JANUARY 24TH 2006 TIME: 1hour INSTRUCTIONS TO CANDIDATES: ANSWER ALL QUESTIONS ANSWER EACH QUESTION IN A SEPARATE BOOKLET

1. A 5 day old full term baby, presents with poor feeding and jaundice of a days duration. The mother lost liquor for 3 days before delivery. On examination, the baby is cyanosed, lethargic with no spontaneous movement and little response to painful stimulus. The respiratory rate is 24 cycles a minute, and irregular. a) List three likely differential diagnoses for this babys condition [6 marks] 1. Neonatal sepsis (early) 2. Severe neonatal jaundice with kernicterus 3. Meningitis 4. Hypoglycaemia b) Name two interventions that must be urgently done to save this babys life [4 marks] 1. check blood sugar and correct hypoglycaemia if present 2. CPR + oxygen c) Outline the remaining management of this baby. [10 marks] 1. Resuscitation: Oxygen, CPR IV fluids-1/5 10% D/S 2. Investigations: a. blood CBC, Blood C/S, SBR, Grouping and Xmatching, G6PD screening, Serum electrolytes, Blood gases b. oxygen saturation, CRT c. LP to r/o meningitis 3. Antibiotics- broad-spectrum to await blood c/s result 4. Phototherapy and/or EBT depending on level of SBR 5. Monitoring- frequent reassessments and appropriate amendments of intervention. 6. Reassure mother, explain each step taken and finally discharge when fully well.

2. A 1-year old girl presents with severe Failure to Thrive (FTT), recurrent episodes of diarrhea for 8 months, and fever for 6 weeks. Stool parasitology isolated cryptosporodial species. Physical examination shows a severely wasted child with generalized lymphadenopathy and oral thrush. A diagnosis of HIV infection is made.

a) What is the clinical stage of the disease using the current WHO 4-staging system? Give reason(s). [10 marks] 1. stage 4: a. severe FTT (-3SD) b. 8 months diarrhea with isolation of cryptosporodial spp c. generalized lymphadenopathy ??stage 1 d. oral thrush ?? Stage 3 e. unexplained fever >1 month _stage 3

b) What is the first-line regimen of Antiretrovirals that should be started for this child? [5 marks] 1. ZDV or d4T+3TC plus NNRT NNRT of choice: 2. 3. if <3 yrs or <10Kg, give NVP

ZDV or d4T+3TC plus PI ZDV or d4T+ 3TC+ ABC

c) Give serious side effects of 2 of the drugs mentioned in (b). [5 marks] d4T 1. Peripheral neuropathy 2. Pancreatitis, Lactic acidosis ZDV 1. Anaemia NNRTEFV 1. Rash 2. Abn dreams 3. Hallucinations 4. Somnolence 5. Agitation 6. Abn thinking NVP 1. Steven-johnsons syndrome 2. hepatitis

3. A previously well 3 year old boy presents with a tonic, clonic seizure lasting 5 minutes. He has no fever. After the seizure he is sleepy for one hour, then he is playful and active again. There is a history of three such convulsions without fever in the last 12 months. a) What is the most likely underlying cause [2 marks] Epilepsy b) What are the principles of management of this condition? [14 marks] 1. History and complete PE to look for aetiology 2. Investigations: EEG to confirm diagnosis and give type, LFTs baseline, Serum electrolytes, serum calcium 3. Drug treatment- carbamazepine, sodium valporate, 4. Counselling of family- about condition, drug treatment + SEs, prognosis etc. c) List 2 other differentials [4 marks] 1. Hypocalcaemia 4. A 4 year old child from a rural district develops fever for 2 days. He convulses on the 3 rd day and he receives amodiaquine for 3 days and once-daily penicillin injections for 5 days from a local health care provider. The convulsions cease but child still remains unwell. The child is brought to your clinic on the 6th day. He at this time has drowsiness, temperature of 38oC and poor feeding. a) Give 4 possible reasons why this child remains unwell after 5 days of treatment [8marks] 1. malaria-treatment failure or partially treated 2. meningitis 3. hypoglycaemia 4. b) List 4 investigations which may assist in the subsequent management of the child. Give reasons for each. a. BF for MPs b. LP- CSF to r/o meningitis c. Blood C/S d. Blood sugar [8 marks] c) Briefly comment on problems with the initial management of this childs illness [4 marks] 1. Amodiaquine: use of monotherapy for treating treating malaria is wrong 2. This could be a case of severe malaria and therefore patient should have been given Quinine instead of amodiaquine 3. Daily dose of penicillin is totally inadequate for meningitis if it was meant for that 4. The case been a severe disease is above the capacity of the local health facility and

the health provider. Patient should have been referred on the very first day and assisted to go to a higher level for care.

5. A well-nourished six year old child presents with generalized oedema. a) Give 4 possible causes for generalized oedema. [4 marks] b) How would you investigate one of the conditions mentioned in a)? [12 marks] c) List 4 complications of the condition chosen in b) [4marks]

G. PLANGE-RHULE E. ADDO-YOBO T. RETTIG A. OSEI AKOTO S. ANTWI D. ANSONG B. BAFFOE-BONNIE

KWAME NKRUMAH UNIVERSITY OF SCIENCES AND TECHNNOLOGY, KUMASI SCHOOL OF MEDICAL SCIENCES CHILD HEALTH FINAL MB.CHB. PART 1B FIFTH YEAR SECTION C January 24th 2006 TIME: 1hour INSTRUCTIONS TO CANDIDATES: ANSWER ALL QUESTIONS ANSWER EACH QUESTION IN A SEPARATE BOOKLET

1. Acute lymphocytic leukaemia (ALL) is the most common childhood malignancy in childhood worldwide. Write short notes on ALL under the following headings: a) Signs and Symptoms [5 marks]

BONE MARROW FAILURE - Leukaemic cells in place of normal haemopoietic elements results in decreased production of rbcs, wbcs, platelets---- Aplastic anaemia ANAEMIA- develops slowly 1. pallor 2. Irritability 3. Decreased activity BLEEDING TENDENCIESa. usually due to thrombocytopenia i. Petechiae and ecchymosis in the skin ii. Mucosal bleeding, such as epistaxis or maelena b. Associated DIC may lead to severe life-threatening bleed- eg in CNS INFECTION due to lack of adequate neutrophils - Fever is the usual symptom/sign - Localised signs may be absent - Infection quickly spreads-bacteremia and sepsis RES INFILTRATION 1. lymphadenopathy- common and may be masssive 2. Hepatosplenomegaly-massive or minimal BONE PAIN-

expansion of marrow cavity or destruction of cortical bone by cells INVOLVEMENT OF SANCTUARY SITES ESP IN RECURRENCE OF DISEASE - CNS diffuse meningeal irritation - Testis- unilateral or bilateral enlargement out of proportion to childs sexual development. b) Laboratory diagnosis [5 marks]

PERIPHERAL BLOOD: NORMAL CBC DOES NOT RULE OUT LEUKAEMIA 1. Normochromic, normocytic anemia with low retic count 2. Thrombocytopenia is common 3. Neutropenia: low(<5000/mm3) in 1/3; normal (5000-20,000) 1/3; high >20,000 in 1/3 4. Blast cells is common BM ASPIRATION- confirmation of diagnosis IMMUNOCYTOLOGY/IMMUNOCHEMISTRY c Prognostic factors Factor Favorable * WBC Age Immunotype Morphology Chromosomes DNA content <10,000 2-10 yrs CALLA+ L1 Normal N/hyperdiploid [5 marks unfavorable >10,000 1<or >10 yrs CALLAL2, L3 Translocations: Hypodiploid, haploid

Bulky disease Sex

Absent Female

present male

c) Principles of Therapy [5 marks] 1. Chemotherapy- Remission- induction, consolidation, maintenance, discontinuation of therapy 2. Radiotherapy 3. BM transplant 4. Supportive- blood transfusion, treatment of infections, fluids, prevention of TLS etc etc. 5. Counselling

2. Write short notes on the following. a) Routine care of the umbilical stump after delivery [8 marks] Daily baths and/or application of an antiseptic- spirit, triple dye etc to the stump And the surrounding skin reduces the incidence of umbilical infection. Infection is serious because of haematogenous spread or extension to the liver or peritoneum. Portal vein phlebitis may develop, resulting in later onset of extrahepatic portal hypertension. Evidence may be minimal (periumbilical erythema) even in the presence of septicaemia or hepatitis. Treatment: prompt antibiotic therapy- amp/gent etc; wound swab for c/s, blood c/s Cord usually dries and separates within 6-8 days after birth. The raw surface is Covered with a thin layer of skin, scar tissue forms and wound is usually healed Within 12-15 days. Delay separation occur if there are saprophytic organisms Umbilical granuloma: persistence of exuberant granulation tissue at the base. Tissue is Soft, vascular and granular, and dull red or pink and it may have a seropurulent secretion. Treatment is cauterization with silver nitrate: repeated at intervals till the base is dry. DD: Umbilical polyp- results from persistence of all or part of the omphalomesenteric duct or of the urachus. Tissue is firm and resistant, is bright red and has a mucoid secretion. May have communicaqtion with the bladder or ileum leading to small amounts of urine or faeces being discharge intermittently. Treatment is surgical excision.

b) Meningomyelocoele [12 marks] A neural tube closure defect that involves the vertebral column. Cause is unknown but genetic predisposition is said to exist. Risk of recurrence after one affected child rises to 3-4% and increases to approx 10% with 2 previous abnormal pregnancies. Nutritional and environmental factors play a role in aetiology- maternal preconcetional use of folic acid supplementation reduces incidence. (in 1st trimester) FA has to be taken prior to conception and continued beyond 12 weeks after conception in order to be effective.

Anticonvulsants eg sodium valporate are known to increase the risk Clinically, condition produces dysfunction of many organs and structures- skeleton, skin, GU tract, peripheral NS and CNS. Location: anywhere along the neuraxis but LS takes about 75%. Extent and degree of lesion depends on the location. Lesion in the lower sacral region leads to bowel and bladder incontinence associated with anaesthesia in the perineal region but intact motor function. Mid-lumbar lesion leads to flaccid paralysis of the lower extremities, absence of deep tendon reflexes, lack of response to touch and pain, and many postural abnormalities including talipes. Constant urinary dribbling and a patulous anus may be evident. Lower motor signs are the usual signs with mid-lumbar lesions. Typically as the lesion goes higher into the thoracic region, the neurological deficits also increase. Upper thoracic and cervical lesions however, have a minimal neurologic deficit and in most cases have no hydrocephalus. Hydrocephalus is a frequent associated complication of myelomeninigocele. Mgt: multidisciplinary approach- surgeons, physicians, therapists; paediatrician should be the coordinator of the programme. Family needs to be counseled thoroughly and they need to be part of each step and decision taken. Repair: Early vrs late Exclusion criteria: marked paralysis of legs, TL or TLS lesions, kyphosis or scoliosis, associated birth injury; other congenital anomalies Prognosis: mortality rate is high: 10-15% even after aggressive treatment. At least 70% of survivors have normal intelligence but learning problems and seizures are common Meningitis affect ultimate intelligence.

3. Write short notes on the following: a) The Pentavalent Vaccine [10 marks] DPTHibHep b) The eradication of Poliomyelitis in Ghana [10 marks]. Linked up with the global effort to eradicate polio

1. Write short notes on the following drugs under the following headings: their use in paediatric practice, their action and side effects.

a) Oral iron [10 marks] Daily maintenance requirement: elemental iron: 0.5-1mg/kg/24 hrs in single or divided dose. Iron deficiency anaemia: 6mg/kg/24hrs in divided doses. Continue for 2-3 months after Hb is normal to compensate for deficits in stores Different preparations with different content of elemental iron b) Frusemide [10 marks] Loop diuretic Inhibits chloride and sodium reabsorption and interferes with urine concentration. Duration of action is x 2 hrs when given IV Dose 2 mg/kg per dose orally; 1mg/kg per dose IV Can be ototoxic 5. Write short notes on the Roll Back Malaria concept [20 marks]

G. PLANGE-RHULE E. ADDO-YOBO T. RETTIG A. OSEI AKOTO S. ANTWI D. ANSONG B. BAFFOE-BONNIE