IDENTIFY DATA: Roy Stanford is a 5 yr old Indian male who presented to the paediatric wards , th Tuesday morning, January

13 ,2014. The history is obtained from the mother who is considered reliable. Chief complaint: generalized swelling, abdominal pain and vomiting x 1/52. History of Present Illness . According to the mother, her son was well about a week ago when she noticed his face followed by his body started to swell. No dyspnea, history of infection or diarrhea. Mother recall child also had a fever, loss of appetite and was irritable. She took the child to Lethem hospital where he was diagnosed and treated for a urinary infection. However, this morning the boy complaint of abdominal pain to the right lower quadrant, accompanied by high grade fever and approximate 10 episodes of vomiting; which the mother describes as slimy greenish content. Child was taken to theatre for appendectomy today. BIRTH HX: Roy Stanford was born on 16th june 2008 weighing 3kg. Child was delivered at 8 months with a

apgar score of apgar score of 5 at 1 minute and 6 at 5 minute. Delivery was vaginal . No history of complications or trauma. child is the second for parents. IMMUNIZATION Hx : Up to date. No associated complications. DEVELOPMENTAL Hx: All milestones achieved at appropriate age . FEEDING Hx: child was breast fed for 6 months. At 10 months he was weaned and presently he eats from the family pot. PMHX: Nil DHx: Nil PSHx: Nil CURRENT MEDICATIONS: Panadol Ramipril nifedepine

MATERNAL Hx: Mother is 28 yrs house wife. She is 0+ and VCT/VDRL negative. No infection or ill health during pregnancy. No use of illicit drugs, smoking or alcohol during pregnancy. FAMILY Hx: Father is diabetic. No history of mental retardation or cancer SOCIAL Hx: A total of rour persons reside in the home. Water source is present in the home and family use it for drinking also. Garbage is thrown away and the family uses flush toilet. No travel history or pets in the home.

REVIEW OF SYSTEMS: A. Weight 3 Kg; current weight change of approximately 1kg. B. Skin and Lymph - No rashes, adenopathy, bruising and bleeding. C. HEENT No concussions, visual problems, ear infections,cold or sore throats D. Cardiac tachypnea but no heart murmurs, or palpitations E. Respiratory no crackles or cough. F. GI diarrhea, vomiting, abdominal pain, and loss of appetite. G. GU no frequency, dysuria, discharge but decreased volume with some foams. H. Musculoskeletal No joint pains or weakness I. Allergy No urticaria, asthma, eczema, or drug reactions J.CNS: child is conscious and oriented x 3

PHYSICAL EXAMINATION: Vital signs: Respiratory rate: 40 bpm; Pulse rate: 120 bp; Temperature: 100.5 F; BP: 130/80

General: dehydrated looking child lying in bed in painful distress. HEENT: normocephalic, pupils equal, round and reactive to light, Clear external auditory canals, No lymphadenopathy, mucus membranes pink and moist. Mild periorbital puffiness RESPIRATORY: Round chest cavity. mild used of accessory muscles Bilateral air entry . No crackles or cough.

HEART: hyperdynamic precordium, no right ventricular heave or thrills. PMI in left midclavicular line in 6th intercostal space. Regular rate and rhythm. Normal Sl with normally split S2 on respiration. No murmurs, gallops or rubs. ABDOMEN: move with respiration, tender to palpation to right lower quadrant,bowel sound present. GENITALIA : Normal male genitalia. Normally placed urethral meatus. Bilaterally descended testes. Not swollen EXTREMITIES: mild pitting edema. No gross deformities.

IMPRESSION: lethargetic dehydrated patient in painful distress with appendicitis and nephrotic syndrome.

DIFFERENTIAL DIAGNOSIS Acute Poststreptococcal Glomerulonephritis Diabetic nephropathy Minimal change nephropathy Angioedema

LABORATORY EVALUATION

Urinalysis Urine protein quantification (by first-morning urine protein/creatinine or 24-hour urine protein) Serum albumin Lipid panel Complete blood count (CBC) Metabolic panel (Serum electrolytes, BUN and creatinine) Chest radiography to rule out pulmonary edema LFT

DIAGNOSIS: Nephrotic syndrome is also known as nephrosis and is defined by the presence of proteinuria, oedema, hyperlipidaemia, and hypoalbuminaemia. It has serious complications and must be on the differential diagnosis for any patient presenting with new-onset oedema. A thorough assessment for the underlying cause of nephrotic syndrome is essential. The diagnostic criteria for nephrotic syndrome are:

Proteinuria greater than 3-3.5 g/24 hours or spot urine protein:creatinine ratio of >300350 mg/mmol. Serum albumin <25 g/L. Peripheral oedema. Severe hyperlipidaemia (total cholesterol often >10 mmol/L) is often present

TREATMENT AND MANAGEMENT

Diet and fluids: o Reduce salt intake in the diet (avoid processed foods and adding salt to food). o Give a diet with adequate calorific intake and sufficient protein content (1-2 g/kg daily). o Fluid restriction is not usually necessary (if severe enough to need this then the patient may need admission). Hyperlipidaemia - does not initially require therapy but may do so if prolonged. Oedema: o Oedema is treated through diuretic therapy with furosemide (~1 mg/kg/day) spironolactone (~2 mg/kg/day). o Check weight regularly to assess response to diuretics and ensure fluid retention is not worsening, or that the patient is over-diuresed. o Patients with very low albumin levels may not respond to diuretics and may require admission to receive intravenous albumin therapy. Some children with severe oedema may be prescribed antibiotic prophylaxis against infection Most children will have minimal change nephrotic syndrome and usually respond to a trial of steroid therapy. Children in their first episode of nephrotic syndrome should be treated for at least three months with an increase in benefit for up to seven months of treatment.[9] In children who do not respond to steroids, and in some adults, treatment may be with other immunomodulatory drugs such as cyclophosphamide, ciclosporin, tacrolimus and levamisole.

DISCUSSION Each kidney has approximately 1 million glomeruli, which are the sites of blood filtration. The layers of the glomeruli include the fenestrated endothelium of the capillary, the glomerular basement membrane, and the foot processes of the podocytes. The main barrier to filtration is the connection between adjacent podocyte foot processes called slit diaphragms. Glomerular proteinuria develops when the components of this filtration barrier are disrupted by disease. The primary insult leading to the development of nephrotic syndrome is the development of highgrade glomerular proteinuria, and the heavier the protein loss the more likely the development of the full-blown syndrome and worsening of renal function. Patients become hypoalbuminaemic due to the urinary loss of albumin. The liver tries to compensate for this protein loss by increasing the synthesis of albumin, as well as other molecules including LDL and VLDL and lipoprotein(a), contributing to the development of lipid abnormalities. [4] Hypercoagulability results from the loss of inhibitors of coagulation in the urine and increased synthesis of procoagulatory factors by the liver. The oedema is due to a decrease in oncotic pressure from the hypoalbuminaemia, as well as a primary defect in sodium excretion. Patients with nephrotic syndrome are also at increased risk of infection due to loss of immunoglobulins and complement and other compounds being lost in the urine.

PROGNOSIS This is very variable depending on the underlying cause. Corticosteroids have reduced the mortality rate in children to around 3%. Outlook for the vast majority of children with minimal change nephrotic syndrome is excellent, with good response to steroids, although there may be relapses and a need to use alternative immunomodulatory drugs. The majority of children who present with their first episode of nephrotic syndrome achieve remission with corticosteroid therapy but over 70% experience a relapsing course.