**4. Our experience**

nutritional support (enteral tube feeding) can be purposed to the patient if nutritional status is unsatisfactory. Vitamin supplementation with folic acid, vitamin A, and vitamin D are

An overall weight gain of 12.5 Kg is considered normal and in CF it is recommended a weight gain of at least 11 Kg. Also gastro-oesophageal reflux, heartburn, nausea, recurrent vomiting, and constipation may occur more frequently in women with CF and required clinicians

A pre-pregnancy diagnosis of CF-related diabetes and gestational diabetes are associated with a poorer prognosis; in literature, it is reported that an unsatisfactory glycemic control in the first trimester is associated with an increased risk of teratogenesis. During preconceptional counselling an OGTT is recommended, if not already performed, and blood sugars have to be monitored during lung exacerbations. Usually, OGTT is repeated at 20 weeks gestation and glycaemia should be measured at every visit: if random values are high, another OGTT could

Insulin is the recommended treatment for diabetes in CF also during pregnancy. A high calorie

Guidelines about when to terminate pregnancy in a woman with advanced CF remain fluid. The indications may be psychosocial (in order to prevent serious injury to the mental health

The only absolute contraindication to pregnancy is a pre-existing pulmonary hypertension with cor pulmonale; also chronic hypoxia could be considered a contraindication to pregnancy. There are no clear indications about the FEV1 cut-off to recommend pregnancy termination.

Most pregnancies in CF end in spontaneous vaginal delivery. Caesarean section is indicated only in case of maternal or fetal sufferance, preferably with spinal anesthesia. In CF, a high proportion (26–46%) of pregnancies end up with a spontaneous or therapeutic preterm delivery, and the usual indication is represented by maternal conditions. Usually, failing lung function and hypoxia occur in patients with a significant low pre-pregnant lung function;

; <85% ideal body weight)

There are also some relative contraindications to pregnancy in CF women:

recommended.

124 Cystic Fibrosis in the Light of New Research

monitoring.

*3.4.6. Diabetes care*

be repeated at 28 weeks.

*3.4.7. Termination of pregnancy*

of the pregnant woman) or medical.

**1.** Poor nutritional status (BMI <18 kg/m2

**2.** Uncontrolled CF-related diabetes

**3.** *Burkholderia cepacia* infection

**4.** Significant liver disease

*3.4.8. Delivery*

intake should also be assessed in diabetic patients.

In our CF Center, we have 230 patients in regular follow-up and 153 of these are >18 years old. In the last few years, fertility issues increased significantly in our population, surely because of the significant improvement in prognosis and the easier access to the assisted reproduction treatments.

During the last 20 years, 12 of our male patients recurred to in vitro fertilization with sperm obtained by epididymal aspiration and in 6 cases had successful reproduction (with twin pregnancy in 2 cases and in 1 case triplet pregnancy). All the newborns enjoyed good health and no recurrence of CF has been detected (all the partners were tested for CFTR mutations before the conception). In the other six cases in vitro fertilization was not successful and one of these three patients decided to use heterologous fertilization by sperm donor, with a subsequent successful pregnancy.

In the same period four of our female patients recurred to in vitro fertilization with two successful pregnancies (in one case twin pregnancy). Also in these cases the newborns enjoyed good health and no recurrence of CF has been detected (all the partners were tested for CFTR mutations before the conception).

In our population 12 of our patients (10 women and 2 men) had spontaneous conceptions with 18 successful pregnancies. Also in these cases no recurrence of the disease has been detected, even if in some cases the diagnosis of CF in these patients had been performed after the delivery and in most cases the partners had not been tested for CFTR mutations before the conception. Three of our female patients, after several attempts to become pregnant spontaneously, decided and obtained to adopt a child.

Actually, no literature is available about scrotal imaging in males affected by CF. We decided to realize an original study, performing scrotal ultrasound examination with high-definition technique, to better evaluate abnormalities in shape, volume, and structure of the testes, epididymis, and spermatic cord in a group of adult patients (>18 years) affected by CF. Preliminary results seem to show an increased incidence of testicular and epididymal abnor‐ malities in comparison to the general population, but in most cases these seem to be secondary to the obstruction of vas deferens [FigureS 7,8,9].

**Figure 7.** US Figure of rete testis ectasia with structure inhomogeneities in a CF patient

**Figure 8.** US Figure of rete testis ectasia with structure inhomogeneities with a little epididymal cyst in a CF patient

**Figure 9.** US Figure of intradidymal cysts (one with corpuscular content) in a CF patient
