labial folds

D- Discoid rash: this is a raised, scaling and scarring rash. It is extremely rare in paediatric patients

S- Serositis: literally means ‘inflammation of a serous membrane’; most commonly involving the heart or lungs in SLE, i.e. pleuritis or pericarditis

O- Oral/Nasal ulcers – this ulceration is usually painless, and is often on the palate or nasal septum

Arthritis – this is non-erosive arthritis involving two or more peripheral joints, characterized by tenderness, decreased range of motion, and effusion.

P- Photosensitivity– rash from sun exposure (notably, sun exposure can trigger a flare of SLE and therefore sun protection is key in patients with SLE).

B – Blood abnormalities – this includes hemolytic anemia (with reticulocytosis), leukopenia, lymphopenia and thrombocytopenia.

R – Renal dysfunction – there are 6 classes of SLE nephritis and a broad range of presentation and severity, from asymptomatic to severe nephritic or nephrotic syndrome.

A – ANA positive – This is anti-nuclear antibody. Essentially all patients with SLE are ANA positive.

I – Immunologic – These are other specific antibodies, including anti-ds-DNA, anti-Smith, anticardiolipin antibodies and lupus anticoagulant.

N – Neurologic – Lupus can present with a wide array of central and/or peripheral signs and symptoms. This can be anything from headaches and difficulty concentrating to seizures and psychosis.

How will you approach history of this patient?

HPI: General description from the patient on the course of her symptoms

• Rheumatologic Review of Systems

• General: Any fever? Weight loss? Malaise?

• CNS: Any headaches? Recent difficulties with concentration,school performance, depression, memory loss, hallucinations? Seizure activity?

• Head and Neck: Very dry eyes and/or dry mouth (SICCA symptoms)? Nose bleeds or sores in your nose? Sores in your mouth? Any hair thinning or falling out?

• Cardiorespiratory: Any chest pain (worse with deep breaths)? Shortness of breath or exercise intolerance?

• GI: Any stomach pain or diarrhea (serositis in SLE can include abdominal lining)?

• GU: Any blood or coke-coloured urine? Any foamy urine? Any swelling in your feet or around your eyes (nephrotic syndrome)? Any headache or visions changes consistent with hypertension?

• MSK: Any joint pain, swelling or tenderness (associated morning stiffness?)? Any muscle weakness or pain?

• Derm: Any colour changes from white-blue-red in your hands or feet consistent with Raynauds phenomenon? Any rashes (specifically after being out in the sun)? As in all cases, a thorough Pediatric History should include: medications (some medications can trigger Drug-induced lupus), Allergies, Immunizations, developmental History, past Medical History

• Family History (often other autoimmune diseases in the family)

• Social History (including HEAADS in adolescent history)

How will you approach your physical exam in this patient?

• Vitals (tachypnea, hypertension)

• Alopecia

• Facial rash or edema

• Oral or nasal ulcers? (Make sure you look on the palate and in the nose because the patient will often not even realize that they have them.)

• Palpate for lymphadenopathy

• Thorough respiratory and cardiovascular exam

• Thorough GI exam

• Complete detailed MSK and neurological exam assessing for arthritis and weakness and other neurological features

• Exam for peripheral edema

• Thorough skin exam for rashes (malar, discoid, vasculitic, periungal erythema)

What is your clinical investigations of this case presentation?

• CBCd, blood film, extended lytes, Cr, Urea, ESR, CRP, urinalysis including microscopy, urine protein: creatinine ratio. AST, ALT, albumin, INR, PTT , CK (myositis)

• TSH, ANA, C3, C4, Immunoglobulin panel, Rheumatoid Factor, DAT (Coombs test)

• Consider infection (either DDx or trigger of a flare of SLE): depending on the patients features -parvovirus, CMV, EBV, blood and urine culture (consider)

• More extensive work-up: ENA Panel (4 Extractable Nuclear Antigen Antibodies), Anti- dsDNA, Anti-phospholipid antibodies

• May consider: glucose, anti-TTG, D-dimer, fibrinogen, cholesterol studies, ferritin, haptoglobin, LDH

• As symptoms indicate: CXR, ECG, echo – other investigations and consultations as necessary

• Opthalmologic Eye exam is essential

How will you treat your patient?

Treatment approach:

Non-pharmacologic – sun protection is key to preventing rashes and avoiding flares

Pharmacologic treatment (these would all be initiated in conjunction with advice from pediatric rheumatology)

- Hydroxychloroquine (Plaquenil)

- NSAIDS (If no renal involvement)

- Steroids (from low dose oral to high dose IV steroids)

- Disease Modifying Anti-Rheumatic Drugs (DMARDS), biologic medications or cytotoxic drugs

Pandu is 10months old boy, brought into POPD with gastro-enteritis which started 2 days ago. You noticed in his Clinic Card that he only received birth Immunisations, the rest was not given. She is RVD unexposed, rapid negative (1month ago), she is thriving along the -1 Z-score WFL (weight-for-length). She had a T:37.6 °C HR:135 bpm RR: 40. She was breastfed for 8months and her mother started weaning her on Formula milk and soft feeds.

Which age Immunisations are outstanding?

6 weeks, 10 weeks, 14 weeks, 9 months

Which Immunisation for the specific ages are outstanding for Pandu?

• 6 wks:

Polio 1

Pentavalent (Diphtheria, Pertusis,

Tetanus, Haemophilus influenza type B,

Hepatitis B

Pneumococcal vaccine (PCV)

Rotavirus 1.

• 10wks:

Polio 2

Pentavalent (DPT,Hib, HepB)

Pneumococcal vaccine 2

Rotavirus 2

• 14wks:

Polio 3

Pentavalent

Pneumococcal vaccine

• 9mo:

Measles

Rubella (MR)

Based on her age which Immunisations are contra-indicated?

Rotavirus vaccine

You are the Medical Intern seeing the patient, you counselled the mother on the

importance of vaccinations. She understands the implication of not been vaccinated.

She agrees for you to catch up Pandu’s Immunisation. Which immunization would you

give first?

Measles vaccine

How would you space the rest of the rest Immunisations and how many doses are

required?

• Polio: 4 weeks apart, (2more doses)

• Pentavalent: 4 weeks apart (3 doses)

• PCV: 4weeks (between dose 1-2) then 8 weeks (between 2-3rd dose) = total 3dose

Mrs Angula, Pandu’s mother further elaborates that Pandu previously had a fever of

38°C the previous day and after her first Immunisation she cried for 1hour, thus she

was reluctant to take her for the subsequent Immunisation.

What would be absolute contra-indications for not Vaccinating?

• Severe allergic reactions (Anaphylactic shock) after a

  vaccine.

• Encephalopathy not due to another identifiable cause

  occurring within 7 days of a Pertusis vaccine.

• Live vaccines should be avoided in patients with Severe immunodeficiency states, like Severe Combined Immunodeficiency (SCID), patients on high dose corticosteriods, patients with malignancy-post Chemotherapy

Which vaccines in the Namibian EPI schedule is live-attenuated?

• Oral Polio vaccine

• BCG

• Rotavirus

• Measels vaccine

14month old girl Dankiros was brought in by her father Mr Matroos to the POPD with 2week history of lethargy, not been playful and swelling of the body. The father reveals that the mother is currently nursing a 2month old baby, thus Agaros was weaned off the breast at 4months when the mother noticed that she was pregnant. Dietary, Dankiros has been formula fed since 4months and drink supplementary Magua (Porriage-based drink), when funds are available she sometimes eat vegetables, fruits, rice and yoghurt. He often pica for soil and ice. On examination Dankiros was irritable, lethargic, with pitting pedal oedema, pallor, desquamating skin rash and hepatomegaly. Her Anthropometry is demonstrated below:

Interpret the above anthropometry

• Failure to thrive with crossing centiles, initially was growing well on the 0-z-score then dropped his Z-score to -2 then -2 to -3 z-score and now less than -3 Z-score. (WFA)

WFA: severe UWFA, <-3 Z-score

HFA: severely stunded, <-3 Z-sore

WFH: severely wasted, <-3 Z-score

What is the most likely diagnosis?

Severe acute malnutrition with oedema

Which other Anthropometry marker would you like to know to confirm your diagnosis?

What do you expect it to be?

Mid-upper Arm Circumference (MUAC) <11.5cm

Supply FOUR (4) reasons for your most likely diagnosis?

• She has Severe Acute Malnutrition with oedema (Kwashiokor) because she is weaned off from breastfeeding early due to mother who had to feed a sibling and was started on carbohydrate based drink.

• Possibly the formula feeding is not adequate or not mixed properly to provide proteins and other micronutrients the patient need to thrive.

• Children with Kwashiokor have pitting oedema in the extremities depends on the severieties.

• These children also have abdominal distension secondary to poor abdominal musculature and hepatomegaly secondary fatty infiltration.

• Skin changes are charcteristics and progress over a few days, become hyperpigmented, dry and then splits open

  when stretched, reaveling pale areas between the cracks

  called crazy paving or flanky paint dermatosis.

Name FIVE (5) major GROUP abnormalities you should correct as part of the

management of this patient?

• Correct Hypoglycaemia

• Correct Hypothermia

• Correct Macronutrient deficiencies

• Correct Micronutrient deficiencies

• Correct electrolytes: Hypokalemia, hypomagnesaemia,

  hypophophataemia, hypocalaemia

• Correct dehydration

• Treat infection

Mr Matroos further states that Dankiros had diarrhea for the past 10days, which (a) TWO (2) supplements will you include as part of his management? (b) Which one has been included in the Namibian EPI schedule to further decrease diarrhea and name one (1) major toxicity signs/symptoms (except Nausea, headache,Vomiting)?

(a) Zinc and Vitamin A

(b) Vitamin A: Raised Intracranial pressure, Alopecia, hepatomegaly, fatigue, dry skin, hyperostosis and irritability.

While examining Dankiros’ abdomen you noticed that it is distended and the liver is enlarged, explain the pathophysiology for hepatomegaly in light of his underlying condition? Total Protein: 70 (43-70) Albumin: 15 (36-51) ALT 20 (18-32)

Hepatic glucose stores are depleted and gluconeogenesis is impaired (especially in more advance disease). Synthesis of lipoprotein is reduced, which leads to a limited ability to mobilize fat resulting in fatty infiltration, the lipid that accumulate is triglycerides and disappears in about 3weeks as the child recovers. Synthesis of albumin is low, but synthetic function of liver is relatively preserved.

You discussed this patient with your Medical Officer, one of the bloods suggested to

do is an FBC? Below are the results:

WCC: 25 X109 /L (6-18)

RCC: 1.85 X 1012 (3.8-5.4)

Hct: 0.096 l/l (0.31-0.46)

Hb: 7.2 g/dL

MCV: 65fl (70-86)

RDW: 26% (11.6-14.5)

Platelets: 600 X 109 /L

Interpret the FBC?

Differential count: Neutrophil predominance, with 15% Bands

Mr Matroos further is concerned why Dankiros likes to eat soil, how will you explain this to him?

Microcytic hypochromic anaemia with high RDW most

likely Iron deficiency anaemia

In the acute phase which supplement should you avoid, based on the underlying problem in the above FBC?

Iron should be deferred until infection is treated and outside acute phase

You decided to start feeding at day 2 admission, however when the father was not monitored, so he decided to feed larger volume feeds, in the hope that Dankiros will gain weight faster and be discharged. On your call you found that after the presumed improvement he had made in the pass 3 days he was now hypotonic from muscle weakness, cardiac failure and had 1 seizure. Bloods taken:

Na: 132 (131-145) K: 2.1 3.6-5.2) U: 3.5 (1.7-6.5) Cr: 52 (27-55) Ca (corrected):

2.3 (2.25-2.73) Mg 0.6 (0.70-1.15) PO4: 0.3 (1.10-1.91)

What is the most likely explanation for his sudden deterioration? How will you explain this to the father?

REFEEDING SYNDROME:

§ This is caused by the metabolic and physiological consequences of the depletion, repletion and compartmental shifts of phosphorus, potassium, magnesium, glucose metabolism, vitamin deficiency and Insulin levels increase after re-feeding. This result in Phosphorus, potassium and magnesium shift to the intracellular spaces, causing a drop in serum levels. Clinical presentation include muscle weakness, seizures, rhabdomyolysis, cardiac arrhythmias, cardiac failure and haemolysis. Thus before re-feeding is instituted the electrolyte imbalance, vitamin and trace element deficiencies should be corrected.

Mention 2 strategies we can use to prevent the above and what is the target weight gain rate?

(a) Feeding slowly and increasing cautiously.

(b) Correct electrolyte imbalance, vitamin and trace element deficiencies.

(c) Supplement Thiamine prior to re-feeding with carbohydrates, since Thiamine utilization increases with re-feeding .

(ii) At least 10g/kg/day

You are working in the Pediatric out-patient department at Intermediate hospital Katutura, Jayden a 6-month-old boy presents with cough and difficulty in breathing. Jayden’s mother who is 15-years-old, reports that she tested positive for Human Immunodeficiency virus (HIV)infection during antenatal care in the last semister and she is on first line treatment. She also reports that Jayden was also given medication post-delivery.

What is the recommended HIV prophylaxis for a high-risk neonate? What is the duration?

Nevirapine and AZT( Zidovudine) Ideally for 6 weeks.

§ If breastfeeding AND mother’s VL > 40 or more or unknown,

continue with NVP daily

§ If NOT breastfeeding since birth or in last 4 weeks, OR mother’s

VL<40, discontinue infant prophylaxis and continue to

monitor mother’s VL as prescribed

What are the THREE criteria you will consider to initiate Jayden on high-risk prophylaxis?

• Born to women with HIV infection who have received less than 4 weeks of ART at the time of delivery

• Born to women with HIV infection with VL >40 copies/ml in the

  3 months prior to delivery

• Unknown VL

• Born to women with HIV infection diagnosed during labour

  and delivery, post-partum or in the breastfeeding period

What is the recommended age in infants to start co-trimoxazole prophylaxis in HIV exposed infants and reasons?

From 6 weeks till HIV status is confirmed.

§ Prevent PCP in HIV exposed infants

§ Protect against common bacteria infections, toxoplasmosis and malaria

When do you stop the co-trimoxazole in an infant who is HIV exposed?

I month After cessation of breastfeeding after

confirmation of DNA PCR negative results

You decide to admit Jayden and treat for the pneumonia. His polymerase chain reactive (PCR) test is positive for HIV even though he was given prophylactic therapy. What are the possible explanations or reasons why Jayden is HIV infected even though his mother mentioned Jayden received prophylaxis and mother was on treatmentduring pregnancy?

Jayden was a high risk patient because his mother started ARV treatment in the last semester possibly he got infected in utero, intrapartum and postnatally through breatfeeding.

The factorS for MTCT is host genetic factors, maternal or infant coinfection, behaviorand nutruition

Genetic factors

§ Fetal Gender and HLA type

§ Maternal – Fetal HLA concordance

§ SNPs for chemokines/chemokines receptors/innate immune factors

Maternal factors

§ Chorioamnionitis

§ STIs: genital ulcer diseases including HSV2 and syphilis

§ Malaria

§ TB

§ Mastitis/breast abscess

§ Oral candidiasis in the infant

§ Resistance to medication

§ High viral load

Behavioral factors

§ Illicit drug use during pregnancy

§ Frequency of sexual intercourse during pregnancy

§ Number of sex partners during pregnancy

§ Infant feeding practices: breastfeeding, mixed feeding, food premastication.

§ Adherence to the prophylaxis medication.

§ Underdosing the medication.

Maternal nutritional status

§ Advanced maternal disease with immunosuppression and malnutrition

§ Vitamin A deficiencies

§ Other micronutrients deficiencies

STI: Sexually transmitted infection

What condition will you investigate before starting Jayden on antiretroviral therapy?

TB

What drug regimen will you initiate Jayden?

Infants 4 weeks to 2 years old ABC/3TC/DTG where Jayden age group belongs

Jayden’s results are positive for cytomegalovirus (CMV) pneumonia, negative for Pneumocystis jiroveci pneumonia (PJP/PCP) and negative for other tests. He is still being breastfed and the mother has a two-months history of cough and constitutional symptoms. What prophylaxis would you give Jayden if you suspect that the mother has tuberculosis and why?

Isoniasid Preventive Treatment.

§ All newborns with no signs and symptoms of active Tb disease that are born to mothers with active Tb disease.

§ All under 12 months HIV-infected children without signs and symptoms of active TB disease and with a known Tb contact.

§ All HIV- infected children who are 12 months or older with no signs and symptoms of active Tb disease.

Signs of allergic rhinitis

1. Allergic shiners: dark discoloration beneath the eyes as aresult of obstruction of lymphatic and venous drainage, chronic nasal obstruction and suborbital oedema.

2. Dennie-mogan lines: creases in the lower eyelid radiating outward from the inner canthus, caused by spasm in the muscles of maxiller around the eye as a result of chronic congestion and stasis of blood

3. Allergic salute: a gesture characterized by rubbing the nose with the palm of the hand upward to decrease itching and temporarily open nasal passages

4. Allergic crease: transverse crease near the tip of the nose

A child presents with 3day hx of puffy eyes, worse in the morning, swollen feet and distended abdomen. Her mother reports that prior to this presentation she had a mild URTI 1 week before. On examination she looks well and apyrexial, but has periorbital pitting oedema, pitting pedal oedema and ascites. Her HR 115bpm, BP108/74, temp 36, with cap refill time 2sec.

UA: blood1+,protein4+,Leuc(-), nit(-), pH6

Bloos Tproit65, alb 19g/dl(38-54), FBC,UEs Norm

What is the most likely dx?

Nephrotic syndrome

What are the 4 classic features?

• Oedema

• Severe proteinuria (3-4+)

• Hypoalbuminemia <25g/l

• Hyperlipidemia/hypertriglycaeremia

List 3 other investigations you will request to make definite dx

• Lipogram

• C3,C4

• UPCR

T/F

The pathophysiology of nephrotic syndrome is both humoral and cell mediate, resulting in inflammation of the glomeruli, which further caused antigen trapping in the basement membrane, this antigen-antibody reaction result in glomeruli damage and protein leakage.

False; this is for nephritc sx

This disease doesnt occur in children with scd

false, SCD pts are high risk and should be screened routinely

Hep B, HIV, TB, congenital syphyllis are secondary causes

True