Assessment and management of an 11 year old girl presenting to ED with abdominal pain, vomiting, 4+ glucose 4+ ketones on urinanalysis

Diagnosis??
1. 2. 3. hyperglycaemia (BG >11 mmol/l); pH < 7.3; bicarbonate < 15 mmol/l and who are: more than 5% dehydrated and/or vomiting and/or drowsy and/or clinically acidotic Glucose +4 Ketones +4 Children who are 5% dehydrated or less and not clinically unwell (mild acidosis and not nauseated or vomiting) usually tolerate oral rehydration and subcutaneous insulin.

Assessment
1. 2. A- airway B- breathing C- Circulation Only if shocked (poor peripheral pulses, poor capillary filling with tachycardia, and/or hypotension) give 10 ml/kg 0.9% (normal) saline as a bolus, and repeat as necessary to a maximum of 30 ml/kg. 3. Comfirm the Dx History - polydipsia, polyuria Clinical - acidotic respiration, dehydration, drowsiness, abdominal pain/vomiting. Biochemical high blood glucose on finger-prick test (>11 mmol/l) capillary blood ketone level above 3 mmol/l glucose and ketones in urine 4. Initial Investigations 1. blood glucose 2. urea and electrolytes (electrolytes on venous blood gas give a guide until accurate results available) 3. blood gases (venous blood gives very similar pH and pCO2 to arterial) 4. Other e.g. PCV and full blood count (leucocytosis is common in DKA and does not necessarily indicate sepsis), CXR, CSF, throat swab, blood culture, urinalysis, culture and sensitivity, etc. DKA may rarely be precipitated by sepsis, and fever is not part of DKA.

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Degree of dehydration 1. 3% - is only just clinically detectable 2. mild, 5% - dry mucous membranes, reduced skin turgor 3. moderate, 7.5% - above with sunken eyes, poor capillary return 4. severe, 10%(+shock) - severely ill with poor perfusion, thready rapid pulse (hypotension is not likely and is a very late sign) Concious level GCS Full examination - Looking particularly for evidence of: 1. cerebral oedema (headache, irritability, slowing pulse, rising blood pressure, reducing conscious level) N.B. papilloedema is a late sign. 2. Infection 3. Ileus Does the child need PICU/HDU? YES, if: 1. severe acidosis pH<7.1 with marked hyperventilation 2. severe dehydration with shock 3. depressed sensorium with risk of aspiration from vomiting 4. very young (under 2 years) 5. staffing levels on the wards are insufficient to allow adequate monitoring

Management
1. FLUIDS a) Volume of fluid By this stage, the circulating volume should have been restored and the child no longer in shock. If not, give a further 10 ml/kg 0.9% saline (to a maximum of 20 ml/kg) over 30 minutes (discuss with a consultant if the child has already received 20 ml/kg). Otherwise, once circulating blood volume has been restored, calculate fluid requirements as follows: Requirement = Maintenance + Deficit fluid already given Deficit (litres) = % dehydration x body weight (kg) Ensure this result is then converted to mls by multiplying by 10. Never use more than 8% dehydration in the calculations.

Maintenance requirements 100mls / kg for first 10 kg 50 mls / kg for next 10kg 20mls / kg for remainder Hourly rate = (48 hr maintenance + deficit) minus (resuscitation fluid already given) divided by (48) Example : A 20 kg 6 year old boy who is 8% dehydrated, and who has already had 20ml/kg saline, will require Deficit = 8% x 20 kg = 1600 mls deficit Maintenance = (100ml x 10kg) + (50ml x 10kg) = 1500mls maintenance each 24 hours (= 1500 x 2 = 3000 mls over 48 hours) Resus fluids = 20kg x 20ml = 400 mls Hourly rate: (3000 + 1600 400)/48 = 87 mls / hour

2. POTASSIUM
Once the child has been resuscitated, potassium should be commenced immediately with rehydration fluid unless anuria is suspected. Levels in the blood will fall once insulin is commenced. Therefore initially add 20 mmol KCl to every 500 ml bag of fluid (40 mmol per litre). Check U & E's 2 hours after resuscitation is begun and then at least 4 hourly, and alter potassium replacements accordingly. Use a cardiac monitor and observe frequently for T wave changes.

3. INSULIN There is some evidence that cerebral oedema is more likely if insulin is started early. Therefore DO NOT start insulin until intravenous fluids have been running for at least an hour. Continuous low-dose intravenous infusion is the preferred method. There is absolutely no need for an initial bolus. Make up a solution of 1 unit per ml of human soluble insulin (e.g. Actrapid) by adding 50 units (0.5 ml) insulin to 50 ml 0.9% saline in a syringe pump. Attach this using a Y-connector to the IV fluids already running. Do not add insulin directly to the fluid bags. The solution should then run at 0.1 units/kg/hour (0.1ml/kg/hour). Some paediatricians believes that 0.05 units/kg/hour is an adequate dose.

 Once the blood glucose level falls to 14mmol/l, add dextrose (5-10% equivalent) to the IV fluids running. DO NOT reduce the insulin. Aim for blood ketone levels of < 1mmol/l. If the blood glucose falls below 4 mmol/l, give a bolus of 2 ml/kg of 10% dextrose and increase the dextrose concentration of the infusion. Insulin can temporarily be reduced for 1 hour. When pH is above 7.3, the blood glucose is down to 14 mmol/l and ketones < 1mmol/l, and a dextrose-containing fluid has been started, consider reducing the insulin infusion rate, but to no less than 0.05 units/kg/hour.

MONITORING TREATMENT OF DKA

CLINICAL Vital Sign Coma Score Sodium, potassium, CO2 or HCO3, venous pH, osmolality Glucose INTERVALS 20-30mins 20-30mins Admission, 2, 6,10, 24 hrs. (or 2-4 hourly until osmolality normal) Hourly bedside; in the laboratory with electrolyte assay, or 1-2 hourly if outside bedside monitor range Hourly if abnormal (<3 or >6 mM/L ) Admission, 12, 24 hrs Admission, 4-6 hourly

Potassium BUN Ketonuria

Rehydration in a dehydrated child with gastroenteritis

Children, especially those younger than 4 years, tend to be more susceptible to volume depletion as a result of vomiting, diarrhea, or increases in insensible water losses. Significant fluid losses may occur rapidly. The turnover of fluids and solute in infants and young children can be as much as 3 times that of adults. This is because of the following: Higher metabolic rates Increased body surface area to mass index Higher body water contents Water constitutes approximately 70% of body weight in infants, 65% in children, and 60% in adults.

Mild Volume Depletion 1. Encouraged to continue an age-appropriate diet and adequate intake of oral fluids. 2. Oral rehydration solution (ORS) should be used. Children should be given sips of ORS (5 mL or 1 teaspoon) every 5 minutes. As an estimate for the amount of fluid to replace, the goal should be to drink 10 mL/kg body weight for each watery stool and 2 mL/kg body weight for each episode of vomiting.

Moderate Volume Depletion Similar outcomes have been achieved in randomized studies comparing ORS with intravenous fluid therapy with fewer complications and higher parent satisfaction in the ORS groups. Moreover, ORS can typically be initiated sooner than IV fluid therapy. With ORS, patients should receive approximately 50-100 mL/kg body weight over 2-4 hours, again starting with 5 mL every 5 minutes. Children in whom ORS fails should be given a bolus (20 mL/kg) of isotonic fluid intravenously. This may be followed by maintenance therapy.

Severe Volume Depletion Patients with severe volume depletion should receive intravenous isotonic fluid boluses (2060 mL/kg) In children with difficult peripheral access, perform intraosseous or central access promptly. Fluid boluses should be repeated until vital signs, perfusion, and capillary refill have normalized. If a patient reaches 60-80 mL/kg in isotonic crystalloid boluses and is not significantly improved, consider other causes of shock (eg, sepsis, hemorrhage, cardiac disease).

Once vital sign abnormalities are corrected, initiate maintenance fluid therapy plus additional fluid to make up for any continued losses. Maintenance requirements 100mls / kg for first 10 kg 50 mls / kg for next 10kg 20mls / kg for remainder

SODIUM DEPLETION? 1. Treat with normal saline or other isotonic solutions. 2. The goal for correction rates for either hyponatremic or hypernatremic patients should be no more than 1 mEq/L/h to prevent the devastating CNS complications of overrapid correction (central pontine myelinolysis and cerebral edema, respectively). 3. Full correction of severe sodium abnormalities usually should be staged over 24 hours or longer.

An 8 year old boy attends OPD as his GP queries precocious puberty and requests your assessment. Outline your assessment.
Early onset and rapid progression of puberty. White european children: <8 y.o in females and <9 y.o in males

Assessment 1. History detailed history should be obtained

1. Age when first signs of pubertal development observed. 2. Which features of puberty are present and in what order did they appear. 3. Evidence of growth acceleration 4. Family history: menarche age in family members

2. Examination
1. 2. 3. 4. 5. 6. Puberty staging Measure height, weight, head circumference Review previous growth records if available Calculate MPH Skin lesions? caf-au-lait (McCune-albright;NF-1) Neuro exams;visual fields; fundoscopy

3. Investigation baseline screening test should be considered.

1. Plasma LH and FSH levels 2. Plasma sex hormone: oestrogen/testosterone 3. Other serum androgen levels 17-OH progesterone, DHEAS, androstendione 4. Urine: steroid profile (sex/adrenal steroids) 5. Bone age X-ray 6. Pelvic ultrasound (testicular masses/ovarioan morphology) 7. MRI scan brain 8. GnRH test: basal and post- GnRH LH and FSH levels as indicator of hypothalamic-ptuitary function.

A 2 year old girl presents to OPD for assessment of unilateral non tender lymphadenopathy. She has recently arrived in Ireland from India. Outline possible causes for her lymphadenopathy and how it should be investigated

 Lymphadenopathy: Malignancy accounts for a small proportion of cases of persistent lymphadenopathy in children. Possible Dx for children in malignancy: acute leukemia, non-Hodgkin Lymphoma, Hodgkin disease, mets from neuroblastoma or sarcoma. Features that may be concren: 1. Diameter >2 2. Persistent or progressive enlargement 3. Non-tender, rubbery, hard, or fixed 4. Supraclavicular or axillary position 5. Associated with other features e.g: pallor, lethargy 6. Hepatosplenomegaly

 Unexplained mass at any site

Following features should raise suspicion of malignancy
Non-tender Progressive enlargement Diameter >2cm Associated lymphadenopathy

Exposure Travel Related

Diagnosis

Arizona, southern California, New Mexico, Coccidioidomycosis western Texas

Southwestern United States Southeastern or central United States Southeast Asia, India, northern Australia Central or west Africa East Africa, Mediterranean, China, Latin America Bubonic plague Histoplasmosis Scrub typhus African trypanosomiasis (sleeping sickness) Kala-azar (leishmaniasis)

Mexico, Peru, Chile, India, Pakistan, Egypt, Typhoid fever, Tuberculoisis Indonesia Central or South America American trypanosomiasis (Chagas' disease)

PHYSICAL EXAM Head and Neck Examine closely for: Scalp infection (e.g. seborrheic dermatitis, tinea capitius) Conjunctivitis injection Oropharynx for pharyngitis, dental problems, HSV ginivostomatitis Ears for acute otitis media Abdomen Examine closely for: Hepatosplenomegaly (this is actually considered part of your lymph node exam!) Abdominal masses (e.g. neuroblastoma) Skin Examine closely for: Any rashes Petechiae, purpura, eccyhmoses (e.g. thrombocytopenia) Lymph Node Exam: When palpating a lymph node it is important to consider the following: Size (measure them) Location Fixation Consistency Tenderness

Investigations 1. Complete blood count, peripheral blood smear 2. Erythrocyte sedimentation rate (non-specific) 3. Rule out infectious causes: Monospot, CMV, EBV, & toxoplasma, bartonella titres, TB skin test, Anti-HIV test, CRP, ESR 4. Hepatic and renal function + urinalysis (systemic disorders that can cause lymphadenopathy) 5. Lactate dehydrogenase, uric acid, calcium, phosphate, magnesium if malignancy suspected 6. Bone marrow, liver biopsies, CT or US guided lymph node biopsy Imaging Studies 1. Chest X-ray. This study will help determine the presence of mediastinal adenopathy and underlying pulmonary diseases including tuberculosis, coccidioidomycosis, lymphomas, and neuroblastoma. 2. Ultrasound of the lymph node 3. CT of the chest and/or abdomen. Supraclavicular adenopathy is highly associated with serious disease in the chest and abdomen. 4. Nuclear medicine scanning is helpful in the evaluation of lymphomas.

Generalized: more than 2 noncontiguous lymph node group

Infectious Viral (most common): URTI, measles, varicella, rubella, hepatitis, HIV, EBV, CMV, adenovirus Bacterial: syphilis, brucellosis, tuberculosis, typhoid fever, septicemia Fungal: histoplasmosis, coccidioidomycosis Protozoal: toxoplasmosis

Non-infectious inflammatory diseases Rheumatologic diseases: Sarcoidosis, rheumatoid arthritis, SLE Storage diseases: Neimenn-Pick disease, Gaucher disease Serum sickness Rosai-Dorfman disease Malignant: leukemia, lymphoma, neuroblastoma Drug reaction: phenytoin, allopurinol Hyperthyroidism

Localized enlargement of a single node or multiple contiguous nodal regions

A. Cervical (most common adenopathy in children, often infectious cause): Infectious: 1. Viral upper respiratory infection 2. Infectious mononucleosis (EBV, CMV) 3. Group A Streptococcal pharyngitis 4. Acute bacterial lymphadenitis (e.g staphylococcus aureus) 5. Kawasaki disease (unilateral cervical lymph node > 1.5 cm) 6. Rubella 7. Catscratch disease 8. Toxoplasmosis 9. Tuberculosis, atypical mycobacteria

Neoplastic: (malignant childhood tumours develop in the head and neck in of cases. Neuroblastoma, leukemia, non-Hodgkins, and rhabdomyosarcoma are most common in those < 6 years old. In older children, Hodgkins and non-Hodgkins lymphoma are more common. 1. Acute leukemia 2. Lymphoma 3. Neuroblastoma 4. Rhabdomyosarcoma

B. Submaxillary and submental 1. Oral and dental infections 2. Acute lymphadenitis C. 1. 2. 3. 4. Occipital Pediculosis capitis (lice) Tinea capitis/local skin infection Rubella Roseola

D. Preauricular (rarely palpable in children) 1. Local skin infection 2. Chronic ophthalmic infection

E. Mediastinal (not directly palpable; assess indirectly via presence of supraclavicular adenopathy. May manifest as cough, dysphagia, hemoptysis, or SVC syndrome this is a medical emergency!) 1. ALL 2. Lymphoma 3. Sarcoidosis 4. Cystic fibrosis 5. Granulomatous disease (tuberculosis, histoplasmosis, coccidioidomycosis) F. 1. 2. 3. 4. Supraclavicular (associated with serious underlying disease) Lymphoma Tuberculosis Histoplasmosis Coccidioidomycosis

G. 1. 2. 3. 4. 5. 6. 7.

Axillary Local infection Cat scratch disease Brucellosis Reactions to immunizations Non Hodgkin lymphoma Juvenile rheumatoid arthritis Hidradenitis suppurativa

H. Abdominal (may manifest as abdominal pain, backache, urinary frequency, constipation, or intestinal obstruction due to intussuception) 1. Acute mesenteric adenitis 2. Lymphoma I. Inguinal 1. Local infection 2. Diaper dermatitis 3. Syphilis