SCJU Constanta
Pediatric intensive care department
Pediatric clinic I

To: Pediatric clinic IOMC “Dr. Alfred Rusescu”

Family and first name: Gitu Mara
CNP (Personal identity number): 6081007134123
Address: Navodari, district Constanta
Period of hospitalization: 26.11. – 17.12.2008
Diagnosis: neonatal cholestatic hepatitis syndrome
(suspected malformation of biliary intra hepatic ducts, D’Alagille arterio hepatic dysplasia or Byler family chronic cholestasis)

Anamnestical data:
Female new born of 1 month and three weeks gets hospitalized for severe skin sclerotic icterus, installed in the fourth life day and progressively accentuate.
APF: second child, born at full term in the maternity Constanta
W: 2800 gr. Apgar 9, corresponding adaptation period, neonatal jaundice installed in the fourth day
AHC: parents deny history of chronic diseases

Clinical data:
Hospitalization examination: eutrophic new born (IP = 0.93), W 3900 gr, afebrile, normotroph, normokinetic, atypical facies, slightly pale, severe skin sclerotic icterus.
FA: 2/2 cm, normal blood pressure, normally developed thorax, cardio- circulatory balanced (AV: 132 beat/ min, regulated, pulmonary without rales, flat soft abdomen, obvious abdominal venous circulation, liver with the lower margin at 2 cm below rib edge and slightly higher consistency , impalpable spleen, hyper chromatic urine, acholic stools, normal sensitivity control.

Evolution
Diminished jaundice, lighter stools with intermittent normal stools, good appetite, with no dyspeptic sd, ascendant weight curve (W at hosp.: 3900 gr, actual W: 4200 gr). The bilirubin values lower than the initial values and staying constant in the last 10 days (we attach the laboratory analyses reports).

During hospitalization the clinical reexaminations and the paraclinical investigations led us to the diagnosis of neonatal cholestatic hepatitis. For the etiological identification we could exclude partial paraclinically the infectious causes (viral and bacterial causes – please see attached the laboratory reports) and the mechanic causes (please see attached results of the echography and abdominal examination). The metabolic etiology could not be analyzed. There is the possibility of the hereditary etiology (Byler family chronic cholestasis ?), based on the clinical look of the mother and sister ( the subicterical skin look)- please find attached the laboratory results.

Sweat test: NaCl: 29mmol/l

The treatment during hospitalization: the treatment with cortisone (HIIC 40mg/day x 15 days) was stopped when the serology tests against Herpes virus arrived, hepatoprotective medication iv (Arginina sorbitol – 70 ml/ day and Liv 52 – 5 ml/day x 19 days), antibiotics ( Ampicilina 500 mg/ day the first 12 days) and afterwards coleretic (Ursofalk : 40mg/ day in the last 2 days). The clinical and paraclinical evolution was initially good, but then it became to worsen.

At the moment: new born with the same general condition, severe skin sclerotic icterus, obvious collateral abdominal circulation, flat soft abdomen, liver with the lower margin at 2 cm below rib edge and slightly higher consistency, impalpable spleen, hyper chromatic urine, acholic stools

Cardio- pulmonary and neurologically balanced

Conclusion: transfer to the IOMC clinic.

Best regards,

Treating physician: LEU Elena 17.12.2008

Resident physician: SUCIU Claudia

Place: Bucharest

Sanitary unit: Clinical Institute Fundeni

Pediatric clinic

Pediatric clinic supervisor

Constantin Arion

MEDICAL CERTIFICATE nr. 430

Year: 2009 Month: 04 Day: 15

Based on the report of Dr. Alexandrina Constantinescu, primary physician gastroenterology, we confirm that:

Name: Gatu Mara Sex: F

Birth date: 07.10.2008

Address: Constanta, str. Unirii no. 112, bl. U2, sc. B, ap. 35, district Constanta

Is in the records of the Fundeni Pediatric Clinic with diagnosis:

1. atresia of extrahepathic biliary ducts – KASAI surgery on 07.01.2009

2. secondary biliary cirrhosis

3. relapsing cholangitis

4. energetic malnutrition

Permanent monitoring and periodic examinations are required.

This certificate is issued in order to be used at the Medical Expertise Committee (Handicap)

Pediatric clinic supervisor, Treating physician,

Prof. Dr. Constantin Arion Dr. Alexandrina Constantinescu

Stamp: Health Ministry

Clinic Institute Fundeni

Hospitalization office no. 2

Clinical Institute Fundeni – Pediatric clinic

Sos. Fundeni 258, sector 2, Bucharest

Tel/ fax: 021- 3180421 (secretary); 021- 3180424 (department)

NOTE OF DISCHARGE FROM HOSPITAL

Name: Gatu

First name: Mara

CNP (Personal identity number): 6081007134123

Birth date: 07.10.2008

Address: Constanta, str. Unirii no. 112, bl. U2, sc. B, ap. 35, district Constanta

Period of hospitalization: 26.11. – 17.12.2008

F.O. 20240

Diagnosis:

- atresia of extrahepathic biliary ducts – KASAI surgery on 07.01.2009 (Q 44.2)

- secondary biliary cirrhosis (K 74.4)

- relapsing cholangitis (K 83.0)

- light energetic malnutrition (B 44.1)

She is on the waiting list for a liver transplant (possible donor is the mother, having the same blood type)

T =59 cm, W= 7300 gr, blood type: B II

PC = 40 cm, PT = 60 cm, PA = 51 cm

Tests:

Biological: Hb = 1.9 – 11.8 g/dl; Hct : 34.8 – 34 6%; leucocytes: 14600 – 14100/mmc (FL, S= 38%, E= 4%, B= 1%, L= 57%, M= 10%). Platelets: 270000 – 232000/mmc; Urea: 17.5 – 14 – 18.1 mg/dl; Creatinine: 0.31 – 0.4 mg/dl; Uric acid: 3.42 – 3.5 mg/dl; F.alk: 922 – 825 U/l; Na: 135 – 137 mmol/l; K= 5 – 5.4 mmol/l; CI= 100 – 101 mmol/l; Fe= 59 – 62 µg/dl; ALT= 181 – 111 – 79 U/L; AST= 98 – 78 – 64 U/l; GGT= 1055 – 648 U/l; I.DH= 181 U/l; VSH= 65 mm/h; Fbg= 425 – 306 – 292 mg/dl; AP= 132 – 111 – 115%; AP= 11.1 sec; APTT= 26 – 24.8 sec; INR= 0.85 – 0.94 – 0.91; Total proteins= 7 g/dl; Albumin= 3.7 – 3.1 – 3.9 g/dl; albumin/ globulin= 1.1; BT= 2.9 – 0.83 – 1 mg/dl; BD= 1.2 – 0.71 – 0.4 mg/dl; BI= 1.7 – 0.12 – 0.6 mg/dl; glucose= 99 mg/dl.

Nasal exam: staphylococcus Haemoliticus (comensual)

Pharyngeal exam (10.04.2009): absence of pyogenes streptococcus; colonization with Klebsiella (same strain, with the same sensitivity as in coproculture)

Urine exam: acid pH; absence of albumin and glucose, normal urobilinogene. Urinary sediment: rare flat epithelial cells, rare white cells, very rare red cells, relatively frequent oxalan mucus

Coproculture (10.04.2009): absence of Salmonella, Shigella; presence of colonies of Pseudomonas aeruginosa, Klebsiella

Haemoculture (10.04.2009): negative at 7 days

Urine test (10.04.2009): sterile

Coproculture (21.04.2009): absence of Salmonella, Shigella and other pathogenic flora

Abdominal echography (10.04.2009): Liver: LSHФAP= 4.8/5.8 cm; LDHФPR= 8.5 cm, relatively clear contour, slightly irregular; hepatic echostructure slightly scratchy diffuse, with visualization of small hyperechogene, imprecisely bounded areas, predominantly central located. One can see CBIH in both lobes, least widened (~2 mm), hardly contoured with hyperechogene margins. One can not see CBP, but do see the bilio-digestive anastomosis, looking permeable. At the gallbladder’s bed level one can notice a gallbladder sketch of aprox. ~ 1/0.3 cm (hypoplasic), permeable VP, 5 mm caliber. Permeable AH with resistance index aprox. 0.75. One can not see the pancreas and the retroperitoneal space, completely covered by gases. Spleen = 9.2 cm, homogeneous echostructure. LK= 6.2 cm, RK= 6.2 cm, both kidneys with the preservation of the cortico-medullary differentiation, with no SPC dilatation, no calculi images. VU reduced quantity of transonic urine. No ascites.

Echocardiography: normal cavities, SIV= 4.8; PPVS= 4.8; VS= 24.5/15.2; good contractions, normal valve. FE= 70%; SIA with little continuity solution type PFO; clean SIV. No gradient. Conclusion: persistency of Foramen Ovale.

Epicrise: The 7 month old patient gets hospitalized by transfer from the IOMC clinic, where she had been diagnosed with atresia of extrahepatic biliary ducts, operated by KASAI technique (07.01.2009 in the Pediatric Emergency Hospital “Gr. Alexandrescu”), biliary cirrhosis and acute relapsing cholangitis, for evaluation regarding the opportunity of registration on the waiting list for a liver transplant. Objective: cheloide post- operative cicatrix, upper abdomen, hepatomegaly (+3-4 cm under rib margin), spleenmegaly (+3-4 cm under rib margin), intermittent acholic stools. Biologically: hepatocitolitic syndrome (ALT= 181 – 111 – 79 U/L; AST= 98- 78 – 64 U/L); cholestatic syndrome (F.alk= 922 – 825 U/L; BT= 2.9 mg/dl; OGT= 1055 – 648 U/L); inflammatory syndrome: (white cells= 14600 – 14100/ mmc; VSH= 65 mm/h; Fbg= 425 – 306 – 292 mg/dl); microcytic hypochrome anemia (Hb= 11.9 – 11.8 g/dl; MCV= 79 fl). Abdominal echography:

hepatic echostructure slightly scratchy diffuse, with visualization of small hyperechogene, imprecisely bounded areas, predominantly central located, CBIH least widened in both lobes (! 2mm); bilio-digestive anastomosis, looking permeable, gallbladder of aprox. ~ 1/0.3 cm (hypoplasic). Following the coproculture and nasal- pharyngeal exam the results revealed colonization with Klebsiella and Pseudomonas aeruginosa, against which treatment with antibiotics is administrated(Ciprinol 150 mg/day and Ertapenem 200 mg/day). The following evolution was good. At the moment of discharge from hospital the cultures are negative, including the haemoculture, and the cholestatic syndrome is improving (BT= 1 mg/dl; GGT= 648 U/L).

Recommendations:

1. Hygiene dietetical regimen

2. Drug treatment:

1. Ursofalk cp 250 mg, split into 5 parts, 150 mg/ a day

2. Liv 52, 2.5 x 2- day

3. Normix cp 200 mg, ½ cp x 2/ day, 20 days/ month

4. Vigantol 4 drops/ day

5. Pharco Calcium 2.5 ml/day, 10 days/ month

6. Fitomenadiona ½ amp/ week, im

7. Polivital 9 drops/day, 20 days/month.

3. Reexamination in one month or whenever necessary.

This note stands also for a medical certificate.

Treating physician

Dr. Alexandrina Constantinescu