Case 1-2

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By Dr. Ayman El-Deeb Case Presentation

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Case 1

Name: ???? : 

Name: ???? Age: 24 Sex: Female The patient was admitted to Tanta chest hospital complaining shortness of breath & occasional cough. She gave a history of epileptic fits and she had splenectomy 15 years ago.

On Examination : 

On Examination Temperature 37oC blood pressure: 120/80 mmHg resp. rate. 25/m The patient looked underweight with signs of hypogonadism & hypothyroidism. She had brown spots covering her face

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Abdominal Examination: Median scar of old abdominal operation (splenectomy) No organomegaly Chest Examination: Reveled crepitations heard over the right L.L. posteriorly Chest X ray P-A: Showed micro nodular infiltration affecting the right lower lung zone.

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Mantoux -ve Thyroid function test (hypothyroidism): EEG: left temproparital epileptic focus Abd. u/s: Liver cirrhosis Investigation done: CBC (normocytic normochronic anemia) reduced platelet count 76000 /cmm

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All these data attracted our attention that she might have a disease that can cause epileptic fits – splenomegaly – liver cirrhosis – pulmonary infiltrates and brown spots on the face.

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We asked for serum ferritin as a screening test for pulmonary hemochromactosis & it was quite high. Normal values for the female 10-140 ng/ml and the patient result was 1867 ng/ml.

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Iron storage disorder due to inappropriate increase in intestinal iron absorption. Resulting in deposition of iron in tissues leading to damage and functional impairment of organs. (liver – pancreas – heart – pituitary -Brain).

Heamochromatosis. : 

Heamochromatosis. The disease was first described in 1865 by Armand Trousseau in a report on a diabetic Pt. with a bronze skin pigmentation, The disease was described as being caused by iron deposition by Von Recklinghausen in 1890.

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Males are usually diagnosed in their forties& fifties while women several decades later because of the regular iron loss in menstruation. There is also a juvenile form of this hereditary disease. The disease is either: Hereditary or Acquired

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Genetic inherited disease : associated with an abnormal gene HFE on chromosome number 6 The gene has two alleles,C282Y & H63D Heamochromatosis type1-3 are inherited in autosomal recessive fashion & Type 4 is inherited in an autosomal dominant fashion. Acquired : with iron overload tissue injury as in thalamenia , sideroplastic anemia & repeated blood transfusions.

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symptoms& signs : according to the organ affected. Malaise, Livercirrhossis, Diabete, CHF, damage to the adrenal gland, dark spots on the skin& neurological symptoms.

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Diagnostic tests: Blood tests:1- Serum transferrin gives a crude measure of iron stores.2- Serum ferretin concentration is a good index of body stores of iron and is a useful non invasive screening test for the diagnosis of early disease. CBC, Renal function ,LFTS &Glucose Tolerance test. Liver biopsy Imaging ct &MRI

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Treatment Early diagnosis is important to prevent organ damage & It is initiated when the ferritin level reaches 300 mg/liter Phlebotomy at a weekly interval until ferritin levelsare less than 20mg/L Treatment chelating agent as deferoxamin given parenterally 10-20 mg/day.

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Case 2

Name: ???? ????? : 

Name: ???? ????? Age: 32 Sex: Male The patient was admitted to Tanta chest hospital on 5/11/2003 complaining of shortness of breath on moderate exertion. He was not coughing and he had no other symptoms. His dyspnea on moderate effort is always with him and he doesn't know when it has started.

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Past History: Is unremarkable Family History: He has no family members with chest problems.

On examination : 

On examination Vital signs temp. 37 oC Pluse: 80/m blood pressure120/80 mmHg Resp. rate 18/m No scars of previous operation seen. The movement of chest wall was limited on the left side There was a marked tracheal shift to the left side felt by palpation. The apex beat was on the left midaxillary line

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By percussion: hyper resonant note was heard over the right side and impaired note was heard over the left lower ribs mainly on the back and let midaxillary region. Auscultation: Apex of the heart was in the mid axillary line 6th space with normal heart sounds.

Chest examination: : 

Chest examination: Normal vesicular breathing was heard all over the right side and the left side except on the lower ribs on the left side from the back side, there was a decrease of the breathing sounds.

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CxR P.A. was asked for and it showed a normal bony frame work with slight narrowing of the interspaces in comparison with the right side.

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There was a marked shift of the trachea to the left side as well as the heart was also shifted to the same side. The right lung appeared over inflated the right copula of the diaphragm is below the 11th rib posteriorly and it appeared crossing the sternum to the left side (herniation of the right lung to the left side). The heart was pulled and shifted markedly to the left side giving the impression of complete collapse of the lower and middle (lingual) lobes of the left lung.

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The patient was asked to have CT scan of the chest and he had it done. The report of the CT gave the same opinion obtained from the P-A view.

The radiologist reported the following : 

The radiologist reported the following Complete collapse of the lingula and the lower lobe of the left lung with loss of aeration and lung volume. Herniation of the right lung through the anterior midline to the left side. Marked shift of all the mediastinal compartment to the left side and he came to the conclusion that the patient has complete collapse of the lower and middle (lingula) lobes of the left lung.

Upon receiving these reports : 

Upon receiving these reports I decided to discuss his condition with my colleagues and we agreed that he should have bronchoscopic examination to find out the cause of the collapse of the left lower lobe bronchus. The patients condition was explained to him and after having his consent bronchoscopic examination was done trans nasally.

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The larynx appeared normal. Vocal cords were mobile. Tracheal rings appeared normal there was no compression on them.

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As I came close to the carina I found that the opening of the left main bronchus was very small and tiny, the mucosa covering the opening and left main bronchus was normal denoting that the patient has congenital agenesis of the left lung with compensatory emphysematous change of the right lung that led to restudy the CT scan which was misinterpreted as it showed also the rudimentary left main bronchus at the level of the tracheal bifurcation.

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Thank You