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Case presentation, Dr iman Ghabn,



Case presentation, baby with hyperglycemia & brain cyst. By dr Iman Ghabn, El Nasr hosp, Port said, Egypt

Case presentation, baby with hyperglycemia & brain cyst. By dr Iman Ghabn, El Nasr hosp, Port said, Egypt



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    Case presentation, Dr iman Ghabn, Case presentation, Dr iman Ghabn, Presentation Transcript

    • Case presentation Dr.Eman Aly MohamedSpecialist of pediatrics & neonatology EL NASR N.I.C.U
    •  Female baby called Ganna neseem ,27 days old. C.S delivery ,not attended delivery ,G2-p2 birth date : 9/12 /2011 Gestational age : 34 w. (+/-) 2 w. was referred to our NICU from other hospital by : Severe respiratory distress. Cyanosis. Persistent vomiting. Persistent hyperglycemia.
    •  Chest xray CBC Blood glucoseTreatment: Antibiotics: cefotax,unictam N.G feeding &O2 by head box.Response to treatment was poor with persistent hyperglycemia and refer to our NICU .
    •  Admission date : 28/12 . The baby was severely hypoactive and depressed reflexes. Central cyanosis Waisted baby with loss of s.c fat of abdominal wall &both thighs Face ex.: no apparent abnormal features except low hair line. Mouth ex.: normal.
    • Chest ex.: Tachypnea, R.R 65/min Intercostals &subcostal retractions Fair air entery on both sides of chest wall Fine consonating &coarse cripits (bilateral)Heart ex.: H.R 135 beat /min. S1&S2 normal. No abnormal sounds.
    •  Abdomen is lax. Loss of subcutaneous fat. Loss of skin elasticity. Liver (++) below costal margin ,soft consistency, rounded border . Passes urine&stools
    •  Lower limbs ex . : Lax, no oedema Loss of subcutaneous fat over both thighs , no deformities. Upper limb ex. : Mild hypertonia,, fisting of both hands no deformities. Back&spines: Normal ,no deformities ,no masses External genitalia: normal appearance
    •  Length: 46cm. Weight: 1.9kg.on admission , birth weight was 2.5 kg. Chest circumference: 29cm. Skull circumferernce: 33 cm. on admission to our nicu. 34 cm. on stay in our unite Anterior fontanelle: soft,slightly depressed,3x5 cm. in size
    •  Plain x ray (chest&heart): bronchopneumonic patches Blood glucose: hyperglycemia Acetone in urine: absent Serum insulin: 2 micro unit /ml. Normal : 6 -24 micro unit /ml. C-peptide : 0.6ng /ml. Normal( 0.9-4) ng/ml.
    • Series Series Series Series Series Series Series Series Series Series Series SeriesSeries PM AM AM AM AM AM AM PM PM PM PM
    •  W.B.C count: 5.7 x 10 3/cubic mm R.B.C count: 2.9 x 10 3/cubic mm Hgb: 10.4 g/L H.c.t.: 30.7 Plt. 35 x 10 3/mm3 Differential count: - Lymph 26.2 % - Mon 21. 5 % - Gra 52.3 %Another C.B.C: normal after blood transfusion
    •  Sodium : 126 mmol/L (135- 150 mmol/L) Potassium: 4.5mmol/L (3.5 - 4.5 mmol/L) Calcium: 10.4 (8.1-10.4mg)
    •  Kidney functions test: Urea : 18 normal :(15-45mg %) Creatinine: 0.7 normal(0.3-1.3mg %) Liver functions test:  SGPT(ALT): 15 normal up to 45u/ml.  SGPT(AST): 39 normal up to 40 u/ml.  S. bilirubin :  total : 5.5 normal up to 1.2n.g  direct: 0.8 normal up to 0.25n.g
    •  Marked hepatomegally with diffuse increased parenchymal density Conclusion: signs suggestive of diffuse parenchymal liver disease for lab.correlation&biopsy.
    • CT brain was ordered because of association of central abnormalities with cases of neonatal hyperglycemia
    •  Large defined cystic lesion of c.s.f density is seen at RT. Tempro-parietal region connected to the atrium of the rt. Lateral ventricle It is seen surrounded by mild degree of interstitial oedema (csf permeation) It measures about 4x3.5 cm.
    •  No evident related soft tissue masses. It exerts mild mass effect in the form of compression upon the third ventricle &minimal leftward shift of the mid line structuresConclusions:Signs cope with large RT.tempro- parietal proncephalic cyst.
    • Neurosurgical consultation:Supratentorial arachnoid cyst attached to the RT. Lateral ventricle &effaced if less likely (pitocyst-AC)for M.R.I&contrast M.R.I Brain :The same finding as C.T.
    •  8 days after admission, the baby starts to develop subtle convulsions in the form of (pedaling & recycling ) alternating with tonic clonic convulsions of both upper limbs ,respond to phenobarbitone . In addition to 3 attacks of generalized tonic clonic convulsions associated with hypoglycemia as a complications of insulin infusion respond rapidly to I.V. GL. 10%
    • Respiratory: C.P.A.P by nasal bronge with pressure 5 mm.Hg, oxygen 21% for 3 days. The baby was Shifted to H.B. 5L/min.for 1 day. Antibiotics : Unictam+cefatriaxone for 7 days followed by Vancomycin +gentamycin Nebulizer with ventolin
    • Glucose : We start with GL.10%with Na &K&Ca , Then reduce gl. concentration to 7% and then to 5%. We modulate G.I.R according to results of blood glucose starting with 7mg/kg/min. till 5.5 mg/kg/min. A.A (panamin g. 0.5 gm./kg/day) lipids (lipovenous 0.5 gm./kg./day)
    •  With persistent hyperglycemia even with G.I.R 5.5mg/kg/min. we start insulin infusion in a dose of 0.02 u/kg/hour. in spite of this minimal dose of insulin the baby developed hypoglycemia so we have to increase G.I.R to 7.5 mg/kg/min. with insulin infusion till stabilization of blood glucose
    •  With stabilization of the baby ,we start gradual nasogastric feeding followed by complete oral feeding . As soon as oral feeding is completed blood glucose returns to normal values.
    •  Blood transfusion :15 ml./kg. Plasma transfusion :15 ml/kg. I.V. phenobarbitone followed by oral phenobarbitone. Surgical drainage of cyst &CSF by shunt operation.
    • After shunt Before shunt